Holy cow, it's been over a year since I posted, that's crazy! It has been a pretty busy year, which I will highlight later. For now, I want to give a full update on Jaxson. This will be a status on all of the specialties he sees. Some of the information will be the same as things are stable, while some will be new, like starting IVIG infusions for his immune system. So yes, this is going to be long, but you'll get a full picture of how Jaxson is doing right now.
Neurology/Neurosurgery: I group these together on purpose, they tend to bounce off of one another. Jaxson's spine and brain are stable. He has central sleep apnea, but we are not treating it at this time because he brings himself back. He still wakes up during the night, but he's finally staying in his bed for the most part. We are watching everything and will do scans and sleep studies as needed. His obstructive sleep apnea resolved with the removal of his tonsils and adenoids. We increased his iron supplement to 2.5mL twice a day in March, but by June his ferritin levels had decreased even with the increase in medication. So we doubled it to 5mL twice a day. He's due for a follow up, but I haven't scheduled it yet. I'm sure they will want to check his ferritin and other iron labs to see if there's any changes.
Craniofacial: Jaxson was initially supposed to have a VPD repair in April, but due to his heart valve and that they could not scope him that day because his INR was high and he'd already had a bloody nose, it was postponed until August. We went back in July so they could scope his nose to look at the gap in the back of his throat, and we decided to hold off on the procedure for now. The gap is actually smaller than anticipated (it changed after his tonsils and adenoids were removed last November). The air escaping through his nose does affect certain sounds with his speech, but we determined that the risks of the surgery outweigh the benefits. He's due for his annual craniofacial clinic visit, so I will be scheduling that soon.
Cardiology: Jaxson had a heart echo in June, which was stable from his December echo. The gradient on his valve is steady at 11, which is in the severe range, but his symptoms are mild. We will hold off on replacing the valve as long as humanly possible to allow him to grow so that a bigger valve can be placed. The hope is that he may only have to do this one more time, but I anticipate two by the time all is said and done. He still turns colors, he still sweats like crazy and he tires easily, but he's happy and the issues are relatively mild.
Pulmonary: All looks good from a lung stand point. We are continuing with his daily Asmanex to help control his reactive airway disease, especially after he contracted two respiratory viruses this spring. We go back in January for a follow up.
Physical Medicine: Nothing new to report here, I received a prescription for a handicapped placard for our vehicle since we do use Jaxson's wheelchair on occasion, but I haven't picked it up yet. He went to the zoo a few times this summer and the chair was an amazing help. It was also used for the Make-A-Wish walk in June and when Jayson took him to see a WWE NXT show. He tires very quickly, so having the chair has been huge in our ability to take him more places!
Nephrology/Urology/Colorectal: These all bounce off of each other. Jaxson has ultrasounds every 6 months on his kidneys and bladder. His kidney stones appear to be stable and the labs related to his kidneys are relatively stable as well. We did increase his potassium citrate this year to try and break up his kidney stones. It appears he may have a new one, but his kidney function is still ok. Urology referred us to the Colorectal clinic due to Jaxson's urinary incontinence. He had a contrast enema done in September which showed that his anatomy looks good, but they were uncertain if he also has bowel incontinence. They said that most patients with bladder incontinence also have bowel incontinence, so it's likely he has it. We started a new med to help control when he has his bowel movements in the hopes that we can get him to stool in the potty vs. his diaper. So far we haven't had any luck getting him trained, so at this point we're at a loss. Rather than use the bathroom, he will hold his stool if he thinks he's going to get in trouble for pooping in his diaper. We don't punish him, though, I just told him I really wanted to see him use the toilet. He keeps telling us he wants out of diapers, but he refuses to use the toilet. We have options for his bladder if we can get him pooping in the potty, but I really have no desire to do the Malone, which is basically a 45-60 minute flush. No way I want to try and keep him on the toilet for that long! We're due to go back this month, so we'll see if they have any ideas. Once we get his bowel squared away, we will likely do a procedure that allows us to cath him through a port instead of his pee pee, which I tried at home once and it was traumatic. Never again. This is likely to be a long process, but I really hope we can get him out of diapers sooner than later! GI is supposed to be part of the Colorectal clinic, so I plan on asking them about malabsorption as well. Increasing medications and having levels drop is really not normal, so I hope we can take a look at this since it's common with Kabuki.
Feeding: We are taking a different approach to feeding with Jaxson. He fights us on everything, and he deals with so many other things that we don't want to fight with him about it. So he gets tube feeds, which he prefers, and we have him eat as much regular food as we can. He still loves french fries, chips, cookies, applesauce and has started eating pancakes and chicken nuggets. I hope that some day we won't need that tube, but for now, it's a battle we choose not to fight.
Neuropsychology: Jax had an evaluation in September and I will get the results next week. One thing I can tell you is that Dr. Rabkin mentioned when we did an eval two years ago that we might have to add some type of intellectual disability to Jaxson's diagnosis list, but it's not something they thought was necessary at the time. He said that based on what he had received from Jaxson's school and just talking with him for a little bit, he doesn't think we will be adding that diagnosis! Cognitively, Jaxson is pretty sharp. He's a bit behind in things like writing and reading, but no intelligence deficit! I do think he will be getting an ADHD diagnosis and probably a med added for that, but I'll know for sure next week.
PT/OT/Speech: All stable here! Meeting his goals and working hard. We continue to do outpatient speech and we added outpatient OT, but we're keeping PT just at school as he's pretty mobile and probably doesn't need the extra help.
ENT/Vision/Hearing: Everything looks good here as well! He had about 6 ear infections this year, but he's been free of them for a month or so now. Vision and hearing are both stable.
Immunology: This is the one with the big update. Jaxson had a bunch of labs done in August and I finally spoke with the doctor a few days ago. Jax saw a good increase in several areas after he got the Pneumovax in 2017. His 2018 labs all looked decent, like he might be growing out of some things, which can happen. But Jaxson being Jaxson, he dipped again. Dr. Lindsley said that he's never seen a kid's IgG jump up the way his did (by 300 points) and then plummet back down to below normal. He said that Jax isn't even doing typical Kabuki stuff for the most part, some of it is, like his B Cell response has dipped which can happen with Kabuki, but some of the other things aren't typical. He's lost his response to tetanus and candida and his flu antibodies were lower than they have been as well. So Jax got his flu shot today at dermatology and he will start the dreaded IVIG infusions next week. We will drive to Cincinnati for 2-3 infusions and then transition to subcutaneous infusions at home. These infusions take about 4 hours, and the first few will come with labs to see how he responds. He will get the first few every 3 weeks, but I honestly cannot remember the home schedule once we get there. I do not look forward to spending all day at Cincinnati where I will have to keep Jaxson AND Skylar entertained, but I will do whatever it takes to keep Jaxson healthy. Dr. Lindsley said that it was up to us if we started these now or waited to see how Jax does this winter with illnesses, and we opted to do it now largely because of his heart. There's already stress on his heart because we are nearing the time to replace his valve, and as I stated above, we will wait until it's absolutely necessary to do his next heart surgery. So in the meantime, we want to keep Jaxson as protected as possible. When we first discovered his heart problem, we ended up having to wait MONTHS for surgery because he couldn't get healthy, and I do not want a repeat of that.
Dermatology: I hoped we wouldn't have to return to Dermatology, which we saw when Jaxson had a rash last year. He was ultimately diagnosed with perioroficial dermatitis and was put on a 3 month round of erythromycin to get rid of it. Well, it looks like it's back. This is the same time last year he got it. The doctor gave us a gel to put on his face should the rash return, but it wasn't responding. In fact, it's gotten worse. So we took him back yesterday and they said he didn't need an antibiotic yet. We got a new gel to try on his face and hope to see improvement in 2-3 weeks, although it could take 2-3 months to clear up completely. We also have to keep an eye on his skin when he's taking a bath or out in the heat because he broke out in hives during his bath yesterday. His skin is so incredibly sensitive, and he's so fair that it doesn't take much to affect it. If he continues with hives, we will probably have to put him on a daily antihistamine but for now we just watch. They were also nice enough to send an exfoliating moisturizer to the pharmacy for the KP on his arms and thighs. Unless something changes, we follow up in one year.
That's all for Jaxson. He's always got a ton going on and I have no idea how I keep up with it.
As for the rest of us, Jayson started a new job this week and I found a job working from home as well, so we're hopeful that finances won't be so stressful going forward. Jeffrey is doing amazing in school, I'm beyond proud of him for being able to function without any ADHD meds. He came home with all A's and 2 B's on his mid-term progress report! I know it's mostly review right now, but Jayson and I are so proud of him and gave him some nice incentives to keep it that way. I hope to get Skylar to the walk-in clinic this week, but if not she has an appt next week. She's been vomiting a lot, and by a lot I mean that if we aren't going anywhere, I don't put clothes on her because they just end up drenched. When we go places, I bring two backup outfits, even if we're only going to be gone a short time. She's arching, she refuses to sleep during the day unless I hold her on my shoulder, although she has a cool chair that allows her to sleep sitting up now too which is nice. She won't take more than 4oz per bottle, and sometimes she doesn't even take that so I know they are going to get on me about her weight. She looks fine to me, just petite like her mama, but you know doctors and their charts. *eye roll* But hopefully we can figure it out soon because when I sent a MyChart message, they said that now it doesn't sound like typical baby reflux. Maybe if they had given me a med for her when I asked for it in August, we wouldn't have gotten to this point! Anyway, other than that, she's beautiful and perfect and super attached to her mama.
I will try to do better with updating the blog and Jaxson's FB page from now on! It's been slightly crazy here, but I think I'm getting a handle on things finally. Have an amazing weekend!
"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." ~Anonymous
