Addy's Story

Adeline Mary Johnson was born,
 fast and furious, at 2:28 a.m. on May 16th. 2012.  She arrived after only about 2 1/2 hours of labor with barely enough time to get to the hospital.  She was tiny and beautiful, weighing only 4 lbs, 4 oz.  She was placed on my chest after birth and seemed perfect.  It soon became clear that she was having significant difficulty breathing and was not "pinking up" like she should.  She was whisked away, up to the nursery to be evaluated and treated while we waited for Liam to arrive.

She received intensive treatment and supplemental oxygen but was still having trouble maintaining her oxygen levels and was working very hard to breathe.  It was quickly decided that she needed more extensive care than could be provided by Wentworth Douglas and transport to the NICU at Maine Medical Center was arranged.  An entire team of doctors, nurses and respiratory therapists was sent for both Adeline and Liam.  Liam was stabilized quickly and left for Maine med first.  While evaluating and stabilizing Adeline for transport the doctors discovered a large pneumothorax (air leak) around her right lung.  She had two chest tubes placed and was intubated before leaving for Maine Med.  I was then discharged and Bill and I made the trip to be with our babies.
   

After arriving at Maine Med, Adeline underwent many tests and was hooked up to an osculating respirator.  The pediatric nephrologist was then called to ultrasound her kidneys.  During the pregnancy she had been followed by a specialist and we were sent for genetic counseling as her kidneys appeared enlarged starting around 12 weeks.  The doctors were unable to diagnose this prenatally as her symptoms did not exactly match up with any specific condition.  When the nephrologist came in to tell us what she had found we were shocked.  As it turned out Adeline's kidneys were enlarged due to a recessive genetic disorder called polycystic kidney disease.  The doctors were very honest with us about her chances with this diagnosis.  Unfortunately, the kidney disease had also caused pulmonary hypoplasia (small lungs).  We were told that if she were to survive she would likely need dialysis and kidney transplants at a very early age and that the mortality rates with this diagnosis were very high.  We refused to give up on her and held out hope that her lungs would be big enough to support her and that her renal function would be enough for her to survive.
     We were awoken by the doctor at 12:30 a.m the next morning as they discovered another pneumothorax around her right lung.  This one was in a position where they were unable to use a chest tube to drain it.  We were told by the doctor that this was a sign that her lungs were just too small to support her life.  We continued to hold out hope though discussed the options with the doctor.  We decided that if she was able to fight we would let her but if she gave us a sign that she couldn't fight anymore we would let her go rather than use any heroic measures that would likely inflict pain rather than extend her life.

 
Sawyer was able to come up to meet both babies on Thursday and was quite taken with both of them, especially his baby sister.  He loved peeking at both of them through the portholes on the isolettes and continually ran back and forth between their rooms to check on them.  We had many other family members visit including my parents, sister, brother in law and cousin and Bill's mom, sisters and nephew.
 
 Adeline was able to maintain her oxygen levels with the maximum
support they could give her.  Thursday afternoon they decided to try turning her respirator setting down slightly to see if she would be able to maintain with slightly less support.  Unfortunately, her oxygen saturation dropped quickly and did not go back up when they turned her respirator settings back up.  After what felt like forever, it became clear that she was not going to recover and this was her sign to us that she couldn't fight anymore.

 We decided to have her switched to a regular respirator so that we could hold her for the time she had left.  She held on for a long time and Bill and I were both able to hold her.  Sawyer was able to be with us as well to say goodbye to her.  Our families were also able to be there to say goodbye.  Once everyone else left Bill and I decided that it was time to let her go.  She was such a fighter and we wanted to let her rest.  We had the nurse remove all of her monitors and the respirator tube and we stayed with her until her heart stopped. Bill then gave her a bath and dressed her in a beautiful layette and held her for a while longer.
     While this is the hardest thing we have ever been through we consider ourselves fortunate to have had so much time to spend with our beautiful baby girl.  We have answers that many parents never get and were able to meet her and get to know her for the short time she was here with us.