Emily Marie, Our chILD Child!

Happy Birthday Emily!

2011-10-01T14:56:00.003-04:00

Emily turned six years old on September 29. I can't believe how fast she is growing up!!!

We baked a cake together after her basketball game, and then we opened gifts. She really wanted a big party, but in the end, we decided against it this year. All in all, I think she had a good birthday!

Happy Birthday Emily! I am so proud of the wonderful girl you have become!

Emily with her homemade Hello Kitty cake!

Make a wish! Wonder what she wished for. She's being very tight lipped about it, but judging from the look on her face, I'd say it was something pretty spectacular!

Blowing out her candles! It took her several attempts, but that is okay.

Emily with a few of her gifts. She got some Hello Kitty stuff, a few outfits, and a scooter.

Growing Up!

2011-09-21T22:23:00.005-04:00

My baby girl is growing up!

Emily started kindergarten last month and absolutely loves it. Her health is doing fairly well except for a recent cold that had her breathing hard all weekend... but hey, we have oxygen, so just turned it up temporarily and she was better after about 2 days. Otherwise, she is more active than ever, her PFT's are up a few more points, and she is doing great.
(Above is first day of Kindergarten)

For homework today, Emily brought home 3 books to read, and when I didn't immediately get to her (I started homework with Ryan), she hand wrote one of the books cover to cover. "I am a rabbit, I am a robot, I am a clown," etc. She is so eager to learn! Too cute! Then she presented me with several other pages of "homework" that she has been working on: her alphabet written over and over, a sheet that said, "I love my mom" about a thousand times, and random other words, letters, and numbers. When she gave that to me, she said, "It says I love my mom because I actually do really love my mom." So sweet! I mentioned that her lower case g needed some work, so little miss perfectionist made another page practicing writing her g's til she got it right. She says she will do "q" tomorrow because it is hard too. She has started trying to spell simple words on her own and has figured out that to make something plural, you can add an s. Doesn't always work out as she planned, but when she gets it right, she will say, "I am so smart!" And she is! I am truly amazed with her progress! She is going to be reading in no time! (Above is an example of one of her homework assignments. She worked on this for days and wanted to be certain hers was the sparkliest. I think she achieved her goal!)

She started basketball last night, and that was just absolutely hilarious! Complete chaos as 4 coaches tried to teach 20 pre-school aged kids and kindergartners how to dribble and pass. The really funny thing was that Emily was far from being the worst player on the team, although she was just as far from being the best. None of the kids had a clue! So adorable and so funny to watch them play at that age!

(Above is Emily's first attempt at defense in basketball.)

As I look at this amazing little girl, I am reminded everyday of how far she has come. When I brought her home from the hospital a little less than six years ago, her doctors made no promises about anything. Her oxygen levels had been so low, we were told that they didn't know how on target she would be developmentally, and I did not think a thing of it that she did not speak more than a few words til she was 3 and a half. I had made my peace with accepting whatever we could get, so I just assumed this was just Emily. But as time goes on, she is really coming into her own more than ever, and as I look at this almost reading, Hello Kitty loving, tomboy basketball playing, bike riding, wrestling, sweetheart of a girl one week before her sixth birthday, I am more certain than ever that she is truly amazing!

"Look Mom, no training wheels!"

2011-06-13T19:34:00.002-04:00

Emily has been working on learning to ride her bike for ages. She is 5 and a half years old, will be six in September, so this might not seem like a big deal to most. After all, Ryan could ride a bike since the first time he touched one at age 3, and there are lots of other kids riding a lot earlier than Emily. But most other kids don't spend their first years tethered to oxygen, so to Emmers, this is a very big deal!

Last summer, she carted her oxygen around in a backpack most of the time, and it really limited her movements. She rode her bike often, with training wheels, often with the oxygen backpack. This summer, Emily has mostly opted to leave the oxygen inside, except for a few really hot days. We also moved next door to some little girls who spend a lot of time on their bikes outside, and Emily is eager to be one of the girls. Apparently, while Dad and Poppy were working on the deck out front the other morning, Emily infomed Poppy that she needs "those stupid training wheels" taken off. Well, he took them off and Emily tossed them in the trash for good measure. Emmers started riding the same day! She is so proud of herself!

Health-wise, Emily remains the same. They started her on Theophylline a few weeks ago to see if it might help with some of the shortness of breath, and the only difference we see so far is that she doesn't seem as swollen as she has. Her weight 2 weeks ago was 64 pounds, and this morning, that was down to 57. Of course, that can all change overnight with Emily, so we're not making much out of that. As for whether the medicine is helping or not, I really don't know. She still seems to pant and takes multiple breaths during each sentence, especially if she is doing something while talking. And the heat is really getting to her! A few days, she has chosen to spend hours at a time sleeping with her oxygen on. Then there have been other times when she has been extremely active, even for Emily, and she has spent a lot of time outdoors the last month or so, so maybe the medicine has had something to do with that!

All in all, Emily remains very well, and we feel very fortunate for that. Her PFT's are pretty good, and she remains stable in the problems she does have. Her breathlessness is not improving, but it is not getting worse either, so we can live with that. It has been over 2 years since she has been hospitalized. She is developing normally and for the most part seems to be a normal 5 year old. We feel so lucky and so eternally grateful for all that her doctors have done to help her over the years!

That said, we do still have our concerns, and so much about Emily doesn't always seem "normal" to us. A lot of it, we choose to ignore, and we have gotten pretty good at "tuning out" her breathlessness. If it doesn't affect her, or if it isn't something that we can change, then why should we worry about it? Still, we are left with questions.

One such question: I walked into Emily's room a few weeks ago and found her sleeping without oxygen, and her neck veins were jumping. I hooked her to the pulse oximeter, and SAT's were fine even without oxygen, but it just looked so odd. I turned on the Oxygen and this stopped. Pulse rate also decreased 30 points with oxygen. I took a short video to demonstrate what I am talking about. So I guess my question is, is this normal? Do you other ILD moms see this in your kiddos? In your healthy kids? My healthy kids do not do this, and Emily does not do this with her oxygen on, but she does when she is sleeping without it. Is this something I should even be concerned about? Is this just another example of Emily's body trying to compensate for her lungs, or is this a case of Mom paying too much attention? What do you all think?

Dad's Graduation, Ryan's race, and 2 Birthday Parties

2011-05-15T17:37:00.020-04:00

Wow, it's been a busy weekend! How many things can you cram into a few days time? And this was actually with skipping Freestyle State Finals! Imagine how crazy we would have been if we'd tried to squeeze that in too!

Friday night was Denny's graduation. We were going to take all 4 kids, but in the end, we opted to leave Emily and Braylon with my Mom, and it was really a good thing we did. There is no way I could imagine sitting there for 5 hours with those two! Ashton and Ryan did get to see their dad graduate though, and we are all so proud!
Above is Ashton, Denny, and Ryan after Denny's graduation. Congrats Dad!
And then above is a picture of me with Ryan.

Then Saturday morning was the Molly Datillo Run downtown, and Ryan competed in the boys grades K-3 half mile. Always the competitive one, Ryan was a little disappointed (tears and all) by his 24th place finish, but he did great! So proud of my Rhino!

Then today was my Grandma's birthday party and also cousin Owen's birthay party. Happy Birthday Owen and Grandma!
This is newest cousin Connor at Owen's birthday party.
Emily playing at "Imagination Station" at Owen's birthday party.
Braylon
Ryan all dressed up in his suit for Owen's birthday party. He said he wanted to look good, and I think he did!
Emily smiles for the camera.
Braylon and Owen play in cars.
All sorts of fun things to do!
Kids at Owen's party. Happy Birthday Owen!
Owen blows out the candles on his really cool cake.
And even Ashton got to play!

We made short appearances at each, then rushed home to finish up on laundry and housework. After our busy weekend, and busy few weeks actually (moving houses plus working overtime the last few weeks), it feels great to just sit down a while!

Emily has been doing well lately, except for a hoarse voice that has gone on for weeks. She has an appointment on the 23rd, so we are holding off til then. I am not sure if it is allergies (although she is taking Claritin) or maybe it is a side effect from her Advair. She has no sign of sickness, denies a sore throat, and looks great! I guess we'll see!

In other news, Emmers is working hard at her letters and numbers and will "graduate" from pre-school next week. She just brought me a piece of paper with a sentence written on it. "My cat is fat." She was soooooo excited to have written this all by herself. :-) Yup, I think my baby is ready for Kindergarten!

March 2011

2011-03-11T09:17:00.002-05:00

Emily's cardiology appointment went well. Other than the little PFO that she has always had, they did not find anything out of the ordinary from a heart standpoint. The PFO is not causing her issues, and they don't reccomend fixing it. They say we should bring her back in a few years just to make sure everything remains stable, but they do not forsee any problems with her heart.

The heart doctor said basically that Emily is out of breath because of her lungs, and any swelling is probably also caused because of her lungs during exacerbations. So as she has always been, I guess. We can deal with that, but it is just frustrating when we go to doctors and have them tell us how great she is, when anyone in their right mind can look at her and see that she doesn't breathe normally at all. What I took away from the pulmonology appointment was that Emily's pulmonary function was pretty much normal, and there is no pulmonary reason for her to be the way she is anymore, so it had to be something else. When they said we should see the heart doctor again, I almost had some hope that it would be the stupid PFO, they could fix it, and she could get on with her life. No such luck.

Maybe I misunderstood at the pulm appointment, but I am getting really tired of everyone telling me she is fixed when she isn't! She's better, yes, a whole lot better than she was as a baby, but she is not fixed. If they can't fix her, I want them to stop giving me false hope and just let her life as she is. She does okay as long as she has her extra oxygen at night and her inhalers, and steroids when she is sick. But if they can fix her, then I want her fixed! I just wish I knew how to express this to the doctors!

Part of me is longing for the old days with our old pulmonologist. I didn't like him because I felt like he didn't listen to us, but up until she was about 2.5, I felt like he always acted in her best interest and honestly was the only doctor I trusted with her life. He always knew the right thing to do. Emily survived the sickest times of her life, and I know he had a huge part in that! I couldn't have kept her going back then without his input, and for that, I am forever grateful!

Of course after the crying I did in his office right after I had Braylon, I don't feel comfortable continuing to see him, and I don't feel he has any respect left for us. Then very next run-in, he declared Emily to be cured and stopped her oxygen....and you see how well that worked out for her. And then very next run-in, Braylon was in the hospital, I had to push some residents around to get Braylon properly cared for (and yes, I am THAT mom, and I will do it again in a heart-beat, you don't mess with MY baby!), so he was angry with me about that, and we had words that resulted in my breaking down into tears yet again. Soooo.....that really isn't an option for us. We see the other doctor, who I LIKE very much. But liking isn't everything....I just want her taken care of. That is the most important thing, and I will cry, beg, scream, fight, do whatever I have to do to make that happen!

Sigh*

Okay, rant over.

So here's the thing....she is sick again, gagging, coughing, wheezing. Inhalers are keeping her going, but not well enough. It is her asthma acting up, and I pray that she improves quickly on her own, without steroids! The boys all have something similar right now too.

I am getting ready to take Ashton to the doctor in a few minutes for something else entirely, what appears to be a broken finger (thank you wrestling practice). The boys are wrestling in the Indiana Folkstyle State Finals tomorrow, so we have to find a good solution for that finger, so that it can be stable, but he can still wrestle. He is already registered, and he has his heart set on Nationals this year...so he needs to go to State to qualify.

Emily is sitting this State Finals out. We're hoping to get her back on the mat in time to qualify for Freestyle State.

Emily's "Lucky Bear"

2011-03-05T20:37:00.006-05:00

Emily's cardiology appointment is on Monday, and I would be lying if I said I wasn't nervous. But we've been down this path before, and other than a minor heart defect (PFO), they have never found anything. I guess we will see if anything turns up this time. Obviously, something isn't exactly "normal" with her, and we are hoping for answers, and an easy fix would be great news too. Fingers crossed, but hopes are not up.

Then next weekend is the folkstyle state finals. Emily qualified a few weeks ago, but I don't think she will be competing at State. Maybe next month at the freestyle finals, but I just don't think she is up for it right now.

This picture below is of Emily all dressed out for wrestling in Jeffersonville a few weeks ago. I went to take her picture, and she pulled this little bear out of her bag...stated it was her "lucky bear." In fact, this bear has been with her from the very beginning, lying next to her in the ICU during her worst days as a baby (other picture). A lucky bear indeed!

This bear has been through so much with Emily, and I was surprised to see she still has it! Maybe we should bring it along on Monday for luck!! Couldn't hurt!

Welcome Baby Connor!

2011-02-27T09:41:00.004-05:00

Yesterday, we went to my mom's for the afternoon, and the kids had a great time playing with their cousins, Emalin, Aleah, and Owen. We also got to meet their newest cousin, Michelle's handsome baby boy Connor James. Braylon was not too thrilled with him, and he stayed right with me the entire day, seemingly afraid that I was going to give him up for that baby. Emily, on the other hand, delighted in holding and loving the sweet baby.

Congratulations Sam and Michelle! And yes Michelle, you do make a pretty baby! :-)

BIG Changes

2011-02-25T17:20:00.012-05:00

Pulmonology appointment went well. Braylon was started on antibiotics and is doing much better. He was also started on inhalers, so mom's life just got a lot easier!!! No more bedtime Pulmicort battle. HOORAY!!!!!

And then there is Emily....Emily blew the very best PFT's of her entire life, which is fabulous except for one little thing. She is still breathing like crap!

So what does that mean?? The pulmonologist says that her symptoms do not seem to be coming from her lungs, and they want us to go back to the cardiologist, especially with the problems she has had with fluid retention the last several months. That appointment is scheduled for March 7. Hoping for answers, although not feeling too optimistic that we'll get any at this point. :-(

I was looking back over pictures today, and wanted to share a few of exactly what I'm talking about. I am hoping the computer will let me load them in the right order, but not so sure about that. I'll do my best.....

Emily in March of 2010 the night before the Folkstyle State Finals:

Now, this is Emily the following month wearing a wrestling singlet at the Boys Club Freestyle City Championship. She's the little girl in red. There had been a several pound weight gain in a few days time, back when her liver had become enlarged, and this picture was actually taken after all that had resolved, so believe it or not, she looked worse about 2 weeks before.

To further demonstrate how fast this came on her at that time, this is Emily on Easter morning.

And Emily less than a week later, so swollen that none of her clothes fit her anymore. This outfit was actually the best thing we could come up with because literally NOTHING fit her. We took her to the doctor, and that's when they found the liver enlargement.

She remained swollen thoughout April:

But was back to normal by May:

And since then, we've learned that it doesn't do it nearly as bad if we treat it with oxygen, although nobody will give us a reason for that. But it's happened over and over since, and she will puff up a few pounds, then lose it. Nothing as severe as that first time.

Here she is on vacation in Florida in July:

And then a few days later:

And there are many more examples, but I think you get the picture. She wakes up one morning all puffy, then one morning some time later, she'll wake up and be back to normal. The left arm and left leg are bigger than the right when she's like this. Not huge differences, but about an inch in her arm and a little under 2 in her leg.

This last example is due, at least in part, to steroids. None of the other examples were though. Emily at her brother's birthday party Feb 12:

And then 2 days later on Valentine's feeling absolutely miserable!

Part of me wants me to allow allow them to convince that she is just a chubby kid and all this is normal. But then I hear the way she breathes..... And when the doctors were telling me she breathes that way because of air trapping and it wasn't something fixable, then it was a little easier to ignore it. But then they tell me now that her symptoms are not caused by her lungs, then someone please tell me what is causing them.....

So what do you all think? Am I overreacting?

Catching up

2011-02-13T15:47:00.003-05:00

It must have been week before last that I first noticed little changes in Emily. She wanted oxygen on more, was a little more short of breath than usual, and just not quite herself. Then on Monday of last week, I noticed little wheezes, which were treated with Albuterol and went away. Monday night, she had a pretty fair bit of trouble while she was sleeping, and I had to turn her oxygen up to keep her oxygen levels in the mid 90's. The extra oxygen helped a lot, and she settled right down. Tuesday, Emily returned to school, and as the bus driver dropped her off that afternoon, she told mom that Emily had been breathing very hard at school and had to use her inhaler. Mom called to tell me this but said she seemed okay to her. When I got home Tuesday night, Emily was coughing her head off and wasn't feeling well at all. As the evening went on, Emily's sats dipped lower and lower, and by bedtime, her sats were 90 on her normal amount of oxygen, and she was not moving much air at all on the left. When she did get air through, it would sound like a very long low-pitched moan, but most breaths, there was no sound at all, not even air moving. And her work of breathing was awful. Very scary!

I've seen this before, and it almost always affects that left side moreso than the right. Not sure why the left. It also responds very well to steroids. I immediately turned her oxygen up, gave her albuterol, and ran downstairs to get her some steroids. I had to dig through the medicine cabinet and combined the remains of 5 bottles to get a teaspoonful, which is what I gave. Within a short time, she was breathing much easier.

She stayed home from school on Wednesday and went to her pediatrician, where they determined that Emily had likely been exposed to the flu, which had set her off. They put her on prednisone and Tamiflu, and she is much better already. Still coughing a lot, and now suffering from steroid side effects (tearfullness, moodiness, anger, flushed face, hunger, stomach upset, hyperness, elevated pulse, etc.). She is miserable from steroids, but she is doing a lot better. We definitely have a steroid love/hate relationship. Love what they do for her, but hate what they do to her.

Yesterday was Braylon's third birthday, and also Denny and my 12th wedding anniversary. We celebrated last night with a little party for the Bray-man. Mom and my sister Michelle came over, and we all had pizza and cake. Braylon got a big wheel and a few little trucks, and he was soooo happy. He played with his toys while the rest of us played Wii. Had a great time!

Then, of course, Braylon didn't wake up on his own this morning. Fearing he might be ill, I woke him up at noon, and sure enough, he was wheezing, coughing, had a fever, and then as time went by, ended up throwing up. So I fully medicated him, and he is feeling much better now.

Pulmonology appointments for Emily and Braylon are scheduled for a week from tomorrow. Fingers crossed that everyone gets back to normal soon. We're so ready for warmer weather!!!

Happy Birthday Ryan!

2011-01-30T14:43:00.003-05:00

Ryan turned 8 this week (the 24th). Hard to believe my little baby Rhino is getting so big! Happy Birthday Ryan! Love you lots!

It just so happened that Ryan was also "Star of the Week" at school this past week. Basically that meant this week has been all about Ryan. As I know it is hard on him being "the healthy one" and having to sacrifice so much attention to his brothers and sister, this was a really good thing for Ryan. I went to the school and ate lunch with him on Monday, he got to take in pictures of himself to show the class, he got to take in a collection of something special to him on Tuesday (he took his wrestling medals, ALL OF THEM!!!, LOL), and finally I got to write a letter to the class explaining what is so wonderful about the Rhino. I must have done alright with my letter because he came home beaming. Great to see!

In the above picture, Braylon waits to eat some birthday cake.

Above, Ryan and Emily give hugs to Mamaw's dog Dante.

And in this picture, Ashton and Ryan were having a little fun in the snow.

Otherwise, the past few weeks have been fairly uneventful. Well, in the comparison to what we are used to anyway. I've always said that if it weren't for bad luck, we wouldn't have any. My truck broke down a few weeks ago, and it still isn't up and running. Rear trailing arms (upper and lower) broke right in two on both sides, so basically the Expedition is ready to fall right apart. Felt like all the wheels were turning as I parked it, and steering was impossible. Thankfully, this happened as I was pulling out of my mom's driveway, and not on the way to the wrestling meet 2.5 hours from home that we were getting ready to leave for. Just so happens that Denny is buying me his mom's old car, and we thought it would be cheaper to put it in the shop to get the brakes done, then I'd have wheels. No such luck. It's in the shop alright, but the job turned bigger than ever, so it's going to be at least another week before we can afford to get it out. Meanwhile Denny was driving us up the hill to mom's house to have a good look at the expedition when his explorer blew up. So yup, just like that, we are down all 3 cars, and I've had to resort to bumming rides to work. Story of my life, but this too shall pass.

There has been a nasty bug going around at work, and I managed to get it a few weeks back and spent 4 days in bed with high fevers. I'm all better now except a little cough and head congestion, but now (of course) Braylon and Emily seem to be coming down with something. They've both been wheezy, pale, and moody for a few days, and Emily has been using her oxygen more often than normal, but otherwise, they seem okay. Emily did have one day of throwing up, which unfortunately began on the bus to school and gave her teacher quite a fright when Emily "turned funny colors and started gasping like she couldn't get her breath." She's over the vomiting now though, just the chest cold stuff. Fingers crossed that this is an easy bug for them to get over.

Emily had a parent teacher conference at house Friday morning, and that went well. Emily continues to progress with her pre-reading skills, and she is right on the verge of putting it all together. The teacher feels she will be reading before she starts Kindergarten in the fall, so that will be good. :)

Oxygen Helps

2011-01-07T23:49:00.007-05:00

They say a picture is worth a thousand words. If that's the case, have a look at these:

This is Emily sleeping off her oxygen. We were watching TV, and she fell asleep on the couch last night.

Note how red her face is and the pale circle around her mouth and nose. She was really working hard to breathe in this picture, and her heart and respiratory rates were all over the place. Neck vein was also visibly pounding so hard, you could see it from across the room.

Now look at Emily sleeping about a half hour later with her oxygen on. Very calm, relaxed, heart and respiratory rates down, and just look at her color! She was sleeping like a baby!

Not sure what it is about that little bit of oxygen that helps her so much, but we are so thankful that we have it available to help her rest easier. We know the difference it has made in her life.

We are having problems with nosebleeds again, and from the amount of blood she's lost lately (soaked most of a towel Friday night), I'm guessing she might be anemic again. We've tried EVERYTHING for the nosebleeds, and they seem to improve for a while, then bam. Of course I know the ultimate answer to that is to wean the oxygen to minimize nose trauma, but with watching her work so hard to breathe every time we try, how can we do that?

As always, we're open to suggestions.

Merry Christmas to All!!!

2010-12-26T13:17:00.007-05:00

We had a great Christmas with family, and the kids had a blast with all their new toys. I worked 5AM-5:30PM Christmas Eve, so by the time I got off, I was wiped out. I knew it was going to be a battle to get them all in bed so Santa could come, then be back up at the crack of dawn to get gifts open and get to my mom's by lunch, so we opted to have Santa come before I went to pick up the kids Christmas Eve. They weren't expecting that at all, so it worked out really well.

I came home and dealt with Santa right after work, then went and picked them up about 7. We drove out to Charlestown, IN to see their Christmas display (yes, in the snow).

We then came home to discover that Santa had gotten there early. Wow, were they ever surprised!

We opened gifts Christmas Eve, then were able to sleep a little longer Christmas morning before going and starting it all again.

We have a lot to be thankful for this Christmas. The biggest thing being that we've had no hospitalizations (knock on wood) this entire year for the first time since Emily was born in 2005. That is huge for us!

Hope everyone had a wonderful Christmas, and we wish you the happiest of New Years! I'll leave you with Emily's ear splitting performance. She says it all!

When You're a Celebrity

2010-12-05T10:22:00.005-05:00

Emily had her school Christmas party last week. All the Head Starts in the area met at the Moose for a lunch with Santa, and they performed their Christmas songs all together there. It was a really good turnout, and the place was packed! Thankfully, as I was coming from work so showed up very last minute, my mom showed up early to help set things up, and in the process, she landed us a table right in the front row. Couldn't have worked out any better for us!

Emily was soooo excited about the whole thing! Every little bit, she'd think it was time to go sing, and she'd make a run for the stage (and I do mean RUN)! No stage fright there! LOL! The singing itself was very short and sweet, and then it was time for lunch and then the long awaited meeting with Santa.

Does Emily believe in Santa Claus? Oh yeah! She could hardly contain her excitement in line and could not wait to get on his lap for her picture. Of course, it was supposed to be a quick picture for the school, but Emily was sure to place her "order" as well. :-) She quickly smiled one goofy grin for the camera, then turned around and started in on him. It was all just too cute, and the newspaper was there and started taking pics.

After they managed to get Emily off Santa's lap, the newspaper man chased her down and asked her what she had been discussing with Santa, her name, and her age. I kind of wondered if she'd make the paper, but of course out of the hundreds of kids there, what were the odds they'd go with that picture? Well, of course, this is Emily we are talking about, so I should have known! Emily made the big picture right on the front page! She was soooo excited, and everyone has been teasing her about being a "celebrity." I hope this link works.

http://madisoncourier.com/main.asp?SectionID=178&SubSectionID=961&ArticleID=60017&TM=38140.95

In other news, we seem to have a tummy bug floating around the house and a nasty cold as well. I've been sick for over 3 weeks now with sore throat and sinus stuff and am hoping I'll get over this soon. Ashton had a really bad asthma attack last week, and I almost had to call 911 for him at 4:30 AM the other day. His airways got so tight, he couldn't even get out the word "help," but he came out of it with 6 puffs of his albuterol inhaler, a neb, and a dose of steroids. That's the good thing about Ashton, he comes out of it quickly. Emily and Braylon have the cold as well, and we've been doing Albuterol nebs on them all week, and Emily has needed extra oxygen. They are still wheezing, but they are holding their own. Nothing a doctor would do any different right now anyway unless we wanted to do steroids, and I don't think we are to that point yet.

Christmas is approaching quickly, and I think we are getting a tree today. We've never been able to do a real tree since the kids have been born, but we are feeling brave this year. Denny was up all night doing homework and is sleeping now, but when he wakes up, he promised to take the boys to cut down a tree. This should be interesting. :-)

Kids are getting ready now, even vaccuuming for me in anticipation of making everything Christmas perfect! They are too sweet!

November 21, 2010

2010-11-21T09:35:00.004-05:00

I came to the realization today that Emily measures time by number of days with Mommy, meaning my days off work. Wow, that really makes me feel like a heel! I hate leaving the kids anyway, but to hear her tell Ryan, "You need to get ready for school. This is the last day with Mommy, and that means you have school." Well, it's actually not my last day before going back to work, and the boys obviously don't have school on a Sunday. I have one more day off this week, but she was certain that she had counted right. Her whole plans for the day had revolved around this being "Mommy's last day" which to her meant my day with just her and Braylon.

Instead, Emily, Ryan, and I went downstairs before the others got up to make cinnamon rolls. Ryan and Emily took Daddy breakfast in bed, and the other boys got to enjoy theirs when they got up. Very sweet morning with my middle two kids. And very yummy cinnamon rolls too. Just what my diet was missing, right??? LOL.

We've all been passing a nasty bug around the last couple weeks. First was Ryan sick for 2 days, then Ashton for 2 days, then Ryan for 2 more. Then we had a couple good days and then Denny got a fever and sore throat for 2 days. Several days later, Braylon came down with a fever but no other symptom. He ran 103+ for 2 days, then got okay. I wasnt' so lucky. Wednesday at work, my throat started hurting. By Thursday night, I was running a fever and was barely strong enough to drive myself home. By Friday morning, I had nasty blisters in my throat, could barely breathe, and could do little more than sleep. Fast forward to Sunday....I'm finally awake! Throat still killing me, but I finally feel like I'm joining the real world.

Only one who hasn't gotten it yet is Emily. Well of course she went to bed early last night claiming she wasn't feeling well and then woke up this morning coughing her little head off. I hope this isn't a sign of what's to come! Keeping my fingers crossed! She can't afford another lung infection.

The pictures below are of Braylon and Ryan taken last night after Emily was in bed. Braylon won these little tops from the school fair a few days ago, and he stood on a kitchen chair for hours last night trying to spin them. He likes them so much, he even slept with them! So cute! And then the other picture is of Ryan and our cat Miss Oreo. Ryan and his cat are nearly inseparable, but this might just be taking devotion to a whole new level!

Results

2010-11-09T21:53:00.003-05:00

Emily had an appointment in Louisville yesterday to get her recent test results. All in all, a really good appointment!

HRCT scan showed the usual chronic changes but overall some improvement from the last one done a year ago. Sleep study didn't show much of anything which is also good...no obstruction, no CO2 retention, pretty good oxygen levels. And last but not least, Emily's PFT's were also up! So it really looks like things are continuing to improve, albeit very slowly. It's been 5 years now since we brought home our little tachypneic bundle of oxygen tubes and cords, and at that time, we had hoped she might be weaned off oxygen within 6 months. And here we are, 5 years later....

Her doctor stressed that he does not yet feel Emily is ready to wean the nighttime oxygen, even though everything else is improving. As we're the ones who live with Emily, I can honestly say this is a good thing as well. While I'd love if we could get rid of it once and for all, I don't want her weaned until she is absolutely ready. Emily still has the shortness of breath, and when she's been off oxygen too long, she still gets very breathless and tearful. And if anyone has doubts, I challenge you to spend 72 hours with Emily without oxygen! She does still need it despite what tests might show, and she will let you know in a very loud, ear-splitting kind of way! And combined with her history and the fact that she is actually improving now, it just doesn't make sense to rock the boat. We are continuing things as is for now. Advair inhaler 2puffs twice daily, Singulair 5 mg at bedtime, and 0.5 liters oxygen at bedtime or when she otherwise needs it. Compared to the huge list of very harsh drugs she was on as a younger child, we've come so far!!!

On a very bright note, I want to offer my hugest thanks to Emily's pulmonologist for actually hearing what we say! That is so huge, especially as we've had issues in the past with other doctors not hearing our concerns and refusing to see Emily as Emily, and instead only seeing the figures and numbers instead. Yesterday, Emily's doctor had another doctor following him, and that doctor did not relate to us very well. He kept asking the same questions over and over like he was reading them from a script, and he just couldn't fathom why Emily was still on oxygen and why we say she gets so short of breath when she looked so normal sitting there on the exam table. When Emily's doctor came in, he attempted to put into words Emily off oxygen versus Emily on oxygen. God love that man! He's never had to listen to her wailing at the top of her lungs for oxygen, and he's never seen her first hand puking in her bed and crying herself to sleep because her belly hurts so bad from essentially no stomach motility. He's never seen her lying on the ground crying at cheerleading because it is too hard to breathe and cheer at the same time when it is hot outside! He's never seen her try so hard to do things other kids do with ease, just to have some sort of normalcy. He has never lived with the real life Emily, but still he can look past the numbers and the figures and the test results and see Emily for who she is, a kid who uses oxygen to help her make the most of her life. I think he really GETS IT, and I REALLY appreciate that!

Trick or Treat!!!

2010-10-30T21:39:00.006-04:00

Had a fabulous night trick or treating! Lots of pics!

It was a beautiful evening for it, and Emily did very well! We were able to walk for almost 2 hours (at a slow pace, of course), and I only had to carry Emily a little while toward the end. Braylon also walked this year and was so cute as he was really just learning what he is supposed to do. The first house, he didn't want to go to the door, but once he did and he got 2 miniature candy bars in his bucket, he grinned really big and turned around and ran towards our house. He was ready to eat it right away! When he realized he got candy at every house, he really got into it. I tried to teach him to say trick or treat and then thank you, but most of the time he would wait until he was walking away to say trick or treat. Emily, Ryan, and Braylon also had to stop to pet every single animal they saw along the way. Oh, and they also chased a poor squirrel up a tree. They really had a blast though!

And a huge thanks to the Winters for coming along and making Ryan's night!

Emily's quote of the evening, "Bray-man, I have a really good plan. When we get home, we're going to eat candy until we fall asleep." Braylon was in complete agreement, of course!

And now for the pictures:

This first one is of the 4 kids together in their costumes. Braylon is Woody, Ashton is Peyton Manning, Emily is a princess, Emily's doll Emma is a fairy, and in the back, Ryan is a very enthusiastic cowboy!

Love the expression on Ryan's face!

My sweet little Braylon Case

Ashton, Logan, and a friend as Colts players.

Braylon and Emily

Love this one of Braylon!

Braylon and Mommy

Braylon and Mommy without the glasses

Emily and Mommy

Ryan and Braylon

Braylon with Ryan's drink. Shhhhhh....Wow, this stuff is good!

Ashton and Emily resting on a bench.

Cousin Eli, his friend, and Denny...2 nerd costumes and one natural!

Happy Halloween!!!!

Testing

2010-10-29T17:04:00.005-04:00

Emily had her sleep study and chest HRCT last night/today. Sleep study was added on a little last minute, and it was a scramble to get the boys' homework finished, get packed, drop Braylon off with my mom, get Emily supper, and make it to Louisville by 7. I found out about the test at around 3 in the afternoon and was at work at the time (scheduled til 5:30). Home is 15 minutes in one direction, then 15 minutes in my mom's direction, then a good hour from there to Louisville, plus needed time to find the sleep center. Thankfully Bridget was able to get Emily showered and dressed before I got home, and my wonderful boss let me leave just before 4 or we never would have made it!

The test itself went okay I guess. I had some concerns with the way they did some things, but we won't get into those. Emily had concerns with all the wires (see pic above!!). I will say that I watched the little CO2 monitor, and the number I saw most of the time was 44.9. I also saw up to 46.9 (middle of the night) and down as low as 33.9 when she was laughing right before going to sleep. Her respiratory rate itself ran in the 30's most of the night and may have been a little higher on the O2 than off (they used mask oxygen and I don't think enough...again with the technique thing). Early this morning, she did have some rates in the teens off oxygen. Not sure what all these numbers mean or what they're going to say about them...Guess that's why we do the sleep study, so they can interpret this stuff!

The HRCT was done this morning under general anesthesia, which meant she had to be intubated. She did fine with it all until it was time for her to wake up and breathe on her own, and she couldn't seem to get her breathing in order. Even though in the end she did fine, it was still very scary for a mom to see. Pic below is when she was finally coming around a little.

She has a follow-up appointment on the 8th of November with her pulmonologist, so hopefully we'll get results then!

2010-10-20T17:53:00.002-04:00

Called the doctor, and they have decided to put Symbicort down as one of Emily's "allergies" as she has tried it twice with the same response both times. Possibly not a true allergy, but she is definitely having some sort of a negative effect from it. So now we are back to trying to figure out if there is anything that can be done for the breathlessness (which seems to be getting worse the last month or so), other than what we are already doing. Next thing we are going to try is restart the Advair and also the Singulair that was stopped in the spring with Emily's liver issues. Honestly I don't see the Singulair helping much (never seemed to before), but hey, it's worth a shot. Only other thing I can think of that ever really helped at all was the Zithromax, but which also had to be stopped with the liver stuff. I don't expect Emily's breathing to ever be perfect, but I'd like to see her back to the point she was last winter and spring. If the Singulair doesn't help, I'll ask about Zithromax again, but I know they don't really want her on any macrolide antibiotics if they can help it. If there is any real risk to her liver, it isn't worth it. HRCT is next week, but insurance is being difficult so we are going to have to wait on the sleep study. :-(

As for Braylon, he was a very sick little boy last night when I got home from work. Weak, sleepy, wheezy, snap crackly popping rice crispies in his lungs, just pitiful. Took him to the doctor and it seems he has a bit of pneumonia. He is now on neb treatments and antibiotics and is already much perkier, although still quite wheezy. Poor little guy! Hate it when my babies are sick!

Other kids are doing well. We had parent-teacher conferences Monday, and Ashton came out with straight A's, and Ryan (who is still graded by number system) has all 4's and one 3 (which is basically all A's except one B). The 3 was because of participation points in Reading class, because Ryan is so quiet in class (seriously??), he rarely takes part in their discussions. Both boys have been behaving themselves in school, and their teachers describe them as "a doll" (Ashton) and "just perfect" (Ryan). Pretty amazing really! And we won't even get into test scores. AShton's math scores were so good, Denny and I are both speechless over that one! Proud of all my kiddos, but so proud of my boys for all their hard work. Now if someone could just bottle some of that good behavior for home!

I wish there was a way for me to stay home with the kiddos. They need consistency, and they need routine. They need me here for them when they are sick and to help them with their homework. There is just so much they do need from me really, and I hate that I have to entrust others with those things. And it really SUCKS that when I get home after putting in yet another 12 hour day with sick dying people who can't breathe, the kids are already in meltdown mode! When I'm home all day, they are on best behavior. It is when I've been working that they get out of hand. I'm tired of coming home to a house that's been trashed day after day. It's a losing battle! If there was some way I could work from home, I would! Or any other way in the world I could juggle their needs and still make money. We have to pay the rent, but I hate feeling like my kids aren't getting everything they deserve, and what they deserve most is a mom! I've got 4 great kids, and their life could be so much easier if I could only be here for them more! Yes, I'm always where there when it counts, but there are times when I have to leave them home when they are so sick, and I just have to pray they will still be there when I get home. Worst feeling ever! Okay, maybe it is just exhaustion talking (6 hours sleep in the last 62, and yes, I know my thoughts aren't flowing, and no, I'm not even sure anyone can follow them, and frankly I'm too tired to care right now), but there really has got to be a better way for families like mine!!!

Thoughts???

2010-10-16T18:39:00.005-04:00

It has been less than a week since Emily started the Symbicort again, and as I feared might happen, she is coughing almost non-stop AGAIN. She was only on it about a week last time, and that is what happened then. She is also wheezing pretty bad. But then again, her brother Braylon is also coughing, so they may have just picked up a bug, BUT on the Advair, even with colds and illnesses, her coughing at least has been under control. Not true on the Symbicort. I am going to try to continue it (if we can) until Monday when the doctor office opens, then see what they want to do. I'm hoping all this is just coincidence, maybe just a transition period, and that she'll start improving. She sounds pretty pitiful though. I hate it when the poor baby is sick!

Pictures below were taken at Ryan's football game last week. Emily and Ashton chose to entertain themselves by tossing a football back and forth while Braylon played with dump trucks.

And this is a cute picture of Emily at Ryan's football game.

This is a picture taken at Emily's "birthday dinner" at Ponderosa (only a week and a half late). We asked her if she could go anywhere in the world to eat, where she would like to go, and she chose Ponderosa to eat macaroni, meat, bread, and ice cream. She wasn't lying either. She ate really well!

And then these were taken back at the football field when Emily was getting tired. She was too tired by the time Ashton's game was over that we went on home.

And then last but not least, I realized I have a pic of everyone but Ryan, so I'm putting in this one of him. This is of serious Rhino helping Daddy with a project for school. What a handsome boy!

News from the Louisville Appointment

2010-10-11T16:13:00.004-04:00

Kids had a good pulmonology appointment today. We saw the nurse practitioner whom we really like! As for Braylon, I asked about his chest, and she said they see that in a lot of preemies and it is mainly just cosmetic. It is not as severe as Emily's and is not a worry. So that's good news. Otherwise his lungs sound good, and all his orders are staying the same. He did get a new nebulizer mask. And his weight is up 3 pounds! Thank you Pediasure!!!

And then there is Emily. They asked me to fill out the little asthma assessment to see whether her asthma was under control or not. Mainly due to her near constant shortness of breath, she scored that her asthma is not under control. I'm not sure how accurate that is considering that she has other stuff going on in her lungs, not just asthma (and she wheezes very rarely), but the nurse practitioner reccomended we try Symbicort for a few weeks and see if that makes a difference. She was on that back in November and coughed the entire time, so hopefully that was just coincidental, but if she starts coughing again, I'm to call her back right away.

Emily attempted PFT's today, but they were not consistent this time, although the best one was a little better than the one from the summer. That's good news. As for specifics, I did not see any numbers so don't really know, although I do know they aren't terrible. Her lung capacity is pretty good, I know. It's the other numbers that are usually "off."

The nurse practitioner also reccomended we get a formal sleep study done (1/2 off oxygen, then the other 1/2 on it) specifically looking for other problems such as her ventilation and also look for stuff such as restless leg syndrome or anything else that may be causing her problems during sleep. They are going to call and set that up.

Then the pulmonologist added that he really wants another HRCT scan of Emily's chest to see how things are progressing (or hopefully not progressing, but improving), which due to the fact that she has ILD and can't control how fast she breathes (and basically can't hold her breath at will), she will have to be under general anesthesia and intubated. :-( This will also help the doctors get the best images so that we can get the most information from the test. We've been through it many times before, but I always hate when they sedate her! This will be done October 29.

Otherwise, it was a great visit. No wheezing, no coughing, no crackles this time. The only other thing is that the doctor came out into the hall before we left and told me that no matter what the tests show, even if everything were to come out completely normal, he strongly advises against weaning Emily off oxygen anytime soon. So for now it stays, maybe just for the time being, maybe forever. Whatever it is, we can deal with it, if we could figure out if there is anything we can do differently for the breathlessness.

That is our only complaint right now, the frequent breathlessness, shortness of breath, tachypnea, whatever you want to call it. That and the tired moody whiney attitude it leaves her with. If we could ever reach a point when we can improve those things, we will be completely happy with where we are. Not that we are unhappy now, because we do know how far we have come and how bad things could be. We've been there, done that, so we will always be thankful for how far we have come, but at the same time, there is still room for improvement.

Wrestling practice tonight, then it's back to work for me tomorrow. *Sigh* Always something!

Interesting Logic

2010-10-06T23:57:00.003-04:00

Wrestling practice went fairly well tonight. Better anyway. The boys looked great, and while Emily didn't do any actual wrestling, she did do a lot of her drills and played a few of the games. Much better than sitting in the corner crying!

After practice, she gave me a little chuckle. We were using the public restroom, and from the other stall, she called out, "Mommy, I think I'm going to quit cheerleading and just do wrestling all the time. Do you know why?"

"No Emily, why don't you like cheerleading?"

"You should know this, Mommy. I only love BOYS, not girls. You know that! I LOVE boys, and there are no boys at cheerleading, but there are lots of boys at wrestling. And at cheerleading, all the girls are all cute and pretty and stuff, but at wrestling, I'm already the prettiest one."

Didn't know what to say to that! Little chicky cracks me up!

As for if she is really quitting her cheerleading team or just all talk, we'll see. She is supposed to have practice tomorrow, and we're going to be there anyway for the boys' football practices, so we'll see....

And there is no night two....

2010-10-05T22:57:00.003-04:00

Emily did pretty well last night sleeping without her oxygen.

That is unless you count the twitching and jerking all night long. I don't think she rested very well, which is pretty typical of what she does without oxygen. I really wish I had a better understanding of why, but I don't. But anyway, her sats stayed up (93-98, mostly in the 94-96 range), and she never really appeared to be in any acute distress.

This morning she got up for school without problem, got dressed, and I even asked her how her breathing was. "My breathing is pretty soft," was her reply. "Soft" meaning the opposite of hard which is how she describes it when her breathing gets bad. Respiratory rate was mid 30's, so I sent her off to school where she remained for 5 hours.

At 12:20, she returned home. Crying screaming tantrum girl had taken over! Spent the next hour listening to her wail because she wanted milk, I put it in the wrong cup, Braylon was looking at her funny, Ashton was supposed to be at school (he was home sick today), she didn't want to stay with Aunt Bridget (I had an eye appointment), that I am HER Mommy and it isn't fair that she never gets me to herself. And then she admitted it, "My head hurts, my belly hurts, I feel too tired, and I need my oxygen." I think pic below says it all!

I carried her back upstairs with her little arms clinging around my neck, checked her sat, and it was 97% (very good), but her heart and respiratory rates were up
(170's and upper 50's), so we put the oxygen on. She cuddled up on her bed for only about a half hour with her cat and favorite doll, and she was happier almost immediately. Then Bridget came over with Daisy and Lilly, so the oxygen was off again.

I did take her to cheerleading tonight, which went better than last time. She cheered for about 5 minutes, then stopped and pouted on the ground. She cried most of the way home and fell asleep right after supper.

I went ahead and put her oxygen on her tonight, and she looks much more peaceful. Her respiratory rate is 22 breaths per minute, her pulse is 82, and her oxygen saturation is 98% on 1/2 liter oxygen. And none of that twitching jerking stuff, she's out like a light!

I wish I had a better understanding of the physiology of what is going on with her and why she can keep her sats up without oxygen, but at the expense of everything else. If you recall, during the time Emily was off oxygen at age 3, she barely spoke at all, was weak, tired all the time, vomited multiple times day and night every single day, and was just not well. That and she practically lived with "pneumonia." A little oxygen turned all that around for her, and while I would like it if she could get rid of it, I just don't know when or if that will ever happen for her.

So our little oxygen weaning experience has failed yet again, this time even quicker than the last. But last time, she spent several hours wailing, and I didn't want to push her that far again...not sure my ears could have standed that! At least we did try, and now I know what to tell the doctor on Monday when he asks.

Night 1

2010-10-05T00:14:00.003-04:00

Night 1 of sleeping without oxygen:

"Emily, are you going to be able to do this?"

"Yeah, I think so. My belly hurts, but my breathing is okay."

Relaxed resting in bed (but awake) respiratory rate was 34, pulse 118, O2 sat 98. Looking good!

She kissed Mommy goodnight, lots of giggling with Braylon, then off to sleep.

Midnight, went in to check her, and she still looks good, but she is starting to show little signs of what she does. Respiratory rate down to 25, pulse 98, and O2 sat is 94-96. Still pretty good. Thing is, everytime the sat drops to 94 (about 3 times per minute), she jerks her hands and legs (a lot like a baby with the startling reflex) which increases her pulse to the 110 range and increases the sat to 96.

Otherwise, she looks good. Not sure how well she is resting with all this jerking, but she looks good. Fingers crossed!

Cooler Weather at Last!

2010-10-04T17:39:00.005-04:00

This is a picture of Ryan and Emily taken when Emily was supposed to be cheering. Note she is on the ground playing trucks instead, and in this pic, Rhino is actually saying, "Emily if you aren't going to do it, then I'm going to take your spot." And he did too for a couple of minutes, til Dez ran him off by kicking him. Gotta love the Rhino!

This is a picture of Emily and her cheerleading team. Emily is the shortest one in the pic, 4th from the left.

This is a picture of Dez and Emily getting ready to cheer in their first game.

And this is Emily's favorite part of cheerleading, making posters! Maybe I'd be better off to enroll her in an art class instead! :-)

And yeah mom, sorry, but I've just gotta include this one! This is an adorable picture of Braylon kissing his Mawmaw through the fence at the football field!

As you can see, it's been an exciting but busy month! Emily had her fifth birthday and Ashton had his tenth birthday all within six days time! Wow, hard to believe how fast my babies are growing up! Emily's health is stable right now, for which we are very thankful. She is still breathing fast and hard (as she always has) and still has issues with pretty bad breathlessness during activity, but she's stable. The other kids have had recent ups and downs (mainly asthma related stuff and viral illnesses), but we are getting through that as well. Ashton and Ryan are both doing amazingly well at school this year which is wonderful to see! Emily is also very happy to be back in school and is doing well, having only missed one day so far this year, and that was just because she refused to get dressed for her dad because she said she had bad breathing that day. So just an "off" day, not a real illness. Knock on wood, hope the good health continues!

Braylon and Emily have an appointment with their pulmonologist next Monday, and wow, I'm already getting nervous about that! I know it sounds silly to get so nervous about an appointment that is basically just a check-up, but it seems like I never know when "just a check-up" is going to turn into something much more stressful, and I feel like there is a limit to what I can say at the pulmonologist's office also. A misunderstanding between the doctor and me just about led me to a nervous breakdown a few years ago. (No, I'm not exaggerating either.) So for back-up, I'm bringing Bridget to help me handle both Braylon (and Braylon's super loud crying) and Emily so hopefully the doctor and I will be able to hear and understand each other well, and also hopefully Bridget will give me a little extra backbone. Don't know why, but I always need a little extra in that department when dealing with specialists. :)

I'm on vacation this week, and since we are still having insurance issues and apparently don't qualify for any additional help either, and since the cost to continue to rent her medical equipment is so much, I am hoping against all odds that since the weather has cooled down to a more comfortable range, maybe this might just be the time when we will finally be able to wean the O2, right now before flu season hits. She's five now, and if it is ever going to happen, now would be the time! And if not, then at least I'll have it documented in writing exactly what happened when we tried to wean her, and it'll be recent enough (since her appt is Monday) that maybe the doctor will be able to help us line up something else. So that's the goal...no, I don't see it happening, but we're going to give it our best shot and see how long we can go, and document vitals AND behaviors along the way, starting tonight! Wish us luck!

Other things I hope to gain from the pulmonology visit...Braylon's chest is beginning to look a whole lot like his sister's, with the protruding sternum, sunken ribs, then flaring ribs at the bottom. If Emily's grew like that due to her lung disease, then what are the odds that Braylon's is also growing that way due to lungs. Right now, he has a diagnosis of chronic lung disease of prematurity, which has always seemed a bit odd to me since other than mild retractions, he never had respiratory issues at all until he got RSV at ten months old. He was a 3 pound preemie and was only on the vent 1 day and on oxygen 1 day and always had an oxygen saturation of 99-100% even throughout being severely ill with NEC as a young baby. He was always fine til the RSV, and only then did he have to do oxygen for a while. The pulmonologist wanted to do an IPFT a few months back, but it was cancelled due to illness, then wasn't rescheduled because he seemed to be doing so much better. I'm kind of wondering if we maybe should consider rescheduling that, just to be sure. He has become quite the fast breather lately as well, although probably still not fast as his sister. Also there has been recent worries by the ped that Braylon has fallen off the growth charts despite very good eating, so we are giving him Pediasure to help that. He has put on a couple pounds since then, but why would his growth have halted after finally having some good catch up growth?? I mean he was tiny for a long time, but once he did catch up, shouldn't he have held to the chart? Of course that may just be some excessive mom worrying. I'll admit it, I do worry about my babies!!!

For Ashton's birthday, we surprised him with the thing he has been wanting, the DSI. Probably the last kid his age in the county to have one, he was soooo happy! But with a perfect progress report, how could we not reward that??

And our little Emmers wanted a baby doll that looked like her! She ended up choosing her own...a brown haired brown eyed doll that she named Emma Lee. Imagine that! :-) That's our girl! I'll put pics from birthdays on as soon as I get them onto the computer.

Until next time, thanks for checking in....

But she doesn't look sick!

2010-09-08T13:34:00.005-04:00

This month will mark a few milestones for Emily. First of all, the child who was not even expected to survive her newborn period will be turning 5 on the 29th. I can't even begin to put into words how special that is. When Emily was a baby and was not doing well, we were not given much hope that she could survive. Okay, that is putting it a little mildly. To rephrase, the doctors had everyone, including me, convinced that Emily's survival was virtually impossible. I mourned the milestones we would never have while praying the doctors could somehow be wrong. After days of that, I even resorted to bargaining with God to let her have just some of those milestones and we would fit all the living we could into whatever time we had. At that point, it became about quality of life more than just quantity. I think we have done a fairly good job with that, encouraging Emily to live life to the fullest and to never be afraid to try new things. And we've been so blessed in that she has done better than anyone ever could have predicted. Her lung disease is now stable, and while she is not exactly normal, she is not going to get worse and is going to grow up and live a full life, just one with obstacles. Obstacles, we can deal with! We feel so blessed!

Other milestones, Emily started a new sport last night...cheerleading! No kidding!!! (See pic above.) Our little wrestling tomboy is now a cheerleader! BUT don't let that fool you, she is still first and foremost one of the boys. I must say that I am so proud of the way she handled herself last night! After practice, we were waiting for Ashton to finish with his football practice, so she went a few feet off to play with some of the other little girls. Definitely the smallest and youngest in the group (other girls were in the 6-8 year old range), she was a follower for a little bit, but then she surprised me. When the group of girls started picking on a little boy because they thought he was wearing a diaper, Emily turned on them and actually pushed the girls off the boy with Ryan and 2 of his friends there to back her up. Not something I expected, but I was pleased to see this side of her emerge. Her explannation was, "I like to cheer with those girls, but they were bad to that boy, and that is not nice. They wanted to pull his pants down in front of everyone, and I was not going to let them do that. They are bad girls!" Could it be? Our little Emmers is well on her way to becoming a good person, taking up for someone else!

Then today Emily returned to her final year of pre-school (pics above). She was so excited! It was a really big day for her, and she met lots of new friends and saw a few old ones too. She got to play in her classroom, go out on the playground, get on the bus and look around, and even have a snack! By snacktime though, I think she was at the end of her rope and really could have used some oxygen at that point, but she suffered through it. About 2 minutes into snacktime, she burst into tears because one of the little boys (now get this) blew on her. That's right, he puffed his cheeks out and blew in her general direction, and Emmers cried about it.

Melting down over nothing...That is a pretty good indication that Emily needed oxygen! She also had those black circles around her eyes and that skin that screamed, "I've had enough." The thing is, we don't have a doctor's order saying she can have oxygen at school. Reason being, Emily doesn't always need oxygen during the day, and the doctor hopes she can get by with just an inhaler at school with the hopes that she will continue to improve. I am really thinking more and more that we should consider having it at school just in case, for times like these (and for times much worse than these when she's surely going to be treated for "asthma"). I am going to discuss it with her pulmonologist at her next appointment, but not really sure how that will go over with anyone.

Emily just looks so good, and I hear over and over from people who don't know and don't understand her lung disease that she doesn't look like there is anything wrong with her. But watching her breathe, I can't even imagine breathing that way for an hour, and she does it all day every single day. Attached video is of Emily just breathing last night. Granted, this is lots better and much much slower than it was in Emily's younger days, but this still isn't the normal way to breathe. This is 22 seconds of video. Look at her neck. How many breaths do you count? If I had to do this for long, I'd be crying for oxygen too! But "she doesn't look sick"??? Maybe not! We work hard at helping her not look sick! That doesn't mean her disease has magically gone away. It is still there, and over the years, we've come to view oxygen as a way to help her not have to breathe like this, as a way to help her feel more comfortable, not as a sign that she is somehow sicker. I just wish the rest of the world could see that too!

August 28, 2010

2010-08-28T17:36:00.004-04:00

Pics above are of Emily taken a few weeks ago. She found a wounded butterfly and was attempting to love it back to health. Love the one with her hugging the butterfly! That's Emmers for ya!

I signed Emily up for cheerleading today, and she starts in September. Can't wait! Funny to think of my little wrestling tomboy oxygen princess as a cheerleader! Very exciting!

On the sign-up form, they made me sign that she was of "normal health," whatever normal is anyway.... I guess that other than her lungs and tummy, she's A-OK normal! I signed the form at any rate as I am fairly sure it is just a formality that I'm not going to sue them! Madison is a small town, and I know people know she has issues, and most people have seen her on oxygen. I guess we'll see how it works out. I can't imagine that she's going to get anymore roughed up than she does in wrestling, and it's not like I'm ever going to be too far away from her! If there is an issue, I'll just pull her out and put her oxygen on her, ya know.... I'm sure that's what asthmatic kids do, come out to use an inhaler. Well Emmers might have to come out to use oxygen. I don't think that's too different in the eyes of a lot of people, is it? Or maybe it is, but there is no way I'm going to tell her she can't be a cheerleader just because she has bad lungs when her doctor says that exercise is her best medicine! And there is no way I'm going to deny her oxygen if it comes up either, just to keep her looking "normal" for the people up at the complex. The heck with that!!!

I took the kids to the football field today for the jamboree. Ashton's team won both their little mini games which was great to see! Great job Madison White! In my haste to get 4 kids, water, the stroller, lawn chairs, diaper bag, and breakfast out the front door, I forgot the oxygen! Of course sitting up in the hot sun, Emily had a spell when she did need the oxygen. She got really out of breath and sat on my lap whimpering and panting for several minutes saying over and over that she needed oxygen, but she finally got okay. I am definitely going to have to be more careful in the future!

Tonight the kids have gone with my mom to Louisville for their cousin Aleah's birthday party. That should be great fun for them!

In other news, Emily is no longer anemic! In fact, her hemoglobin is now on the other end of the spectrum and is almost high at 13.8! It was almost 4 points lower less than a month ago! This doesn't really surprise me as it seems that we have finally gotten the nosebleeds under control, and also her hemoglobin has always been rather high due to compensation for her lung disease. Blood with more hemoglobin in it can carry more oxygen. Another way Emily's body works around having damaged lungs. Whatever works!

Emily will be going back to school soon, and she is soooo excited. She's already planning out what she will wear and who she will get to play with. She can't wait!

Other than that, not much to report. The boys are back to school, Denny is back to school, and I'm working as always, although I did get my hours cut a little! Braylon is doing well, a bit underweight so the doctor has me actively trying to fatten him up, but otherwise, he's doing great, jabbering up a storm always eager to learn new things.

All in all, life is pretty good right now! The usual worries, money, kids' health, etc., but (knock on wood) we are really enjoying some good healthy months! Thanks for checking in....

Got Milk????

2010-08-14T11:52:00.000-04:00

Above are pictures from the fishing tournament last week. Ryan got the biggest fish of his age division at 9 pounds 6 ounces, and Ashton won his age division. They both had a fabulous time, and Emily and Braylon had a great time watching the boats from the river bank! Huge thanks to the Chelsea Bassmasters for all they did so that area kids could have such a fabulous experience!

I took my older boys to the doctor yesterday for physicals, and it was a total nightmare! I won't get into it (and the boys are fine, just bad behavior), but I'd have rather taken kicking screaming crying Braylon to the doctor. At least I am still physically stronger than he is. I am so embarrassed!!!

As for Emily, I am again convinced that milk is the source of her GI issues. We've tried her off milk before without much results, but maybe we were missing hidden sources of milk, I don't know. But anyway, what I do know is that yesterday when I got home from work, her belly looked great. It was so soft and normal looking, I couldn't believe it! We were out of milk and had been for two days. I went to the store last night, and by bedtime, her belly was round and hard.

Then this morning, she came to see me in my room bright and early to say that she wanted yogurt for breakfast. I looked at her tummy, and it did not look too bad (bigger than yesterday afternoon, but fairly soft). I gave her one of the little Scooby Doo yogurt squeeze things, and nothing else. Within a half hour, her belly was hard as a rock. Going to try her off milk again, and see how it goes.

She is still having nosebleed issues, but we are trying a new product (thanks Stefanie), and hopefully it is starting to help. About an hour ago, she told me her nose was hurting and felt like it might bleed. I put some of the gel up in there, and there was definitely blood in her nose, but it did not pour out like a faucet like it normally does. I am hoping that might be a little sign of improvement!

Today, we are taking the kids for a picnic (and probably a swim) at Hardy Lake. Emily is already breathing hard, so we will definitely be taking oxygen.

August, 2010

2010-08-11T19:22:00.000-04:00

Not too much to update....just more of the same. Summer is here, and with it has come the heat and humidity. Heat index of 115+ degrees. Way too hot for normal healthy people, but Emily in particular does not seem to be handling the hot weather very well at all! A few minutes outside, and she is just so breathless. It is sad to see her struggle with this, especially as she wants so badly to just go outside to play.

We have had a few decent days, and we were even able to take her hiking a few weeks back (picture above). But with the super high temps the past few weeks, even short walks down the street are out of the question. I did give in and took her for a little walk on Monday morning, and we weren't a block away when she said her oxygen wasn't blowing hard enough. I turned her up, and she only made it almost another block when we had to put her in the stroller and wheel her back home. Her legs were shaking, and she literally looked like she was ready to collapse. It is just too much for her.

In other news, we did find out a few weeks back that our Emmers is anemic. She is now on vitamins for that. As for a cause, we will assume dietary (although Emily eats everything), but as she is having so many nosebleeds lately (several the past few weeks, another 2 today), I have to wonder if the nosebleeds could be contributing to her anemia. Makes sense that it could be anyway. These aren't exactly little nosebleeds.

GI stuff is still messed up too....no more puking but big belly (VERY big belly after meals), frequent undigested poops, abdominal pain that often leaves her screaming and shaking after she eats, and something yesterday that looked suspiciously like blood in her poo (hoping she just ate something red). The boys have their yearly check-ups at the doctor this Friday, so I am going to bring it up to the pediatrician again then.

Hopefully we can get her all straightened out soon because it is back to school in a couple weeks!

Worries

2010-07-23T21:54:00.000-04:00

Never a dull moment around here.

I got a call at work yesterday morning that Emily was screaming out in pain saying her belly hurt. Her Dad wanted to know what to do, so I told him to give her a Prevacid and watch her. Later in the day, I got a call that she had been throwing up green liquid but that it eventually stopped. Poor kid! I felt so bad for her, but by the time I got home last night, she was fine. Great relief.

Then early this morning, she started screaming from her bed again. Being known for her bedtime puking abilities, I immediately assumed I'd find puke, especially after yesterday. I was very pleasantly surprised to find a bloody bed instead (only mom to a kid with DGE would say that!). Poor Emmers had a bloody nose and the sight of blood had scared her half to death. Got the bloody nose cleaned up, and she is fine in that regard. Well, not really. The doctor has told me a few weeks ago that her nose is in very rough shape, and we are doing nasal washes for that. But at least she isn't bleeding. Emily can't stand the sight of blood.

Then there is the breathlessness. How should I tackle that? Truth is, there are no easy answers. We do exercise, we do oxygen, we do inhalers, and nebs when needed. It is sad to think that this might be as good as it gets with her. Huge improvement from her baby days, but still, there is room for improvement! At her request, we tried to wean her oxygen last weekend, and we failed miserably. Her sats stayed pretty good, but her respiratory rate went way up, and she cried for about ten hours straight on Sunday. She's just not ready, and at almost 5 years old, I must admit that I am starting to fear she might never get there.

The belly thing worries me a lot right now too. Her abdomen is still really big in proportion to the rest of her. She is a good eater, so I am sure some of it is just that, but I'm not convinced that is it entirely, especially after all the liver issues in the spring and now the belly puffing back up again and the green puke. Guess I will watch her. Not much else I can do.

It is just so frustrating because I don't really feel like we have a doctor to turn to for reassurance for all this. I feel in my gut that there is more to it than the doctors know but feel powerless to do anything about it. I could take her to the pediatrician, but they would look at her smiling face and say there is nothing wrong with her. She could be breathing 80 or more breaths a minute and puking all over, and she'd still smile at the doctor...that's just Emmers. The only doctor I used to really trust with my daughter's life...I hated him, but I trusted him because he was very smart and knew how to handle Emily (but was an incredibly arrogant pompous ass, her former pulmonologist)...one day all that changed and he stopped listening and took her oxygen away without looking at the whole child and refused to hear what her father and I were telling him...now I don't trust him anymore. It is just unfortunate.

On the bright side, I look at this child and laugh and laugh at her antics and adore her sweet nature. She is happy, smart, funny, active, all the things I feared she would never have a chance to be. For that, I am so thankful.

Below is a pic of my two middle kids, Ryan and Emily, taken on vacation.

Fabulous Trip

2010-07-23T01:43:00.000-04:00

These are pics from our trip to Florida for my sister Miranda's wedding. We had a beautiful trip, and Emily actually went 2 whole days in Florida without her normal breathing difficulties which was amazing to see. Of course she did return to normal after a few days, but it was still nice to see Emily get a vacation of sorts from her lung disease. All in all, a fabulous trip! Congratulations Miranda and Jason!

Some Good Results

2010-07-09T10:18:00.000-04:00

Bronch results are very good so far. We are still waiting on some results concerning her immune system, but no idea when they'll be back in. But the big news is that there are no nasty bugs growing (some bacteria but nothing that needs treated), and no sign of bleeding this time! Fantastic news! We can now leave those fears behind us and move on toward better things-- like my sister's upcoming wedding and our trip to Florida! So excited! Now if only I could keep Emmers out of the packed suitcases!

Bronch results are very good so far. We are still waiting on some results concerning her immune system, but no idea when they'll be back in. But the big news is that there are no nasty bugs growing (some bacteria but nothing that needs treated), and no sign of bleeding this time! Fantastic news! We can now leave those fears behind us and move on toward better things-- like my sister's upcoming wedding and our trip to Florida! So excited! Now if only I could keep Emmers out of the packed suitcases!

Bronchoscopy

2010-07-02T18:10:00.001-04:00

Emily had her bronchoscopy today. We had to starve her all morning for anesthesia, but she was such a trooper. I actually kept her up late last night so she would sleep this morning while I took the boys out for breakfast and dropped them off with my mom. Then I came home and dressed her and took her to the hospital. She was none the wiser about food having ever been offered to anyone else, and she did much better than I had expected.

When we got back in the pre-op area, the doctor came in and talked with us before the procedure. Then they came and doped Emily up on Versed, and I think they expected her to lie down, get drunk, and relax. Not our Emmers! She was singing "Bringing home a baby bumblebee" so loud that everyone could hear her! That's our girl!!! She got drunk for sure, but she did not chill out. She is a very loud, singing at the top of her lungs, happy little drunk! It was very hard to keep her from falling out of her bed because she kept wanting to stand up in it and dance along with her singing. I wish I had a camera!

Then they came and took her for the bronch. The doctor came back a few minutes later to say that things went well and that her airways looked pretty good. There was a little bit of redness in one area and also some visible granulation tissue deeper into her lungs, but it didn't appear to be blocking her airway. She also has a weird variant of fewer than normal number of airways in her right upper lobe, but we were told that the last time and is nothing at all to worry about. Overall, things looked fairly normal. Of course we didn't expect that part of her lungs to really look bad. Her issue is deeper down in her lungs, in her bronchioles and alveoli, and a bronch doesn't go that deep.

They did collect samples for tests which was the main reason for doing the bronch, and results should start trickling in next week. They will be looking for hemosiderin and/or lipid laden macrophages (especially the hemosiderin as she has a history and we really need to be sure her lungs don't have any bleeding, even on a microscopic level), cultures for infections, and also looking at her immune system by checking her t and b cells in fluid from her lungs.

Although things went so well, it did take Emily about 3 times longer than they had anticipated for her to wake up. She was a sleepy little girl, and she woke up a very very drunk little girl. Denny and I had joked that the first words out of her mouth would be asking for food, and sure enough, after lots of grunting and groaning and jaw quivering (which they said was just a really odd looking side effect from anesthesia), she looked at me and said, "Let's go get a Happy Meal from MissDonald's." (She always says miss, never Mc.) Had to laugh at that!

We eventually did escape the hospital and Emily got her happy meal (which, holy cow, she ate every last bite of!!!!), but she is still so drunk. We got home in time for the Regatta parade traffic, and determined not to miss anything, drunk little Miss E is looking out the window watching what she thinks is the parade (which is really just a never ending stream of cars, LOL).

All in all, a long, tiring, but very productive day. Thanks so much for checking in on our girl. I will update again when results start trickling in.

Good Pulmonology Visit

2010-06-10T20:55:00.000-04:00

Emily and Braylon had an appointment with their pulmonologist today, and WOW, what an appointment it was!

All the way to Louisville, both kids were angels, singing and playing games as we rode along. Emily would sing, "5 little monkeys jumping on the bed" while Braylon followed with "Monkey bed." Then Emily would laugh and say "macaroni" to hear Braylon repeat it back, then they would both laugh. Then they followed up with "spaghetti" and "bird" and anything else Emily could think of. Then they would go back to singing. It was pretty funny watching the kids have so much fun at just saying words back and forth.

But the moment Braylon realized we were at a doctor's office (which occurred as we were walking in the front door), he switched gears into screaming, crying, shrieking, temper tantrum Braylon. He screamed the entire time we were at the appointment, kicking, hitting, throwing himself on the floor, and with his shrill voice...let's just say my ears hurt! *sigh* He's my kid, and I love him, but I hate when he does this, and I was powerless to calm him. He really dislikes doctors that much, and I had a very hard time communicating with the doctor while trying to sooth the Bray-man. Next time, I need to bring some backup with me to help in handling him at least while the doctor sees Emily.

The appointment itself went well, except for all the Braylon fits. Braylon got checked out first with hopes that he would calm down once it was over, but no, it couldn't be that easy! LOL. The poor doctor was such a trooper though! Anyway, Braylon had a good check-up and doesn't have to return until October.

Emily started off by doing PFT's, then she was checked out by the doctor. Her PFT was the best it has ever been, a shocking 91%. The doctor feels that the oxygen is helping her finally grow some healthy lung tissue instead of scarred damaged lung tissue and also helping to prevent further vascular remodeling. So for now, he encourages us not to be in any hurry to wean her oxygen. That is fine with Emily, and certainly fine with us if it means that it is going to help her lungs grow healthier.

Her lungs sounded good, and as she has been doing fairly well lung-wise the past few months, we set to discussing her other recent issues and the things we need to get done. The doctor asked many questions about her recent liver scare. Her liver has since returned to normal, and her weight is more stable also. We are still assuming the liver thing was related to medication, although the doctor kept asking about what they thought caused her swelling and if they had looked for infectious causes. I assume they did; I know they did at least do blood cultures, but to be honest, I took her pediatrician's word for it that she "tested for everything."

At any rate, Emily will be having a bronchoscopy July 2. It was scheduled for last month, but with the liver issues, we postponed. Now, it has come up again as something we need to do. The doctor will be looking for signs of bleeding as the last bronch showed a high number of hemosiderin laden macrophages. This will be done to rule out pulmonary hemmorhage. The doctor is also going to wash out any trapped mucus and culture it to make sure there aren't any bad bugs growing in her lungs. Fingers crossed that all goes well!

Issues and no answers

2010-05-18T17:40:00.001-04:00

Been a little while since I updated last. Not sure where to start.

Emily is still having issues, but no answers. She had an appointment with the lung doctor in Cincy, and they are very pleased with her lung progress...so much that she does not have to go back there anymore. She is to follow up as planned in Louisville with pulmonology next month. Cincy doctor did reccomend we get Emily an echocardiogram due to all the swelling (to rule out pulmonary hypertension), and that thankfully came out okay. That is great except Emily is still puffy, weight fluctuates 3-6 pounds up and down, her left arm and leg at times are much larger than her right, and at times the veins in her neck stick way out and visibly "pound" (and she whines and complains that this really hurts when they are doing this). At other times, she doesn't look too bad. What we have found that what helps somewhat is wearing oxygen most of the time (her weight holds steady at 47-48 pounds on oxygen, but it jumps to 51-53 after being off for more than a few hours. When her weight goes up is when she starts breathing harder, whining a lot, complaining of the neck pain, etc. That seems to be when her arm, leg, face, and belly puff up too. Why this is happening, we don't know. This is very frustrating for us, especially as we don't feel like anyone seems to care to find the cause. I think the doctors have written it off as "side effects of medication," but I would think it would have gotten better by now. I do find comfort in the fact that at least she is doing so well, other than these "minor" complaints.

Otherwise she is doing great! She finished out her wrestling season with a Freestyle State Championship...very exciting! We are not signing her up for any additional sports right now until we feel more comfortable with whatever is going on with her health. We also have her follow-up bronchoscopy on hold for the same reason. I'm not about to rock the boat!

She continues to go to school and is doing very well. She has developed a lot of pre-reading and math skills and is even trying to spell out easy words. Not bad for a kid who has only been talking 16 months! We went to parent teacher conferences and her teacher is pleased with her. Emily will not be going to kindergarten next year due to her late birthday (missed the cut-off), but that's probably for the best anyway. She has missed a LOT of school this year, and I am not sure how "real" school will deal with that, and frankly, we're not too anxious to find out. :)

Grizzly Iron Man

2010-04-24T23:40:00.000-04:00

The boys wrestled at the Grizzly Iron Man Tournament today and did great! So far, Ryan won the Freestyle division and got second in Greco, while Ashton brought home the silver medal in each! Tomorrow is the Folkstyle portion of the tournament, so we will be up and at 'em early again tomorrow.

Emily did not wrestle today, but she came along and played with and cheered for her brothers and teammates. She must have peed 45 times today, and I am hoping that might mean that her weight will be down some...will check it in the morning.

Pics of the kids posted, in no particular order. The first is of Emily resting in a lawn chair between the boys' matches, then Braylon early this morning, Ryan with his medals (so far, hopefully there will be another one tomorrow), then the others are of Emily with teammate Skyler. Emily says Skyler is her "boyfriend," and he is a great kid to play along.

Back to a Normal Routine

2010-04-21T22:46:00.000-04:00

New pictures taken the past few weeks are above. Some were taken on the Heritage Trail in Madison, then the others were at the Boys Club with cousins Daisy and Lilly. Funny, Emily immediately thinks she is supposed to get into her wrestling stance to pose for any pic at the Boys Club. Silly girl!

All in all, Emily is doing fairly well right now. She returned to wrestling practice tonight but didn't last very long before she plopped down on the ground on her belly during the middle of a drill and refused to move. Usually this is a sign of de-satting and needing oxygen, and I fully expect that is what happened this time, but as Daddy had forgotten her oxygen bag and pulse ox, there is no way to tell for sure. At any rate, she came and sat on the bleachers with me and would not go back out there the rest of the night.

She did weigh in for next week's tournament at the Boys Club...weight still up a bit at 48 pounds. It will be interesting to see who they pair her up against next week for Championship Week.

And this is totally unlike her, but she is choosing not to wrestle at the Grizzly Iron Man this weekend in Franklin even though she knows there are trophies and other prizes up for grabs. Her reasoning is that she does not feel too good and thinks it might be better to just watch the boys this weekend, so long as she can still go to State next week. She came to this conclusion after clarifying that the Iron Man would have boys for her to wrestle, whereas State Finals would only have girls. I think she is still feeling skiddish about wrestling boys after being thrown last month.

When we got home tonight, she pretty much went straight to bed, choosing to skip dinner. Not like her at all! I'm hoping that maybe she is just tired and not coming down with something.

Good News!!!

2010-04-19T15:26:00.000-04:00

The GI office called this morning, and Emily's alpha 1 antitrypsin test came out normal. Yay!!! Not that they really thought it was that anyway, but it is always a relief when a test like this comes out okay!

Then Emily had a follow-up with the pediatrician today. Her liver size has returned to normal, and it is no longer firm. Hooray for that as well! The pediatrician and GI doctors STRONGLY feel Emily's liver enlargement and edema issues were caused by medicine. Which medicine? We don't know...possibly the Erythromycin, possibly a combination of all the meds she has taken over the years for her lungs. Right now, I am pretty much afraid to give her any meds, just giving her Advair and have given her a couple doses of Prevacid this week, pretty much just when she was vomiting though. This whole mess has me terrified to give her anything!!!

She follows up with Dr. Young next week, so I am hoping to get clearer instructions on what is medicine is absolutely necessary for her and what isn't! One thing I can tell you, NO MORE ERYTHROMYCIN, never ever again!!!!

For now, we are just so thankful that she is doing better. :-)

Update on Appointment with Liver Specialist

2010-04-13T17:39:00.000-04:00

Emily had her appointment in Cincinnati today with the liver specialist. The good news is that her liver is smaller than it was Friday, although it is still enlarged. Her weight is also down to 47 pounds. They think she is going in the right direction. They don't think this is caused by a disease, although they are doing tests just to be sure. The ultrasound and doppler were good, showing no blockages. They did a lot of labs just to make sure things are going to be alright, including alpha 1 antitrypsin....I don't know how long the labs will take, but they will call with results. They have a few ideas about what could be going on....a weird reaction Emily's body had to some virus or some medicine, or that she drank too much milk washing too much protein out of her intestines (Emily loves milk). At any rate, it has been a very frightening few weeks, and we are going to work hard to get her body back to normal. We are to limit her milk consumption (which is the exact oposite of what the nutritionist reccomended for her in October), and also we are holding all meds except Advair for now. No need to overwork the liver if it isn't absolutely necessary. And no sports of any kind for at least another week. And as for the question of could this have been caused by her sports and activity, the answer is no....they said she would be in SEVERE pain if that were the case, and she is not. This is just something weird that happened, and we hope it is something we can get under control and will never happen again. So all in all, a pretty good day!!!

Appointment Set for Tomorrow

2010-04-12T16:35:00.000-04:00

Appointment with the liver specialist and GI has been set up for tomorrow morning at Cincinnati Children's. Emily will also have another ultrasound looking for anything Madison might have missed. Fingers crossed we will get some good answers!

I went ahead and called off the bronchoscopy, which was scheduled for Friday. I figure there is no way I am letting anyone sedate her until they can tell me more about what is going on.

Will update more when I know more.

Waiting for Monday

2010-04-10T23:00:00.000-04:00

Emily remains bloated today, and she has a few new symptoms as well. She is complaining intermittently of abdominal pain, feeling like her "guts might pop" if she bends over, occasional complaints of chest pain, frequent little pale yellow poo's with blood, and now also wetting her pants every few hours when she hasn't done that for years! No severe distress though, so I'm still watching her at home, but we are very anxiously waiting for Monday, when we can get back in touch with the doctos. I don't know what we are going to do with her!!!

She was able to go watch the boys in their tournament today, and then this evening she rode in her little cart behind Denny's bike as we went bike riding. She also played a little in the park before dinner. I am just glad she is still happy and able to play considering everything.

I am attaching a few pics for effect below. The first was one month ago at the Folkstyle Wrestling State Finals, when she was barely 44 pounds. The second is today in the park, 49 pounds. What a huge difference! I really hope we can figure this out quickly so I can get my girl back to normal!

Emily's Having Issues

2010-04-09T22:27:00.001-04:00

Well, I guess it was a few weeks ago that Emily's belly started looking distended. She gained a few pounds, jumped from 42 to 44 or 45 pounds. She then started having trouble with bowel movements, and her vomiting during the night came back. I thought she was surely having motility issues again, as Emily has a diagnosis of severe gastroparesis. We had discussed motility issues with a specialist a few months ago, and he gave us a list of options to try...next step was to try Erythromycin to increase her motility (although the doctor really wanted to go ahead and fix her pyloris, but we want to avoid surgery if at all possible). So I talked to the pharmacist to see what he thought, I discussed it with my husband, and we decided we'd give it a go. Good news is that Emily started going to the bathroom easier, and she stopped puking....I hoped the problem was solved.

The only thing is that Emily continued to swell. Her belly got bigger, and in a few days time, she jumped to 51 pounds!!! Her belly was swollen, her arms and legs were swollen, and even her face was puffy. Something was up, so I have spent the week trying to track down the right doctor for the job....Today Emily had x-rays and labs. X-rays showed her liver is huge. Not knowing why, we went back to the doctor for ultrasound...which showed a normal liver (no masses, etc), just a really large liver.

So again, I heard the words I have grown too accustomed to hearing over the years where Emily is concerned. "I'm sorry, but this is nothing I've ever seen before. This is a mystery."

Solution: Emily will be seeing a liver specialist next week. In the meantime, I am to closely monitor her for signs of worsening of her condition. Change of respiratory status, increased dyspnea, a wet cough, vomiting, change of mental status or lethargy, and complaints of abdominal pain would all be signs that warrant a trip to the hospital. And in the meantime, Emily is forbidden to participate in her sports. She is not a happy camper!!!

Emily Gets a Win!

2010-04-05T23:11:00.000-04:00

This is a very short video of Emily wrestling her friend from school, Sammie. Emily was so excited to win this match! But she still maintained very good sportsmanship, going over to Sammie and giving her a hug and telling her that she did a good job afterwards. Proud of my Emmers!

In other news, Emily has been doing fairly well, except for the usual breathlessness. Still on oxygen at night and during a lot of activity, and still has issues with her tummy. She actually started a new medicine for her GI motility, Erythromycin. Keeping my fingers crossed that it helps her!

She will have another bronchoscopy next week looking for any further signs of lung bleeding. In her last bronch, there were 15 to 20 percent hemosiderin macrophages, a finding that is not quite normal and indicates that her body has been cleaning blood out of her lungs...Source, we are assuming right now that it was "just" an airway injury, so keeping our fingers crossed there will be no sign of bleeding this go round! Bronch will be done next Friday morning in Louisville. Then the last Wednesday of this month, Emily will go back to Cincinnati to see Dr. Young. I know Emily is looking forward to her Dr. Young (Dr. Lung as Emily calls her)...she wants to show off her medal from State. :-)

Emily's Exciting News!

2010-03-08T21:10:00.000-05:00

Emily has some big exciting news! She wrestled at the state finals folkstyle tournament in Indianapolis on Saturday, and she placed second in the girls PW50 weight class. Emily actually only weighed into the PW45 weight class, but she didn't have any opposition, and she really had her heart set on wrestling girls (she normally wrestles boys, and this was her first chance to face little girls). So she could have claimed the title of State Champion in the PW45 weight class automatically if she wanted to, but that's not Emmers' style. She wanted to earn it, so we bumped her up to the next biggest weight class. Emily ended up wrestling a six year old girl (Em is only 4) in the championship match who was bigger and stronger than she was, and MUCH more experienced. Our little oxygen princess didn't really stand much of a chance at all against her, but still Emily went out there with a smile on her face, fought bravely, and after losing in a very quick, very ugly match, she still exited the mat very graciously, shaking hands with the other little girl and then her coach, and then smiling for cameras all the while. Silly girl really is such a ham for cameras too! LOL! And she is tickled with her runner-up medal! We are so proud of her!

I will say that Emily has been more breathless than normal the past week or so, and she has used TONS of oxygen this week. We are talking more tanks used in the past week than we have gone through in the past 3 months! No idea why that is, if it is the extra exertion or what. She doesn't appear sick at all, just more short of breath than normal. But she hasn't let it slow her down!!!

Go girl power!!!!!

Emily's brothers also wrestled at State. Ryan had 21 people in his weight class and unfortunately did not place this time (you'll get 'em next time though Rhino), and Ashton placed 5th in the I140 weight class.

And Braylon was a very good boy for his Aunt Bridget.

I am so blessed and just so proud of all my kids!

Growing up with chILD

2010-01-30T18:05:00.000-05:00

We already knew she was a fighter, now Emily is a wrestler!

2010-01-10T22:17:00.000-05:00

Emily participated in her first ever ISWA "boys" (they didn't have a girls division) folkstyle wrestling tournament today. She weighs 42 pounds, so at 4 years old, she wrestled in the Peewee 45 weight class. She got 4th place and a yellow ribbon for her efforts! She is so excited, and she didn't cry at all! Such a brave girl!

Her wrestling itself, not so pretty. She'll get it though, and even if she doesn't, the fact that she is out there at all is such a victory! My girl never ceases to amaze me! I am posting a video of her first ever wrestling match. Might just be the shortest match of all time! Still....She has come so far, I can't help but beam with pride!

The boys were great as always. Ryan got 4th, and Ashton brought home the silver medal. So proud of all my little wrestlers!

New Medicine Worries

2009-12-02T17:08:00.000-05:00

Emily had an appointment last week with her Louisville pulmonologist. That went well, except for her PFT's were a lot worse than last time. They gave her some Albuterol,waited a little bit, then repeated the PFT's, and that showed some improvement. That is a good thing, that some of her airway issues can be reversed, but it means she has asthma on top of everything else and her current meds were not controlling it. We have known this since she was a baby though, no big surprise. This child has taken so many different meds over the years including massive doses of oral, IV, and inhaled steroids, and we have never gotten her to "normal," so I've pretty well just given up on that. She wasn't wheezing at her appointment, and she actually seemed to be doing fairly well that day even though her PFT's were worse, so I really hated to change her meds based on a test when she actually looked pretty good for once in her life. BUT I would do anything just to get her as close to normal as possible. I see her struggle day in and day out, and if this new med could help that, it would be such a relief for her and for me too.

So anyway, the doctor decided to change her QVAR to Symbicort, and I admit that I was immediately terrified about that for a few reasons, and maybe I am being quick to say that I don't like the Symbicort because of my fears. I don't know. What I do know is the following: The QVAR has been such a big help to us the past few years, and before it, we did Pulmicort as her inhaled steroid. I may as well have been giving her water in that nebulizer, the only thing Pulmicort ever did was make her breathing more labored. But within days of starting QVAR, her breathing improved dramatically. Her hospitalizations were immediately decreased by more that 100% as well. So to hear that her pulmonologist thinks the best thing for her is to stop a med that has helped her so much over the years, and to replace it with a med that has Pulmicort as the steroid component makes me scared. So as I said, maybe I am quick to judge (and possibly misjudge) the Symbicort.

Almost immediately after the switch though, Emily started coughing and gagging all the time (every 15 to 20 seconds approximately), she is hoarse, and her lung sounds are very odd. That is coming from me, a person who listens to lung sounds day in and day out for a living. I don't really have a phrase to describe her lung sounds. Mainly clear (like weird super dry clear, like a kind of clear that is fake and just odd), with a component of the sound you get when you let go of a balloon and all the air comes out in a hurry, that flop flop flop sound. Not as loud as a balloon though obviously, but you get the idea. (Or maybe you don't, I don't know??) Her cough is soooo dry too, way drier than her normal dry cough. And her O2 sats are low 90's today, and that is okay I guess, but it is worrisome as she has been running much higher than that during the day. I just don't think the med is agreeing with her.

So today I called the pulm back today, and I spoke to the nurse practitioner. I think she thinks I am crazy. She said that would be really odd for it to make her cough more and not less (but I swear her cough is practically non-stop! and this only started after starting the new med and stopping the QVAR). But she said they are changing the Symbicort to Advair anyway due to insurance reasons, and that maybe the Advair would do better for her. So I just gave her her first dose of that, and I guess we will see if it makes any difference.

Tonight, Emily has started running a temp of 101. She is still cough cough coughing too, and nothing we try is helping. Respiratory rate 64 breaths per minute. Oh, I hope this is not the start of a downhill turn for her.

In more pleasant news though, Emily had her school party with Santa today, and it was too cute! Pics are below.

The first one is of Emily as she spots Santa near the back door. The look on her face says it all. This one is of Emily shaking hands with Santa. It is Santa! WOW!!! Emily and Mommy at the Christmas party. This one is of Emily singing in her program. She is fourth from the right, wearing all pink. She really had a great day!

Look, New Gloves!

2009-11-17T21:33:00.000-05:00

Cute picture of Emily.

Horrible Week!

2009-11-15T11:30:00.000-05:00

It has been a nightmare of a week. Ashton started getting sick a week ago on Wednesday, and he ran a fever from Wednesday thru Sunday. I took both him and Emily to the doctor on Thursday of that week hoping they had not come down with the flu. They swabbed Ashton for the flu, and it came up negative, and as Emily's lungs sounded so bad, they did an x-ray of her chest which was also negative. I took them home without so much as a cough medicine prescription for either. Then Braylon started sounding bad, but Ashton and Emily slowly improved. By Monday, Braylon was needing more oxygen than his sister, so back to the doctor we went. He was diagnosed with a sinus infection, but not the flu, so he was started on antibiotics. I was also told that it was okay to send Ashton back to school at that point. Ashton returned to school on Tuesday, but as the day progressed, Braylon started struggling more and more to breathe. Emily still sounded bad, but she was handling it well for her. Ashton got home from school and he was feeling kind of tired but insisted that he wanted to go out to the college for an after school party he had been invited to, so I relented. Denny drove him out there while I was tending to the younger kids. About an hour and a half later, Denny and Ashton got home, and Ashton was in very bad shape. He just came in and flopped down, and Denny called out to me to come look at him. We could barely keep him awake, and we couldn't get him to answer questions very well. What we did get out of him was that he could not breathe, his chest hurt so bad he didn't want to breathe, and he was so tired that he wanted us to leave him alone. We had a horrible time keeping him awake more than ten seconds at a time. Braylon the baby was also struggling very badly, and I had actually been considering taking him to the ER, so when Ashton came in like this, we packed both Ashton and Braylon up and went to the the hospital. It turned out that they both had pneumonia most likely secondary to the H1N1 virus (even though they both tested negative), and Ashton had positive blood cultures as well. They both earned a night in the hospital, IV's, and high doses of antibiotics. Emily is slowly improving on her own, Ashton and Braylon are also now improving, and now Ryan has come down with a nasty cough but no other symptom as of right now. And then Friday night, Friday the 13th of course, I came down with a fever and the worst cough ever, and now I feel like I have been hit by a truck! Most definitely the flu! As I said, it has been a nightmare of a week!

Not Quite Right

2009-11-05T18:36:00.000-05:00

Something is definitely going on with Emily. I have been watching her very closely for the past week, and I am afraid she has come down with something. She gets a little worse every day which is pretty typical for how she goes into one of her sick spells. She started off breathing faster and harder than normal about a week ago and wanting her oxygen and inhaler more. Then about 4 days ago, she started wheezing on her right side. The next day, she started wheezing on both sides. Albuterol was helping, and the wheezing was coming and going. Now she has really harsh breath sounds throughout, and the Albuterol isn't really helping a whole lot anymore. Pretty typical for Emily. Ideally, I would like to start steroids at this point as I am fairly confident she will not fight this off without them, but I am waiting on her doctor to call back. I took both her and Ashton to the pediatrician today, and they swabbed Ashton to check for flu, and that was negative, thank goodness (he is coughing like crazy and running a fever). They also did a chest x-ray on Emmers due to the way she sounded, and they were going to call me back with results and instructions in "about an hour." When the pediatrician still had not called at 4:50 PM, I called back to make sure she hadn't forgotten, and I was told that she had not had a chance to look at the x-ray yet, but that the nurse had it pulled up waiting for her, and the doctor would be returning my call shortly. That was 2 hours ago, and still no call. Actually, I am fairly sure at this point that she will not be calling, but this is beyond frustrating. Why did I even bother to take her to the pediatrician?? Of course I know the answer is because that is what the pulmonologist would have wanted me to do, and I know it is frustrating for the pulm to have to be bothered by little problems like this. But knowing Emily as I do, I know good and well that if we don't do something pretty quickly, it will inevitably turn into a very big problem. If I haven't heard back from them by morning, I will call them back, and if I don't get anywhere, I guess I will skip straight to the pulm. I know they hate that, but gotta do something. Way beyond frustrated here!

Moving onto happier thoughts, I am going to share a couple of photos.
Below is a pic from Halloween Trick or Treating! My gorgeous bunch of kiddos, Ashton (the alien head thing), Ryan (death), Emily (a witch), and Braylon (my pumpkin).

And this is a picture of my favorite flag football player "Rhino" with his football trophy. Ryan's team got second in the tournament this year. Ryan, you are awesome!

Thumbs Up!

2009-10-29T21:28:00.000-04:00

These are some new pictures of the kids. There is one of Emily on our front steps, and then the rest are of the kids at Ashton's football game this evening. Emily and Braylon wore their costumes. Emily is a witch, and Braylon is a pumpkin. They wanted to stop by my work to show off their attire, but we ran late at the game, so they will just have to wait til trick or treating Saturday. They are so excited! Emily's breathing has been pretty rough the last few days. Not really sick, just breathing really hard and extra fast which is why the oxygen. We are watching her of course, and she says she feels "a little sick but not too sick." She agreed to the O2 in public which must mean she doesn't feel great, but I am just hoping maybe it is just the weather and all the exertion yesterday at her field trip rather than an actual illness. This last thing is a funny little video of Emily tonight at the ball game. She was showing her Grandpa a dance they did at pre-school which she calls the "thumbs up dance." What she is doing is trying to put her thumbs up, elbows back, feet out, knees together, head up, stick out her tongue, close her eyes, and turn in a circle all at the same time. And with oxygen tubing, that just adds to the fun even more. She thinks it is the greatest "dance" ever! Typical Emmers sense of humor though.

Looking Better

2009-10-23T22:04:00.000-04:00

Emily's face is getting back to normal after her little accident, but her speech isn't quite there yet. She is so funny talking with lots of "sh" sounds. When she grows up, she wants to be a "t-shirt" (teacher). For Halloween, she wants to be a "wish" (witch). But she is looking a lot better, and her teeth are getting tighter in her mouth on their own. We took her shopping last night, and she got a "pretty wish dress." Now we can't seem to keep her out of her costume! She is so excited and just can't wait to go "Shrick or Shreeting!" LOL!

Otherwise, we are becoming even more concerned about the flu as our neice has H1N1, and our kids still haven't been vaccinated. I called the health department today, and they said it would be mid to late November before they could vaccinate Emily and Braylon but they would do Ashton and Ryan sooner than that at school. I know the flu to any kid can be devastating, but I am so worried about what would happen if Braylon (chronic lung disease and asthma, former 30 weeker preemie) or Emily caught it. And even the older boys don't handle respiratory infections very well, Ashton with asthma, and Ryan who has had bronchiolitis more times than I care to remember. And they are my healthy kids! It is a scary time, but we continue to pray our kids will remain healthy.

Emily's Accident

2009-10-15T19:10:00.000-04:00

Emily was involved in an accident last night at the grocery store. I had tried to wait it out until Denny got home from school last night before going after milk, but at 8:30 I gave up, and I loaded all 4 kids up and went to the local store before they closed at 9. So we went in and bought a few things and were on our way back out right at closing time. This young guy (around 18-20 years old) was behind us in line, and apparently he was in a hurry. Both of my older boys were walking on my right, the baby was in the cart, and Emmers was on my left. The young guy came running full force out the front of the store and zipped right by us on my left side to jump into his truck. He nearly stepped on Emily, and it scared her, and when she jumped, she got her feet tangled up and landed hard right on her face on the pavement. Poor little thing bloodied her nose, bloodied both top and bottom lips, bruised her gums, knocked her upper teeth loose, and her nose and mouth swelled up something fierce! We took her to the ER to make sure she was ok, then today had to take her to the dentist. Apparently she checked out ok, and the dentist thinks her teeth will tighten up on their own. But she can't eat anything harder than jello. She is just pitiful to look at! And her speech is certainly different, but she is still happy enough.

Through it all, her biggest concern was that she would have to miss pre-school today (which she did due to being at the ER half the night). She didn't want to miss because her normal teacher Ms. Krissy wasn't going to be there, and there was going to be a "very special smart teacher" (a sub) there who was going to teach her lots of new things. She said none of her regular teachers would be there today, and she didn't know why, but she thinks maybe they are all going on a bear hunt. She went on to say that she hopes they don't cook bear for lunch on Friday because she doesn't think that sounds very good, so she will give hers to her friend Hannah because Hannah will eat anything. That's Emmers for you. Always thinking! LOL!

Emily Talking and Playing at the Park

2009-10-14T15:34:00.000-04:00

The boys played football all day Sunday, and between their games, we took the kids for a picnic in the park where Braylon and Emily played their own "football game." The coughing and gagging (and eventual vomiting) is pretty typical of what Emily does during activity when she starts to get short of breath. Dr. Young says it is air trapping that causes this.

Emily's Story

2009-10-11T03:20:00.000-04:00

Emily Marie was born a healthy baby on September 29, 2005. She was born five weeks before her due date, but she was big at 6 pounds 7.6 ounces. We were told that her due date had probably been wrong and that she did not appear premature in any manner. At age 3 days, she was released from the hospital and was brought home to begin her life with her family.

We were overjoyed to have our little girl home, and for a few short days, we really gave all appearances of the perfect family. Mother, father, two sons, a daughter, a three bedroom house in the country, what more could anyone ask for? But then things began to change, and it became apparent to me that there was something seriously wrong with my little girl. She was losing weight at an alarmingly fast rate, and no matter how hard I tried, I just could not get her to suck from her bottle. As the days went on, she became weaker and more lifeless, and I kept trying to get her to eat without success. I had her home from the hospital for a total of five days, and I took her to the doctor four of those five days. Something was just not quite right. The doctor just kept reassuring me that I needed to make her eat more, and I kept trying to get her to eat, but she would just lie there and let it all run out the sides of her mouth. I didn't know what else to do, so on her fifth day she was home from the hospital, her eighth day of life, we went back to the pediatrician’s office, this time seeing a different doctor.

Although it broke my heart, at age 8 days, Emily was hospitalized for "failure to thrive." By that time, she was down to 4 pounds 12 ounces. The pediatrician told me that they planned to put in an NG tube and tube feed her for a few days until she was strong enough to eat on her own. He also increased the calories in her formula and changed it to something that was supposed to be easier to digest. He reassured me that her feeding issues were probably related to her slight prematurity, and I believed him. Emily was not my first preemie, and I really thought it would just take a little time and then everything would be okay.

I was so wrong! Just two days later, everything changed. Emily had finally started sucking a little from her nipple, so I thought, “Oh good. She's going to be okay.” But then they drew labs.... And then they came in and said that they needed to draw the labs again, but not to worry because the really strange results they had gotten the first time had to be lab error. They were just too weird to be real results. My husband was there with Emily at the time, and I wanted to go home to get changed, so I left the hospital for just a few minutes. I wasn't even two minutes from the hospital when the cell phone rang, and my husband was frantic. He said, "Get back here now! They are transferring her to Louisville!" What?? She is fine. I had just left her!! I just couldn't believe what was happening!

I arrived back at the hospital not even five minutes after I had left it to find the doctor, two or three nurses, and two people from the lab standing around my baby. My husband was in the corner looking very pale with his mouth hanging open as if in shock. I asked the pediatrician what was going on, and he said that Emily had very strange electrolytes and severely low sodium levels. I asked him what that meant, and he replied that he wasn't really sure, but he thought it could indicate a serious infection. I asked him infection of what, and again he said he doesn't really know, but then he indicated maybe of the GI tract. The pediatrician had a sick look on his face, and I just knew at that point that this was going to be BAD news!

The ambulance came to get us a few minutes later. They said that I could ride in the front of the ambulance so that I could fill out papers when we got to the other hospital, but my husband had to drive down. As we were about to leave, I heard the pediatrician tell the ambulance driver in kind of a low voice to make it a fast trip. They did! We drove 80-90 MPH the entire way there!

By the time we arrived at Kosair Children's Hospital in Louisville, KY, Emily was saturated in watery diarrhea. She was lethargic and floppy, and she looked like a little rag doll. During the short time it took them to get us to a room, she had diarrhea all over everything. I was trying to clean her up, and she just kept going and going.

Finally a doctor came in, and he said they were going to do some tests but promised that she would be alright. They did a spinal tap, and they were going to run some more tests, but first they came in and drew some blood, and next thing I knew we were being whisked away again, this time to the Pediatric Intensive Care Unit. Her sodium level was down to 112 at that point, way too low! The ICU doctors worked with her all night long. They determined at that point that she had oxygen requirements, so she was started on oxygen via nasal cannula. They also determined that she did have something going on in her intestines, possibly necrotizing enterocolitis. She also had blood in her stool. They stopped feeding her immediately, and they put a tube in through her mouth to suck everything back out of her stomach. They put in several IV's, an arterial line, and stuck her over 30 times trying desperately to get a central line before finally succeeding, and they shaved part of her head trying to get even more IV's. It was unreal all the things they had running into my girl at once! And I just sat there watching in disbelief sobbing my eyes out. Then this one young doctor came over to me, put his arms on my shoulders, and looked me right in the face. "This is not your fault," he said. "You did nothing wrong." After so many days in a row of beating myself up for not being able to make her eat, I really did need to hear that.

That was one of the longest nights of my life. Emily had blood transfusions, test after test after test, and they tried desperately to get the sodium level up. Nothing they tried budged it. It stayed around 112 all night. Finally, someone gave the order to give her hydrocortisone, and within minutes, her sodium level was at 120, still not normal, but safer.

Emily's oxygen requirements continued, but by the next afternoon, her intestinal problem had resolved. Either she never had the necrotizing enterocolitis to begin with, or it had really resolved very quickly. The tube was taken out of her mouth, and the following day, she was again allowed to try to eat. Her doctors still didn't understand completely what had happened to her, though, so they kept her in the ICU. They said it was because they just didn't "trust" her. We started feeding her again, and she became much sicker again. Her respiratory rate increased into the 90's, then 100's, then 110's, then 120's. It was so painful to watch. The night before she was placed on the ventilator, she had returned to that limp, lifeless state, again had diarrhea going everywhere, exactly the same way she had been the first night we had arrived. Her sodium level was also low again. I was afraid to hold her because every time she was moved, her oxygen saturation would creep lower. We had her lying on her back, her head tipped back on a pillow to open her airway, she was breathing over 120 weird chest flailing breaths per minute, she was a very dusky gray color, and her oxygen saturations kept dipping down near 70. They cranked her O2 up, tried a couple different kinds of breathing machines, and nothing helped. Early the following morning, she was intubated. She was two weeks old.

The first time I saw her after she had been put on a ventilator, I was overwhelmed by how much better she looked. I told the doctor that I was so glad they had gone ahead and done it because she looked so much more peaceful, like she was finally able to rest a little. He responded, "No, she is not breathing on her own now. She is not peaceful." But to me, she looked better, and I really wished he could have just let me believe that she was more comfortable than she had been the night before.

Over the next several days, Emily continued to deteriorate. Her blood gases were horrible, and she lost the ability to get rid of carbon dioxide. Her pH was way too acidic, so they had to give her repeated doses of another medicine to help neutralize her pH every little bit. They drew labs every fifteen minutes around the clock. She had her own ICU doctor who only left her room to use the bathroom and to eat. That doctor stayed in that room for 3 days continuously, and I don’t know when or if she slept at all. There was another ICU doctor in and out, and Emily also had two nurses at a time. These people worked tirelessly around the clock to help my daughter hold on, and I am so thankful for them. They tried several different vent settings, and even tried bagging her (with a mask and ambu bag) and then drawing labs, then bagging her again and drawing more labs. They did this repeatedly without success. The hope was that they might find something, some rate, some depth, anything at all that might help, and then they would try to duplicate that with the life support. Nothing they tried worked. They had to sedate and paralyze her to keep her from fighting the vent. She saw I know more than 100 doctors and specialists, and those specialists were also consulting nationwide trying to find answers. Each day, I would sit by her bedside as the parade of specialists would come and go. I heard over and over, "I am so sorry, but I have never seen anything like this before." Nobody had a clue how such a perfect little baby could go from healthy to this in such a short time. She did not have an infection we were told, her heart was okay, her brain was fine, her kidneys were good, it didn't appear to be her lungs on x-ray (a few infiltrates noted but not bad enough to explain this!), and even the endocrinologists and rheumatologists said it was nothing they had seen before. Basically none of the specialists would step in and claim this illness as one of theirs.

We saw other children come in and out of the ICU. I remember being so jealous of parents whose kids "only" had open heart surgery because at least they knew what was wrong, and someone was trying to fix the problem. Each time they ran a test, I hoped it would come back positive, just so I could have an answer. I knew that no matter what kind of answer we were to get, it would not be a good one, but any news would be better than none at all. They just kept telling us over and over that Emily was a puzzle, that they'd never seen anything like her before, and that they were just so sorry.

October 16, 2005 was a really rough day. That is also the day we met one of Emily's pulmonologists. He walked into Emily's ICU room accompanied by one of the ICU doctors. The doctor who had been keeping vigil at Emily’s bed side stepped out. Unlike the rest of the doctors we had seen who were all just "so sorry" but didn't know anything either, this doctor didn't mince words. Emily was lying on her little table with her tubes and wires going every which way, and as usual, I was sitting on my little stool stroking her little head. They asked me if I wanted to go to the family conference room. "No way," I thought to myself. I knew they gave bad news there. There was no way they could have dragged me into that room! I told them no, that I was fine where I was. Then they began to tell me the hardest news I have ever received in my life.

They had done a CT scan of Emily's lungs, and the results of it were "quite impressive." Somebody really needs to tell those doctors not to use that word to us parents!!! Impressive, that's good, right? NOT WHEN A DOCTOR SAYS IT! I just nodded. He went on to say that the reason they could not find a setting on the ventilator that would work for Emily was that she had progressed beyond respiratory distress, that this was severe respiratory failure. The CT had shown that Emily had no normal lung tissue left. Needless to say, I was in shock by this point, turned my attention to my daughter and just continued to stroke her head trying hard not to cry as he started talking about the importance of finding the cause if she was to stand any chance of surviving at all. The pulmonologist looked sad as he struggled through every horrible word and just kept going on and on about how sick my daughter was. I tried so hard to process what he was telling me, but I just couldn't understand. How could this be happening?? He then looked to the intensive care doctor, and I heard him say in a really harsh voice that he had done the hard part and that she could document it. He then looked back at Emily and me and said something, and he looked very sad by this point. I was beyond understanding words, but I did understand that awful look on his face and the shakiness in his voice. And I couldn't help but just glare back at him. I was so hurt and so angry at him for saying those awful things about my daughter. He then left in quite a hurry.

The intensive care doctor then tried to repeat to me what he had said. She knew I hadn't taken it all in. They were going to do a lung biopsy the following day. It was Emily's only chance at survival, but even then, her chances were very poor. It appeared that Emily had a severe lung disease that for the most part was considered to be "incompatible with life." She then started talking about surfactant deficiencies, and at the time, I really did not understand it all. It seemed that Emily's best hope was that her biopsy would show some other cause, but with the damage that had already been done, it was still very unlikely that she could pull through no matter what the biopsy showed. But if biopsy did not confirm their suspicions and it proved that she still had some chance, they may consider using ECMO (heart-lung bypass machine) for her to let her lungs rest and give them a chance to heal. But as it looked at that point, ECMO was not an option for Emily because they didn't feel it could change the outcome. Emily's best hope at that point was that biopsy would show a horrible devastating infection which at best would leave her a very slim chance of making it. So the following day, we sent our daughter into biopsy praying that she would have pneumonia. How awful is that??

The night before the biopsy, I began to accept that this might just be all the life my little girl would ever get to experience. It seemed to me that all the preparations at home had been for nothing. She would never again see the room I had painted for her. She would never get to wear the little pink dresses and sleepers I had bought her. I had other sons, but she was my one and only daughter, the little girl I had always dreamed of having, and she may never even again get to live life not attached to machines. It was at that point that I decided that she had to know that life was worth living, that there was more to life than pain, and I finally left her side that evening for a few hours so I could go to the store and pick her up a few things she might be able to appreciate right then and there even in the sedated paralyzed state she was in. I really feared this was all the time we would have left, and I was going to make every second count. I returned a few hours later with a little pink hat, little soft pink slippers, a soft pink blanket she could lay on, a teddy bear with her name on it, a CD with classical music lullabies, and a baby book so I could fill it out and have proof that she had indeed lived. That night, the nurse and I made footprints on her hospital photo (the one that showed my beautiful healthy daughter that I had brought home from the hospital only a few weeks before), we filled out that baby book as accurately but as positively as possible considering all she was going through and her young age (examples: favorite food was TPN, favorite toy was that teddy bear I had just bought her, favorite music was that CD I had just bought her, etc.). We also took many pictures of her "all dressed up" in her new hat and slippers and played her lullaby CD over and over for her. Maybe if she could just feel how much I loved her, she could hang on just a little bit longer until someone could find an answer for her. And amazingly enough, Emily held on through the night and next morning until it was finally time to say goodbye so they could take her to biopsy.

Minutes before the procedure, the intensive care doctor came and got us. She wanted us to see Emily's CT scan for ourselves. I think she knew that I still was clinging to my own reality, that this was something that would pass. So anyway, she showed the CT to my husband, my mother, and to me. My mother left crying pretty quickly upon just viewing the images. The doctor said, "See how consolidated everything is. Her lungs appear more liver-like than like anything else. There just isn't any healthy tissue there to exchange gases." I knew there was more she wanted to say, so I asked her, and she told me that Emily probably would not even survive the procedure, but she had deteriorated to the point that after biopsy if ECMO still wasn't an option for her, they may ask us to consider discontinuing life support. She had a very pained look on her face, looked me right in the eyes, and said there just may not be any point in putting her or ME through anymore.

I had known it was coming, but by that point, things seemed pretty hopeless to me, and I no longer understood why we were being asked to put her through biopsy if she was going to die anyway. I mean here we were sending our little baby off to lung surgery, going to let them cut her open, and then if she survives that, they're still probably going to bring her back and unplug her! That was what I was hearing. So I forced myself to choke out the words and asked her why we should put her through it. Her only answer was that the doctors don't like to give up until there is absolutely no hope. Right now, there was still a chance, a very slim chance, but still there was some hope. She then said that if this turned out to be something else, recovery might be possible but would be very long and hard. That's all I needed to hear. I told her that we were up for the challenge, that I knew that Emily wanted to live, and it didn't matter to me if I had to put my life on hold for the next several years, I would sit right there in that hospital and wait if that's what I had to do!

Then we got our miracle! Emily's initial biopsy report was inconclusive, and her blood gases started to improve on their own. She returned from the biopsy slightly more stable than she was when she went in. They had to hold on a little longer and continue to wait and see what the final word was on the biopsy. Emily never was placed on the ECMO machine as they really felt that even it could not save her, but a few days later, she started holding her own a little better on the ventilator. And by my 28th birthday, October 27, 2005, Emily was extubated, and I was allowed to hold her again. Best birthday gift I have ever received! She again tried the Vapotherm machine (didn't work for her) and then went to CPAP. And after a few hours of that, it looked like she was going downhill again and had another air leak forming, and they were starting to consider going back to the ventilator and placing a third chest tube. But then, by complete accident, they began to suspect that her lung disease was steroid responsive, and by simply changing the steroid they had been using to support her sodium level to a stronger, different type of steroid, it enabled them to wean her to nasal cannula oxygen. That enabled us to bring her home in November 2005, oxygen and steroid dependent, but very much alive.

At our first pulmonary appointment after Emily was released from the hospital, we were told that she had an interstitial lung disease and that her biopsy had shown diffuse alveolar damage (DAD). The cause was unknown but may have been an unidentified virus. ILD in children is extremely rare we were told, and there was no real prognosis for her. She would either improve as she grew, would continue to struggle, or she would develop wide-spread, possibly end-stage fibrosis. You've got to be kidding me! I had finally found out what was wrong with my daughter, and they still didn't know!!! But there just had not been enough research done for the doctors to know what would happen in a child like Emily.

As it has turned out, Emily remained on oxygen 24 hours a day for a long time, and she actually learned to crawl and to walk tethered to an oxygen machine. When she was 3 months old, we learned that her ILD was getting worse again, and they changed her medications at that point. The following few months were bad for her, and she was hospitalized at least once a month those first five months for ILD exacerbations until spring finally came around and she started to improve. The first CT that showed improvement came when Emily was around seven months old, and her doctor literally jumped for joy in his office as he told us the news. Since then, she has endured countless battles with illness (the sniffles to any other child almost always equaled pneumonia in Emily) and several hospitalizations. Through it all, she has always remained a very happy, pleasant, sweet, playful little girl. At somewhere around 14-16 months old, there was a change in Emily, and she became able to go longer periods of time without oxygen during the day. And in April 2008, after 2 years 6 months and 16 days of in some way or another being attached to supplemental oxygen, we got another miracle. Emily was finally weaned off oxygen.

I hoped the fact that she was off oxygen might be the end of her ILD story, but as it turns out, it is not. Emily was off oxygen for a total of eight months, and during that time, her speech stopped progressing. Actually, a more accurate description would be that her speech went away. At her third birthday, she could only say a few words, and that was if you could convince her that it was worth the effort. She sweated a lot especially at night, her shortness of breath became so severe that she could barely walk up a flight of stairs to her bedroom, and she had no energy. She also vomited all the time, even in her sleep multiple times each and every single night. She continued to have frequent “asthma” spells that would require steroids or hospitalization. Still, her pediatricians and pulmonologist assured me that she was going to be fine and that I needed to stop worrying. I was told that this was asthma, but as my son was asthmatic and Emily was completely different from him, I began to doubt it. Her pulmonologist asked me one day why I worried so much about her now when she was so much sicker as a baby, and I couldn’t give him an answer at the time. I have now realized that it is because then when she was a baby, I expected her to be the way she was because I knew she was recovering from severe lung damage. The reason I was stressing after a few years was that there was still something wrong, but her doctors no longer saw it. They had taken her oxygen away, and I had no way at home to help her. Basically, I was treated as if I had become a neurotic parent, and they actually convinced me that maybe I was. I tried to do everything they said and just took their word for it that she would get stronger if I listened to their advice. They said Emily no longer had ILD, and her big issue was now only poorly controlled asthma, so I believed them.

Then in December 2008, Emily got sick, and she could no longer keep her O2 saturations up in the 90’s during sleep. I took her see her pulmonologist, and he started steroids due to her wheezing, but her O2 level at the doctor’s office was fine. So again, there I was, left looking crazy. Of course at the doctor’s office, she had been awake, not asleep. Time passed by, and I noticed awful jerking during sleep. This was happening every night, and she was barely getting any rest at all. So back to the pediatrician we went, and surprise, by then her O2 level was only 85 awake. I was told that was because she had been sick and when the steroids got into her system, she would get back to normal. They were not overly concerned. I did not trust that, so I again scheduled an appointment with one of her Louisville pulmonologists. It took a week or so to get her in, but when we finally saw him, Emily was diagnosed with an ILD exacerbation and pneumonia. She was hospitalized for almost a week, and she received high doses of steroids to get things back under control. During that time, they were again unable to wean her completely off oxygen. So again, we brought our daughter home on oxygen, this time only at night and during illness or physical activity.

Since that time, however, she has really thrived. She has more energy, and she started talking complete sentences almost immediately. Her shortness of breath continues, but it is a lot better. Her oxygen levels are back up, and according to her oxygen saturations, she probably no longer “needs” the oxygen, but due to the complete change in her for the better, her pulmonologist is now unwilling to even consider weaning her oxygen again until sometime next spring to summer (2010). She does continue to have sick spells every few months that respond only to steroids, but she has had no further hospitalizations since January 2009.

In May 2009, she had another HRCT, and it showed new changes including early bronchiectasis and cysts in her lungs, changes which were consistent with ILD. I had been told for months that Emily’s big problem was currently just asthma, so I was a little taken back when the same doctor told me that no, it was ILD after all and things were changing for the worse. No longer knowing what to believe or whom we could trust, we decided to get another opinion. At that point, we decided to take Emily to a chILD center. This pulmonologist went over Emily’s case very thoroughly. She carefully listened to everything her father and I had to say and spent hours going through everything. She really seemed to care. She ordered a few tests, and it turns out that Emily does still have a form of ILD.

She also re-reviewed Emily’s lung biopsy from before, and she showed it to us during a recent appointment. I had heard in the past that it was very bad, but after seeing it myself, I better understood just how bad it was. The doctor says it is one of the most dramatic biopsies she had ever seen, and she is almost positive that Emily’s disease was caused by an unknown infection and sepsis as a baby. She could think of nothing else that could have caused such severe damage. She went on to say that most people including adults who have diffuse alveolar damage (DAD) do not make it any longer than a month or two, and of those few who do, they almost always remain symptomatic. She went on to say that Emily’s DAD was very severe, and her biopsy actually showed necrosis (dead lung). With tears in her eyes, she indicated that the fact that Emily survived at all just goes to show the regenerative powers of a human body, especially in a baby. She also indicated that she is not surprised at all that Emily remains symptomatic.

And then at long last, in October 2009, after four years of scratching our heads wondering why our daughter breathes the way she does, why she needs oxygen, and why we can’t control her “asthma” no matter what we do, we finally got an answer. The chILD specialist had us repeat Emily’s HRCT scan, and I had thought maybe it might show changes one way or another (she has been doing fairly well since summer, so I hoped it might show improvement), but I never thought we would get a firm diagnosis out of it. But then, there it was, at long last we had our answer! Emily has Bronchiolitis Obliterans! What this means is that she has scarring in her bronchioles (smallest airways) which is causing her to trap air making it harder to breathe. This scarring likely came from her initial illness as a newborn. Since scarring is permanent, she will never completely recover. The doctor predicts that Emily will always get very sick and need steroids with any illness clear into her teenage years and beyond, and her oxygen needs will continue to come and go as they do now. However, since she has improved over time, it should not progress any further, and she may improve more as she continues to grow healthy lung tissue the next few years. But she will never recover. The doctor likened her condition to cerebral palsy, only of the lungs.

In her short lifetime, ILD forced little Emily Marie to evolve into a little girl who understands things far beyond her years. Twice per day, Emily willingly takes her medicine because she is afraid she might get sick again if she doesn't. If we forget it, Emily does the reminding. She knows which inhaler she uses for what purpose. She can put together and take apart the nebulizer and has shown more than one sitter where to put the medicine in it. She has come to understand that oxygen helps when she is short of breath, and she can put it on, take it off, and adjust the flow all by herself. Emily’s life has not been an easy one, but she has learned to adapt and actually considers herself a completely normal kid. She has many friends at the local Boys and Girls Club, and they do not even bat an eye when she wants to put her oxygen on. It is just part of who Emily is.

And it is so wonderful to see her do things that at one time, I never thought would be possible. Little things like walking, talking, and running and playing with other children are such a blessing. She is such a special child. She is an extremely proud and helpful big sister to her baby brother and an adoring little sister to her two older brothers. She goes to all their sporting events and practices with them and delights in watching them play hoping that one day soon, she will be out there with them. She intends to join their wrestling team this fall, although that is certain to be a challenge with her breathlessness and oxygen needs. She is definitely a high spirited girl. Her doctors would shudder to hear it, but Emily adores animals and desperately wants a "buppy" (puppy) of her own. She likes to sing and is so funny dancing around in front of the television as her older brother puts on a show with his "Guitar Hero" game. She likes to go shopping and loves to get dressed up in pretty clothes. And she believes she is a real fairy tale princess. She gives the sweetest hugs and her enthusiastic "yeah" with two syllables is the cutest thing. She has become a celebrity in town, and wherever she goes, everyone knows and loves Emily. Even the ladies at Wal-Mart get so excited when they see her coming. You can hear them call out, "Emily is here!" I don't think there is anyone who has ever met Emily who does not adore her outgoing nature or sweet disposition, and I am so blessed to have her in my life. She is my own personal miracle.