In today’s #apaperaday, Prof. Aartsma-Rus reads and comments on the paper titled: Adult North Star Network (ANSN): Consensus Document for Therapists Working with Adults with Duchenne Muscular Dystrophy (DMD) – Therapy Guidelines
Today’s pick is from the Journal of Neuromuscular Diseases by Narayan et al on consensus for therapy guidelines for adult Duchenne patients generated by experts of the north star network doi 10.3233/jnd-210707.
You may remember another Journal of Neuromuscular Diseases paper on adult care that was published on World Duchenne Awareness Day. The paper published now focuses on physiotherapy, occupational therapy and speech and language therapy but should be seen together with the other paper.
As with that paper I recommend you read the whole paper – I will provide highlights here, but details are important. Context: Duchenne patients live into adulthood now due to improved care. Adults with Duchenne require specialized multidisciplinary care as well.
However, while pediatric care is often well organized, transition to adulthood often means less experienced healthcare professionals and reduced access to services. Also while pediatric care focuses on the family, adult care focuses on the individual with Duchenne.
The consensus document was generated through a reiterative process with experts in adult care from 28 centers in the UK looking after 650 adult Duchenne patients. Authors stress these guidelines are based on UK system – they can be adopted elsewhere depending on resources available
One example of a challenge is that Duchenne patients have a chronic need for physiotherapy (PT), while in the UK for adults PT is given in blocks of 6 weeks. Authors stress also that respiratory PT should focus on maximizing ventilation and airway clearance.
Respiratory tract infections & dysphagia (swallowing problems) are common causes of morbidity & mortality . Patients should be assessed annually by a respiratory PT. Physiotherapy also focuses on managing contractures – stretching should happen daily and be tailored to the patient
Splints can also help prevent contractures. Posture in the wheelchair should be tailored to the individual patient as well to ensure optimal breathing, eating and communication. Likely position during the night should allow optimal sleep.
Mobility influences independence & participation so patients should have an electric wheelchair when being unable to walk less then 10 meters – ideally this is anticipated based on the patient’s trajectory. Note that patients generally will have a wheelchair before reaching adulthood
Exercise is very important. However, patients should not overdo it as that will cause excessive muscle damage due to the muscle pathology. But, inactivity will also mean losing muscle tissue so it is important to exercise regularly.
Occupational therapy aims to optimize engagement with society. This involves both physical help (e.g. arm support) but also psychological help (to cope with losing one function after the other). For some patients also help is needed due to behavioral and cognitive issues.
Engaging in social activities and work (paid or voluntary) is important (I think we all realize how important this is after the pandemic). Patients should be empowered to engage, but as a society we should also help them (e.g. wheelchair access etc).
Dysphagia (swallowing problems) occur in many adult Duchenne patients. It is poorly studied. There are multiple problems: due to swallowing difficulties patients may eat less & become malnourished. Food may also go into respiratory system which is a risk for infections & choking
Furthermore, when mealtimes become very long this may reduce social dinners etc. Multiple small high caloric meals are suggested. Speech is also important for social participation. Due to respiratory problems and weakness, this may be difficult for patients.
Here suggestions like a microphone or meeting in areas with low background noise are suggested. All in all, authors suggest a holistic multidisciplinary approach with regular assessments and tailoring care to the individual patient’s needs.
I commend the authors for publishing this document. I expect that like the pediatric care guidelines this will not be the last version, but that with increased expertise in caring for adults with Duchenne, additional versions will appear in the future.