Columbus, OH - March 2016
Before the creation of the Muscular Dystrophy Clinic at Nationwide Children’s Hospital in 2012, patients received the care they needed. They had access to world-class physicians known for their research into neuromuscular disorders. Respiratory therapists performed spirometry at appropriate times, and cough assist devices were introduced when required.
What patients did not normally get, though, was all necessary care coordinated in a single day.
Services for a typical muscular dystrophy patient often include Pulmonary Medicine, Neurology and Cardiology, plus an occupational therapist, a physical therapist, a nutritionist, a genetic counselor, a wheelchair specialist, a social worker and more. This meant that a patient would have many appointments over the course of several days, an especially difficult burden for families travelling from far away to Nationwide Children’s.
A specific clinic that brought all of those care providers together at one place and one time could help lift that burden. So the Nationwide Children’s Muscular Dystrophy Clinic was born.
“Most importantly, we are trying to make life easier for patients and their families who deal with chronic disease,” says Richard Shell, MD, a pulmonologist at Nationwide Children’s who cares for patients at the clinic. “There is also great benefit to having all of the care providers essentially in the same room. Now, if I see a patient for respiratory concerns, but I think it’s primarily a heart issue, I have the cardiologist nearby who can do an echocardiogram and assess the patient right there.”
In 2014, the clinic was the first to be named a Certified Duchenne Care Center by Parent Project Muscular Dystrophy, the leading advocacy organization in the United States dedicated to ending Duchenne muscular dystrophy. In addition, the clinic treats patients with all forms of neuromuscular disease who could benefit from multidisciplinary care.
The clinic is held Wednesday mornings. About 20 patients are scheduled per day. Most visit every six months or more often if needed. A comprehensive visit at the Muscular Dystrophy Clinic can make for a long day, so some entertainment is provided, and parents know to bring toys and snacks.
“They have all of this interaction at one time, not ten different times,” says Kavitha Kotha, MD, a Nationwide Children’s pulmonologist and member of the Muscular Dystrophy Clinic team. “Instead of getting a snapshot of information at each different specialty appointment, you can incorporate all the recommendations and present a unified care plan for the family.”
The clinic’s pulmonologists, a group which also includes Grace Paul, MD, tend to see a muscular dystrophy patient for the first time at about 4 years old, Dr. Kotha says. Pulmonary function is generally still good at that age, but education begins then regarding respiratory signs and symptoms to watch for, such as colds lasting longer than usual, poor anesthesia recovery, snoring, morning headaches and sleepiness. Patients receive lung function testing, or spirometry, on a biyearly basis as an objective measure of lung capacity.
Over time, as a patient begins to need a wheelchair, respiratory management becomes more necessary. Patients often develop hypoventilation, especially during sleep, and a bilevel positive airway pressure machine (BiPAP) is introduced to alleviate the disrupted sleep. Muscle weakness can lead to impaired cough mechanisms, so early in the disease process, a cough assist device is introduced to mechanically stimulate a cough and aide in mucous clearance.
While this is occurring on the pulmonary medicine end, cardiologists and neurologists are managing other parts of the disease process. Orthopedic surgery may be recommended for scoliosis, and dysphagia and other issues may lead to gastrointestinal interventions.
Providers at the clinic also work on end-of-life issues as a group. Social workers are crucial in this area, as many muscular dystrophy patients become adults but remain at home with their parents, Dr. Kotha says. Figuring out advance directives and power of attorney while patients can still explicitly state their desires is an important goal.
The Muscular Dystrophy Clinic will continue to evolve as the standard of care does, Dr. Shell says. But patients and physicians alike have embraced the comprehensive clinic approach.