A couple of months ago I posted about Jaxson's heart rearing up and having to do some things with Neurosurgery. Since then, I've only been able to post on his Facebook page a few times, so I'm going to section everything out again.
Cardiology: After the 24-hour EKG, Jaxson was diagnosed with a wandering pacemaker and PVC's. A wandering pacemaker happens when the electrical signal that tells the heart to beat fires from the bottom of the atria instead of the top. PVC's are early heartbeats. While these are things to monitor, they are not putting stress on Jaxson's heart. Good news.
Urology: Jaxson's urodynamics test showed that his bladder is smaller than anticipated and it spasms early. The imaging ordered by neurosurgery did not match his bladder issues, so we are going to see them in September. We are going to discuss medication to help his bladder calm down so that he can be potty trained and not have to wear a diaper all the time. One of the side effects of these meds can be retention, meaning he won't void his bladder completely. So I will have to learn to cath him in case that ever happens. Not looking forward to that.
Neurosurgery: Jaxson was finally presented at their conference and the plan has not changed. Because imaging does not match his symptoms, they don't want to do anything yet. I'm totally fine with that. They do, however, want to monitor him closely. He will have a repeat sleep study in October, another spinal MRI and a cervical spine X-Ray done. Right now, the sleep study is scheduled after the NS appointment, but we're on the cancellation list and hopeful to get it done beforehand. Once that information is in, we'll see if anything changes.
Craniofacial: Jaxson had craniofacial clinic at the end of July. Everything looks good, except there is noticeable air leakage through his nose when he speaks. They discovered that he has a gap in the back of his throat, which is not uncommon for kids with a cleft palate or with Kabuki kids. Their solution is to have Jaxson's tonsils and adenoids removed. At least, that's the plan right now. A message was sent to ENT to have Jax come in to be assessed, but it looks like that's the direction for now.
Dermatology: I haven't posted many photos of Jax lately because he's got a nasty rash on his face. It began on May 31 and was finally diagnosed as perioroficial dermatitis by dermatology about 2 weeks ago. They put him on a 1-3 month round of antibiotics to clear it up, which of course is screwing with his Coumadin so I have to pay VERY close attention to his Vitamin K intake. Dr. Huston was nice enough to send me a list of the Vitamin K content for various foods so that I can continue making Jaxson's food. I just got that list on Friday, and I haven't had a chance to make food for him yet, so he's been on Pediasure for a couple of weeks. Drinking it, though, we're really trying not to go backwards here!
Speaking of going backwards, I just happened to think about him having surgery on his throat. I have no idea how he's going to react to eating after that. It's going to be a disaster. Maybe not, he might surprise me. This morning he told me he wanted cereal and applesauce for breakfast! We didn't have applesauce, but I made him multigrain cereal with his Pediasure so he still got what he needed AND got to eat. So it's definitely possible he could surprise me and want food, I guess I'm just not holding my breath.
I think the biggest difficulty with all of this is that Jaxson has to go in 3 days early for ANY surgery. Because his mechanical valve is the mitral valve, which is the smallest valve in the heart, cardiology will want him to come in early to be put on a heparin drip instead of bridging with lovenox shots. Honestly, I'd rather not give him the shots anyway because I hate doing it, but having a mostly healthy 6 year old in the hospital for 3 days sounds exhausting. And then there's the recovery process, which includes switching back to Coumadin from heparin. Last time we did that was right after his valve was placed, and his INR shot up to 9, which means his blood was extremely thin. It kept him in the ICU because it's so dangerous. Jean, who manages Jaxson's INR, thinks it was a dosing issue, but my sister had the same issue when she was pregnant with her daughter. Last time the INR kept Jax in the hospital an extra week, so I'm very nervous about this one. I pray Jean is right and she can control the dosing so that we don't have that issue, but it's a very real concern.
This summer has been crazy with appointments and unknowns. Right now, there's nothing I can do bu make sure Jaxson gets to his appointments so that the doctors can gather all of the information they need to make a plan. Trying not to think about the "what ifs" is hard, so I mostly try not to think about it at all, which I know is not the most healthy way to approach this. I was really hoping I could get a part-time job this fall, and it's possible that I still can once we get a plan in place, but this being in limbo is killer. We were so excited about the prospect of having additional money coming in, so hopeful that we would be able to finally get out of having to alternate which bills to pay and just be able to stay on top of things, that this is devastating.
On a final note, a petition has been started for children with rare diseases and genetic conditions. It was started by the founder of All Things Kabuki, and it is designed to get the attention of the leaders in Washington to take notice. There is only ONE Kabuki clinic in the entire US. It's in Boston. Travel arrangements are hard enough, but there are organizations out there which can help. The problem is that once you get there, no one will cover the medical part. Shouldn't our kids be allowed to visit a specialty clinic, even in another state, to ensure they are getting the proper care designed by the experts in the syndrome? There's no reason for our kids to suffer through Medicaid wanting to "exhaust all local resources" before allowing anything else to be done. It's not only a waste of everyone's time, but Medicaid is actually losing money on these kids because the specialists they are referred to aren't familiar with the syndrome, and therefore no one is comfortable making a decision because they don't want to be wrong. There are Kabuki kids who have suffered for YEARS without getting the help they need because we can't afford to take them where they deserve to go. Please, this doesn't cost you a thing, just a moment of your time. Please sign this petition so all children with rare and genetic conditions can get the care they so desperately need and deserve!
https://petitions.whitehouse.gov/petition/rare-disease-and-medically-complex-children-need-access-quality-specialty-clinics
As always, thank you for your continued prayers and support! I know very well how much more difficult this would be if we didn't have those things.
~"Life is not the way it's supposed to be, it is the way it is. It's how you cope with it that makes the difference." -unknown
