Sidelined by rare diseases, she became an advocate for better treatments
Sharon Rose Nissley has lived with chronic pain since she was a young child, but even now, at age 46, it’s still hard for her to find the care and support she needs. “Finding compassion and adequate care for chronic pain has been the most challenging obstacle in my life,” she says. Two years ago, she started using medical cannabis, and has begun to experience some relief.
‘I will never forget that moment’
In her late 30s, Sharon Rose started to experience nagging pain in her upper body, shoulders, and neck. She had already been living with chronic pain from knee deformities since childhood (and undergone some major knee surgeries) but this seemed worse than anything before. It was interfering with her work as an interior designer, a physical job that required her to be on her feet.
Sharon Rose visited a chiropractor, who took an X-ray. His response was shocking: He told her the bones in her neck were fused together, handed her the X-ray film, and rushed her out of the office.
“In the parking lot, I sat in my car, looking at my X-ray held up against the window. I had no idea what a cervical spine X-ray was supposed to look like, but I knew it wasn’t supposed to look like that,” she says. “I will never forget that moment. I didn’t understand, and the fact that he was so alarmed and in such a hurry to get me out the door was very scary.” She cried in her car. “I felt embarrassed. I’m different, I’m not normal. Something is wrong and I’ve never known. I had always thought my knees were the big hurdle in my life; now look at this!”
That night, she looked up “congenitally fused vertebrae” online and came back with scarce results, but did find something called Klippel-Feil syndrome (KFS).
Getting a diagnosis (and then some)
Sharon Rose went to see her orthopedic knee surgeon, who made the official KFS diagnosis. Even with his level of expertise, he had little understanding of KFS; he gave her a flimsy neck brace and a pack of steroids, and told her she would be fine.
But soon after, she had a dizzy spell at work; it felt like her nerves were a tangle of wires sending mixed signals. She developed head pain that has never gone away: “My head feels like a concrete block that I am lugging around, tight and pressured, 24/7.” She started missing more work, and developed more symptoms. Within a couple years, she was also diagnosed with cervical dystonia and Ehlers-Danlos syndrome (EDS), both of which also contribute to chronic pain.
And she found that almost no one knew what KFS was, or how to treat it. A rare degenerative musculoskeletal disorder, it causes the bones from the bottom of the skull down through the vertebrae to be deformed and fused together from birth. Debilitating head and upper body pain, fatigue, and dizziness are hallmarks of the condition.
Sharon Rose also has vasculitis and a bleeding issue, which add complications. In fact, it was her dermatologist who first recognized that her excessive bleeding was abnormal. Later, her pain management specialist and hematologist together determined that EDS (also a rare condition) was contributing to her bleeding issues. (All of these contribute to her pain.) It’s seemingly random which specialists have an understanding of rare conditions, and Sharon Rose has been to a wide variety of health care providers in order to get answers.
Facing multiple chronic conditions—and becoming an advocate
“My life was turned upside down by chronic pain,” Sharon Rose says. “I have had to completely remodel and repurpose.” She is no longer able to work full-time in the career she loved; her employers have stuck with her, though, helping craft ways for her to work a small amount each week. She has had to cut down much of the time she used to spend on hobbies like gardening, reading, and creative pursuits. She isn’t able to walk her dogs anymore (two beagles, Mags and Lyla Bell), but they do give her joy and a sense of purpose—and strength to face the day.
“I am home a lot more, so I make sure my environment is pleasing. I read, but a book can last two months. I plant flowers—just not five flats’ worth. I set a goal that each month, I’ll do one thing that is a stretch, whether it’s seeing a friend I’ve lost touch with or a day trip. Last year, I took a trip to Austin, Texas, something I thought I couldn’t do anymore—it was empowering!” Sharon Rose is working hard to not let chronic pain get the best of her.
During the past few years, she’s also found a passion for advocacy. “While facing disability, I have been determined to find solutions,” she says. “I developed the leading international network of patients and families, which advocates and provides resources and support to improve the lives of those affected by KFS. Klippel-Feil Syndrome Freedom can be found on Facebook.” She also speaks to federal legislators about the need for more research. (Only 5% of rare diseases have an FDA-approved medication or treatment.)
Sharon Rose had never tried cannabis before—had even prided herself on that fact—when her chronic pain began. But a few close friends and her mother all brought up the idea when her condition worsened. “I have an adverse reaction to opioids, which narrowed my options,” she says. “I had to put the stigma of cannabis to rest personally.”
She did some homework and decided to give it a try two years ago. “I did not want to smoke it; I decided a vaporizer would be best for me. I went really slowly. I always thought being ‘high’ would be a hugely altering experience, but now I feel that using marijuana is no big deal at all. Even if I do use a little more than I feel comfortable with, it passes.”
After a little experimentation, Sharon Rose has settled on using a combination of a vape pen (she likes that it is convenient and discreet, with no preparation and a quick effect) and edibles. “I have a sweet tooth, so chocolates, gummies, or hard candies are a perfect method for me—I can go slow to control the effect I get,” she explains.
“I am currently microdosing with chocolate, which keeps a low level of cannabis in my system throughout the day with less noticeable high,” she says. “This helps me focus on things I need to do, or rest my body and mind, without being ineffective.” Sharon Rose says that she’s taking the learning process slowly. “Even though I’ve had my prescription card for two years, I am far from an expert—I learn something new every time I go to my dispensary.”
She likes that cannabis eases the anxiety that is sometimes created by her pain. “When using cannabis, I can’t focus on the pain; I feel the pain but it quickly goes out of my mind,” she explains. “That’s the main benefit for me: feeling at ease, relaxed, calm, and carefree. I’ve been using cannabis in the middle of the night to curb pain and anxiety and aid in restful sleep.”
Why is cannabis demonized?
Sharon Rose says the stigma around using cannabis keeps people from accessing medicine that could truly help them, in a safer way than pharmaceutical drugs.
“Growing up, I thought cannabis was this dangerous, bad drug, but it is no big deal,” she says. “The medications we are given—and told we can drive and live normally while taking—are rat poison. I have had terrible experiences with some pain medications, and terrible times weaning off them, often with little support from my doctors. Prescriptions like this make it dangerous to function, yet are considered ‘safe’ and valid options. It makes no sense.”
Sharon Rose says she still sometimes struggles with the stigma she grew up with. “I still sometimes don’t feel at ease about using it. It was ingrained in me that marijuana is this terrible thing.” But she knows it helps her: “Cannabis is something that you have control over, and if you don’t need it, you don’t have to use it. Plus there is no fear of withdrawal effects. I like that cannabis is natural.”
Keeping her options open—and close at hand
Sharon Rose also uses many other things to take care of herself, including some medications (but much less than she did before cannabis), other pain therapies, topical creams, mindfulness, guided meditation, breathing exercises, creativity, gardening, music, time spent outdoors—she keeps a printed list of her chronic pain “tool kit” and recommends others do the same.
“There is no magic bullet. Pain management is a combination of therapies that may change on any given day based on your pain experience,” she says.
It is sometimes completely overwhelming, but there is always something soothing that you can reach for—there is always hope for relief.
Klippel-Feil Syndrome Freedom: facebook.com/KlippelFeilSyndromeFreedom
Global Genes: globalgenes.org
Rare Disease Legislative Advocates: rareadvocates.org
Sanford CoRDS Research: sanfordresearch.org/SpecialPrograms/CoRDS
U.S. Pain Foundation: uspainfoundation.org