Musings of a Single Dad

For those of you who have had the misfortune of having gone through this, you already know what I’m about to describe… The Death Roller Coaster of Terminal Illness.

This is what happens as the person you love gets closer and closer to the end. When it all started, and as their sickness progressed, you go up the hill of hope… some medicines are working, they’re getting a little bit better and they’re starting to act like their own self and are able to go home.  Right when you think you’re safe, BAM things take a nosedive, down into the scariest hill of despair, as your loved one suddenly gets worse than they’ve ever been before… and just when you think it can’t get any worse, it suddenly banks and throws you into a dark tunnel loop of “what the hell are these new symptoms and where are they coming from?!?” This leads to the short straightaway of unease before banking again into “new disgusting symptoms scaring everyone!” Followed by another drop into what appears to be unknown waters (and when the hell did this roller coaster turn into a water ride and why is it shit’s creek and what happened to our paddles???) Only to bank again into the “even the doctors don’t know what’s causing this fever or that rash” set of rapid up and down hills.  We all know how this Roller Coaster ends, but the unknown twists and turns are what makes it so terrifying, horrifying, physically, mentally and socially exhausting.

In 2021 my Dad started getting sick.  It was a cough and a rash that wouldn’t go away.  At first doctors thought it was cellulitis, so they treated it but it didn’t go away… and the cough persisted.  Then one doctor did a biopsy of my Dad’s rash and said it was Adult Onset Still’s Disease, which is an auto-immune disorder. But it turns out, that he actually has something called VEXAS Syndrome. VEXAS is an anagram which stands for Vacuoles, E1 enzyme, X-linked, autoinflammatory, and somatic (VEXAS). It is a syndrome that is an autoinflammatory disease that affects adults and is associated with somatic ubiquitin-like modifier-activating enzyme 1 (UBA1) mutations. Inflammation is the main symptom of VEXAS syndrome, and it can affect many organs, including the skin, joints, cartilage, lungs, and blood vessels. For example, skin rashes can be painful and resemble those seen in Sweet syndrome. Joint inflammation can cause arthritis, and cartilage inflammation can cause pain, swelling, and redness in the ears and nose. Inflammation in the lungs can cause cough and shortness of breath, and inflammation in the blood vessels can cause blood clots and low blood oxygen levels.  The problem with VEXAS is that it is also associated with a new form of Myelodysplastic Syndrome, aka MDS aka Leukemia light.  These are what my Dad is currently fighting.

A few weeks ago, my Mom called the ambulance because my Dad had a horrible episode that I won't get into here.  MDS, the blood cancer my Dad has, has basically turned his blood into Koolaid, as the ER doctor described it.  His hemoglobin level was hovering around 7, when normal levels are around 12.5 to 15 for someone of his age.  His platelets have been hovering around 11,000… and normal is between 150,000 and 400,000.  So the fact that his body basically has no platelets to stop the bleeding makes things super scary, especially when the new symptom deals with bleeding and you don't know where it is coming from today.  My Dad's episode happened while he was on his portable toilet (he's been in and out of the hospital so often over the last 6 months that his muscles have atrophied terribly).  He essentially wasn’t responsive.  My brother in law ran over to help my Mom as they waited for the ambulance to get there.  They got my Dad back on the bed and they tried to talk to him to see if he'd respond when the ambulance finally arrived. My Dad has a specific DNR directive. He doesn't want to be kept alive by machines, but they can do CPR if he codes, but only for a few minutes. I'm telling you this because he coded twice, once on the way to the ER and once in the ER. My oldest sister walked in and he wasn't responsive or breathing and she said "Dad, I'm here." and he opened his eyes and took a breath. We all rushed to see him. My oldest sister lived the closest, only a mile from the ER, then my Mom arrived, then I came up from NJ and my middle sister finally got there after from southern MD as apple maps had her going all over God's green earth to the Wrong ERs in the hospital's system.