Hello, Fellow Eaglets!
It has been quite some time since I last jumped into the conversation here on the forum, so I thought I’d share an update to let you know what life has been like for me 16 months after my surgery. The short version is I have found some joy again after long-suffering due to vascular ES. That said, I have developed a new medical “mystery” too, which has left me feeling like “here we go again.”
But first, the joy…I can move freely again! I should actually say I can move more freely than I did when my vascular symptoms were at their worst. I’m not running any marathons (or running at all for that matter), but I have been a gardening fool this summer! Pushing a lawn mower, planting flowers, pulling weeds, and outside socializing with neighbors admiring my handiwork: None of these things were possible prior to surgery, because my head would’ve exploded. Back then, I was afraid to sneeze or bend over to tie my shoe for fear of blowing out my left eyeball. And now, I’m 40 pounds lighter simply because I can get up and outside in my garden.
I gained 50 pounds over a period of approximately 6 years being sedentary, because of vascular ES. That significant weight gain alone wreaked havoc on my body both physically and psychologically. I had never had high cholesterol before, so seeing my labs gradually improve as I’ve slowly lost the weight over these last 16 months has been a real win too. I expect that with continued light activity, I’ll be back to my former healthy weight by the end of the year. I should add for clarification that I am not dieting - at all - or using any weight loss drugs, although I will say that weaning off gabapentin has probably helped. But shedding the 40 pounds is truly owing to the ability to simply be active once again without paralyzing pain.
As for the medical “mystery,” I mentioned that, immediately following my surgery, I felt like I had a lump or balloon just under my incision. Here I sit today, over a year later, with the balloon that has taken up permanent residence in my neck. Up until very recently, I was experiencing total déja vu contending with dismissive and totally indifferent medical professionals, who either gaslighted me or essentially shrugged as if to say good luck with that.
Thankfully, I had my annual follow-up last month with Dr. Taka, who did my invasive angio last year. My latest CT/A shows my veins and arteries are patent with no sign of aneurysm. It also clearly shows what’s been causing the ballooning sensation. The radiology report completely disregarded my complaint by merely stating post-surgical “anatomical changes.” But Dr. Taka looked at the images and pointed out an enlarged area of soft tissue on the left side of my neck, just under my scar, that is much larger than my right side. He said I should return to ENT to have the mass examined.
I have an appointment in a couple of weeks scheduled with a DO in the neuromuscular department here. Because just about every ENT at what is supposed to be an academic medical center failed or otherwise refused to diagnose vascular ES, my primary and I have very little confidence in the department. He’s hoping that perhaps neuro might provide some answers. If not, it looks like I’ll have to take a trip back to Cleveland Clinic. But now that I have the images, thanks to Dr. Taka, I’m feeling optimistic that the road to diagnose and treatment won’t be as long as the initial vES odyssey was.
While the balloon might be a continuation of the vES odyssey in some respect, I’m also aware that it could be due quite possibly to some other disorder. Having been diagnosed last year with fibromuscular dysplasia and found to have a variant of unknown significance for vascular Ehlers-Danlos syndrome (my mother has the same VUS), I was referred to a well-known genetic specialist, Dr. Fong, who ordered whole genome sequencing. Given my long complex medical history along with that of my family, it’s clear that I have a connective tissue disorder, but it’s also evident that there’s more going on. He essentially referred to Hickam’s dictum, “Patients can have as many diseases as they damn well please.” Put another way, it’s possible to have multiple disorders simultaneously.
Dr. Fong wants to try to get to the bottom of the mystery of why the long history of medical complexity through genetic testing. The ballooning could be the result of a surgical misadventure, a complication of vES, vEDS, or some entire other as of yet diagnosed disorder. I’ll have to wait to see what the test bears out. In the meantime, I’m grateful to be up and moving again. Hopefully, many of you are as well or will be again too very soon!
Blessings 
