Our Unexpected Journey

If I'm going to document our story, I have to start at the beginning.  Buckle up or grab a drink...this might take a while. 

September 12, 2020, was Andy's birthday and the day that we officially became empty-nesters.  It was also the day that he first started experiencing some balance issues; problems with his vision and gait soon followed.  Initially we thought that he was dealing with vertigo or Meniere's Disease. Difficult and annoying?  Yes.  Profoundly life-changing?  No.  However, as the weeks progressed, his symptoms quickly worsened and by the end of October he was losing his vision, unable to walk unaided, having tremors, losing feeling in various extremities, and slurring his speech, along with numerous other symptoms.  

As the fall and winter months continued, his medical team had grown to include: his PCP, an ENT, a neuro-ophthalmologist, a primary neurologist, a research neurologist, and the internationally-known head of Neurological Sciences at UCSD.  Fancy, huh?  By the end of January, his tests had included: five MRI's, a PET scan, a lumbar puncture, a bone density test, specialized eye testing, and more blood draws than we could count.  Additionally, he had received six high-dose steroid infusion treatments.  Tests revealed that he had "innumerable" lesions on his brain and spinal cord, as well as active inflammation in the spinal cord.  They knew the what, but they didn't yet know the why.  

While we moved through those months, they initially suspected that he had a brain tumor.  Then we were told that it was likely MS.  After a few weeks, they said that it wasn't MS after all and the possibilities were paraneoplastic syndrome, various rare forms of lymphoma, and a few other spooky things.  There were quite a few sleepless nights around here and it was a complete roller coaster.  I will say that since I was binge-watching House at the time, I was quite adept at all of the neuro lingo.  Oddly, it really did help - I wasn't thrown off by all of the big medical words.  

Speaking of fancy words...  By early March, 2021, the prevailing theory was that Andy had CLIPPERS syndrome.  Are you ready?   It stands for Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids.  It is an extremely rare auto-immune disease, a type of encephalomyelitis, that presents with neurological symptoms.  It was just discovered in 2010 and at the time of his diagnosis, there were fewer than 100 confirmed cases worldwide.  Not much is known about the disease since it is so new, so for the time being, the treatment was large doses of steroids.  As I mentioned, he had the six infusions, and he had begun daily oral steroids as well.  It wasn't ideal as steroids do their own, not insignificant, damage.  They also left him severely immunocompromised so he had to take prophylactic antibiotics daily and well as other medications to combat the steroid's side effects.  Thankfully, though, we saw improvement in his symptoms; they weren't gone completely, but things were definitely getting better!  

If you've hung in there with me this far, you deserve a medal!  Currently, he is beginning to stabilize.  Symptoms flare and then subside, more of that nasty roller coaster.  The fluctuations are difficult; some days he struggles to function, others are reasonably normal.  They initially had him on 60mg of Prednisone daily, with the idea being that over time they could wean him down to the lowest possible effective dose (without producing a symptom flare).  As of today, he is taking 40mg daily.  He has had to change up his diet and use a glucose monitor to keep his blood sugar in check as the drugs have made him essentially diabetic.  He has lost over sixty pounds...yes, sixty!  The disease is definitely taking a toll on his body.  

These are latest action items for him as of his 03/02/21 neuro appt:

• dropping the prophylactic antibiotic from daily to 3x weekly - any medication reduction is happy news
• slowing his steroid taper - they initially had him dropping a few milligrams every two weeks, but now they've decided to have him slow to tapering monthly in hopes of not bringing on another flare
• he needs to schedule another lumbar puncture - there are more tests that they want to run, including ruling out yet another kind of cancer
• they have ordered another set of (three) MRI's for April - he is becoming a regular at the radiology center

One of the doctors did give us a few words of hope.  He said that if they do indeed stay with CLIPPERS as his diagnosis, that there are potentially some other drugs that he could try in the future and that they might be able to take the place of the steroids.  He also encouraged us that other than this, Andy's overall health is excellent so he is in a good position to fight this disease and be able to manage it long-term.  Oh, how we are clinging to that hope!  Our lives have been so upended that any positive words are profoundly welcome.