November 2020 Update

My calendar is overrun with appointments - I had to get one with bigger squares! *sigh*

A quick breakdown for those who don't want to read the whole thing:

1. Stoma dilation and PEG replacement were successful.
2. I have a new Rheum on the team - he will fill in the gaps left by my SSc Specialist.
3. My second round of eye duct plugs are going well.
4. My yearly Echocardiogram showed a Pericardial Effusion and thickening of two heart valves.
5. I was diagnosed with immunodeficiency and will be beginning IVIG or SCIG.

My GI system has continued to deteriorate over the last month. I know I say that every month - but it's true. As mentioned, we placed a new larger diameter tube to assist with gastric drainage. The surgery went smoothly and the new PEG works well. It's huge which is esthetically not pleasing, but at this point, I really don't care. I am desperate for anything that will improve my day to day life. I am having to drain my stomach contents multiple times a day, occasionally I even get woken up by the pressure and nausea in the middle of the night. Apparently, I often tell Noel, "I am nauseous," in my sleep - I am a big sleep talker.  This is not food or drink, it is just bile and air my stomach isn't passing. It is incredible what it can produce when given absolutely nothing. 

Thankfully, I am still tolerating my J tube feeds, though I am no longer absorbing all the vital minerals which they contain. My labs have been blahhh and my chronic dehydration, due to my lack of absorption, has become a serious issue. As mentioned I have been going to the infusion clinic twice a week (though it should be three) for hydration which, for now, includes a mix of calcium, potassium, dextrose, and sodium - though other minerals may need to be added in the future. It has become too difficult for my body to make the 2 hour round trip drive twice a week so I moved to the hospital's satellite location about 30 min closer to my home. Unfornutly, this satellite location is not where I feel safe, sanitary, or well taken care of - that is why I held off the switch for so long. I will continue to fight for home infusion but I have been assured that it will be impossible. Apparently, Medicare "never" approves at home hydration. Awesome...

I'm not sure if I have made this clear, but these infusions and intestinal feeds are what is keeping me alive. It is a palliative care measure - without them, I would not be able to live for more than a few weeks. It is disheartening to me that a patient can reach this point and still be denied a method of more convenient treatment all because the insurance company won't be, "reimbursed enough to make it worth their while." 

I met with a new Rheumatologist Monday. Well, actually, not new to me, but one I haven't seen in ages. His role will be to quarterback my care and to fill in the gaps left by my Scleroderma specialist who only practices here in Seattle once a month. This is actually the physician who initially diagnosed me 9 years ago. He remembered exactly who I was stating that I was the first, "incredibly severe scleroderma patient" he had seen. (This is probably the least preferable method when it comes to being remembered by a doctor haha) After looking over my chart and history he was a bit concerned - there really are no options my specialist hasn't already tried to get this disease under control. He believes another clinical trial would be my best bet. He actually mentioned another transplant, specifically an allogeneic (donor) since the autologous didn't seem to resolve the autoimmunity in my blood... but I kindly declined. I am not interested in going through another transplant and will not change my mind on that matter. For now, we will still try to move forward with the Actemera infusions which my specialist recommended back in September - though we are still battling insurance for them. He also ordered some blood panels and a follow up in one month. 

Later in the week, I met with my Ophthalmologist to trial some new tear duct plugs. This time we gave the dissolvable plugs a try since my eyes did not tolerate the more permanent ones well. So far so good. It is actually quite exciting to see how white my eyes are after just one day! Normally the whites of my eyes are always pink from irritation, even with using artificial tears 8 - 10 times a day. 

I also had my yearly Echocardiogram. Not much has changed, my pulmonary pressures and ejection fraction are still stable, though two of my valves (aortic and mitral) are starting to thicken from the Scleroderma. Their motion is still normal at this point though, which is good. The one thing of significance was a posterior Pericardial Effusion, aka fluid around the heart. This can, unfortunately, be somewhat common in autoimmune disease patients with heart and lung complications. This fluid would probably explain the chest pain and tightness I have been experiencing since last week. There isn't enough fluid to really drain so my body should naturally reabsorb it. However, we will need to keep an eye on it.

This morning I met with my Immunologist to go over the results of the vaccine we introduced to assess my humeral immune memory response. Unfortunately, my body did not "absorb' the external antibodies. I actually showed a whooping 0% percent repletion. This is bad news. In laymen's terms, my immune system no longer creates its own immunoglobulin or responds to the external introduction of protective antibodies, such as vaccines. This is categorized as an Immune Deficiency Disease. There are over 400 types of Immunodeficiencies - my physician believes my subset is a secondary immunodeficiency due to so many years of immunosuppressive treatments. It is a rare side effect but can happen. So, once insurance decides what they will and won't cover, I will be beginning either subcutaneous immunoglobulin (SCIG) or Intravenous Immunoglobulin (IVIG). These interventions should help bolster up my immune system against foreign contagions. Maybe I will finally get a break from my near-constant sinus infections! Whoop!

I will have to meet with my Scleroderma Specialist again this month and, due to this new diagnosis, will likely be unable to continue with the path of traditional immunosuppression for autoimmune disease management. Long story short, it is a confusing and transitionary time.

Xoxoxo, Chanel