Our daughter was 8 months old when she passed away at Texas Children's Hospital in Houston, Texas. She had been through so much in such a little time. She was born on January 26th, 2004. The birth was quick, everything appeared to be fine and there was no indication of any problems during the pregnancy. Daddy went with Ava to the nursery where she was accessed, cleaned and bundled up to be released to our recovery room. One of the nurses noticed she was a little blue around the lips and nose, after checking they found that her oxygen saturation level was a little bit low so they wanted to monitor her. Eventually her oxygen level improved and we were relieved. However, the following day we were told that a blood test had revealed that her bilirubin level was higher than normal and they wanted to take a second blood test. Like a lot of people, this was the first time we had heard the word bilirubin, but we all now know how that word rules your life.
The second blood test revealed a slightly lower number but still elevated and the neonatologist said that they had contacted a GI and they were going to do an ultrasound on her belly. We weren't sure what to think, we had no idea what was in store. I went with Ava during the ultrasound and remember them talking about the gall bladder. It turns out, of course, they couldn't find it. Since Ava had eaten prior to the ultrasound, they waited awhile and performed another ultrasound just in case her stomach was blocking it. That was the second day of her life and we still had no grasp of the seriousness of what was happening.
The following morning, a heart murmur was detected on Ava and an echocardiogram was performed. That afternoon was when we were bombarded with the possibility that Ava had biliary atresia. However, they still held out hope because she had a cyst near the liver and they thought that maybe the problem. At the same time the GI was talking to us about possible scenarios, the neonatologist walks in and says that Ava has a problem with her aorta and she will be taken to the NICU. It turns out that she had a narrow spot on the aorta and the condition is called coarctation of the aorta. We spent several days in NICU and then transferred to Children's Hospital of Austin to await surgery to correct the aorta. In the meantime, further blood tests, ultrasounds and HIDA scans fail to convincingly diagnose biliary atresia. Therefore, after her heart surgery and recovery, exploratory surgery takes place to definitively diagnose the bilirubin problem. Ava was found to have biliary atresia and also malrotation of the intestines. The Kasai was performed and her intestines were adjusted at 22 days old. This was February 17th and just the beginning of our journey. We dealt with the same issues that every other parent here has dealt with. The constant hospital admissions, infections, feeding problems, feeding tubes, IV'S, blood draws, etc.
Unfortunately, her liver disease progressed for the worse and Ava was put on the transplant list on August 11, 2004. Two days later were her 6 month immunizations and since one was a live vaccine Ava had to be put on hold status for 4 weeks. Later, the transplant coordinator had said two livers did come up, during this time period, but Ava had to be passed over because of the live vaccine. She was put back on the list September 11, 2004 and we got our first official call 3 days later. Ava and Daddy were already at the hospital being discharged on this day but they said we weren't going anywhere because we got a liver. Unfortunately, after waiting 9 hours, we got the call that the liver was too big, even for a split liver.
Ava had many complications the biggest being her weight gain and size. After stalling at 12 pounds, Ava had a central line placed in early August and she was finally gradually gaining the weight and getting bigger. Ava was initially listed with a PELD score of 18 and it had increased to 20 with all the complications, so we felt secure that at some point we would get the real call. Throughout all of this, Ava was still her usual adorable self and took everything in stride since she was too young to know any better. The doctors felt that with 2 offers during her hold status and the one call after being back active, that the right liver would come soon. We had time to allow her to keep gaining the weight and get bigger.
On September 20th, we were admitted back to the hospital with ascites again and this time it is decided that we will just stay in the hospital until transplant since we were just waiting for the call. The ascites is dealt with, but Ava is found to have another urinary tract infection, so she has to get antibiotics again for that. Ava was found to have a urinary tract reflux, which was causing the infections so surgery was planned to fix the problem she was having with her bladder. On Sunday, October 3rd, we were put into the ICU because Ava quit urinating and we were told later that Ava's kidneys had shut down and she needed to get hooked up on dialysis. This made any possible transplant secondary as this problem now needed to be worked on before a new liver or living donor portion could be transplanted into Ava. The first attempt at administering the dialysis had to be stopped as Ava's blood pressure and oxygen saturation levels dropped to dangerous levels. During the second attempt at starting dialysis, Ava passed away as her little body just got tired of fighting all this.
We learned much more about liver disease than we ever thought we would. Ava touched so many lives and is an inspiration to us. There will always be an empty space in our hearts, but we know she is in very capable hands free of pain and in true peace. We thank her doctors, Dr. Berhane at Children's Hospital in Austin and Dr. Karpen, her transplant doctor in Houston. We appreciate everything they did for our little angel.
We all miss you baby girl.
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-The support, information and encouragement provided by the PPFL parents is not meant to take the place of medical advice by a medical professional. Any specific questions about care should be directed to a health care professional familiar with the situation.