April 2019 Update

During times of discouraging health set backs I like to remind myself just how far I have come. Two and a half years ago I was literally dying and relying on Social Security Disability benefits for income. Now, I am a mostly functioning young professional directing the entire client relations department of a multinational company. Those are big, BIG strides... but it's still difficult to not get discouraged when I experience set backs. Recovery, especially from clinical trials, is not a short, sturdy stair case to a perfectly safe balcony. Recovery from a clinical trial is more like a steep sheet of ice to a rickety old deck, you make a bit of progress, slip back down, make a bit of progress, slip back down, etc. Sometimes, when you finally get to the top, the deck collapses out from under you. It's frustrating and messy. Did I mentioned frustrating?! But no matter what, your only option is to keep crawling the slippery ice while praying that old deck will hold up as long as possible.

Lately, I feel like I have been clawing my way up that slippery ice only to find the rickety deck swaying from side to side while nails silently slip out of their holes. I'm thrilled that I have made it to the top, but I have no idea when the nail will slip that was holding the last few boards together.

As many of you know, I reached my two year post HSCT mark back in January, however, my blood work is still horribly awry. My WBC is still well below normal and my IgA, IgM, and B cells are completely non-existent. This are all solid indicators that my bone marrow never fully recovered post transplant, especially at two years out. There is really nothing to be done besides being overly cautious of germs - and to be put on antibiotics at the first sign of any sort of sicknesses. (Which is a whole can of worms in itself.) The last thing I want to do is is live, "over cautiously," avoiding crowds, public places, pools, certain foods, etc. So, the truth is, I am not. I am just living. I am fully aware of the implications BUT I didn't fight this hard to live only to sit at my house basking in hand sanitizer. Please understand, I am not disrespecting my bonus life, nor am I completely throwing all caution to the wind. I am being smart. I am being safe. I am simply enjoy this second chance to the full extent possible.

I saw my Scleroderma specialist last month who noted swelling of the hands and increased GI distress. The good news is my skin score is still stable, but the bad news, I am clearly aspirating and backsliding in regards to GI progress. Wet, ripping coughs accompanying each meal. Tightness in the chest. Food and phlegm coming up hours, sometimes days latter. Recently, after a particularly bad bought of aspiration I ended up with a nasty chest infection requiring antibiotics. My biggest fear is aspiration pneumonia, which has taken the life of three of my best friends.

I was given an emergency referral to my Digestive Disease Specialist who was wonderful as always. After a rectal exam no one enjoyed, she noted the months of Pelvic floor therapy I did was a total failure. The muscles in my pelvic floor are shot. Being unable to defecate due to a lack of muscle tone causes extreme constipation, which eventually leads to horrible diarrhea and leakage. (Cute I know.) There is literally nothing they can do. My dyssynergia and loss of muscle tone is just too great. So... home enemas every three days for me! As for my upper GI tract, my esophagus has been desperately holding on to food. Take for example, burping up a piece of egg I ate 3 hours ago that never made it to my stomach while driving to work. Ew. Likely, I have a stricture in my esophagus causing the retention of food - plus the severe dysphagia which doesn't help. I am scheduled for an Upper Endoscopy in May to dilate my esophagus. We are also working on scheduling another swallow study to asses my pharyngeal muscles which are likely contributing to the aspiration along with the strictures. While at my GI we noticed my resting oxygen sats were down which is never a good sign. So I have been referred to my Pulmonologist next month as well. I will be so unbelievably bummed if my GI tract has begun ruining my lungs - they were finally stabilizing in the high 70s! I am losing weight, nauseas, and a literal burp factory.

....Urg. Insert all the curse words.