Ataxia Telangiectasia

I don’t know how else to title this one, so I’ll just put up what it is. Around 2pm my phone went off thinking it was just an notification from some annoying app on my phone. As I turned my phone over, lab results from Mayo. Last month based on the recommendation from the immunologist at Mayo, I underwent the Primary Immunodeficiency panel done by Invitae. This test looks at over 405 genes in total. The hope here was to make sure the CVID diagnosis was right. Well. It’s sort of right. Logged into the Mayo app and my anxiety started going through the roof as it loaded. 

The 9 page report showed up under results, that’s when the first thing my eyes saw was “Positive”. As I slowed down and just read the report, Ataxia Telangiectasia. What the fuck is that?  Well. I remember seeing it listed as a PI, but never read into it since I was hyper focused on CVID. The more I read about A-T, I just stop reading. 

I think we can all agree that getting answers helps people “solve problems”. This is the type of answer you don’t want. It’s up there. From the case studies that I’ve read, the red streaks that have appeared all over my face, chest, etc are called Telangiectasia. Which are enlarged blood vessels. To some people they just come with age and bear no significance, but for me, it’s the telltale sign of this disorder. Most sites describe A-T as a progressive, degenerative disorder which involves neurological, immune, and other body systems. It has been well documented that it’s the primary driver of people who cannot build antibodies from polysaccharide vaccines, or who have low IGg, Igm, Iga. Recurring infections. So with that being said, I think that checks all the boxes so far. 

As I read, the more I had to re-read. Fuck this is a lot to process. Another thing it’s known for is the eventual shutdown of the T-Cell system of the body. Not to be grave, but that’s basically a death sentence. Having a B cell problem is one thing, but now combine the two. That’s no way to find, or fight infection. It’s basically SCID. Bubble boy.  

As I read on, they started to expand on the motor/neurological impairments this causes. Now remember I said that this is a progressive thing. 

I stopped after the first 3 paragraphs and just emotionally broke down. Again. You’d think with everything Ive been through the past 3 years would have some sort of light at the end of the tunnel, or a finish line.  Not my luck. As I collected myself, I thought of the kids. I sent them both the results, and a small explanation of what this is. As I talked to my daughter I just started crying. My daughter has been through a lot. Having to send her this broke my heart. “Nothing like throwing more gas on the fire”. I know it’s not my “fault”, but this hurts either way.   I wish I could make it easier, but that’s just not gonna happen. 

Secondly I emailed my immunologist with the results, with the text of “WTF?”  I’m sure this is a pretty big piece of the puzzle we were looking for,  it this is the shittiest piece ever made. I’m sure that I will be having a deeper conversation with him on figuring out the approach. I know that I will be on SCIg for the rest of my life, but I think at this point, it’s what is keeping me alive to be honest. If you want to read more about what this is, please go here:


https://primaryimmune.org/about-primary-immunodeficiencies/specific-disease-types/ataxia-telangiectasia/

TLDR;

o Development of Telangiectasia or enlarged blood vessels

o Immune disorders ranging from CVID-like to SCID

o Motor function interruption including speech, swallowing and walking

o Severely increased susceptibility to cancers including pancreatic, prostrate, lymphatic systems

As for an update of everything else, pus was once again coming out of my face. Fortunately I had taken (stolen) culture swab kits from my GP’s office last time I was there. I woke up one morning to just my face burning. I went to feel my ear and wham. Pus. 

I immediately shot out of bed frantically looking for the culture sticks. Man. It was a lot. Probably a pea-sized worth. I got as much as I could on the two culture swabs (with the help of the wife), and jammed them into the culture gel. Sealing away what I could only hope for a good culture this time. Every time before it’s been nothing, or dead skin cells. 

Reluctantly my GP at first didn’t want to do anything with it, however , he finally caved and took them. If he didn’t I would have just taken it to lab core and filled out the paperwork myself and paid cash. I’m sorry but I need to get to the bottom of why this shit keeps coming out, and what is it. After a long 2 weeks, it came back positive (finally). I cultured:

o Cutebacterium Avidum

o Yeast

o Entercoccus Faecalis

Fucking finally. Something. My GP at first wanted to do Vancomycin, however, I had to remind him oral wouldn’t work, only IV. He immediately went with Augmentin. The report said that both the cutebacterium and Faecalis were susceptible to penicillins, to which I was reluctant to try since it doesn’t seem to work. To my surprise it’s been working. Even the swelling in my face is going down. So that’s a relief. A small win I guess, but I’ll take it. The pain is still there, but I’ve been tapering down on pain meds. It’s not as bad as it was, but it will still flare / hurt. So I’ll just have to keep an eye on it. 

Outside of that work has been ok. They started having people come back into the office. I wonder what it’s like. It’s probably not the same that’s for sure. For the time being, due to all my immune stuff, the CDC still marks me as a high risk for COVID so I have to be careful. I filled out paperwork which still allows me to work from home (to which I am so grateful for). 

As for next steps, I am waiting for the Genetic Consultant to get back to me on what the game plan is next. I’m pretty sure it will be what I am already doing therapy wise, however, I’ll probably have to start scheduling routine labs every 3 months to be proactive about the whole cancer thing. 



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