Synopsis and Update 4-4-12

Hi All, decided to switch formats for updates on Claire so look here from now on. I will do updates as I am  able and this will not be every day. As I'm sure you realize, there is a finite amount of time and energy available. If you're just joining us, here is a synopsis:

Claire was born March 16th at Providence St. Vincent. She came about 6 weeks early after Mom was on bedrest for about a week. At first, we thought she would only be in the NICU a short time- just long enough to get "caught up" and then we could take her home no problem.

However, Claire began to show signs of illness- something that could not be attributed to her simply arriving early. Her thyroid function was very low and she was started on thyroid medication as a result. The doctors tell us that she will likely be on this medication for her lifetime. Claire then began having heart rate and breathing troubles and began to need increased breathing support as a result. The doctors initially suspected infection and began her on several broad spectrum antibiotics and also tested for viral infections. Claire's digestion also slowed down and the function of her pancreas came into question. Her blood cell counts were also very abnormal, with very low white blood cell counts.

On March 25th, Claire needed to be placed on a ventilator that did all of the breathing for her. Her feeds were also stopped and she began receiving all of her nutrition through her IV because nothing seemed to be moving through her system as it was. Later that same day, she needed to be placed on an even higher level ventilator. We were told that there may come a point when they are no longer able to support her.

Throughout last week, Claire managed to hold steady. The doctor continued to say that it seemed like Claire had dug her heels into the sand. She made some small gains, and remained on the ventilator. None of the tests that they had done for infection of any kind came back positive. The doctors began to lean more towards an underlying syndrome or disorder that would be causing her to be so ill as opposed to infection. Just to clarify, none of her major issues have stemmed from her being premature alone. The doctors have told us many times that babies of her gestational age can do extremely well if they are healthy.

On Saturday, Claire's CO2 level continued to climb, despite adjustments made to the ventilator. The doctor told us that we should stay close. Fortunately, Claire stabilized and has remained steady since. The goal is to be able to wean her as she can tolerate from the high level ventilator. They have tried to make some adjustments, but she has tended to need to go back to the levels she had started on.

On Sunday we authorized a small skin biopsy to obtain cells for further genetic testing. The two syndromes that they are testing for specifically are Pearson's and Shwachman Diamond- two very rare conditions. The test for SD should be back in a couple of weeks; Pearson's should take about a month. The testing was prompted because her doctors have been unable to find other explanations for her condition and because her symptoms seem to line up with both syndromes. Jerre and I are looking at these tests as beneficial to at least rule out or confirm these possible diagnoses, although we have been told that a diagnosis for one of these would likely not change what they are doing to support/treat her currently.

We also learned this week that Claire has a small blood vessel going from her heart to her lungs that is still open- it normally closes off in newborns. Although this is not a root cause of her problems, it is probably not helping anything because it puts more pressure on her pulmonary circulation. It is possible to do a quick bedside surgery to correct the problem, but the doctor is concerned about how she might tolerate this in her fragile condition. They will repeat the echocardiogram tomorrow to get another look. The doctor may also restart feeds tomorrow if things are looking good.

So, that was a lot of medical information in a very short period of time- and that is the short version. We are so thankful for every moment with Claire, especially when she opens her eyes and looks around with wonder and curiosity. She knows the sound of our voices and cues in on us when she is awake. She enjoys her pacifier, squeeszing our fingers, yawning, and finding her fingers. She has beautiful hair and a precious countenance. Looking at her, it is hard to believe that she could be anything less than perfect. Thank you for your thoughts and prayers as we navigate these ups and downs and search for answers that have been elusive thus far. We are living a life right now that is far from normal and is fraught with chaos and uncertainty. It means everything to have you pulling for us.