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.
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.
