by Lorine Parks
“An old drug gets a new price: $89,000 every month, to combat DMD.”
A outrageous price tag like this is just one of the challenges Matt Kinley faces, in trying to help his son Ryan get as much out of life as possible after being stricken with Duchenne’s Muscular Dystrophy. It is a disease that does not get any better. And only the symptoms can be treated, until a cure is found.
Matt, a past president of our Rotary Club of Downey, discovered 19 years ago that 2 year old Ryan had Duchenne’s Muscular Distrophy, a rare but severe genetic disorder which strikes one in 3,500 boys. Right from the start, Matt decided to be open and frank about Ryan’s condition. If educating people can lead to finding a cure, he wanted to take that chance. And he wanted to destigmatize DMD, so people would treat Ryan just like any other kid.
Matt has faced Ryan’s awesome diagnosis with him, and recalls their odyssey, “A Superior Life’s Journey.” The upside of this story is the love and support Matt gives Ryan, a happy handsome young man, every single day, as the story of their 2015 Haunted House party will show.
Genetics-wise, DMD means a faulty recessive gene on the X chromosome, which causes muscle cells to be flawed and easily damaged. The body doesn’t produce dystrophin, a protein that helps protect muscles.
Without it, muscle fibers start to break down and are replaced by fibrous and fatty tissue, causing the muscle to weaken. Structurally, muscles are the body’s cushion and the glue for the skeleton.
The diagnosis is made in early childhood, by observing generalized weakness, late walking, being a toe walker, enlarged calves. Because of muscle weakness, a tell-tale clinical characteristic for Duchenne muscular dystrophy is Gowers' sign. Children with Duchenne muscular dystrophy find it very hard to get up from a sitting or lying position on the floor. They first pull up to their hands and knees. The child walks his or her hands up their legs to brace themselves as they rise to a standing position.
Only women can be the carriers of the faulty X chromosome, but women rarely get the disease.
Treatment is simple, but it does not make the patients get better. Patients can be made more comfortable and symptoms relieved, though, and the progress of this neurological condition can be slowed. Managing the symptoms includes stretching exercises; possibly scoliosis surgery; steroids, although that can have side effects such as immune system repression, Cushing syndrome appearance and cataracts.
Until recently life expectancy ended in the teens, but now some young men can hope to go to college, get married and have children. So far, Ryan, 21, has graduated from Wilson High School in Long Beach, and is currently a student at Long Beach City College.
Matt interspersed his slide presentation with quotations from many philosophers, such as Bugs Bunny, but also one from novelist C. S. Lewis. This one begins stoically, “What do people mean when they say, 'I am not afraid of God because I know He is good'? Have they never even been to a dentist? “It doesn't really matter whether you grip the arms of the dentist's chair or let your hands lie in your lap. The drill drills on.”
If the name is familiar, it’s because Lewis, a British novelist and lay theologian, is best known for having written the fantasy trilogy, Chronicles of Narnia, especially the first volume which was made into the popular movie The Lion, the Witch and the Wardrobe.
Most people became aware of this neurological disease back in 1966 with Jerry Lewis’s Labor Day telethons, which raised money and awareness for “Jerry’s kids.” Johnny Carson’s sidekick Ed McMahon, was a longtime co-host of Jerry Lewis' annual muscular dystrophy telethon.
Matt is active in Parent Project, a DMD organization that is leading the fight to end the Duchenne-type dystrophy. Their mission can be summed up as, “Everything we do—and everything we have done since our founding in 1994—helps boys with Duchenne live longer, stronger lives. We will not rest until every young man has a treatment to end Duchenne.”
Matt showed us pictures of Ryan. “He’s a great kid,” Matt said, and there was Ryan as a healthy appearing young child, then again, always smiling but in a wheelchair, which he has had to use since he was 11. In it, he and Matt join in the annual Belmont Shores Christmas Parade.
Ryan is 21 now, and Matt spends a lot of quality time with him. They have taken cruises: “Cruise ships are very accommodating to handicapped passengers,” Matt said. Spiderman movies are another favorite pastime.
Ryan wanted to have his scalp shaved with a swath left in a Mohawk cut, and pictures show him sporting his signature style, dyed orange red, and at other times a bright teal blue. The latest blond six inch upright locks of hair, teased and gelled and sprayed till they don’t dare bend, makes Ryan look like a cheerful golden cockatiel with a moustache. He and Matt love it.
Matt and Ryan’s adventures were chronicled in a 2015 Press Telegram feature story, “Long Beach Teen Gets Own Haunted House For Hallowe’en.” With an appropriately macabre sense of humor, Ryan likes to dress like a clown in a black and white skeleton costume and a grinning mask with sharp teeth.
Matt rented a vacated storefront below Pier 1 at the Marina Pacific Mall and, with family and friends, did an extensive decorating job to turn it into a haunted house. 800 guests visited the vacant storefront decorated inside as a haunted house. Matt was dressed as a giant rabbit with blood splattered on his furry chest.
“Ryan loves Halloween and all the characters. It’s his thing,” says Matt
Matt closed by reflecting on Parent Project. For example, the Muscular Dystrophy CARE Act Amendments of 2014 address issues “to ensure the law continues driving toward improved outcomes for all Americans impacted by the muscular dystrophies.” It works toward achieving the scientific discoveries and breakthroughs that are necessary to develop treatments and therapies.
The MD CARE Act mandated that the National Institutes of Health (NIH) would support Centers of Excellence focused on muscular dystrophy, “exemplifying what can be achieved through genuine public-private partnerships to transform the biomedical research and drug discovery landscape.”
How does such a genetic disease really affect a community? Matt and Ryan’s
positive attitude sees a lasting effect. Matt let C.S. Lewis have the last word, summing up how father and son approach each day: “Suffering is not good in itself. What is good in a painful experience, to the sufferer, is to submit to the will of God. To the spectator, there is the opportunity for compassion and acts of mercy.”