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:
- Drooling during the day
- A sensation of too much food in the mouth to swallow
- Food coming out of the mouth while eating
- Food or liquids coming out the nose while eating
- Choking on solids or liquids
- Coughing or sneezing while eating solids or liquids
- Suddenly taking a breath while swallowing
- Food coming back into mouth after a swallow
- Feeling a lump in the back of throat
What are the physical problems that cause swallowing difficulty in HD? Experts break down the swallowing problems into separate parts:
- Preparatory Phase: Getting food from plate to utensil to mouth includes correct bite size and pace of eating. Chorea and impaired fine motor skills interfere with the mechanics of of food delivery, making it harder to control bite size and the pace of eating so that "gulping" occurs. A care partner who cues or assists in feeding is essential.
- Oral phase: Main problems include chorea of the tongue, and uncoordinated movement of food to the back of the throat. There is delay in starting the swallow, repeating of the swallow, or food left in the mouth after the swallow. Food consistency in each bite is important; a bowl of cereal is a set up for choking because the liquid may get to the back of the throat faster than the solid. Placing softer food in the cheek by the molars helps overcome the slowness of propelling food to the back of the throat. Starting the meal with something sour may help speed subsequent swallows. A "chin tuck" position of the neck is probably preferred for protecting against aspiration.
- Pharyngeal: Choking, coughing, aspiration: Small bites, thickened liquids, softer moist foods, progressing to smashed, then pureed consistencies. Part of this is due to "respiratory" chorea, which causes breathing during a swallow.
- Esophageal: Regurgitation of food, or vomiting. There is also evidence of muscle dysfunction in the esophagus that may cause pain. It is important to stay upright for at least an hour after eating.
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.
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