In December of 2018, before Jennifer Dargan flew from her home in Montana to the National Institute of Health in Washington, D.C., she knew she wouldn’t be able to travel with her usual oxygen tank. “It’s like a little metal missile,” she says. “My backpack weighs ten pounds with that thing in it.”
However, she needed the oxygen to keep her brain and eyes from becoming hypoxic, so she got a loaner portable oxygen concentrator with enough batteries to power it for 18 hours. Within four, while Jennifer was still in the air, 45 minutes away from D.C., they were all dead.
Thankfully, Jennifer wasn’t travelling alone. She’d received financial support from the Friends of Patients at NIH program, which is funded by private donations, primarily from Race for RP. The travel grant covered her and her daughter, Shaina, who took charge of the situation as Jennifer looked for a socket to plug in her oxygen concentrator. “When my brain and eyes aren’t getting enough oxygen, I can’t comprehend anything,” she says. “My eyes roll up in my head and I start to go into a deep sleep.”
By the time Jennifer made it to the NIH campus, she’d been without enough oxygen for roughly two hours. This was a long time, even by the standards of Jennifer’s long history with the disease, which included many overlooked hypoxic episodes. “You know that thing they put on your finger and it registers your oxygen level?” she says. “Well, mine can read 97 percent, but I can still be hypoxic.”
That night, the concentrator again malfunctioned, and when Jennifer arrived at the NIH in the morning, she started her marathon day of testing by having 28 vials of blood drawn. On an empty stomach and with already low levels of oxygen, she “started to feel like a zombie again.”
If she had been back at home, her doctors might have missed the warning signs of her hypoxia. “It’s not because they’re bad doctors,” she says. “It’s because no one’s ever seen anything like this.” At NIH, though, Nurse Jill quickly recognized what was happening. “You’re not okay, are you?” she asked Jennifer, who shook her head. Jill cranked up her oxygen while Wendy Goodspeed, a research nurse in the RP department, took her to the hospital to be admitted and put on continuous oxygen.
Unfortunately, treating the rest of Jennifer’s symptoms wasn’t so easy. The eye muscle cartilage in her eyes has been inflamed for years, and at times her right eye feels like it’s being pushed from her socket. She also has pulmonary hypertension, fluid around her heart, a bit of pancreas inflammation, and collapsing airways. Because of her trigeminal neuralgia, even a light breeze on her face is painful during a flare-up, and when they’re inflamed, her Achilles heels feel as if they’re being ripped from her bone. “There’s nothing in my body that hasn’t been touched,” she says.
But, being unable to breath was, perhaps, the scariest of all her complications. When her airways first started collapsing, in 2012, she says it felt like a boa constrictor was wrapping around her chest and neck. Without an official diagnosis, Jennifer had to learn how to cope on her own, so she taught herself meditation. She says, “It’s taken me the last eight years to learn controlled breathing and how to not freak out.” However, this skill introduced its own complications. “I don’t huff and puff or gasp for air,” says Jennifer, “so it’s hard to get medical staff to believe I am truly struggling to breath.”
With an official RP diagnosis from NIH, Jennifer was able to start comprehensive treatment, including a plan for if her airways worsen. She’s also been able to start Remicade, which “has settled my body down,” she says.
“You can feel RP in your body and what it’s doing and where it’s damaging you.”
The diagnosis also gave her confirmation in the face of a lifetime of skepticism from doctors, who had explained her pain by saying she was depressed, imagining symptoms, or faking an illness for attention. A few years before her visit to the NIH, a doctor told her, “There is not a goddamn thing wrong with you” and recommended she see a psychiatrist.
Because RP reportedly affects so few people (roughly three to five per million), every patient who goes to the NIH makes a much-needed contribution to the disease’s research. “I also have it in places where it’s not documented yet,” Jennifer says, “but we have proof that it’s there.” Jennifer’s pulmonologist has told her that, by going to NIH, she’s helped many other patients.
Like most of those other patients, though, Jennifer wasn’t in a position to pay for a trip to Washington, D.C. “If NIH hadn’t helped me, I could not have gone,” she says. “I take like 20 medications, so my money goes to medicine to keep me alive. And oxygen!” she adds, laughing. She also points out that, though the RP community has been an irreplaceable source of emotional support, it’s difficult for fellow patients to support each other monetarily. She says, “We spend all our money on medical, so when it comes to donating, there’s nothing left for some of us.”
Because her health insurance company refuses to cover most RP treatments, Jennifer says she’s “lucky” she also has inflammatory arthritis, whose medications work for RP as well. However, her care continues to be complex, so she’s looking forward to her next visit to the NIH. In preparation for her trip last year, she’d prepared a list of questions, but then the hypoxia struck. “I waited two years for this visit,” she remembers, “and when I finally see the doctors, all I can say is, ‘I don’t know my medical history well.’”