Blogs to Boards: Question 16

Mr. J is 58 year old diagnosed with ALS 6 months ago.  He is referred to your clinic by his primary care doctor to help discuss options to treat a progressive weight loss.  He currently lives alone in an apartment, is independent of ADLs although he has been having difficulty feeding himself due to proximal arm weakness. He complains that he occasionally bursts out crying or laughing, but denies feeling depressed. His forced vital capacity (FVC) has remained at 70% for the last 3 months.

The best next step to help treat his progressive weight loss?

  1. Riluzole
  2. PEG Placement
  3. Mobile arm supports and modified cutlery
  4. Non Invasive Positive Pressure Ventilation (NIPPV)

Discussion: Correct answer is (c)

  1. Riluzole is the only available disease-modifying therapy for ALS.  Based on clinical trials, riluzole likely prolongs median survival in patients with ALS by 2-3 months compared to patients taking placebo.(1)  It does little to improve functional outcomes or bulbar symptoms.  There is no evidence to suggest that it is beneficial for weight loss.
  2. PEG placement should be discussed with any individual diagnosed with ALS, although attempt to reverse other common reasons for weight loss is warranted before PEG placement.  Ideally, PEG tubes should be placed before FVC falls below 50%. While PEG may be indicated for this patient in the future, currently C is the best option.
  3. Individuals living with ALS may have difficulty with the mechanics of both cooking and putting food from the plate to the mouth. This may often contribute to weight loss, especially for those individuals living alone.  Occupational therapy may help maintain adequate nutrition by supplying devices such as mobile arm supports and modified cutlery.
  4. NIPPV confers a survival benefit and improves quality of life in patients with normal or moderately impaired bulbar function, although it does not improve weight

References:

  • Miller RG, Mitchell JD, Lyon M, Moore DH. Riluzole for amyotrophic lateral sclerosis (ALS)/motor neuron disease (MND). Cochrane Database Syst Rev. 2007 Jan 24;(1):CD001447.
  • Mitsumoto H, Rabkin, JG.  Palliative Care for Patients With Amyotrophic Lateral Sclerosis “Prepare for the Worst and Hope for the Best”. JAMA. 2007;298(2):207-216