Worries

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

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

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

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

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

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

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.