Life goes on….Jonathan’s story

So we had a plan.

Jonathan would be delivered via c-section. Within an hour of his birth, he would be transported to the Regional Center Newborn Intensive Care (RCNIC) at CCHMC. They would start him on his ‘cocktail’ of  IV meds. They had the dialysis team on alert. They did not know if his ammonia would be high. They truly did not know what to expect – they had never had a baby diagnosed prenatally before.

December 22, 2004, Jonathan Earl was born.

They whisked him away and I didn’t get to see him for 2 days. He was born during the worse ice / snow storm the Cincinnati area had seen in years. We were lucky to have gotten to the hospital early and to have had the whole thing planned.

I was also  lucky to have a doctor willing to discharge me 2 days post c-section.  (Of course, if you’ve had a c-section – you might disagree with the ‘lucky’ part!) I was discharged on Christmas eve, we got to spend an hour or so with him, then we had to get back to my mother in law’s house. She had electricity, we didn’t. The whole area was under emergency alerts. I think our electricity was out for 5 days.

Jonathan did very well. His initial ammonia level was 53. He responded well to his IV meds and did even better with his low protein formula and oral meds. Jonathan spent 14 days in the RCNIC. We were sent home with a very strict feeding schedule. 3 oz every 3 hours. His sodium phenylacetate and arginine were mixed into his required formula on a daily basis.  Jonathan never needed an NG tube or a g-tube for feeding.

When he reached 5 kilos, we scheduled his transplant evaluation.

The transplant evaluation process was grueling. It was 2 full days of tests. Blood work, MRI and meetings. We met with the dietician (needless to say post transplant he would be able to eat anything he wanted).  We  had a clinic visit with his GI doctor. We met with his transplant coordinator. We met with the surgeons, again. I had already met with the surgeons while I was pregnant. We met the chaplain, the child life specialist, the social worker and the financial counselor.

We learned about how the actual surgery is performed. We learned about medicine required post transplant. Precautions and expectations post transplant. We learned about the services offered through the hospital. We toured the unit he would be spending his time. And they learned about us. The social worker and transplant coordinator really spent a great deal of time getting to know us. Our family history, our expectations of them. Our fears.

And they all met with Jonathan. He was only 3 months old, so it was easy for him to capture their hearts. They saw how aware he was, how he interacted, how he smiled at everyone. Their main goal was to keep him developmentally on target, as much as they could. They were a little skeptical about performing a transplant on a seemingly healthy child. He was responding well to his low protein regime and tolerating his feeds very well.  They agreed to present him to the group at a future grand rounds meeting. The surgeon was on the fence, he saw the need to preserve brain function – but at what cost? How do you injure the body to save the brain? My question was how can you not? How could they decide to wait until after he’s had several rounds of high ammonia. How could you wait until after his brain is destroyed and the body is no longer worth saving? They knew from our experience with Nathaniel that the lack of enzyme was severe. Some kids can tolerate low protein diets better than others. No one believed he would tolerate his diet and med regime for long, but everyone was leery about being the one to commit him to this life altering surgery.

He was presented and approved for a spot on the transplant waiting list. He was given the average number of points and we were told not to expect anything anytime soon. Most kids waited 6 months or more for an organ to become available. So with our pager a constant companion we moved forward.

Life goes on.

Until it stops.

The Friday before Mother’s day, Jonathan was lethargic. He refused his bottle, he was limp and lifeless. He was 5 months old and I knew he was having his first hyperammonemic episode.

I called the hospital, talked to the genetic doctor and told them I was on my way. 2 hours later we arrived in the ED.

His ammonia level was 458. He was limp and lifeless in my arms, they couldn’t get the IV started, he never made a peep when they stuck him 4 different times to get that IV started. I was determined to remain calm and level-headed. They finally got the IV started, they got the meds going, they got us settled in a room and we waited.

Waited and itched.

Well, I itched. I had a severe case of poison oak. I had a red, itchy rash all over the place. I’m a city girl, no one warned me about poisonous vines and the fact that you shouldn’t take a shower – water spread the rash ALL OVER!!   There wasn’t anything I could do either. I was stuck sitting in a hospital room with my son sick and me itchy. The rash was so bad  – the attending doctor even commented on it  – I think he was more concerned with me being contagious than uncomfortable!!!

So that’s how we spent out first Mother’s Day together, Jonathan and me, him recovering from the high ammonia and me trying not to scratch myself raw.

We were discharged Monday. I had asked the doctors if there was a reason why he suddenly had this episode – he hadn’t been sick, nothing had changed. They didn’t know. They thought it was probable that for whatever reason his body had attempted to break down its own protein reserves, overloading his liver and making him sick.

Life goes on.

Come September, 3 things happened simultaneously, he got his 1st flu shot, 3 days  later he had another episode and hospital stay and UNOS changed their listing rules for kids with urea cycle disorders. Up until then urea cycle kids were put on the list and they waited and got sicker, like everyone else on the list. Now when a urea cycle kids gets listed – they have 30 days, if they don’t receive an offer, the child can be bumped to the front of the line – to a Status 1B. Someone realized these kids couldn’t afford to wait. By this time, Jonathan had been on the list for 5 1/2 months.

2 weeks after being bumped to Status 1B – we got the call. A donor had been found. Drop what you’re doing and get down to the hospital. That was Sunday, October 9, 2005.

He had his surgery on Monday, October 10, 2005. Columbus Day. That was the first day of his new life.

Life goes on.

We spent 3 weeks at CCHMC.  Jacob spent 3 weeks at grandma’s house. I think Jacob had more fun.

Jonathan came home from the hospital on Halloween. We settled into our changed life. Medicine schedules, central line care, home care nurse visits, lab draws, clinic visits. It was a lot of work. It was worth every minute. The fear of brain damage was no longer in the equation, I was up for anything that came my way.

We made it 8 weeks without any problems. Then Jonathan spiked a fever, the day before his birthday. This was our first of many “48 hour” ruleouts. Since he had a central line anytime he had a fever of 100.4 or more, we were required to bring him in for at least 48 hours. They would send blood cultures to the lab, put him on broad spectrum antibiotic coverage and wait.  If there’s no growth and no new fevers they let us go home. If there’s a bug, they reduce his antibiotic coverage specific to that bug. Support him with fluids and anything else indicated by his blood work.

So he spent his 1st birthday in the hospital, but he made it home for Christmas.

Life goes on.

2006 brought Jonathan a lot of hospitalizations. He had several infections, he had several liver biopsies to ruleout rejection. He had one episode of rejection, 168 days after his transplant. He had a 105 degree temperature that precipitated a seizure – a 90 minute seizure. That landed him in ICU for a week and anti-seizure meds for a year. Because he had had the 2 episodes of hyperammonemia prior to transplant, they treated the seizure as being related to the citrullinemia. Normal fever-related seizures do not last 90 minutes. During this time they did an MRI of his brain and found he had a delay in the myelination. Myelin is like a sealant for the nerves in the brain. This too, they attributed to the citrullinemia and his 2 episodes of high ammonia.

By the summer of 2006, Jonathan was 18 months old and it was evident that he had delays. He barely spoke, he couldn’t tell you what he wanted, most of the time he couldn’t even point to what he wanted. We had him evaluated by speech, occupational therapy and physical therapy. He was a happy baby, he was able to play on his own, we would spend endless hours sorting his little metal cars or putting his puzzles together. He was very complacent, though. He let Jacob take his toys away and bully him and he never fussed or put up a fight. By fall, Jonathan had started all 3 therapies. He continued with OT and PT until he was 4. He still receives speech therapy weekly through the elementary school.

At this point,  Jonathan’s hospital stays are becoming fewer and farther in between. Life has started to feel normal. He was weaned off the anti-seizure medication by Christmas of 2006. He started to make some real progess with his speech therapy after that.

Life goes on.

Until January of 2007.

I. am.  pregnant. Again.

I told you the Seton center knew me very well.

 

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