It is Still March, 2021

Like the title says, it is still March, yet here we are with another update lol Digestive-wise, things have been going really well! I've been searching for any and all information on the GI tract and human immunoglobulin therapy lately. It has been a fascinating ride. From what I can understand, human immunoglobulin, which is a blood plasma component, is linked to antibodies and other substances directed at nerves. The immune system and nervous system, especially autonomic, are heavily intertwined. In autoimmune disease, the immunoglobulin fights the autonomic-neuropathy, aka, the dysregulation of the autonomic nervous system due to autoimmunity. This syndrome is heavily present in almost every autoimmune disease, and for me, what took out my digestive tract. Here is a GREAT article about it! https://www.karger.com/Article/Fulltext/498858

With my GI nerves sort of healing, which if you recall, also happened for a short time after my transplant due to a similar short-term resolution of this autonomic-neuropathy, I can definitely take more by mouth. We have actually moved my hydration infusions back to as needed, instead of every day! Whoop! In all honestly, I have been trying to use my J tube for feeds less and less. I even went a few days without feeding through the J - though I am definitely still using it for meds. However, I may be being a bit too optimistic *sigh* My latest labs showed lower than normal potassium, calcium, etc. Which means I am not absorbing all the nutrients I need from the food I am keeping down. My J tube formula is all predigested proteins, etc. so my body doesn't have to do so much work to digest. With food, that is clearly not the case. I actually have quite a bit of residual food in the morning which drains from my G tube  - which means some food still isn't leaving my stomach. I am also aspirating more than I expected. I have a ripping, wet cough all day every day at this point. I am going to be consulting with my GI doc regarding what steps are best since I need to ensure my nutritional needs are being met - but also don't think I need to be on all-day feeds anymore. So good news, bad news. 

My latest labs also showed the continuing decline of my WBC and ANC. My last ANC was down to 0.7 which is really, really low for someone not on immunosuppression. Normally, anything under 0.5 they want you in the hospital for your own safety... Yikes. My docs still have no clear idea as to why this is happening. So, we are running another panel this week to see if there are any changes - if it continues to decline I've been told to expect a bone marrow biopsy. 

The PFTs that I had performed at the very end of last month looked good. Everything was stable beside the DLCO which did drop about 12%. However, I typically find varying percentages of about 5-10% are pretty standard for me in that arena. So, my Pulm isn't too concerned about it for now. We will repeat again in 6 months instead of a year as we had originally planned. 

That's about it in my world. Besides taking care of my health, I have been spending a lot of time roller skating. It's been a lovely workout for me, as well as an enjoyable new hobby. Though, it is quite hard on my osteopenic bones lol I already broke an elbow! Luckily, it was a clean break and is healing quickly. No surgery or cast needed!

Hugs, Chanel

March 2021 Update

Hey All! Sorry I left you hanging in regards to all that blood work with the Hematologist. It all came back inconclusive regarding the reasoning for my continuing low ANC/WBC counts. At this point, since we cannot pinpoint a reason for this issue, it is being coined a Rituxan side effect. My team is split on this explanation, however. Half of my physicians think it is an underlying issue we haven't found yet and the other half believe Rituxan can have this effect for up to six months. Considering my last dose was 7 months ago, I have a difficult time feeling that this is in fact a Rituxan side effect as well. Truth be told we may never know. At the moment the low ANC/WBC count isn't harming me so I am not really that concerned. I'm technically not neutropenic, though I am hovering right above it. If I do dip into neutropenia my team decided they want to do a bone marrow biopsy - so let's just hope it doesn't dip. lol 

In other news, things have actually been going really well lately! I started noticing fewer GI symptoms a couple weeks ago. I was taking my nausea meds less and staying better hydrated via mouth. I was wracking my brain for what could have caused this improvement and the only thing I could come up with is my weekly SCIG dosing. So, naturally, I started reading medical journals and clinical trial reports to back up my findings. Low and behold, Immunoglobulin treatments have been successfully used in patients with Gastroparesis and Chronic Intestinal Pseudo-Obstruction! (Why had this never been brought up before!?) I was only able to find a handful of reports (probably why it was never brought up) and one ongoing clinical trial, but the results were all overwhelmingly positive. WHAT A HAPPY SURPRISE! While none of the studies necessarily cured the small subset of patients, almost all found that their quality of life was improved and their ability to take food by mouth was more prevalent. 

Here were the studies and one trial I found if you're interested:

• https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4792859/
• https://www.elsevier.es/en-revista-revista-colombiana-reumatologia-english-edition--474-articulo-successful-treatment-with-intravenous-immunoglobulins-S2444440517300560
• https://clinicaltrials.gov/ct2/show/NCT04208828

So treatment for a completely separate issue, my Immunodeficiency, has lead to an improvement in my GI symptoms. I think that is pretty awesome! So far here is what I have noticed, my nausea is insanely reduced and I can take more by mouth. It is not always constant, and I am still not absorbing much if any of what I eat, but it sure is enjoyable! I started with soft things like cream of wheat and bananas and then moved to things like small bits of bread and mashed potatoes. I still have trouble swallowing, so things have to be relatively small/soft; I also haven't noticed any improvement in the GERD, so things cant be too spicey. However, anything is exciting at this point! I did notice that I am not gaining weight at all with the extra oral intake and (TMI warning) that my stool is very fatty stained and often has undigested food. So, while I don't think I'll be sustaining life without a tube anytime soon, I can now eat a bit "recreationally." Woohoo! I still require nausea meds most days, but haven't thrown up (not counting brief regurgitation) in almost a week! That's a record in my household. lol The only downside of being able to tolerate more orally is the risk of aspiration. *sigh* I woke up Sunday morning, after having some ice cream a bit too late in the afternoon, to horrible coughing fits and shortness of breath all day. So, I will need to be safe and limit my oral intake times and amounts to protect my lungs. 

All and all, I think things are looking up! So are my SED rates, unfortunately... see what I did there? hehe But that is fine, while my inflammation levels may be up my quality of life still feels loads improved. Right now we are still iffy on what treatment, if any, to pursue. We have few options since almost all of them are immunosuppressants which is the last thing we want to be doing in my current state. It will likely all hinge on the results of my most recent PFTs. If they show a large decline in scores we will really have no choice but to return to immunosuppressants. We are also looking at TPE, Therapeutic Plasma Exchange, as an option which, while unconventional, has shown some promising results in Scleroderma patients. Let's just hope the PFTs are stable.

Once I get my results I will be sure to give an update. Hugs!