Jamie St. Clair

May 24, 2001

Jameson Keith St. Clair was born on December 19, 1989. I had worked the day before as an Oncology nurse at the hospital. I had worked up to the delivery of my other 3 children so I had also planned to do the same with Jamie. On Sunday the 17th as I got up to go to work my water broke. Jamie was not due until January 10, 1990.

Jim, my husband, says "Now what are we going to do?", "Well, I replied, I guess we are going to have a baby!".  I called off of work and spent the day puttering around. I did some laundry and we took a leisurely walk downtown. I had contractions on and off but nothing consistent. I drank water all day to keep hydrated. Nurses are the worst patients, or so I am told! I did not bother to call the doctor and kept my very early appointment for the next day. Well, I was in BIG trouble. After he deemed that I really was OK, and scolded me anyways, I was sent to the hospital to be induced. This was a new experience for me since the others came out without help! I was on an IV drip of Pitocin to help the contractions. Lets just say, Jamie did not want out! But, we were patient. He was finally born on Tuesday the 19th at 5:30 a.m. He was a giant among premies weighing in at 6 pounds 7 ounces! They declared him to be 4 weeks early. He had some problems feeding due to this and some blood sugar problems and was slightly jaundiced. We were able to bring him home on Christmas Eve night!

He was able to nurse every other feeding due to tiring, but seemed to be doing ok. I can look back now at pictures and see signs that should have been picked up like his clenched hands, holding his thumb into the palm of his hand. He was growing fine and seemed to be developing on time. At about 4 months he began to have feeding problems with reflux and coughing while nursing. We went and saw a doctor who did a bronchoscopy on him. He said it was due to the cartilage in his trachea being soft because he was premature. Another sign.

We were followed by the neonatologist at the preemie clinic. He continued to grow and develop normally considering his prematurity. By 9 months he weight 18 pounds! I remember saying to the doctors that something was just not quite right. I was told that I was basically a worrywart. Every child develops differently. He is just fine. I can remember his 1st year check up quite clearly. Looking back again, I remember that he was developing a bit of a scissors walk and doing some tiptoe walking. Another sign. But again they deemed him just fine.

It was shortly after his birthday that our world began to crumble apart. He began to drag his left foot in the walker. Then he began to hold his left arm oddly, like a stroke victim. Being a nurse, I caught on to this right away, but when I called to get into the clinic it was going to be a few weeks wait since he was not sick. By the time we were able to see the doctor, he was unable to sit up without assistance. We were sent immediately (funny how it was important then) to the hospital for a CAT scan. I was so nervous. I was told that they were looking for a brain tumor. Looking back again, this would have seemed like an ok thing. However, the CAT scan was negative. They could not find anything wrong!

We were referred to Children's Memorial Hospital in Chicago to see the Neurology Department there. I was told it would be a 6-week wait!! I stressed the urgency to them. This boy had been walking around furniture a few weeks ago and now could not even sit up! They were not about to bend any rules. I called our clinic back to tell them this. The doctor on call was the best. He said to us, "Now don't tell anyone, but if you just show up in their ER tomorrow while I am on call, I'll give the hospital permission to treat so it will be covered by your HMO. That's the only way to get seen sooner.

And so we did. I wondered why, when I told them about his problems on the phone I had to wait 6 weeks. After we were in the ER, you can't even imagine the attention we got. Right down to the Chief of Neurology, Dr. Swisher, coming to see us! He said we would need to be admitted right away and we were. They did the usual battery of tests, spinal tap, blood draws etc.. He looked at our CAT scan films and decided to do a MRI. It wasn't done until 10 pm. The technologist assured us that they couldn't see anything glaring out at us.

In the morning, however it was a different story. A mass of doctors and students began a ceaseless questioning. Were we related? What? Yes, they wanted to know if Jim and I were cousins or something like that. Needless to say we were speechless. No! We told them over and over. Then the neurologist explained what they were looking for. He knew it was some type of Leukodystrophy because of the sclerosing of the white matter on his MRI. He rattled off a few names of different types, which I wrote down. I wanted to know all about this disease. He then told us from here it was a simple blood test to find out which one. I do remember that he never informed us of the gravity of our situation. We were discharged to home and began the weeklong wait. In the meantime I researched the library at the hospital I work at. I was not prepared for what I read. Jim refused to read the papers I had photocopied after I gave him a brief review.

Dr. Swisher called us at home on Friday January 17, 1991 in the evening. A phone call. No come in and sit down so you can have this broken to you gently! What we were told was that he had Globoid Cell Leukodystrophy and we could expect his demise in about 11 months as children with this disease rarely live past age 2 years. That was it. I really dislike this man still today. Later on his office gave us the name of the ULF. We got lots of information and help from them in the beginning, but they seemed to care more about the more well-known or common Leukodystrophies. That summer we were able to meet with Doctors at the annual meeting and ask about treatment and medications. By this time Jamie was extremely irritable and not sleeping at night. We had been using Chloral Hydrate, prescribed by the pediatrician. They knew next to nothing about Krabbe's. But, after the conference we went back to them with our newly found info and Jamie started on Elavil for sleep and Valium for irritability. Things seemed better emotionally, but we had been having feeding problems and he had not gained any weight since 9 months.

At age 18 months we decided to have a G-tube placed. This was a good thing. We could now give medicine without forcing it down him and he started to gain weight. He really hadn't lost more motor control, but he was still at a 3 month old level of development, physically. Mentally he was as sharp as tack. About this time he began to open his mouth and smile for YES and keep a straight mouth for NO. We were determined to make this last year his best. He stayed with us were ever we went. We got our 1st wheelchair.

Now, we were surprised when age 2 and age 3 came and he was still doing fine. We found a new Neurologist and he too was amazed. At about age 3 Jamie began to have jackknife seizures. He was started on Klonopin, and is still on it today. That helped a lot. He would fall asleep and they would come in the middle of his sleep and he would be obviously upset and start to cry. And he continued to grow.

At age 5 Jamie began Kindergarten. He was in a special education room with other handicapped kids. He was a little clingy, but I think that was because we never left his side. We had no idea what a little social butterfly he would become! At the end of the school year it was decided to put him in a regular classroom. He has been there ever since! His classmates help him with writing and computering and reading. There is a list because otherwise they fight over who will help him for the day! He thrives on them! He has a head switch on his wheelchair that can activate many electrical things like tape recorders, computers, staplers and paper shredders. He helps in the office when the other kids are doing things he can't do. He is always picked to be on a team for Kickball at recess!! And he loves it so much. I can't say enough kind things about our school system!

He has developed different seizures, as he has gotten older. He is on many medications for this. He has had pneumonias on and off since age 5. Some were aspiration and some bacterial and sometimes both! At age 8 we had a Nisson Fundiplication procedure done to help with his reflux pneumonias. That has been a big help, but he still can vomit and has reflux. He has severe scoliosis When he was 10 he developed atalectasis (collapsing of part of his lung/s). We started on BiPap, which is a ventilator of sorts that uses a facemask. That was a big help too but did he hate that!!

Last fall he developed a cough. And it lasted and lasted and at Christmas he was still sick. In January he got pneumonia and was really sick. Our usual hospital stays are about 7-10 days. We were in for 32 days, and all but 3 days were in the Peds ICU. We had early on decided that we would not put a tracheostomy tube in place. This admission changed all that. He struggled to breath one night and the look of fear in his face was all that was needed. He is still very alert and very much aware of what is going on and being said.

So on February 14 of this year he had a tracheostomy done. I noticed immediately that the cough was gone. It was easier to suction him. However, his seizures got worse. They started him on 2 more seizure meds. That put the total up to 6!! He was very lethargic (but seizure free!) and that his how we took him home, 9 weeks ago. Prior to that admission he only needed O2 at bedtime. He is now on continuous O2. He rarely needed suctioning unless he was ill. He now needs to be suctioned several times and hour. We have weaned him off 2 of the seizure meds and he is now his old happy self! He started back to school full time 3 weeks ago.

We are back in the hospital as I write this now. We were barely out 8 weeks! We have pneumonia, again! I know that what ever has passed will be nothing like what is in the future for us. We have been luckier than most parents and we know it. But we have also been waiting and waiting for 10 years now! For the end that never seems to come. We have watched him grow up. We even started shaving his mustache this year! He is still alert and interactive. I look at him when he sleeps and he is so beautiful. He loves life like no one I ever knew. We will continue to do the best we can and our goal always is that he is comfortable and happy.

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