I wanted to write a post to first of all, update everyone. When we see family and friends who have followed his story from the start, they always are so thoughtful to ask, "Hows Jake?" So many people ask, because not much has happened, and for Jake that is a wonderful thing. I also wanted to post an update because so many other KT kids and families are about to take the leap with a new medication they have found to help our KT kids, Sirolumis (aka Rapamune). We have had such a positive experience with it and so many concerned parents post on the KT pages asking for any advice. Here is our experience.
In the fall of 2013, Jake spent his longest time in the hospital. We had just attended the Vascular Birthmarks Foundation Conference in Irvine and had a wealth of information. We left with so much promise and hope. Just a few days later, we landed in the hospital, yet again, with another infection. This time, it was serious and we couldn't get the pain under control. I turned into a mama on a mission. I gave his doctors down at Rady Children's San Diego a list of names with the doctors we had just met with. I wanted a change. I couldn't continue to bring my 3 year old in to the hospital every 2-3 months to be hooked up to IVs, pumped with medication, poked tons of times, scared and miserable. I wanted to take him to San Francisco for treatment, I wanted to take him to New York. I was not leaving with him until we had a plan. I felt like this was what our life was going to be if I didn't speak up. We stayed at Rady for 2 weeks and after many doctor conferences, phone calls, visits and too many IV pokes to count, went home with a plan. They started Jake on Sirolimus. This medication is typically used for patients with kidney transplants. Somehow, it has worked for kids with KT as well. The hope was that it was slow the growth of the lymphatic portion of the malformation, which is what made his bottom and right leg so swollen and large.
After starting on it, we got to know an entirely new Jake. Jake had been the kid that threw major tantrums, scream and cry constantly, wake up and cry in the middle of the night, it had been rough from day one. We never knew when we would head into the hospital. Each day was unknown. He couldn't tell us when he was in pain. Jake also peed blood every time he used the bathroom, which is why he was 3 1/2 and in diapers still. He sometimes screamed and shook when he peed because it hurt so bad. I'll never forget him standing in his pull-up shaking and crying as he tried to urinate. It was unbelievably heartbreaking. This was all due to the malformation in his private area. I had to learn his leg and the changes it made, warm red sports and fever mean infection, hard nickel size knots were clots, bruising was usually pooled blood which then got an infection in it. Once he began the Sirolimus, the tantrums lessoned, the blood in the urine stopped and he was funny and happy. I know his pain lessened once the medication kicked in. I think he grew and the lymphatic portion didn't grow as much, which meant less pressure on the inside of his abdomen and leg. We did weekly blood draws until his levels were where the Drs wanted them, typically between a 6 and an 11. We now do monthly blood draws. We also did MRIs every 3 months to check the growth of this leg. The size and swelling of his leg lessoned dramatically. He's grown taller without the leg growing larger. Granted, his leg is still greatly larger than the other.
Since November 2013, we have been hospital free. Going from every 2-3 months in the hospital since birth to not having to carry the worry of another hospital trip has been unbelievable, although that fear is never far from my mind. Just last night Jake spiked a fever and Travis and I went in to panic mode. Trying to figure out how we'd get Bryce to and from school, what we would do with Trenton, how would we manage as a family all being apart? Scared us. Thankfully he woke up just fine. We do know that a hospital trip is imminent. As this is a chronic illness that will follow Jake for his entire life.
There have been a few downsides from Sirolimus, but no where near enough that it's not worth it. Sirolimus does lower a childs immune system dramatically. In the beginning, I was very careful about where we went and what we did. You cannot live that way tho. By last summer, we were spending our days at the trampoline park, the zoo, the beach and camping. We were out and busy and Jake had no complaints. I think by November, 1 year since starting the medication, things were so good that I became lax on hand sanitizer and keeping him germ free while we were out and about, although its not all avoidable. He's had the chicken pox, tons of colds, stomach flu and pink eye. We have Jake on Bacterium on the weekends to keep his immune system up, but it can't all be avoided.
Overall Sirolimus has been a blessing to us. Dr. Geddis at Rady Children's Hospital said out of the 11 patients she has on this medication, Jake has had the best reaction. She said she wished all of her kids had his reaction to it. We are so grateful for that. Come October this year, we will start to wean him off. The longterm side effects of the medication are unknown. To say I am nervous about taking him off slowly is an understatement. Jake starts kindergarten in the fall and although he will have a health plan in place, I'd hate for him to be in and out of school due to pain, bleeding or hospital trips. The future is unknown but we are grateful for now.
Please message me if you are a parents who's child will be starting the medication soon and if you have any questions. Ill answer them to be best of my ability.
((Jake takes 1mL 2x a day with a little cup of OJ)
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