Mastodon ALS Practice Parameter Updates ~ Pallimed

Thursday, December 17, 2009

ALS Practice Parameter Updates

Neurology recently published an update to a 1999 evidence-based review of issues in the management of patients with amyotrophic lateral sclerosis (ALS). The update is divided into two parts with the first focusing on drug, nutritional, and respiratory therapies while the second part focuses on multidisciplinary care, symptom management, and cognitive/behavioral impairment.

Many of the areas reviewed that are relevant to palliative care have insufficient data to fully inform clinical practice (nothing new under the sun for our field) but I'll summarize some of the conclusions.

The pathophysiology of ALS remains refractory to attempts to modify it significantly. Rilozule is the only medication that may slow disease progression, although in the four "Class I" studies (controlled clinical trials), survival is only prolonged 2-3 months on average.

Potentially life-prolonging therapies, therefore, include PEG tubes and non-invasive/invasive ventilation (which, according to the authors, may both be "underutilized.") PEG appears to stablize or improve weight and likely prolongs life, but evidence that it improves quality of life is entirely lacking. Some lower quality studies suggest that if you're going to do it, consider it before the patient's functional vital capacity falls below 50% predicted, because there may be more complications after that point. The effect on QOL is mentioned as an area for future research.

Several lower quality trials suggest that non-invasive ventilation (NIV) can have a positive impact on energy, dyspnea, daytime somnolence, depression, concentration, and fatigue. Patients who had a tracheostomy rated their quality of life to be similar to those who used NIV and the vast majority indicate that they'd choose ventilation again. Interestingly, some evidence suggests that caregivers rate their QOL to be lower than the patients.

Communication is given fleeting attention in the guidelines, with a focus on breaking the news of the diagnosis. In the few studies referenced, patients reported lack of empathy, insufficient explanation of the diagnosis and the course of the illness, and lack of information on where to get help. But "there have been no controlled trials of breaking the news in ALS" and "there is insufficient evidence to support or refute any specific method of disclosing the diagnosis in ALS." A fair conclusion in some ways, but this suggests the method for disclosing ALS may be different than the method employed in a plethora of other settings in which life-changing news is delivered to patients. It isn't, and I wouldn't bat an eye before applying evidence on methods of discussing "bad news" or prognosis in cancer or ICU patients to an ALS population. What is needed, perhaps, is further evidence of what the specific "information needs" of this population are. (For example, the method for determining that an individual is ready to talk about ventilation or PEG is already informed by research in other populations, but when are most patients ready to talk about these topics? What are the top 10 concerns of patients 1 month after diagnosis is disclosed? etc.)

The evidence (or lack of evidence) behind the management for several symptoms is detailed, including sialorrhea, pseudobulbar affect, fatigue, cramps, spasticity, depression, anxiety, insomnia, cognitive impairment, pain, and dyspnea. Also, "no evidence" guides the use of hospice or the "optimal method" for withdrawing ventilation.

The issue of prognostication (and lack of evidence to guide physicians) is not mentioned.

Perhaps by the time the next practice parameter comes along, the role of interdisciplinary palliative care teams in the care of ALS will be better least well enough to receive a more appropriate mention than in the present guidelines. For example, in Figure 2 of the first article (an algorithm detailing respiratory management), the first vague reference to palliative care is at the very end of the algorithm after the patient has "failed" non-invasive ventilation at least twice. Then, you're suppose to either intubate and/or trach 'em OR "refer to hospice for palliative care." That's too late. Palliative care could be introduced well before then, but which patients benefit the most and when? To the authors credit, there is a brief mention of palliative care being provided concurrently with other therapies.

Anything in these guidelines surprise you? As a palliative care practitioner, are you getting timely, appropriate referrals of ALS patients? What about as a hospice provider? What challenges have you faced with this population?

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