June 2021 Update

Happy Summer Everyone! I am so thrilled to see sunshine in Seattle. This will be a short and sweet update - likely the last for a good while too. My Jejunal tube was pulled this month as I am now sustaining 100% orally. While my nutrition and diet aren't exactly a perfect science at the moment, it is enough to sustain a mostly functioning human. I am BEYOND thrilled to have one less medical device in my body! My Gastrostomay tube was swapped out for a lower profile button as well. This will continue to be used for draining residuals. As of now, I am no longer, 'the tube-fed wife.' ...You know, getting rid of your lifeline is actually quite frightening. My head was swimming with the, 'what ifs.' What if this treatment suddenly fails? What if I need my J tube again? Remember how terrible that surgery was!? But, I have decided I cannot live in the 'what ifs,' all I can do is move forward. So here I am, marching forward with one less tube, ready to take on whatever comes next. I sincerely love and appreciate each and every person who has followed my long journey via this blog. However, I hope we never have to meet here again! lol Let's connect on another life path that has less to do with failing health... Shall we? ;)

If you're interested in my next adventure, please find me on Insta @thetubefedwife

Signing Off,

Chanel

May 2021

Hello Everyone, no news has been good news in this case. It seems like a sole monthly update (perhaps even less) will be pretty standard from this point moving forward. What an exciting milestone to reach! Things have continued to improve, from my lungs to digestive tract, thanks to my Immunoglobulin therapy. It has been nearly 8 weeks since I used my J tube for nutrition. I have been able to sustain orally now without any enteral assistance. While my intake of supplements has been increased, and I still continue to require lactated ringers throughout the month, this is HUGE! I am officially no longer reliant on artificial nutrition. So, what is the game plan?

In mid-June I have an appointment to remove my J tube! This will leave me with the G tube, which will stay indefinitely. The G tube allows me to remove undigested food from my stomach - creating a better quality of life. Less nausea, less regurgitation, less bloating, etc. While yes, I am sustaining orally, that does not mean my digestion is suddenly perfect... or even close to on par with that of a normal human being! lol I definitely will still need help from my G tube, oral supplements, and IV hydration to keep me functioning. Immunoglobulin therapy is not a cure all - though it seems to be the closest thing I have received in the past 10 years!

Our biggest concern up to this point was aspiration. I continue to have a wet cough throughout the day. My Pulmonologist and Gastroenterologist worked together to assess my aspiration risk and ensured it will be safe to pull my J tube next month. The good news, even though I am coughing, my lungs have NOT been damaged or lost function since I began oral intake. This means my cough could simply be acid reflux or, it is a mild aspiration that doesn't seem to be causing damage. As long as my CTs and PFTs continue to look this good though, I am not worried. My PFT results were actually the best they have ever been! 

My blood work continues to be off in the white blood cell category. I met with a new Hematologist this week actually. She didn't believe this leukopenia was derived from past immunosuppressive treatments, but instead, a side effect of my actual autoimmune disease. Apparently, in rare cases, Scleroderma and its overlapping conditions can cause leukopenia on their own. If my numbers continue to drop, we talked about starting a medication to stimulate white blood cell growth. However, that is not quite necessary yet.

I am so incredibly grateful to the thousands of plasma donors who have made this incredible recovery possible. Thanks to their generous donations I am able to truly live again. Eat again! I cannot express my gratitude enough. Thank you so very much. 

Hugs, Chanel

April 2021

Happy Spring! The weather has been rather lovely here in Seattle. It's been a refreshing and much-needed change from the super dreary winter we had. Things have continued to improve with my GI tract thanks to the Immunoglobulin treatment. Drumroll, please... At this point, I am sustaining COMPLETLY ORALLY! I have not used my J tube for nutrition in a couple weeks. While my diet is not completely normal, it is enough to sustain human life and keep my weight stable. My GI doc is of course monitoring me closely during this transitory time. My blood work will be closely monitored to ensure nothing essential goes out of whack. I was started on a few heavy liquid multivitamins to ensure I am getting everything I need from my limited diet as well. My G tube is still being utilized daily for draining the excess things my body just cannot digest, aka residual left in the morning from the day previous. My GI doc says if I can go 6 weeks without utilizing the J tube for nutrition, it can be removed! However, the G will stick around indefinitely. This is SUCH exciting news! The only thing we are a bit considered over at the moment is the aspiration - so ill be connecting with my Pulm soon to assess.

Side note, my email subscription services are changing over the next few days. You as the subscriber dont need to do anything, though you may notice my email updates will come from a new platform and/or look a bit different than they have in the past.

Hugs, Chanel

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! 

January Update 2021

Not much to update, honestly. That is a great thing though! My health hasn't been declining or improving really - I would say it's just stagnant. My treatments are almost exclusively at home right now due to the pandemic. So, under my physician's supervision, I continue to utilize Lactated Ringers with D5 and IV Zofran 5-6 days a week as well as my Hizentra (IVIG) subcutaneous at home once a week. 

The Hizentra has been going well. I am not a fan of the subq infusions, they simply are uncomfortable. Though, it really doesn't matter as it seems to be working. My immunoglobulin is finally trending in a good direction. I cannot explain how satisfying it is to see such quick, successful results from a treatment! I wanted to put my lab results on the fridge with a little gold star lol My Immunologist is very happy with my results as well and decided I will continue with this treatment at this 10mg dose. I will continue getting monthly labs drawn to asses my IgG levels. The other thing we addressed at our appointment this week was my continually reducing Neutrophil count. I was under the impression that improving my immunoglobulin count would somehow help improve my WBC count. However, apparently, they are not connected at all lol My Immunologist actually wants me to see a Hematologist regarding the poor WBC count - so back to the Hematologist, I go. 

Next week I'll do a haul of blood work with the Hematologist to assess my counts and then see her the following week for results. Other than that I have a bunch of labs to draw for my Rheum who I see later this month. A pretty quiet month in my world ;)

Xoxoxo,

Chanel