According to two recent studies from the United Kingdom, bodily discomfort (which refers to total body physical suffering) and pain (which refers to localized physical suffering) are problems in Huntington's disease (HD) that significantly affect quality of life. Both studies, which included a total of 140 home-dwelling individuals in middle stages of HD, reported that more than 50% suffered from at least moderate discomfort/pain, and about 10% more severely. This is a large number, much higher than occurs in an age-matched general population. Unfortunately, very little is known about this symptom in HD . . .
The Studies: Both of these studies, published in June and August of 2013 [Calvert M 2013] [Arran N 2014] found that many HD individuals reported body discomfort/pain as part of surveys on quality of life in HD. These surveys focus on 5 major factors: impaired mobility, impaired ability to perform self-care, impaired ability to perform usual activities, anxiety/depression, and pain/body discomfort. While much is known about the first 4 factors that contribute to poor quality of life in HD, surprisingly little is known about pain/body discomfort.
Why this knowledge gap?
- Pain is not a visible symptom or sign in HD
- HD researchers have not routinely asked about pain; for instance, there is no question about pain/body discomfort in the United Huntington's Disease Rating Scale (UHDRS), the test that for decades has been used to describe and measure the symptoms and signs of HD
- Doctors probably don't routinely ask their HD patients about pain
- HD patients probably don't complain about pain
- HD patients have difficulty describing the discomfort/pain
Pain problems are not unique to HD: Quality of life studies in other neurodegenerative diseases have shown similarly high rates of pain/body discomfort [Calvert M 2013]. Other pain studies, particularly those done in Parkinson's disease (PD), may be informative with likely similarities between PD and HD pain. In PD differing types of pain syndromes are described that are probably caused by a combination of nerve cell abnormalities from the periphery (skin), spinal cord, and a multitude of pain processing cells in the brain.
- Musculoskeletal pain syndromes occur most often in areas of dystonic movements of the back and shoulders [Kim YE 2013]
- Chronic widespread discomfort/pain, a condition similar to fibromyalgia in which pain is not localized to a specific site [Toda K 2010]
- Central neuropathic pain/discomfort for which the patient has difficulty describing the character or location of pain [Sophie M 2012]
Other studies in PD suggest that physical pain/discomfort increases as the disease progresses. Another consistent finding in PD (which is likely true for HD) is that pain complaints are under-treated when compared to the same pain complaint in the general population [Kim YE 2013].
Pain and Agitation in late stage disease with dementia: Though there are no HD studies on discomfort or pain in late stage HD patients, recent studies done in nursing home patients with dementia suggest that agitation behaviors (pacing, screaming, restlessness, aggression) respond to pain treatment [Husebo BS 2011, Husebo BS 2014]. In these situations, it is thought that discomfort/pain is experienced, but damage to nerve pathways prevents the mental processing that cognitively interprets a painful (or uncomfortable) stimulus. One of my patients who had broken her clavicle (collar bone) could not localize her pain and thought it might be coming from her foot. Because this type of person cannot accurately describe discomfort, pain too often is not treated at all. My experience with Hospice and HD patients suggests that discomfort/pain in end-stage patients is often not treated adequately.
Author Comment: Clearly all of us in the HD community have discounted discomfort and pain in HD and this important symptom deserves more attention and study. Both researchers and doctors should routinely ask about pain -- and like other manageable symptoms of HD, it should be treated.
Calvert M, Pall H, Hoppitt T, Eaton B, Savill E, Sackley C. Health-related quality of life and supportive care in patients with rare long-term neurological conditions. Qual Life Res. 2013 Aug;22(6):1231-8. doi: 10.1007/s11136-012-0269-5. Epub 2012 Sep 23. PubMed abstract
Arran N, Craufurd D, Simpson J. Illness perceptions, coping styles and psychological distress in adults with Huntington's disease. Psychol Health Med. 2014;19(2):169-79. doi: 10.1080/13548506.2013.802355. Epub 2013 Jun 14. PubMed abstract
Kim YE, Lee WW, Yun JY, Yang HJ, Kim HJ, Jeon BS. Musculoskeletal problems in Parkinson's disease: neglected issues. Parkinsonism Relat Disord. 2013 Jul;19(7):666-9. doi: 10.1016/j.parkreldis.2013.03.002. Epub 2013 Apr 16. PubMed abstract
Toda K, Harada T. Prevalence, classification, and etiology of pain in Parkinson's disease: association between Parkinson's disease and fibromyalgia or chronic widespread pain. Tohoku J Exp Med. 2010 Sep;222(1):1-5. PubMed abstract
Sophie M, Ford B. Management of pain in Parkinson's disease. CNS Drugs. 2012 Nov;26(11):937-48. doi: 10.1007/s40263-012-0005-2. PubMed abstract
Kim YE, Jeon BS. Musculoskeletal problems in Parkinson's disease. J Neural Transm (Vienna). 2013 Apr;120(4):537-42. doi: 10.1007/s00702-012-0960-2. Epub 2013 Feb 19. PubMed abstract
Husebo BS, Ballard C, Sandvik R, Nilsen OB, Aarsland D. Efficacy of treating pain to reduce behavioural disturbances in residents of nursing homes with dementia: cluster randomised clinical trial. BMJ. 2011 Jul 15;343:d4065. doi: 10.1136/bmj.d4065. PubMed abstract
Husebo BS, Ballard C, Cohen-Mansfield J, Seifert R, Aarsland D. The response of agitated behavior to pain management in persons with dementia. Am J Geriatr Psychiatry. 2014 Jul;22(7):708-17. doi: 10.1016/j.jagp.2012.12.006. Epub 2013 Apr 20. PubMed abstract