Men with Duchenne MD find independence within their reach
Recently, the dad of a young boy with Duchenne muscular dystrophy (DMD) was trying to explain to a child psychologist about certain educational choices his son needed to make in order to be well prepared for adulthood.
"She looked at me and asked, 'How long do you think he is going to be with us?' She thought that death by 20 was always the case. She thought that she was well-informed. She was trying to get me to realize that, in talking about preparation for adulthood, I was being completely unrealistic in my expectations."
But it was the psychologist, not the father, who was being unrealistic. Today, many people with Duchenne muscular dystrophy not only are surviving, but thriving into their late 20s and, in some cases, well beyond.
"Boys with Duchenne are living longer and with greater ability than in the past," says Lawrence Z. Stern, director of MDA's Mucio F. Delgado Clinic for Neuromuscular Disorders at Arizona Health Sciences Center in Tucson. (Girls occasionally develop DMD, but their disease course is generally less severe than that of boys.)
When Stern gives a diagnosis of DMD, he tells parents, "Not only are boys living longer, but their quality of life is often excellent. In the last 20 years, their life experience has improved considerably."
This improvement primarily is due to better general care, Stern says, especially pulmonary and cardiac care, as well as overnight assisted ventilation. In some cases, scoliosis surgery also is a factor. And where medicine leaves off, advanced adaptive equipment takes over.
But as is clear from the experience of the father described at the beginning of this story, societal attitudes haven't always kept pace with reality. Low expectations can be just as deadly to the human spirit as low oxygen is to the body.
In the following pages, Quest presents the stories of five men with DMD who refused to lower their expectations, and who have achieved levels of independence unheard of two decades ago. Though DMD still steals their strength, it hasn't touched their freedom to be who they want to be.
| Drew Johnson in his Chrysler.
Absolutely nothing in American culture says "independence" like having your own car. Just ask 18-year-old Drew Johnson — if you can catch up with him between runs to school, the video store, church and dates with friends.
"When it came time that I needed to drive and my older friends were starting to drive, I starting thinking, 'What am I going to do?'" drawls the Zachary, La., high school senior. The answer was a black 2000 Chrysler Town & Country minivan with a lowered floor, and a DigiDrive II computerized gas/brake/steering system that allows Drew — a master Nintendo player — to drive with a joystick. With the flip of a switch, the car can be converted back to standard driving controls, meaning that the family takes Drew's van on trips because everyone can drive.
"When Drew was diagnosed (at 21/2 years old), Teri and I made a commitment that whatever we can provide, we will," says his father, Sam. For Drew's growing up, this meant snow and water skiing trips, mission trips for the church, and two years as MDA National Goodwill Ambassador in 1991 and 1992.
"When he wanted to drive, we were blessed financially that we were able to provide that opportunity, which was very, very expensive," Sam says. The van with modifications cost $88,000, plus 50 hours of special driver training for an additional $4,500.
The family started looking into driving possibilities when Drew was 15, taking him for a driving evaluation at the biomedical research department of Louisiana Tech University in Ruston. Drew, who uses a power wheelchair, has some mobility in both arms and doesn't use any ventilation. His custom driving system allows him to handle the horn, blinkers and air conditioning with his left hand and control the joystick with his right.
As it does for most adults with disabilities, technology plays a big part in Drew's independence. In addition to the van, his power chair "lets me do my own thing — it's fast, reclines and handles bumps really well." A cell phone keeps him connected to his parents, as does instant messaging with friends on the Internet. He can self-transfer thanks to a lift in his bedroom and a water-powered lift in his pool.
At the opposite end of the spectrum, Drew enjoys the centuries-old "technology" of playing the bagpipes.
Drew, who is MDA's 2002 Louisiana Personal Achievement Award recipient, plans to attend college next year, then go on to seminary to become a youth minister. He already travels and speaks at Baptist churches in his area, sometimes to up to 2,000 people at a time. "I think that's why God has blessed me with the van, so I can travel and speak to others," he says.
Like all parents of teens who drive, the Johnsons have had some white-knuckle moments. "Merging on the interstate — wooo-hoo!" Sam exclaims. But they have seen Drew go farther than predicted by his doctors so many years ago.
"He's done far better than we were told he would do. We just take it one day at a time and are thankful for today."
As for Drew, his license plate says it all. It reads simply, "FREDM."
Jon Whitmer is still chuckling about the road trip in 1996, when he and some friends broke down on a desolate stretch of highway between Billings, Mont., and Seattle and were towed by a degenerate Texan in a beat-up Dodge.
"That was a hell of a night, it just sucked," he laughs. "He was just whipping us around, we were up on two wheels. Then he ran out of gas. We were in tears laughing hysterically but we didn't want to laugh around him because we thought he might kill us."
Attention, parents: If the idea of having your vent-dependent, almost completely immobilized son bumming rides from strangers on the highway at night is a bit disturbing, stop reading here. Because it's guaranteed that Jon Whitmer, 31 and still rocking, will never stop going on the road.
"For me, there's God, family, music and the road," says Whitmer in his laid-back, storyteller fashion. "The highway, getting out on the open road, leaving all your troubles behind, being yourself. One week a year, I'm calling the shots, I'm on the road."
The fourth of six boys, Whitmer received his DMD diagnosis at age 6 and was in a wheelchair by 8 (none of his brothers have DMD). Severe respiratory complications in his late teens almost did him in, and he battled to breathe for about a year before finding a compatible mobile ventilator. In addition to having a g-tube for nutrition, Whitmer also uses head controls to drive his power chair and a toe switch to brake it. But this summer he broke both legs when he couldn't reach the switch and rammed into a wall, so now he brakes with his tongue.
"That's a funny little story," he chuckles. "First thing I thought was, there goes my Fourth of July."
Whitmer tried a year of college but it wasn't for him. He prefers to be setting off glorious (but illegal) July Fourth fireworks shows, or camping and fishing with his family, or dragging his nieces and nephews around behind his chair, or keeping his brothers in line when their bands play nightclubs, or being the "designated shopper" for family gift giving, or deejaying local parties to help finance his yearly trips.
Named MDA's 2002 Personal Achievement Award recipient for Montana, Whitmer is active in various civic projects, including a new skate park. His advocacy also helped change state laws regarding personal care attendants, or personal assistants, who are an essential component of his independence. In one instance, Whitmer was involved in changing a law that stipulated that only medications, not food, could be given by attendants through a feeding tube.
In another case, he helped change regulations that prevented state-funded attendants from traveling out of state with their clients. "I said, 'Am I on probation because I'm handicapped? Does my disease end at the state line? How am I supposed to go buy illegal fireworks in Wyoming if there's no one to drive me?'"
Whitmer, who lives with his parents in Billings, also looks after the needs of others. When the bride-to-be of one of his attendants developed cancer, Whitmer organized a benefit concert that brought in $1,200 to help with her expenses. "It's not a whole lot, but we did it," he says. "It's like the Lynnrd Skynnrd song that says, 'If you think you got it bad, take a look around.' There's always someone worse off than you. That's how I've gotten as far as I've gotten in my life."
Last year, about 150 people celebrated Whitmer's 30th birthday and contributed to his next road trip to Seattle to see a Mariners game. "I believe God gives me some of these things to keep me going," he says. "He gave me Lynnrd Skynnrd to keep me going. He gave me Mariners baseball. I got a whole new lease on life after I discovered the Mariners."
His next projects? To exploit his shopping skills by advertising for jobs as a personal shopper, and to write lyrics for a melody his brother composed especially for him.
Whitmer had no doubts that he would make it to age 30 and expects to see 40. "I don't plan on going anywhere. I just ordered three more years of Rolling Stone magazine." He invites anyone who wants to talk to e-mail him at the firstname.lastname@example.org.
When Ryan Tyrrell was about 8 years old, his family moved to Orange County, Calif. Because he had DMD, the new school system put him in a self-contained school for children with cognitive disabilities. Looking back now, his mother thinks it may have been a blessing.
"He hated it. Hated it," says Terry Fontenrose of Long Beach. "The only way they would let him into a regular school was if they put him back a grade. I think that really started Ryan's quest for independence. When he had to go back a grade just because he was disabled and not because of his mental abilities, he became more interested in being what he calls 'able-bodied.'"
Now 26, Tyrrell is very much an independent soul. A graduate of the University of California at Berkeley with a degree in sociology, he lives in his own home, hires and pays his own attendants, and is actively searching for employment.
"It's difficult, but not as difficult as it would seem," says Tyrrell, who isn't vented and has the use of one finger on his right hand. "I've always been really independent. When I was living with my mom (a single parent), I came home and stayed alone until Mom got home at 6 p.m. I got used to being on my own and doing things for myself."
"Being on my own" means living in an accessible mobile home in Oxnard, Calif., which was purchased for him by his parents. Tyrrell pays for his rent, utilities, food and attendant care using income from Social Security and California In-Home Supportive Services. His parents help out with emergency expenses, like repairs to his van.
Although he admits that living on his own sometimes can be "a little boring," Tyrrell prefers it. "I didn't really have a problem with living at home," he explains. "But you feel more independent and have more privacy when it's just you, with your attendant."
California's state program pays for Tyrrell to have about 200 hours of attendant care a month, although it pays minimum wage, while attendants charge about $9 an hour. He has two live-in attendants for evenings and overnight (who also get free rent), and a morning and an afternoon attendant during the day.
For about three hours a day he's alone, but it doesn't bother him "as long as I know I have people coming, and as long as I'm up in the chair, not in bed." His home is set up with everything at the right height to facilitate independence, and he's learned little tricks like raising his arm up onto a table by maneuvering his power chair. His biggest problems arise when an attendant gets sick or gets another job.
Finding "good-quality attendants who will do the job thoroughly and not try to get through it as fast as they can" is a skill Tyrrell learned his first year in college. UC Berkeley teaches students with disabilities how to find, hire, train, pay and fire attendants.
"It was hard his freshman year because he was used to me knowing everything about him," Fontenrose says. "By the end of his first year he called and said, 'Mom, I got my own apartment with three other disabled guys, and I'm staying for summer school.'"
After graduation, Tyrrell interned for a summer with the U.S. Navy, editing technical manuals, and now is searching for an in-home, part-time secretarial job. His social life is mainly online.
Terry Fontenrose grew up with a brother with DMD, who died of pneumonia at age 15. She always expected that her son would have a better life than her brother, "but I had no idea he would graduate from college and do all these things. As each thing happens, I just think 'oh good! oh good!'" she laughs.
"I think Ryan feels in his mind that he is pretty normal," his mother says. "That's part of his strength. Everything is in the mind — nothing is really in the body. The mind is really what you have to work with."
|Tom Downs (center) with his wife Cheryl and their son, Joshua
Tom Downs wasn't looking for love, just a trip to a fund raiser for a friend with leukemia. But when a group of people met at a Milwaukee Park 'N' Ride lot to carpool to the event, Cheryl Lee just happened to get into Tom's van. "And the rest is history," he says with obvious enjoyment.
Tom, 40, and Cheryl have been together for eight years and have a 6-year-old son, Joshua. Their relationship was "blessed" by their pastor on Oct. 15, 1994.
Settled into Cudahy, Wis., they lead busy lives. Cheryl manufactures her own line of children's clothing called Little Traditions. Tom, who elected to have a tracheostomy in 1999, tutors fourth-graders and coordinates With a Little Help Inc., which provides camping and recreational activities for adults with muscular dystrophy. He also serves on several state committees, and is very involved with raising his son.
If it all sounds incredibly normal, it may be because Tom's family has had a lot of experience with DMD, going back five generations. Setting and accomplishing goals in spite of limitations is kind of a family tradition.
"If I wanted to do it for myself, my family wouldn't butt in and try to help me," Tom says. "They weren't overprotective. That is one of my frustrations — how sheltered some kids are. They should go do what they want and live a normal life as best they can. Then a lot of good things will happen."
That was the attitude Tom's parents took even before he was born. Says his mother, Carole, "I had a brother and an uncle with Duchenne who died when they were 141/2. I knew I didn't want my child to grow up like my brother did. He stayed home most of the time and my mother was very protective of him." Carole and her husband, LaVerne (who died in December 1986), resolved that if they had a child with DMD, "We weren't going to hold them back. If there were things they wanted to try, they could try."
This meant not rushing in when Tom would fall, not forcing him to use a wheelchair despite the frequent stitches, not letting him give in to occasional bouts of anger or bitterness. And when he announced as a young adult that he was moving out to live with a friend/attendant in another town, it meant remembering their promise and letting go.
"I've been blessed that my health was really good and I've done a lot of stuff for myself all these years that's helped me achieve my independence," Tom says. "My mom has been really supportive." With the help of attendants, family and determination, Tom ran his own disc jockey company, Downs Sounds, for 10 years and earned a degree in human services from a technical college.
Despite her belief in her son's abilities, Carole never imagined that he would one day make her a grandmother. "You know — he didn't either!" she laughs. When Tom and Cheryl's relationship became serious, she counseled Cheryl not to be Tom's sole caregiver.
"I told them it would be too hard on her. They had to make sure there were people that could help him, and not just Cheryl doing it all. They've done just wonderfully," his mom says.
Someday Tom and Cheryl plan to start their own business helping others with disabilities gain their independence. "It would be like a group home where people could come and be away from family and start a new life," Tom explains. Until then, he will continue enjoying his own hard-won independence, tutoring, fishing, being with his son and rooting for his favorite teams.
"My advice to young men with DMD is 'set goals for yourselves.' Keep fighting to accomplish those goals. There are still ways to get it done.
It's 1998, and it's dark and quiet in the forest in the middle of the night. Brian Butler sits alone in his power wheelchair, tending a fire. In order to become a Vigil member of the Order of the Arrow (a Boy Scout honor society, with Vigil being the highest rank), Butler must remain by himself, without talking, and keep the fire going all night.
Because the 17-year-old has only limited mobility in his arms, a washtub of wood has been outfitted with a ramp, so he can use a small rake to push a log into the fire. It takes some maneuvering, but he works through it with typical doggedness. Come dawn, the flames are still crackling.
"To me, it was OK. I didn't have no problem with it," Butler, 21, says in his laconic style, looking back on his experience. In fact, the words "no problem" could be Butler's motto, or maybe "no stopping" or "Watch out, here I come."
"Whatever stopped me, I would try to get around it in some way," he says in explaining how he was able to become one of the elite 4 percent of Boy Scouts who achieve the rank of Eagle Scout. To get there, Scouts have to fulfill requirements in the areas of leadership, service and outdoor skills. "I could do almost everything anyone else could do," Butler says. "If there was a merit badge I couldn't take, they would replace it with one that was equal to it."
In 1999, Butler was one of 10 Boy Scouts with special needs chosen to represent the United States in an international Special Needs Jamboree in Japan. His two-week visit presented lots of accessibility challenges and a few culinary ones. "It was pretty neat but I didn't like the food much."
The determination and adaptability (and ability to eat strange foods) that was nurtured in Scouts now is being applied toward his career. A junior at the University of Houston at Clear Lake, Texas, Butler is studying communications.
He is the "technical person" for all manner of presentations, and also creates videos of MDA summer camp for his local office. "I like having the control," he says with relish.
Butler recently quit his part-time job with a check-processing firm to concentrate more on his studies. He still lives at home, but is independent on campus, which he attends twice a week. His goal is to start his own advertising or video production business. "I like to be my own boss, and to do the things I want to do when I want to do them."
Butler is the first — and currently the only — Boy Scout Eagle Commissioner who helps other Scouts with special needs become Eagle Scouts. His advice to Scouts and others with DMD is the same as the creed by which he lives: "Try to do everything you can. Live life the way you want to."