Although he made it to 29 years old with Duchenne muscular dystrophy, Jason Williams was not expected to survive more than another year.
But this week, doctors announced that Williams no longer has an expiration date. Thanks to a groundbreaking surgery that's never been performed on a Duchenne patient in the United States before, there's no telling how many more birthdays -- and Cincinnati Reds seasons -- he'll be around for.
"The most important thing this device has given him is hope, because without it, we know what's going to happen," said heart surgeon Dr. David Morales, who implanted Jason's Left Ventricular Assist Device, or LVAD, on Sept. 5 with a team of doctors at Cincinnati Children's Hospital.
Duchenne muscular dystrophy is an inherited disease characterized by progressive muscle weakness because of a mutated protein gene. It only affects boys, and usually sets in when the child is between 2 and 6 years old, first confining the child to a wheelchair and ultimately weakening his heart and killing him by his late teens or early 20s.
"He's going to be with us longer than we ever anticipated he would be," said Williams's mother, Karen Jones, as she stood next to her son, who wore a Reds T-shirt and Cincinnati Bengals pants during the news conference. Williams gazed out at the crowd from his wheelchair, puffing from a tube to help him breathe. When reporters asked what he was looking forward to, he told them: the Reds.
Williams was diagnosed when he was 7 years old, and has been a patient at Cincinnati Children's Hospital 70 miles away from his home in Peeble, Ohio, ever since.
When his doctors asked him to be the first Duchenne patient to receive a LVAD to help his weakened heart muscle pump blood to the rest of his body long-term, he delved into research before deciding to commit.
"I just thought it might improve my health," he said in a telephone interview the night before the conference. During the conference, he added, "I wanted what would give me more energy."
Adding to that motivation, Williams's younger cousin has the same disease, his mother said. Experimental surgery on Williams could mean progress for his cousin and other younger boys with Duchenne. About 2,500 boys are born with Duchenne every year.
Although LVADs are often temporarily implanted while patients await heart transplants, something Duchenne patients are not eligible for, doctors hope to use LVADs in patients like Williams until stem cell therapy becomes a reality. Doctors will study how the LVAD affects the right side of Williams's weakened heart and how the availability of better blood flow to the rest of his tissue improves or prevents his condition from progressing.
"I hope that doctors and surgeons can learn from my surgery and by my recovery and be able to offer this treatment to other men and boys with Duchenne," he said.
So on Sept. 5, Williams went under the knife for doctors to implant the LVAD, which functions like a left ventricle to take blood out of the heart and pump it to the aorta, Morales said, holding a contraption with one long white tube and one short grey tube up to his chest as he spoke.
But recovery wasn't easy. Less than a week after Williams' surgery, doctors had to operate again and replace his LVAD with another LVAD because of a complication, Jones said. Since then, his recovery has been a struggle but he's now feeling about the same as he felt before the surgery.
Williams and Jones have lived at the hospital for more than a month, but Williams hopes to be home in time for Thanksgiving, Jones said.
"There have been times through this that he's been very frightened, and there have been times that he's said, 'I wish I hadn't done this,'" Jones said.
Williams said it is too soon to tell whether the surgery helped him, but the hope is that he'll have more energy and be able to breathe easier soon.
Although non-Duchenne LVAD patients usually report drastic changes to activity, such as suddenly being able to climb stairs, it will be harder to measure in Williams' case because of his disease. In time, he should start to feel better, though doctors don't quite know what that means yet.
"I'm excited because there are lots of things that Jason would like to do but he's not been able to because of his lack of energy," Jones said. "I am just happy that now I think he's going to be able to do more."