Unless you’ve been living under a rock these past few months, you’ve likely heard and seen the Ice Bucket Challenge (for my favorite click here: Ice Bucket Challenge with glacier water) and perhaps even doused yourself with some refreshing ice cold water to support the cause. This viral social media challenge has made Amyotrophic Lateral Sclerosis (ALS) a household name (and significantly reduced the number of puzzled blank stares I receive when telling people what I do for a living!). ALS is a fatal, neuromuscular disease causing rapid degeneration of the motor neurons in the spinal cord, brainstem and corticobulbar tracts. Typical age of disease onset is 55 years and average survival ranges from two to five years and is largely dependent on disease onset type . Thirty percent of people with ALS (PALS) present with speech and/or swallowing impairments (bulbar-onset type) and 85% of PALS eventually demonstrate dysphagia [2, 3]. Typical dysphagic symptoms reported by PALS include: increased mealtimes and associated fatigue with eating; weight loss; choking; food getting stuck in the throat; difficulties swallowing saliva; and frequent respiratory infections.
Role of the SLP
The Speech-Language Pathologist (SLP) plays a crucial role in the management of communication and swallowing function in PALS. Timely and proactive education and counseling on communication, voice banking options, nutritional requirements, safe swallowing and feeding tube placement are of the upmost importance to the SLP working with PALS. Such an approach affords PALS the best likelihood of maintaining effective communication, ensures the ability to ‘bank’ ones voice (prior to degradation of speech function when this is no longer a viable option) and facilitates optimal nutrition thereby decreasing the likelihood of malnutrition. It is equally important for SLPs to counsel and educate PALS whose disease involvement is limited to the limbs, as the majority will eventually experience deteriorating speech and swallowing function, and because all PALS are at high risk for malnutrition regardless of swallowing function. The purpose of this article is to highlight the importance of nutrition and of the significant impact early and timely feeding tube placement can have in this unique and challenging patient population.
PALS AND MALNUTRITION – THE PERFECT STORM
PALS are highly susceptible to malnutrition due to two confounding factors:
- Hypermetabolism: PALS have a higher resting metabolic rate [4-8] and therefore function with increased energy needs and require more calories to maintain weight and nutrition.
- Reduced Caloric Intake: Reduced oral intake of foods and liquids is common in PALS due to dysphagia; fatigue with eating and drinking; limb weakness that impairs the ability to self-feed; loss of appetite; and avoidance behaviors associated with meal times [1, 9-11].
A higher baseline metabolic resting rate combined with reduced caloric intake creates the perfect storm for the development of malnutrition and further muscle wasting in PALS. If preventative action is not taken early in the disease to ensure adequate nutritional intake (either oral, non-oral or both); a vicious cycle of decreased eating, weight loss, malnutrition, and further muscle wasting ensues that go beyond the catabolic effects of the disease itself. This potential complication underlies the importance of the SLP, in concert with the dietitian and neurologist, to stress the significance of nutritional intake as soon as a patient is diagnosed with ALS. Higher baseline body mass index (BMI) at diagnosis  and maintenance of body weight during the disease  have been identified as prognostic predictors of longer survival in PALS. Furthermore, malnutrition has been noted to increase risk of death by nearly eight times in PALS . Therefore nutrition is a top priority in the management and care of PALS at the ALS multidisciplinary clinic I work in at the University of South Florida.
PEG Use in ALS
An obvious remedy to prevent malnutrition in PALS is placement of a feeding or percutaneous gastrostomy (PEG) tube. PEG placement in PALS is a generally a well-tolerated and safe procedure and recommended for use in ALS by the American Academy of Neurology (AAN) and the European Federation of Neurological Societies . Its use in the management of PALS appears to have increased as the number of PEG placements doubled between 1990 and 1998 . Current practice guidelines in the United States recommend that PEG placement occur in PALS when: 1) a loss of greater than 10% of body weight has occurred and/or 2) before Forced Vital Capacity (FVC) falls below 50%  to minimize surgical risks and complications. Since these AAN guidelines were published, however, a number of emerging studies have reported that respiratory function (FVC) at time of PEG placement does not impact complications or post-PEG survival [11, 15, 16] These findings suggests that PEG placement in PALS is safe, even in those with a compromised respiratory status.
Impact on Survival?
Because early PEG placement ensures a dependable means to maintain nutrition and hydration, it is not surprising that the presence of a feeding tube has been noted to increase survival in PALS. Indeed, PEG placement is one of only a few interventions noted to significantly impact survival in this patient population. Several studies have documented that PALS who undergo PEG placement live, on average, approximately four months longer then PALS who do not undergo PEG placement [11, 16, 17]. Although these numbers may seem low, comparatively, this represents a 50% greater treatment effect than the only FDA approved medication (Riluzole) to treat ALS of 83 days. Interestingly, the impact of PEG placement on survival was seen to be greater in spinal onset PALS (8 months) vs. bulbar onset PALS (3 months) in a review of 150 PALS with dysphagia . This year, in collaboration with Laurie Sterling and Dr. Stanley Appel, we studied outcomes associated with PEG placement in consecutive patients attending the MDA/ALS Clinic at Houston Methodist Neurological Center between1992 and 2014. This retrospective review of 2172 cases represents the largest dataset to examine the impact of PEG placement on survival in PALS to date. Our analysis determined that PEG placement was protective and increased survival, on average, by 107 days (PEG vs. No-PEG). When we further divided PALS into disease onset type, similar to Spataro and colleagues earlier work (2011); we noted that spinal onset PALS benefited most from PEG placement (+314 days). Current data indicate a strong relationship between nutrition, wellbeing, quality of life and survival in PALS. Therefore, consider early PEG placement to stay ‘ahead of the curve’, to prevent malnutrition and to ensure a ‘back up’ nutritional source if oral intake fails to meet the increased nutritional requirements. In addition, placement of a feeding tube allows for the administration of comfort measures (pain medications, hydration, etc.) at the end of life in PALS.
My practice patterns at the University of South Florida are based from the best available data. Although effective treatments for PALS are lacking; attendance at a multidisciplinary clinic , maintenance of adequate nutrition and weight [9, 10], and gastrostomy tube placement [11, 16, 17] are favorable prognostic indicators. Therefore, I routinely recommend for PALS to attend a multidisciplinary clinic that functions like a “one stop shop” in the management of the multiple facets of this complex disease. For PALS, attending multiple single specialty clinic sessions can be extremely tiring and taxing since these individuals function within the context of a diminished set of resources. In a multidisciplinary clinic, the patient can be seen by up to eight different medical specialists. Such a setting facilitates coordinated medical care while affording energy conservation and minimizing fatigue for PALS.
I am proactive from my initial encounter in educating the patient and caregiver/s about the importance of nutrition and the inherent high risk for malnutrition. This education is emphasized in my patients with low baseline BMIs, who are at an even greater risk for becoming malnourished. I talk about the benefits of having a feeding tube placed early while highlighting the fact that a gastrostomy tube does not preclude an individual from eating by mouth, can play a prophylactic role in preventing nutrition-related weight-loss to stay ‘ahead of the curve’, and that it can prevent further decline in muscle mass. For those PALS with safe swallowing abilities who do have a PEG or who are considering PEG placement; I discuss the importance of oral intake to keep the swallowing system functionally engaged and refer to the “use it or lose it” principle of plasticity  to keep the muscles of deglutition actively engaged and to prevent further disuse atrophy. For my younger patients, I highlight the option of a Mic-Key (“button”) feeding tube that is cosmetically less conspicuous since its opening lies flush with the skin, is capped, and has no outer tubing.
At the end of the day I acknowledge that placement of a feeding tube is a highly personal decision that incorporates a multitude of factors including cultural, religious, and personal beliefs and values. While current data suggest that PEG placement in PALS is protective, I also acknowledge and understand that for some PALS, this is not a desirable treatment option. As I see it, my role is to ensure that patients and caregivers understand the inherent risks of malnutrition and its impact on disease progression, current gastrostomy tube options and the associated outcomes with these interventions in PALS. The patient and family members then can make an informed and educated decision. I can appreciate that for some PALS, regardless of what the data say, having a feeding tube does not fit with their personal beliefs, views or values.
Free Patient Resources for PALS
We have developed a set of free downloadable resource to promote safe swallowing and to provide information on AAC devices in PALS. These can be accessed at: http://nssrlab.org/patient-resources/.
- Kuhnlein, P., et al., Diagnosis and treatment of bulbar symptoms in amyotrophic lateral sclerosis. Nat Clin Pract Neurol, 2008. 4(7): p. 366-74.
- Carpenter, R.J., 3rd, T.J. McDonald, and F.M. Howard, Jr., The otolaryngologic presentation of amyotrophic lateral sclerosis. Otolaryngology, 1978. 86(3 Pt 1): p. ORL479-84.
- Chen, A. and C.G. Garrett, Otolaryngologic presentations of amyotrophic lateralsclerosis. Otolaryngol Head Neck Surg, 2005. 132(3): p. 500-4.
- Bouteloup, C., et al., Hypermetabolism in ALS patients: an early and persistent phenomenon. J Neurol, 2009. 256(8): p. 1236-42.
- Desport, J.C., et al., Factors correlated with hypermetabolism in patients with amyotrophic lateral sclerosis. Am J Clin Nutr, 2001. 74(3): p. 328-34.
- Desport, J.C., et al., Hypermetabolism in ALS: correlations with clinical and paraclinical parameters. Neurodegener Dis, 2005. 2(3-4): p. 202-7.
- Funalot, B., et al., High metabolic level in patients with familial amyotrophic lateral sclerosis. Amyotroph Lateral Scler, 2009. 10(2): p. 113-7.
- Kasarskis, E.J., et al., Nutritional status of patients with amyotrophic lateral sclerosis: relation to the proximity of death. Am J Clin Nutr, 1996. 63(1): p. 130-7.
- Limousin, N., et al., Malnutrition at the time of diagnosis is associated with a shorter disease duration in ALS. J Neurol Sci, 2010. 297(1-2): p. 36-9.
- Ngo, S.T., F.J. Steyn, and P.A. McCombe, Body mass index and dietary intervention: implications for prognosis of amyotrophic lateral sclerosis. J Neurol Sci, 2014. 340(1-2): p. 5-12.
- Spataro, R., et al., Percutaneous endoscopic gastrostomy in amyotrophic lateral sclerosis: effect on survival. J Neurol Sci, 2011. 304(1-2): p. 44-8.
- Desport, J.C., et al., Nutritional status is a prognostic factor for survival in ALS patients. Neurology, 1999. 53(5): p. 1059-63.
- Miller, R.G., et al., 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(15): p. 1218-26.
- Forbes, R.B., S. Colville, and R.J. Swingler, Frequency, timing and outcome of gastrostomy tubes for amyotrophic lateral sclerosis/motor neurone disease–a record linkage study from the Scottish Motor Neurone Disease Register. J Neurol, 2004. 251(7): p. 813-7.
- Czell, D., et al., Outcomes of percutaneous endoscopic gastrostomy tube insertion in respiratory impaired amyotrophic lateral sclerosis patients under noninvasive ventilation. Respir Care, 2013. 58(5): p. 838-44.
- Sarfaty, M., et al., Outcome of percutaneous endoscopic gastrostomy insertion in patients with amyotrophic lateral sclerosis in relation to respiratory dysfunction. Amyotroph Lateral Scler Frontotemporal Degener, 2013. 14(7-8): p. 528-32.
- Chio, A., et al., Safety and factors related to survival after percutaneous endoscopic gastrostomy in ALS. ALS Percutaneous Endoscopic Gastrostomy Study Group. Neurology, 1999. 53(5): p. 1123-5.
- Traynor, B.J., et al., Effect of a multidisciplinary amyotrophic lateral sclerosis (ALS) clinic on ALS survival: a population based study, 1996-2000. J Neurol Neurosurg Psychiatry, 2003. 74(9): p. 1258-61.
- Kleim, J.A. and T.A. Jones, Principles of experience-dependent neural plasticity: implications for rehabilitation after brain damage. J Speech Lang Hear Res, 2008. 51(1): p. S225-39.