I had a great pregnancy, delivered full term. Hunter was a healthy
baby 7lbs 14oz, 19in long. Everything was going great. He slept and
ate all the time. Well all of a sudden he develops a squeak when he
would breathe. The doctors weren’t worried. Then he starts having a
problem eating, he just didn’t want to eat. He had weekly weight
checks and he kept losing weight. We went in to his doctor one day and
hunter was looking really pale. His Dr told us to take him to the
Children’s Hospital ER and that was the beginning of Hunter’s journey.
June 10, 2009 I will always remember that day. Hunter was 2 ½ months
old. He was admitted to Seattle Children’s Hospital with respiratory
distress. Everyday seemed to get worse. He was put on oxygen. Then he
refused to eat, so they put a feeding tube down his nose to his tummy(
a NG tube). He was really uncomfortable and cranky. The night of June
15th Hunter crashed and was moved to the PICU ( pediatric intensive
care unit). There he was put on CPAP (a high flow of oxygen through
the nose) for maybe a day. Then they intubated him for a procedure and
decided to keep him intubated, because he was comfortable. He stayed
sedated for a long time, otherwise he would try to pull the tube out
of his throat. They tried to extubate him a few times and he always
failed. They decided to give Hunter a tracheotomy in August of 2009.
Hunter was their little mystery, they ran every test they could think
of and everything came back fine. One day a
Neurologist comes in and he says that Hunter has the symptoms of
SMARD. I thought no way, my baby will be fine and he will just grow
out of this. He did the test and we went about our business learning
everything about how to care for Hunter. Before we are about to take
Hunter home I ask someone if the results are in and they said he was
negative, I was so happy. We took Hunter home and everything was
great. We went back for a check up and the Dr told me that Hunter did
have SMARD, the person who told me read the results of the SMA test.
Well I just lost it right there, I just couldn’t believe it, I thought
my little man was going to be just fine.
Well we went home and adjusted to life with Hunter. We were only home
for 2 months when Hunter almost died. I had a nurse who I trusted to
care for him while I slept. One night this nurse was negligent with
Hunter’s care and almost killed him. To sum it up She messed up his
tubing, so he wasn’t breathing and she didn’t do anything about it.
She waited to wake me up and I went in his room and he was lifeless
and I saved him and brought him back to life. He then had a seizure
and was taken to the hospital. He was unresponsive and had seizures on
and off for a few hours. Then he finally came through and was his
happy little self again.
We went a whole year without Hunter having to go to the hospital. Then
flu season came around and he has been in and out since then. Recently
we found out that hunter has a little amount of brain damage and
seizures all from when that nurse almost killed him.
So that is Hunter’s story. I have been putting off from writing it. It
is always emotional thinking back. I always thought when I would have
a baby I would be married and my baby would be healthy, would be able
to walk and say I love you mommy! No matter what though I know my baby
loves me, I know he will walk one day, and he will be healthy one day.
That is what keeps me going strong knowing that my baby is going to be
Jan and I were married twelve years before Christopher was born in July of 1989. He was our little miracle.
From a guys perspective the pregnancy progressed pretty well though Jan was sick a lot and we were quietly concerned that the baby didn’t seem to move as much as other babies. I know Jan has lots more insight into these times than I do. She was watched closely because there were concerns.
When he was born, he was small. I remember 4lb 6oz but I’m probably off a mile. I just remember he was tiny. He seemed normal though his cry was a little bit quieter but he seemed fine. I remember his being able to do a pushup in his bassinet. Over the next bit though we began to think something was wrong. He would occasionally turn a bit dusky but we couldn’t put our finger on it. I remember going to a Lamaze reunion with an apnea monitor on a long cord and marveling how much the other kids were squirming. By now we knew something was wrong and we went from doctor to doctor trying to get someone to believe us. At the three month mark our then pediatrician was out of town on a family emergency when Christopher crashed. It’s as though he could eat or breath but not both. They did a blood gas in emergency and slapped him on a ventilator and he’s been on one since. That was in October of 1989.
He spent the next four months in ICU undergoing multiple tests that pretty much showed he was normal. I don’t think there was any such thing as SMARD back then. I don’t think kids that had it generally lived long enough to get a diagnosis and, of course, they hadn’t sequenced the human genome yet. From our and their perspective it was an orphan disease. I remember several times throughout the years MDA would have nothing to do with us because we couldn’t give them a diagnosis (actually, they never did give us any help at all – It’s hard to explain how frustrating that was, having a child with such major muscle/nerve problems that he couldn’t move or even breath and because we didn’t have a name for it we were literally on our own. You can see I have issues with them. I hope others have fared better).
At seven months of age Christopher came home on a hospital ventilator because the portable vents in those days were not adequate for a kid that small. Ventilators have changed a lot since those days. What he has now is half the size of a brief case but superior in nearly every way to that first vent.
We were on our own. No one had ever done what we were doing (that we or the children’s hospital knew of). If the internet existed, we, and just about everyone else, had never heard of it. We converted a smallish bedroom into a hospital room with extra wiring and a sink installed against the wall.
And so began our new lives. I am a dentist and Jan had, a number of years earlier, changed majors two and a half years into a four year nursing program because she couldn’t stand nursing (one of those little ironies). We knew the basics about cross contamination. I knew a good deal about anatomy, physiology, metabolics, endocrinology and pharmacology. [In the area of metabolics and nutrition Jan has far surpassed me. She has been doing research for years. This brings up a point. Jan is very willing to share anything she has learned but she is every bit as interested in learning what you all have learned as well. Nobody knows better than us how a bit of info can make a difference.]
As I look back I think what helped a lot, in addition to our faith, is that Christopher was small. We could easily move him around. I could hold him in my arms and put him in my lap. He could still move a bit for a couple of years and I can, in my mind, still hear him say “pa pa”. He has been unable to speak or move for years and now and he weighs 160lbs. [Another aside: Jan and I differ over the issue of “he can’t move from the chin down”. If we suspend his arms he can get them to swing back and forth enough to activate switches but he is unable to do much else. He can’t turn his head or breathe independently at all. What I find fascinating is if it is innervated spinally it doesn’t work. If it’s innervated cranially he’s OK. He can smile, smirk, look around laugh etc but it’s all facial and head muscles. He can turn on and off the most beautiful smile like a light switch. His slight arm movement seems to be the result of some accessory innervation of the shoulder girdle from his cranial nerves. I guess, on this point I’m glass half empty and she’s half full.]
We have had Christopher at home now for over twenty years. He has only been back to the hospital twice since he came home. The first time it was for a neurogenic bladder. We discovered he was not voiding well enough and his blood pressure went nuts until we could figure it out. He now gets cathed several times a day. The second time was for his lungs. That was a couple of years ago and we almost lost him. He was in ICU for a month but he pulled through.