Jayne Johnson\u2019s house is like that of most 16-year-olds.<\/p>\n
There\u2019s a television in the family room, and a computer well-stocked with video games in the den.<\/p>\n
There are her two wiggly little dogs, Taffy and Tyrone, whom she dotes on.<\/p>\n
There is her mom, Joey Sexton, who loves her very much.<\/p>\n
But for Jayne, that\u2019s where her life, and that of a normal 16-year-old\u2019s, sharply diverge.<\/p>\n
Jayne, one of a few thousand people in the world with a rare and possibly fatal bone-marrow disorder, has a house that is a combination sanctuary and prison.<\/p>\n
It\u2019s a sanctuary because that\u2019s where she rests, away from communicable illnesses like the flu and colds.<\/p>\n
It\u2019s a prison because, thanks to a failing immune system and constant pain, she can\u2019t go anywhere else.<\/p>\n
\u201cI want to be done with sickness, to be done with the hospital,\u201d Jayne says, when asked what her greatest wish is. \u201cI just want to be independent.\u201d<\/p>\n
Jayne\u2019s illness is called Severe Chronic Neutropenia Kostmann\u2019s Syndrome.<\/a> What it means is a messed-up bone-marrow system that doesn\u2019t give her the disease-fighting abilities the rest of us take for granted.<\/p>\n Bone marrow has a critical job to do: the specialized cells in the center of our bones are what build our immune systems, generating scores of powerful disease-fighting cells that patrol our bodies through our blood, wiping out foreign invaders, whether they are bacteria, fungi or viruses.<\/p>\n In Jayne\u2019s case, her marrow doesn\u2019t create enough white-blood cells called neutrophils, which fight bacteria.<\/p>\n \u201cWe have (about) 5,000 of those cells,\u201d Joey explains, sitting in the family room, while Jayne watches her, sprawled on the couch in the attitude of a typical 16-year-old.<\/p>\n \u201cFor Jayne, she\u2019s lucky if she has 500 of them.\u201d<\/p>\n Joey knew from the day Jayne was born that she was special. Blood tests taken shortly after birth indicated that Jayne was woefully low on neutrophils. But the newborn wasn\u2019t sick, either, so Joey was allowed to take her home, and the first four months passed largely without incident.<\/p>\n At five months, though, Jayne started getting boils on her hands. And at 9 months, \u201cshe got really sick,\u201d Joey said.<\/p>\n Seattle Children\u2019s Hospital<\/a> did a bone-marrow aspiration, extracting marrow from Jayne\u2019s bones, to evaluate what was going on with her immune system.<\/p>\n The diagnosis came back: Severe Chronic Neutropenia Kostmann\u2019s Syndrome.<\/p>\n \u201cIt\u2019s a super-rare disease,\u201d Joey said, noting that when children do make it to adulthood, the outcome is usually some form of leukemia, a cancer of the blood or bone marrow.<\/p>\n The only cure is a bone-marrow transplant.<\/p>\n Fortunately, at the same time Joey was grappling with the fact her daughter had a dire illness, researchers at the University of Washington were developing a potent drug to treat it. By putting Jayne on a registry for the syndrome, Joey was able to ensure that Jayne would have a free supply of the otherwise expensive drug.<\/p>\n \u201cThat drug has been delivered on our doorstep, free of charge, every month for the last 15 and a half years,\u201d Joey said, noting without that assistance, it would cost about $10,000 per month for the medication.<\/p>\n With the drug forcing her bone marrow to create more neutrophils, Jayne improved. But she was not like other kids. The usual spate of mild illnesses that come from being in a public setting could send her to the emergency room with severe fever and infection. Doctors suggested Jayne be kept at home.<\/p>\n When Jayne was 4, Joey, who had done her homework about the disease, made an appointment at the Fred Hutchinson Cancer Research Center<\/a> in Seattle and asked if it were possible for her daughter to have a bone-marrow transplant.<\/p>\n Hutch officials told her that because Jayne didn\u2019t have a matchable bone-marrow donor, and because she didn\u2019t have a cancer diagnosis, the risks of a transplant outweighed the necessity.<\/p>\n So Joey went home and made a decision: Jayne was going to have as normal a life as possible, while she still had one.<\/p>\n \u201cWe decided to start life, just cautiously,\u201d she said.<\/p>\n That meant Jayne went to daycare, and then to school. All Joey asked was that teachers call her if there was a sick kid in class, so that she could take Jayne home. Jayne thrived in the social setting at school, although she wound up going to the emergency room multiple times, and on scores of antibiotic treatments, when she got sick being around the other kids.<\/p>\n Jayne loved sports, too, especially basketball.<\/p>\n \u201cI played in as many sports as I could, then when I came home it was ice packs and ibuprofen,\u201d Jayne says, noting the aches that would come from the physical exertion and her sensitized bones.<\/p>\n But by seventh grade, Jayne was in too much pain to play sports. She wound up with a hip problem nobody could diagnose, and on crutches.<\/p>\n It was the beginning of a long slide backwards, as her bone marrow began to fail, even with the medication she was getting. The pain only increased in subsequent years, as her condition worsened, and the side effects of the once-helpful medication grew.<\/p>\n \u201cI have very bad bone pain,\u201d says Jayne, noting she\u2019s usually between and 8 and a 10 on a pain scale of 1 to 10. \u201cIt\u2019s the whole body that hurts.\u201d<\/p>\n She hates it, but doesn\u2019t whine.<\/p>\n \u201cThere\u2019s no use in complaining when you can\u2019t stop it,\u201d she says. \u201cI might as well keep my mouth shut and deal with it.\u201d<\/p>\n Jayne left school last fall, due to ongoing issues with the pain. The medications she was given, at various times, left her completely wiped out. She slept constantly, at one point, due to the heavy-duty meds that staved off the terrible ache in her bones.<\/p>\n Today, her life consists of sleeping, laying down, and playing video games \u201cto take my mind off the pain,\u201d she says.<\/p>\n In short, there\u2019s not much quality left, for a teenager who normally would be having slumber parties with friends, and discovering boys for the first time.<\/p>\n But Jayne has a glimmer of hope that she can put all of this behind her, so that she can live in the same world as everyone else.<\/p>\n Last September, her doctors found two units of umbilical-cord blood that could work for Jayne, so that she could receive a stem-cell transplant, using the unformed cells to become specialized bone-marrow cells.<\/p>\n And Joey was able, with Jayne\u2019s doctors, to successfully present a case to Children\u2019s Hospital as to why Jayne should have the stem-cell transplant, even though she doesn\u2019t have cancer.<\/p>\n On July 6, Jayne will be taking her first steps toward that new life. Accompanied by her mom, Jayne will be moving to a special apartment complex in downtown Seattle, set up right near Children\u2019s Hospital. She\u2019ll be getting prepped for what she hopes is her only chance for a normal life.<\/p>\n A new immune system.<\/p>\n It will involve killing off, with chemotherapy, her current failing system. And then, with the stem cells in the umbilical-cord blood, creating a new, normal immune system, that hopefully will remain with her the rest of her life.<\/p>\n If all goes as planned, she will be home by Christmas, and then will need a year to fully recover from the treatment.<\/p>\n There are plenty of risks, though.<\/p>\n Jayne\u2019s body could reject the stem cells. Or the stem cells, in turn, could reject her body, attacking her organs. Because the source is a finite one – two units of cord blood – Jayne could be left with a destroyed immune system, and nothing to restore it, unless doctors can revive it to some degree with some of her failing marrow, extracted before the rest of her marrow is destroyed.<\/p>\n \u201cThere\u2019s a 50 to 70 percent chance she could survive,\u201d Joey said of the procedure, noting doctors have been up front about her daughter\u2019s chances.<\/p>\n \u201cThey looked her straight in the face and said, \u2018you could die.\u2019\u201d<\/p>\n Jayne\u2019s response to them: \u201cIf I die, I die trying to live.\u201d<\/p>\n At home, Jayne is businesslike about her decision to go through with the treatment, even with the risks.<\/p>\n \u201cIt\u2019s better to be moving forward, actually doing something for myself,\u201d she said. \u201cIf this is my only shot, I guess I am going to take it.\u201d<\/p>\n Of her current life, virtually incapacitated from the pain and the medications, she\u2019s pretty much fed up.<\/p>\n \u201cIt\u2019s tiring, annoying and completely not fun.\u201d<\/p>\n She dreams of the life she could have.<\/p>\n \u201cI love my parents, but I\u2019m moving to Florida,\u201d Jayne says of what she wants to do, as soon as she\u2019s well enough to be on her own.<\/p>\n Florida is warm, she says, and she had a wonderful time in her younger years, when she visited on vacation.<\/p>\n She wants to live there for a year, before returning home to Washington. Her hope is to attend the University of Washington, to be an oral surgeon.<\/p>\n \u201cI will achieve it,\u201d she says. \u201cI want to make a good income when I am older, to provide for my wishes. And I will be helping people.\u201d<\/p>\n And if her patients complain to her of pain?<\/p>\n \u201cI will never doubt anyone,\u201d she says.<\/p>\n<\/p>\n YOU CAN HELP<\/p>\n Family and friends have started a fundraising campaign to assist with expenses, including a wig that Jayne Johnson is hoping to have made from her own hair, so that she can wear it when she loses her hair to upcoming chemotherapy.<\/p>\n You can make your contribution at any U.S. Bank to the Jayne Johnson Fund, or www.savejayne.com.<\/a><\/p>\n Visit the Puget Sound Blood Center<\/a> to learn more about donating bone marrow and blood.<\/p>\n