My name is Erin Ackerly and my husband is Joe. On August 20Th 1998 we were blessed with a beautiful baby boy. After already having our daughter we were excited and ready to welcome another addition to our family. I have always wanted a boy and a girl so you can only imagine how happy we were when our sonogram clearly revealed we would be having a boy. Joe and I were ecstatic and could not wait to meet our son. The pregnancy was normal it was the delivery that was not quite as it should have been. I was in labor for about 8 hours and when my son was finally delivered he was extremely blue and very low tone. I was unsuccessful at stimulating him to breathe so they took him from me and began O2. I spent almost 4 hours pushing and he was born with severe facial bruising from being in the birth canal for so long. When we brought him home everything seemed fine. He did all the things that a normal newborn did.
We noticed some fisting but I remembered my daughter doing this so we thought nothing of it. At the end of the first month we noticed that his crying was more often and he seemed to be more and more irritable. The second month was one of almost constant crying throughout the day and night. He seemed to startle at the softest noise and the only rest that Auggie got was when he was to exhausted to cry anymore. His naps were short,15-20 minutes only to wake and cry for another 2 hours. He cried so much that his eyes were almost always red and swollen. So swollen he hardly opened them. He smiled for his Daddy only twice and then it just stopped. Auggie never attempted to hold his head up and his tone was still that of a newborn. We were constantly taking our son to the Doctors telling them there is something wrong . The only answer I got was, "He has colic". I knew in my heart that they were wrong. When he was just about three months old I decided to see another Pediatrician and was told the same thing, my son has severe colic and this was the reason he has not yet met any milestones. She put him on a protein hydrolysate formula that was pre-digested to help with the colic. I returned the next week and met with the nurse practitioner. I was determined to leave that office with some answers. I explained the past two months in detail, after examining my son she called his Doctor in and we discussed what he was and was not doing. After an hour our Doctor told us that our son almost definitely has Cerebral Palsy and she was going to admit him for testing to rule out any other diseases that are similar to CP. I was devastated. I looked over at my mom and said to her, "It could be worse, They could tell us he is dying." and I was ready to fight this C.P. head on and help my son be all he could be. Little did I know that this was a battle that could not be won. We spent over a week in the hospital doing various tests. They did blood work, a spinal tap, an EEG, MRI, and more blood tests. As the results came in one by one the pieces to this puzzle were finally coming together. We learned that Auggie's gag reflex was diminishing, he could no longer suck from a bottle with out choking on the milk so he had a NG tube put in to assist with feedings. His reflexes in his arms and legs were very weak and we were beginning to suspect that his vision was impaired or maybe even absent. We had an Ophthalmologist examine Auggie, he told us that structurally the eyes were normal but the brain was not processing the information. Every day proved to be more bad news and we never knew what to expect but nothing could ever prepare us for the news we got on Thanksgiving morning. We learned that our son was dying but we did not know what from. We knew he had some sort of Leukodystrophy but a name was not yet known. Three days later our neurologist told us that Auggie had Krabbe's disease and life expectancy was 6 months to a year. I thought CP was bad but you could not believe how bad I wanted that to be all he had. When we finally got a diagnosis we were able to get on the Internet and learn all we could about this disease. We learned about the cord blood transplants that were being done at Duke University but after consulting with Dr. Kurtzberg we learned that this would not be an option for Auggie because the disease had progressed to far and the damage to his brain was to bad. Thanks to the Krabbe's web site and all the families who share their stories we were able to get information that was not available to us through our Doctors. This was something that they had never seen before and knew nothing about. Fortunately we have a wonderful group of Doctors that quickly learned all that they could. They have been extremely supportive and are always willing to go to any length to help us keep Auggie as comfortable as possible. I believe that it is extremely important to have doctors that listen to you and trust your judgment and instincts as a parent. When it comes to helping make our children as comfortable as possible we surely do not need Doctors with large egos standing in our way telling us that we don't know what we're talking about. I've done that already and it got me no where. When one did not listen I moved on to the next and found our son the best.
In February Auggie had a Fundoplycation surgery and a G-tube put directly into his stomach. The Fundo. or stomach wrap is a procedure that wraps the stomach around the esophagus and ties it off to prevent reflux. Because he lacks a gag reflex he is at high risk of developing phenomenia through aspiration and after discussing the procedure with Auggie's Pediatrician, Gastroenterologist, surgeon and other families we decided that this surgery would be the best for our son. They were able to do the procedure laproscopicly rather than cutting a large incision down his tummy and in turn was much more comfortable for him. The G tube is much better than the feeding tube that was in his nose because it is less irritating and more comfortable for him. In April Auggie's surgeon will put in what is called a button or Mickey that is small and rests just next to the skin. When he needs to be fed we simply attach a tube to the button and remove it when feeding is done. Much easier than having to work with and maneuver around 10" of tubing. Our main goal right now is to keep our son as comfortable as possible through medication, physical and massage therapy. His physical therapist comes once a week and the rest of the week we work with him on our own. Daddy and I do all of his massage and I believe this is what is keeping him from becoming stiff.
His medications include Klonopin (Brand Name Only) to control seizures, Valium (Diazepam), Propulsid and Prilosec. Because the Klonopin increases secretions, the Prilosec is used to help keep them under control. We have him on a continuous feeding pump throughout the night and we give him bolus feeds 4 times throughout the day.
Some of the things we have found that Auggie really enjoys are walks, music from his music box and vibration. When August was an infant we noticed we could calm him down by jostling him. We would put him in his carrier and let it hit our legs as we walked, I thought he was going to get shaken baby syndrome but it was one of the only things that kept him calm. We found a stuffed animal through a friend, it looked like a beanie baby but it vibrated. We had that on his tummy every where we went day and night, We were going through batteries sometimes once a day. He also had a vibrating bouncy chair that he loved. We were able to find a bigger one for him that is a baby hammock suspended by rubber bands and a vibrator in one and this has proven to make him very happy. He also loves the sunlight, lucky for him we live in California, he gets all he wants here. Auggie is not able to giggle or smile and he may not be able to reach out to us but he sends us his love in another way that can only be felt. It is not happiness we see with our eyes but with our hearts when we hold him and he, in his own way hugs us back. He hears his Daddy's voice and his eyes light up the room and when his sister kisses him on the head it is obvious to me he is smiling inside. Every day he tells me he loves me with his eyes and the love he has shown me is all I will ever need to feel truly complete. He is our precious Auggie Bear and though physically he may not always be here with us, his spirit and his will to fight this ugly disease will always keep us strong.
I will not spend each day in despair because I know I will lose him, I will rejoice and celebrate in each new day that the Lord blesses us with his loving presence .
Sweet little Auggie earned his angel wings on March 28, 1998, at the age of 7 months old.
E-mail Joe and Erin