First Week of Hizentra

I mentally feel a lot better than I did during my last post. I had been really struggling with a lot of frustration over the last few weeks. Mostly with my insurance for deciding to make everything so incredibly difficult recently. Though, I think the realization that I have been fighting for nine straight years sort of crept up on me too. Nine years of literal life and death - I think my poor brain just shut off for a couple weeks there. lol Thankfully I have a wonderful therapist, palliative care team, and army of family and friends willing to hold space for me during my moments of grief.

My insurance finally approved the SCIG which began this week. This is a blood product, called Hizentra, created by thousands of plasma donors which will build back my depleted immunoglobulin. It will be infused subcutaneously at home once a week. This means four small needles will be placed into my stomach while thick fluid is pumped under the skin just above the muscle. Yes, it is about as pleasant as it sounds. The subcutaneous version of immunoglobulin has a lesser side effect profile than the intravenous version, IVIG. IVIG boasts many severe, life-threatening reactions - I had been on IVIG once in the past for a bought of GBS and did not fare well. My physicians hope I will tolerate the subq version better since it is received in lower doses more frequently as compared to one large dose a month. The subq version I am receiving is not necessarily painful, but quite uncomfortable as the fluid builds under the skin. I am left with red welts until the fluid dissipates over about 24-48 hours. The one-hour infusion feels pretty similar to rubbing stinging nettles on yourself - sort of itchy and prickly. Besides being very tired the next day, I seemed to do just fine. 

I met with my Rheumatologist to go over some blood work and to discuss treatment options moving forward. Due to this newly diagnosed immunodeficiency, we decided to hold off on any immunosuppressive treatments, as they would be contraindicated at this time. We will be reevaluating my treatment options once we get this current, "fire" under control. It is quite odd prioritizing "fire." Letting one fire burn so you can snuff out another feels counterintuitive, but in reality, it is often the only way people like me survive. I have read a few studies that showed promising improvements in MCTD patients on therapeutic levels of immunoglobulin though, so maybe this treatment will kill two birds with one stone? That would be rad... My blood work was pretty typical for someone with serve GI issues. Low vitamin D, low potassium, etc. I also showed some alarmingly low blood sugar trends, low white blood cell counts, and of course, a formidably high autoimmune panel. Like I said, typical stuff when you reside in a failing body. I'll see my doc again in a couple months to evaluate how I am doing on the SCIG and to run more blood panels. 

After further discussion with my team about hydration at home, it looks like the only way forward in the fight with Medicare for treatment coverage is to accept full cost responsibility which will generate an, "official denial." If you are scratching your head saying, "that doesn't make any sense," I too had that reaction. Essentially, my doctors cannot fight Medicare until I am officially denied payment for treatment. Once I receive the denial letter from Medicare I can then have my physicians send in all the documentation on why at-home treatment will be more beneficial in my case. It's backward I know, but it currently is the only way forwards. Hopefully, we won't have to front the cost too long and can get the denial overturned asap. At least for now, I am receiving my D5LR safely from the comfort of my own home. 

I know this was a super short update, but I just wanted to pass along that things are moving forward finally! Xoxo, Chanel

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