Nobody knows what’s wrong with Jenny Ray McGee.
If you saw the 20-year-old Lawrence High School graduate on the street, you wouldn’t guess that as a baby she was deathly ill and spent her childhood stuck in a wheelchair.
“Everyone looks at you like you are normal, and you are expected to be normal,” Jenny Ray starts, but interrupts herself.
“Normal. I hate that word,” she scoffs. “But you’re not normal. You’re trying to fake it, and everyone thinks you’re normal, but then they find out you’re not normal, and then they don’t treat you the same.”
There is something wrong. But whatever causes the numbness in her hands and feet, frequent falls and lack of balance, the inability to run, the frequent exhaustion and abnormal gait doesn’t have a name.
She knows what she doesn’t have. She lists the complicated names of her past diagnoses like a tired, frustrated mother reeling off the names of naughty children: ataxia, porphyria, chronic immune mediated poly demyelinating disease, congenital myasthenia gravis.
Congenital myasthenia gravis made sense. This rare form of muscular dystrophy could have caused her weak and improperly functioning muscles. CIPD made sense. This could have caused the numbness in her extremities. Ataxia made sense. This could have caused her lack of balance. And porphyria made sense. This rare blood disease could also have caused her muscle pain, numbness and weakness.
But none of these names was right.
Some might refer to these names as misdiagnoses, but they are really more like dead-end roads for Jenny Ray. Roads that looked for a while like they might lead her to freedom from sickness. But each time the treatments and medications stopped working, and the joyous skipping down the road to recovery became a difficult hike down a trail of tears as Jenny Ray struggled to come to terms, again, with the fact that she is sick.
Three years of skipping
In 2005, the name given to her road to recovery was dopa-responsive dystonia, a diagnosis by her doctor at Children’s Mercy Hospital, Dr. William Graf, chief of neurology. Graf says he disagreed with the previous diagnoses because a milder form of the disorder also affects Jenny Ray’s biological mother.
“This should illustrate the importance of an accurate diagnosis versus an initial hypothesis,” he says.
The DRD medication worked amazingly well, and a profile story on Jenny Ray that ran on the Children’s Mercy website carried a headline proclaiming “Diagnosis of Dopa-Responsive Dystonia Ends 16 Years of Health Challenges.”
For three years Jenny Ray was skipping. But then she started stumbling, and then falling. The medication had stopped working. Jenny Ray says she bounced around various doctors and hospitals, including Lawrence Memorial Hospital, in search of doctors who would just renew her prescriptions for the DRD medication and not request more tests, even though she herself had begun to doubt that DRD was the correct diagnosis.
Jenny Ray says she knows it sounds dorky, but during some research on DRD, she found some online videos of kids who had been correctly diagnosed with DRD and miraculously healed.
“They were in a wheelchair, now they’re doing back flips,” she said. “I was in a wheelchair. But me? Not so much on the back flips.”
So Jenny Ray’s aunt persuaded Graf to see her again. She says he, too, had begun to doubt that DRD was what she really had. And since DRD is an illness that is diagnosed by observing how the patient responds to the medications, there is no definitive test that could prove this is what she had. So the best diagnosis that Jenny Ray gets, for now, is that she is the victim of an undiagnosable genetic condition.
“Undiagnosable? Maybe,” Graf says. “Or ‘yet-to-be diagnosed.’ We could probably label her condition as CMT (a complex group of hereditary sensory motor neuropathy disorders) and just leave it at that, but I think Jenny and her birth mom are more complicated. And we cannot begin to discuss targeted therapy for the condition until we understand it biologically.”
Will to live
Jenny Ray was never supposed to live. Debbie McGee, who adopted Jenny Ray as a baby, had to stay up almost around the clock for days and nights on end taking care of her.
“This is not the type of kid you put to sleep and take care of in the morning,” Debbie says, adding that Jenny Ray “absolutely” would have died during the night without the constant care. “But I am not a hero. I have never personally met a parent that wouldn’t have done for their child.”
Debbie says that even though the doctors weren’t able to diagnose Jenny Ray, they were able to take amazing care of her and help keep her alive. She adds that support from family, friends, doctors and nurses is why Jenny Ray is still alive today. Plus, Jenny Ray, even as an infant, had an amazing will to live.
“I was told that other babies would have just rolled over and died. But Jenny Ray would not,” Debbie said.
The assistance from hospitals, nurses, friends and family went beyond emotional and medical support. Debbie says it was only through this support system that she and her husband were able to struggle through paying for Jenny Ray’s care.
“Insurance companies are after one thing,” Debbie says. “And that’s profit. Plain and simple.”
Debbie says that the insurance companies never wanted to help, and that they would always delay paying for Jenny Ray’s care as long as possible. “I couldn’t talk to the insurance companies. I would just get so furious. And I needed every ounce of energy to take care of Jenny.”
But, Debbie says, they always managed somehow to get by. The family worked overtime, never took vacations and always put Jenny Ray’s needs first. She says Children’s Mercy would get as much as possible out of the insurance companies, and then ask the family once a year to pay whatever they could toward Jenny Ray’s bill. The rest would be taken care of by the hospital.
Sometimes it was friends and family stepping in to help. Or the family’s church at the time, Baldwin Gospel Chapel. Or the Visiting Nurses Association of Douglas County.
“We somehow, always, managed to get by,” Debbie says.
The wild years
Jenny Ray’s struggle for survival, though easier now, is far from over. “It’s the suffering that makes me sad,” Debbie says. “Every day is a day of suffering for Jenny Ray. There are no days without suffering.”
Even though her illness still has no name, Jenny Ray’s overall health has improved greatly compared with when she was younger. Though she tires easily, she rarely falls over anymore when she walks. She doesn’t have to spend long stays in hospitals receiving infusions or getting tested. And she says she is never going to use a wheelchair again.
Three years ago, when Jenny Ray seemed to be on the path to almost full recovery, she started what she calls the “wild years.” For the first time in her life, Jenny Ray was in control of her body, and she wanted to take full advantage of it.
“I was gonna do what I wanted to do,” she says. “I just didn’t care after 17 years of having your own body as your own prison: not being able to tie your shoes, brush your teeth, comb your hair, stand without assistance. I had so many feelings, and I didn’t know what to do with them, so I just drank them away.”
Freed from her prison, Jenny partied, drank and got in trouble “a lot,” and a rift developed between her and her mom.
“What am I doing with myself after they kept me here?” Jenny Ray says she finally asked herself one day. “So many people fought for me. They don’t have a label for it, but I am still here, I’m still walking and I’m still breathing. And I’m a lot better than a lot of other kids. A lot of people would look at me and be like, ‘What are you complaining for? Do you know what we would give to have been in a wheelchair and then be able to walk, and still be getting better?’”
Inside of a large blue corner house in Lee’s Summit, Mo., in late January, Debbie and Jenny Ray tease each other like teenage sisters. Jenny Ray moved from Lawrence to Lee’s Summit last year in order to work and go to school while waiting to enroll at the University of Missouri, so Debbie has to drive from Lawrence to pick her up. Debbie is taking her daughter to Children’s Mercy for her appointment, where Jenny Ray will be getting back the results of a new series of tests.
In the car, Debbie puts on a CD. Over a slow, acoustic intro, Debbie explains the song. Six years ago, when Jenny Ray was 14, she had a really hard winter — winter was always the hardest season. Every night Debbie sat with Jenny on her lap in the same recliner rocking her to sleep to the song. “It didn’t make dying OK,” Debbie says. “But it seemed to make the emotions OK.”
On the morning of her appointment, Jenny Ray says she is skeptical that she will ever be fully cured. “That word is —” Jenny Ray says, but stops and sighs. “I don’t think that word applies to me anymore.”
She says she is happy with the amount of strength that she does have, and that she is grateful for the amount of time and energy everyone — family, friends, nurses and doctors — has spent trying to make her better.
In the hospital room of Children’s Mercy’s neurology department, though, her frustration is obvious. Tears roll down her cheeks as Dr. Graf explains that the technology for identifying previously unidentifiable genetic conditions is improving rapidly. During this latest round of testing, 20 percent of people with previously undiagnosable genetic conditions received a diagnosis, for the first time in their lives.
Not Jenny. All her tests came back normal, so all she can do is wait. Wait for computer technology to advance, Graf says. His best guess is a couple of years. A couple more years of waiting. At that point another 20 percent or so of people tested will likely get diagnosed.
“Because of expense, we are testing Jenny for individual genes step-by-step,” Graf says. “Her gene test for the GJB1 gene is pending now. In the future, this could be performed more effectively and efficiently by whole genome sequencing.”
Never giving up
Graf believes Jenny Ray’s story is a story about fortitude, and “not giving up the chase and not being satisfied with a poorly, or incorrectly, defined diagnostic label. I am sorry that we cannot fix her disorder. But I see my job as finding the right diagnosis through the right approach, in addition to supporting her.”
As soon as Graf leaves the room, Debbie walks over to comfort her daughter. Jenny Ray sobs as Debbie rubs her back. Jenny Ray finds some comfort in having grown up in hospitals seeing children much sicker than she, who “would give anything to trade spots with her.”
But this comfort is short-lived because Jenny Ray is tired. Tired of waiting. Tired of falling. Tired of starting over.
A few days later, Jenny Ray’s frustration and anger haven’t receded much.
“I know (Dr. Graf) has my best interest at heart, and I know that, in the end, he will probably cure me,” she says. “But I do not want to just be a sitting duck for two years.”
She continues by imagining a conversation with her doctor. “What are you going to do,” she asks “if next week I get a cold? And I’m not going back in that wheelchair. I will lay in your office for three weeks straight until you give me something else. I’m not ever going back in a wheelchair again.”