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Dysphagia, or difficulty swallowing, is a big problem in Huntington's disease (HD), but surprisingly little is known about it. It is not known how early it begins, or how symptoms and swallowing dysfunction progress over the stages of HD. Further, even less is known about techniques that might prevent, alleviate or treat it. As such, we welcome the recent attention given this symptom by investigators from Europe.

What are the symptoms of swallowing difficulty? After an earlier extensive review of studies relating to swallowing difficulty in HD, investigators from Denmark developed a scale for measuring the level of swallowing difficulty a person with HD experiences [Heemskerk AW 2014]. As part of this study, they identified several important characteristics:

What are the physical problems that cause swallowing difficulty in HD? Experts break down the swallowing problems into separate parts:

Should a formal swallowing study be done? At least in this doctor/author's opinion if that study will not change a diagnosis, or what I recommend for a patient, I don't think it should be done.

Important other considerations:

Limiting distractions during mealtime is vital. This means no TV, no phone, no difficult conversations. Safe eating requires concentration. And as such, eating can be exhausting. More time is required because the eating process is slowed. Frequent small meals can be helpful.

Many medications used for Huntington's cause worsening of swallowing. Antipsychotic drugs (like Haldol® or Zyprexa®) and Xenazine® (tetrabenazine) are frequent culprits. Alcohol, or any drug that causes sedation can interfere with the concentration needed for more safe eating practices. A trial of tapering doses of these drugs may improve swallowing.

What to do when swallowing difficulty becomes severe: In this situation, there is often fear of choking, which in itself is a significant distraction. At this stage of disease, individuals are often receiving palliative and hospice care services; this doctor/author has found that providing small doses of oral morphine prior to a meal can decrease both the anxiety and frequency of choking, and prolong the time that a person can enjoy eating.

What about feeding tubes? No matter how vigilant the caretaker, swallowing difficulty will progress to not being able to swallow at all. Is the next step a feeding tube? Though there are no studies done specifically in HD, we can learn from studies in other diseases. In a landmark study of 339 individuals with advanced dementia residing in nursing homes [Mitchell SL 2009], feeding tubes did not prolong life, did not prevent aspiration, and did not improve function or comfort. Feeding tubes were associated with more agitation and more skin sores. Based on this study, the American Geriatric Society officially recommends against placement of a feeding tube in this situation. In this same study, those individuals who received enhanced oral feeding, one on one in a calm environment with between meal supplements lived longer and more comfortably. Though not all studies agree, there is more evidence that feeding tubes do not prolong life even in Amyotrophic Lateral Sclerosis (ALS)[Heffernan C 2004].

There is no study to give us guidance regarding feeding tubes specific for HD. End of life choices should always be discussed with the patient and family, and education given on the potential for benefit or risk and then be based on personal preference of the individual.

References

Heemskerk AW, Verbist BM, Marinus J, Heijnen B, Sjögren EV, Roos RA. The Huntington's Disease Dysphagia Scale. Mov Disord. 2014 Sep;29(10):1312-6. doi: 10.1002/mds.25922. Epub 2014 May 23. PubMed abstract

Mitchell SL, Teno JM, Kiely DK, Shaffer ML, Jones RN, Prigerson HG, Volicer L, Givens JL, Hamel MB. The clinical course of advanced dementia. N Engl J Med. 2009 Oct 15;361(16):1529-38. doi: 10.1056/NEJMoa0902234. PubMed abstract

Heffernan C, Jenkinson C, Holmes T, Feder G, Kupfer R, Leigh PN, McGowan S, Rio A, Sidhu P. Nutritional management in MND/ALS patients: an evidence based review. Amyotroph Lateral Scler Other Motor Neuron Disord. 2004 Jun;5(2):72-83. PubMed abstract