Carlie Brinker of Millersburg, Ohio, tries to walk to the mailbox and back a couple of times a day and to her sister’s house, half a mile away, if the weather is good.
It’s not easy, because calcifications (mineral deposits) and contracted joints from her autoimmune disease, dermatomyositis (DM), impair her mobility, as does her weight. She’s now carrying more than 200 pounds on a 5-foot-1-inch frame, because of the prednisone she’s taken for some 11 years to treat her disease.
Brinker, 19, lives on a family farm with her mother, Wanda, and her father, Russell. During the day, she reads, makes scrapbooks, follows stock car racing and takes photographs. She’s preparing to take the GED exam to get her high school equivalency diploma, not having attended school since the sixth grade, when health problems and anxiety caused her to switch to home schooling.
When she’s feeling up to it, she participates in 4H Council, a youth group with an emphasis on agriculture, attends car races, and has even been elected Early American Days queen at a local community event.
Trying something new
Recently, she’s been going to Pittsburgh, three hours away by car, to participate in a clinical trial that may, she hopes, change her life. The trial is testing rituximab (Rituxan), a powerful immune system suppressant that’s been used to treat lupus and rheumatoid arthritis.
“I don’t know if I’m on rituximab or the placebo,” Brinker says. “And I won’t know until the study is done.”
Meanwhile, the doctors in Pittsburgh have been reducing her prednisone dosage by half a milligram every month. She’s now on 12.5 milligrams a day, along with several other medications, such as methotrexate (Rheumatrex), hydroxychloroquine sulfate (Plaquenil) and aspirin, to treat her DM.
At age 8, when she started losing the ability to dress herself or ride her bike, her alarmed pediatrician referred the family to Akron (Ohio) Children’s Hospital.
“They ran MRIs, EKGs, X-rays and all that, and put me on 60 milligrams a day of prednisone and left me for a year,” Carlie recalls. “My blood pressure went sky high, and I started gaining double the weight that an 8-year-old is supposed to gain.”
Then, when Carlie was 9, Wanda Brinker encountered an MDA Lock-Up event at a department store.
“She talked to the lady in charge. She said she needed to hurry up and go home and get me. We went in, Mom said the lady was going to help me, and I just started crying.”
An MDA staff member at the Lock-Up referred them to the MDA clinic at MetroHealth Medical Center in Cleveland, where they would meet Irwin Jacobs, neuromuscular disease specialist and clinic co-director.
On his recommendation, she also went the following week to Cleveland Clinic Children’s Hospital, which has an extensive pediatric rheumatology (inflammatory joint disease) department.
“They did a whole bunch of tests, and the doctor said, ‘I know what you have.’ And at that time,” Brinker says, “I knew something terrible was the matter and that I was going to have a challenging life.”
Brinker still goes to the MDA clinic at MetroHealth, where she sees Jacobs.
Living with side effects
Although her prednisone dose has been drastically reduced from its original 60 milligrams a day, many of its effects remain with her; most notably, obesity, stomach ulcers, frequent respiratory infections, sleep problems and psychological distress.
“It causes you to eat all the time,” she says. “They want me to keep as physical as I can, but they also want me to use caution and not do too much. If I do too much and agitate the calcifications, they can get infected.
“They say eat small portions and drink lots of water, and I do that. I’m not a sweet eater. I used to eat a lot, but my doctor in Pittsburgh gave me a really good heart-to-heart talk and said that if I were to eat a whole bunch I wouldn’t be able to move, and he didn’t want that to happen. So I’ve quit eating a lot.
“I have a very low immune system, so I can’t be around anybody that has a cold or any pneumonia or I’ll get it right away. I get a lot of infections, like bronchitis every winter.”
She takes an antibiotic three times a day to ward off such illnesses. She also takes medication to lower her blood pressure, offset bone loss, decrease stomach acid and help her sleep.
“My Mom is a big role model in my life who just tells me that it’s going to be OK and that she’ll be with me to get through all this.” Most of the time, she considers her mother “just a really big supporter,” but, she says, “there’ll be other times when I’m biting her head off, from the prednisone. On prednisone, I can be happy one minute and sad and crying the next minute.”
Brinker started seeing a psychotherapist two years ago, and that’s been helpful, as has Ativan, a prescription tranquilizer.
“I can’t say,” she replies in answer to how long she’ll be on prednisone. “I was off it once before, and I got totally weak and was in a wheelchair, so they hurried up and put me back on. If I go completely off it, the disease gets really active, my muscles get weaker, and I’m not able to do anything for myself.”
She’s pinning some hope on the rituximab trial, but for now, she takes things one day at a time.
“You have to live each day to the fullest that you can and not get all upset, because there’ll be better days ahead. I have more good days than bad.”
|Taylor Fetrow with his two sisters|
Holding steady at 13
Ten years ago, Taylor Fetrow was, to casual observers, a normal, active 3-year-old, says his mother, Stephanie. The difference between his motor skills and those of other boys his age were almost imperceptible.
“If you put him side by side with one of his peers and said, ‘1,2,3, run,’ you could see it then,” his mother says. “If you said, ‘Stand on the side of the pool and jump off,’ you could see it then. Those were about the only ways.”
But the Fetrows, who sell real estate in the Dallas area, where Taylor’s father, Chris, also teaches fourth grade, were well aware that all was not well with their son. A year and a half earlier, a blood test had revealed that the level of his creatine kinase — an enzyme that leaks out of damaged muscle cells — was “through the roof,” Stephanie recalls.
“We got hooked up with the MDA office and they got us in to see Dr. Iannaccone through Scottish Rite,” Stephanie recalls. (Susan Iannaccone, now director of the MDA clinic at Children’s Medical Center of Dallas, was then directing the clinic at Texas Scottish Rite Hospital for Children.) At 3, Taylor was found to have Duchenne muscular dystrophy.
The next few years were unremarkable. Other than being very cautious in his play (“he wasn’t one who liked to go and ride bikes or climb trees”), Taylor continued on a fairly normal path. He climbed stairs, but not as fast as most children could. He could swim, but couldn’t jump into the pool.
Then, in 2003, when he was 7 years old and in third grade, he started having difficulty walking long distances. His parents got him a manual wheelchair to use on special occasions.
“We wanted him to conserve energy for when we would get to where we were going,” Stephanie says. He soon started using it at school to go from his classroom to the cafeteria and playground.
A flabbergasting change
“At that point I was starting to get a little worried that we were starting to see the disease, finally,” Stephanie says. “Because up to that point, we hadn’t. But when he started having some issues with his energy, more than his ability, we talked to Dr. Iannaccone about steroids. She had talked about them a number of times, and we had said ‘No, the side effects scare us.’
“But we’d been hearing things about weekend dosing or days on, days off dosing. The reports had been favorable, and the side effects had been minimal.”
Taylor started on 40 milligrams a day of prednisone on Fridays, Saturdays and Sundays only.
“Immediately, we saw an increase in his energy,” Stephanie says. “Then about six months into the steroids, we realized that we had seen a big increase in ability and strength and stamina. He got to the point where he didn’t want the chair around at all and didn’t request it.
“He wasn’t tired after a day of school. He was doing more walking, more running. Since it was coming up on summertime, we started doing more swimming -- and he was able to jump off the side of the pool.
“It just amazed us what he was doing. Really, instead of just stopping the progression, he really gained back some strength. We were flabbergasted. Dr. Iannaccone was too. She was kind of in awe of what it had done for him,” Fetrow says.
“When he started fourth grade at age 8, we took the wheelchair to school for transport, and he looked at me and said, ‘Mom, there’s no way I’m getting in that chair.’ So we put it in the closet at school, left it there and took it home after two weeks. It’s stayed in our closet in the garage since then.”
Now 13, Taylor seems to be holding his own, without any change in his prednisone dose or schedule. He has, however, gained some weight, and he hasn’t grown as fast as his peers.
“He was a string bean when we started steroids,” his mother says. “He was probably close to 20 pounds underweight, which was good. He’s put on some pounds, but he has slowly put them on. He’s about 4 feet 10 inches, and he weighs 119, so he kind of hovers around the overweight level.”
His behavior changed with the steroids, but it’s been hard to say exactly what factors were at work.
“We had this easygoing, quiet, meek, little, sweet angelic child who all of a sudden became a typical boy,” Stephanie says. “I think he kind of came into his own. We dealt with a little bit of that in third grade, and then in fourth grade, because I think he felt empowered.
“There wasn’t anything real severe. He did have some outbursts where he’d get really mad, whereas before he didn’t know how to express that anger. He would hit the wall, or he’d punch the table.”
The family spent some time with the school counselor, who told Taylor, “I’m going to teach you some ways to safely let your anger out.” The best thing she taught him was, “If you’re mad and you’re at home, go to your bed and punch the pillow, because you can’t hurt your pillow and your pillow can’t hurt you. It’s OK to be mad.”
Schoolwork has never been easy for Taylor, and his parents elected home schooling for sixth and part of seventh grade. “He needed some maturity and he needed some focus on reading,” Stephanie says, noting that he also struggles with math.
“We feel like we accomplished a lot in a year. When we put him back in school, they started him in a resource class; but after the holidays, they moved him out to mainstream classes, because he was doing so well.”
Today, Taylor is for the most part maintaining his strength. “He’s slightly weaker than he was at 8, but not considerably,” his mother says. She and Taylor’s doctors are keeping a close watch on Taylor’s spine, which is beginning to develop a small curve.
His heart and lung function have been excellent, he continues walking, and his blood pressure and blood sugar are normal. “He’s fully self-sufficient,” Stephanie says. “Dresses himself. Makes his own lunch. Makes his own breakfast.”
So far, the wheelchair has remained in the garage closet, which, Stephanie says, “is an awesome thing.”