John Brandsema, MD, discusses the importance of multidisciplinary care for patients with spinal muscular atrophy, including neurologists, primary care providers, physical therapists, and social workers.
Crystal Proud, MD; John Brandsema, MD; Julie Parsons, MD; Nancy L. Kuntz, MD
PUBLISHED June 10, 2020
Current Series: Advances in the Treatment of Spinal Muscular Atrophy
Crystal Proud, MD: John, we've now heard about the pulmonary and nutritional care needs for our patients with SMA [spinal muscular atrophy]. Can you review the role of therapies like physical therapy and occupational therapy for our patients?
John Brandsema, MD: The key here is that it really does take a village to care for an individual with SMA, and we want to make sure that we are respectful of their care context because this isn’t universal depending on where somebody lives with SMA in terms of access to all of these different options. The primary care provider plays an essential role in terms of things like vaccination for things like pneumococcus, maybe RSV [respiratory syncytial virus] prophylaxis when they're younger, and the annual flu vaccine.
They are often the first person that patients access if they have concerns about new symptoms, so they need to be abreast of any care considerations. In most places, a neurologist is the care home, leading a team of people in the clinic to try to optimize function for people with SMA. This is where our rehabilitation colleagues are so essential because weakness is universal in SMA.
We really need to optimize day-to-day function as much as possible in the context of that weakness and do appropriate therapeutic interventions, whether it be bracing, stretching, or different therapeutic exercises to both maintain function and avoid development of things like contractures, which can end up being limiting over time, especially if somebody is gaining strength on some of these new treatments that we have.
It's also common in our patients with more severe forms of SMA to develop scoliosis of the spine and develop contractures that need intervention in various joints, whether it be hips or ankles. This is where our orthopedic colleagues can often be very helpful if the scoliosis is progressing by helping with stabilizing the spine either through bracing or through surgical intervention.
In terms of the rehabilitation management, that's one aspect of an interdisciplinary team. Pulmonary and GI [gastroenterology] would be others, but there are lots of other folks who we end up involving in the care as well.
We haven't explicitly said it yet, but for the vast majority of patients with SMA, cognition is not affected whatsoever, but the psychosocial aspects of living with this disorder are really important to keep in mind. This is where our social workers and sometimes even behavioral health intervention is needed at various stages, whether it be for the person living with the disorder themselves as they go through stressful transitions in their life or are trying to cope with other stressors that come up in the context of living with weakness. There are also caregivers and loved ones and those in the community who interact with them who may also be affected by different aspects of living with a disorder like SMA.
This part of the care is very important to keep addressing and following up with patients. Be very open in addressing it because it can lead to significant quality-of-life issues if it's not addressed appropriately.
Crystal Proud, MD: I think that your comment that it takes a village is incredibly true, and there are multiple members of our team who make it possible for our patients to survive and thrive and develop, especially in an era where we now have the opportunity to treat.