The ALS multi-disciplinary clinic
Motor neuron disease is the term neurologists use to describe amyotrophic lateral sclerosis (ALS), but it also refers to other central nervous system disorders like Kennedy’s disease, also known as X-linked spinobulbar atrophy. And when patients receive this diagnosis in the clinic, they may not really get it. But when they hear ALS, or Lou Gehrig’s Disease, their whole world gets turned upside down. A disease characterized by gradual muscle and nerve wasting, with preservation of awareness, and death within 3-5 years, ALS is among the worst news you can get at a neurology appointment.
But there is hope. In the US, hundreds of multi-disciplinary care clinics have been established for the sole purpose of treating patients with ALS. With physical therapy, occupational therapy, nutrition, respiratory therapy and palliative care services, the ALS patient can be reassured that no stone in his or her care won’t go unturned. In this week’s BrainWaves episode, Dr. Lauren Elman describes her experience running a multi-disciplinary ALS clinic.
The first appointment at the ALS multi-disciplinary clinic is probably the hardest. For the patient and the provider. Often patients aren’t even told of their diagnosis, or if they are told, they aren’t aware of what it means. The updated 2009 Practice Parameter put out by the American Academy of Neurology does not make specific recommendations about “breaking the news,” but it’s probably not a bad idea to make sure the patient comes to this appointment with family or friends who would support him or her. Reading the situation is very difficult, and we’ve already talked about Therapeutic Privilege but I might suggest you be completely transparent about the anticipated course of the illness. Goals of care need not be addressed so soon after the diagnosis is made, but a mutual understanding regarding the importance of quality of life should be reached.
After the news is broken, quantity and quality of life become the two greatest priorities. Initiation of riluzole therapy, which is done more frequently in ALS multi-disciplinary clinics than the standard neurology office, is the only neurotherapeutic which may prolong life in ALS–by a median of 3-5 months. This is a relatively straightforward process, so the remainder of the clinic visit and all subsequent visits will focus on symptom management, meaning quality of life. In the episode, Dr. Elman describes her approach to secretion management, tracheostomy placement, timing and need for percutaneous feeding, and the importance of “energy conservation.” While most neurologic diseases benefit from aggressive, and energy-demanding physical therapy sessions, ALS is a disease where PT actually focuses on rest and passive mobilization exercises. High energy activities may only exhaust the patient without allowing for any real strength or stamina training. Motor units are breaking down far more rapidly than muscle mass can possibly build up. So it is important for patients and families to realize that relaxation is extremely important in order to maximize quality of life, and minimize fatigue, for the patient with ALS.
For more interesting tips on how to support your patient with ALS, check out the 2009 Practice Parameter published by the AAN. And for those who like the more relaxing approach, I recommend checking out our episode on BrainWaves.
Miller RG, Jackson CE, Kasarskis EJ, England JD, Forshew D, Johnston W, Kalra S, Katz JS, Mitsumoto H, Rosenfeld J, Shoesmith C, Strong MJ, Woolley SC and Quality Standards Subcommittee of the American Academy of N. Practice parameter update: the care of the patient with amyotrophic lateral sclerosis: multidisciplinary care, symptom management, and cognitive/behavioral impairment (an evidence-based review): report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology. 2009;73:1227-33.
Miller RG, Jackson CE, Kasarskis EJ, England JD, Forshew D, Johnston W, Kalra S, Katz JS, Mitsumoto H, Rosenfeld J, Shoesmith C, Strong MJ, Woolley SC and Quality Standards Subcommittee of the American Academy of N. Practice parameter update: the care of the patient with amyotrophic lateral sclerosis: drug, nutritional, and respiratory therapies (an evidence-based review): report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology. 2009;73:1218-26.