Dear Evie Jayne Jayne,
I realize if I'm going to have time to blog through last year before your birthday next weekend, I'm going to have to consolidate a little!
Last fall was a busy one. About two weeks after we brought you home from the hospital we thought you were having seizures. It was a Friday night and you were having some weird body movements, with no explanation. We took you to the ER, and after talking to a few neurologists, they told us that babies do weird things. Really? Well okay then! Sunday the spells were worse so we took you back... and we were right. In an effort to dialogue what you were doing, we grabbed the video camera so we could accurately describe it this time. The physicians admitted you immediately, and the video we took may be used in future research and teachings on severe infantile HPP with seizures.
This was our first hospital stay at Children's, and it was a scary one. You had many EEGS, a stay in the PICU, a slew of tests, and were unresponsive for a 24 hour period because of the seizures you had been having. During this visit Dr. Lutz came by several times and told us he may have a diagnosis for your "disease." He explained hypophosphatasia, the symptoms, the clinical presentations, and that he remembered hearing about a treatment at a rare diseases conference of some sort he had been to. We read through the information, and although thorough, the prognosis was not great. Infants who have HPP with seizures that do not undergo treatment have a 50% mortality rate by 18 months... and 50% was not going to fly in our book. Within that week we found the treatment, began the initial process for getting started, and had you on vitamin B6 for your seizures. This was our first admission where I realized how amazing your pediatrician, neurologist, and geneticist were. When the three of them were gathered in your room at 2am on a week night, I knew God had provided us with an amazingly brilliant and caring group of individuals. I also realized how amazing your dad is. When we found out it could be a few weeks before we could get you started with treatment in Nebraska, he was looking for flights and lodging in Massachusetts, where the drug company is based. After a few phone calls they assured us it would be better to stay home, although you are so blessed to have a dad willing to relocate for 6 months to get you the care you need (:
During the months of October and November we closely monitored your blood and urine levels with bi-weekly visits to the pediatrician. You quickly became a celebrity because... well you are so dang cute! (: The first week of December we started the clinical trial (treatment). Because there is no treatment already approved by the FDA and this treatment had only produced positive results with no negative side effects we decided it was the best way to go. Monday, Wednesday, and Friday for these 3 months we would go to the Med Center to their LIED treatment center where you would receive your injection, and we would stay for 2 hours of monitoring. It ended up taking at least 9-10 hours a week when all was said and done with check-in, treatment, and check-out. I honestly didn't mind. It was nice to have you in a safe place with wonderful nursing care, consistent treatment, intense monitoring, cookies and fountain soda (: Your dad would take Lyla home after the first hour and we would have a few hours to spend together. It's funny to look back and think about all of the marvelous things I thought I could accomplish in those hours, but all we ever accomplished was snuggling and making friends in the treatment center. Every time we checked in there was a waiting room full of patients, mostly there for cancer or transplant treatments. It was very healthy for me to take a break from the fast paced outside world and get to know people who were really in need of some extra compassion and sometimes just a listening ear.
In January we were driving home from treatment one afternoon, you were upset in the car and suddenly there was an eerie silence. I knew something was wrong so I pulled into the nearest parking lot to find you not breathing in the back seat. I somehow called 911 and got you inside a furniture store. I started CPR at the advice of the emergency hotline. You (and I) were so scared. I'll never forget the look in your eyes when I could tell you couldn't get your next breath. We had lights and sirens on the way to the hospital in the ambulance, and you were slowly breathing by the time we got there. After another week stay, you failed the car-seat test... the seat put too much pressure on your lungs, and with upper airway congestion you just couldn't breathe. In that same visit you "coded" during an upper GI test (more CPR), while checking your digestion tract. The tube they put down into you belly somehow hit a nerve that responded in the plummeting of your vital signs.
My dearest Eve, I will never take a day with you for granted. When your dad and I got married we had a pretty easy going life, and I thought that being comfortable was pretty important. I knew God would take care of me through the good times and bad, I just didn't know how much of either he was going to throw our way. It is His will for you to be here with us sweet girl, and you have made me a more compassionate, loving, thoughtful human being. Your life has changed my perspective.
I love love love you,