This is David Roper, Ashley’s dad, writing to update everyone.
Two years ago a friend texted me, “I’ve heard of a surgery that one woman did that seemed to cure her POTS symptoms! I thought I’d let you know because she sounds a lot like your daughter, Ashley!” And the odyssey began.
I was skeptical at first. “How can a surgery fix what Ashley has? Her connective tissue is just made poorly.” But my friend’s text led me to learn about Jennifer Brea, a woman with, amazingly, all the same rare diagnoses as Ashley … hypermobile Ehlers Danlos Syndrome (hEDS), Postural Orthostatic Tachycardia Syndrome (POTS), Chronic Fatigue Syndrome (ME/CFS), Idiopathic Intracranial Hypertension (IIH), Atlanto-Axial Instability (AAI), Mast Cell Activation Disorder (MCAD), Tethered Spinal Cord, Gastroparesis … to name a few. Jennifer created an incredible documentary titled “Unrest” (available on YouTube) which documents her struggles with Chronic Fatigue Syndrome (and the numerous doctors that didn’t believe her and/or tried but had no answers). But one doctor saw her film and reached out to her saying, “I think I know what’s wrong with you. I think you have hEDS. And I do a surgery that might help you.” (You can read Jennifer Brea’s fascinating story here.)
Ultimately, this doctor explained what I have come to learn:
Tendons hold muscles to bones and ligaments hold bones to bones. What holds tendons and ligaments in place is “connective tissue.” In people with hEDS, their connective tissue becomes lax, not holding things in place very well. Our brains and skulls are very heavy and sit atop our spinal column, held in place by connective tissue, ligaments and tendons. Over time, as we turn our heads thousands of times a year (and potentially have some traumas from falls, sports, etc.) that connective tissue becomes increasingly lax. Over time, as that connective tissue gets more and more lax, some people who have hEDS experience their head gradually “settling” lower, compressing the top vertebrae (C1) into the second vertebrae (C2). However, there is a crucial artery and nerve that runs to/from the brain to the rest of the body that exits between C1 and C2. If C1 is being compressed into C2 (as in when they stand or sit up, they can experience significant problems as a result of diminished blood flow and nerve performance. (Ashley struggles with chronic headaches, migraines, “brain fog”, intracranial pressure, intermittent vision issues, significant fatigue, slow healing, gastrointestinal issues and pain, hot/cold sensitivity, difficulty with loud noise and bright lights, etc.)
Hence, a C1-C2 fusion surgery (in which they take 2 small bones from a cadaver and screw them onto the sides of C1-C2 to keep that space open the proper amount) can help some patients tremendously! Jeff Woods, a person who has had this surgery, lays this out beautifully in greater detail on his website. (His story is pretty remarkable, with him being in the hospital for four months and figuring out the cause for his problem but multiple doctors refused to read the medical journal articles he had highlighted right next to him on his bedside, explaining what he believed to be the solution … a C1-C2 fusion. His story and Jennifer Brea’s are similar to Ashley’s in so many ways … pain, struggles, some doctors trying, other doctors not believing, struggles to live life, etc. Remarkable to have found people who’ve had similar life experiences … and found something that actually helps!)
So, Ashley will have the C1-C2 fusion surgery on April 29, 2025 via Dr. Fraser Henderson in Maryland, the same neurosurgeon who handled her tethered spinal cord release surgery. Recovery will be hard for 3-6 months and may continue for another 6-12 months after that.
Note that this is not a “cure” for her hEDS; you can’t “fix” your connective tissue. And there is much she’ll have to do following her surgery and recovery to maintain improvements (extensive PT, etc.). But she (and we) are hopeful that this surgery will significantly improve her quality of life and she can get back to loving on children, hanging out with friends, and engaging with peers in church and bible studies … things that she’s been increasingly unable to do.
Yet she is a courageous, resilient young woman who continues to persevere and believes strongly in Isaiah 41:10 (“Fear not, for I am with you, for I am your God. I will strengthen you and help you.”). Your thoughts and prayers are much appreciated, and Ashley loves receiving your encouraging text messages! (Just don’t expect her to respond while she is preparing for surgery and healing.)
Many blessings!
David
