Anosognosia is the medical term for lack of self awareness of one's own disability or disease symptom. This condition, which is different from denial, occurs in many brain diseases including Huntington's disease (HD). Anosognosia is caused by nerve circuit damage to brain structures that interpret and organize information. Lack of awareness in HD creates problems that can range from refusal of therapy: "I don't need treatment if I'm not ill" to more extreme situations that can be dangerous for HD individuals or others. Families know that dealing with this symptom when it is severe -- is like walking through an emotional minefield, knowing the explosion is likely to come.
How is anosognosia different from denial? Though they have similarities, denial and lack of awareness are not the same. Denial is a psychological defense response to extreme stress that occurs in the absence of neurologic disease. An example is the shock or numbness felt just after the death of a loved one. Described as the first stage of grief, it helps us to survive loss by letting in only as much grief as can be handled. Denial is usually present for the short term, days or weeks. Anosognosia is different; it is a long term neurologic condition due to brain circuit damage the impairs the ability to recognize the nature of one's own illness. In this situation, the person can observe and recognize the illness state in others, but literally cannot see it in themselves. It shouldn't be surprising that troubles arise when HD individuals -- who literally can not see the problems -- and care-partners disagree.
Anosognosia is variable: As with any symptom in HD, there is a wide range of severity and variability of anosognosia among individuals. Lack of awareness can occur with any symptom, as with chorea, dystonia [Snowden JS 1998], and/or for cognitive, emotional, social, or functional abilities [Hoth KF 2007]. Though some individuals with HD are very aware of symptoms, others aren't. Because lack of awareness can occur, HD doctors and other medical professionals routinely solicit information from both HD individuals and care-partners to guide therapy. This is particularly true for behavioral symptoms. Though it could be said that this practice interferes with patient confidentiality, this is thought necessary for gathering medical information needed to guide care. And it shouldn't be surprising that problems can arise during or after medical visits when the HD individuals and care-partners disagree. Nor is it surprising that HD individuals in this situation might lose trust in medical providers too.
When anosognosia is severe, this symptom is one of the hardest in HD. It is frustrating and provokes anger on all sides, and can badly damage relationships. It can be like walking through a minefield, with serious and dangerous consequences for HD individuals and their families.
What can be done? For such a big problem, there is surprisingly little research or expert advice to help guide us through the anosognosia minefield in HD. However, there has been more study done in schizophrenia and Alzheimer's where anosognosia is common, and there we learn mostly what not to do. In these diseases, discussion is not helpful. In fact, efforts to rationally convince individuals of illness or the need for medical intervention more often causes distrust, paranoia, then anger, which can lead to aggression toward care-partners. Or less commonly, if awareness of the illness is achieved through discussion, it can lead to depression and suicidal behaviors.
If rational discussion isn't helpful, then what can be done? The advice is to listen and try to find common ground to work within so that trust can be maintained. Don't be adversarial as this can worsen behaviors. And it is important to realize that from the medical-legal point of view, there isn't much that a care-partner or provider can be do until there is harm or threat of harm to self or others. In the extreme it take an explosive misstep to trigger a hospital intervention. Why is this? Because self-determination is a cherished and protected liberty that includes the right for adults with diminished capacity to make their own decisions. Even when the need appears obvious, treatment can not be forced because unrequested intervention is an infringement of civil liberties -- until there is a documented threat.
Author's comment: Dealing with the consequences of severe anosognosia is one of the hardest of situations in HD. Though there is painfully little evidence on which to base recommendations, I advise:
- Get education about anosognosia and behavioral modification strategies very early: Though I have no proof of this, my bet is that early education of at-risk HD individuals and HD families about anosognosia might alleviate some of the suffering -- by proactively learning and utilizing behavioral modification therapies. If an at risk individual "learns" that this can be a symptom, this might provide a "side door" neural pathway for dealing with the problem.
- Develop empathy and understanding: Imagine what it would be
like to have others telling you that you are ill when you
can't see it yourself. Calling it "denial" blames
- Really listen: What are the most problematic issues as identified by the HD individual. Is it loss of a job, or independence, or driving, etc. Imagine what it would be like to experience these losses but not understand why.
- Show concern, don't be adversarial.
- Utilize engaging distraction: Encourage involvement in activities both inside and outside the home. Exercise or PT programs, church related activities, volunteer programs, local Y or senior programs.
- Seek help: Engage the support of friends and family, church groups, support groups,
- Have frequent and ongoing contact with professional care providers: This has been helpful in Alzheimer's [Villars H 2013] where a "team approach" with sub-specialty doctor, nurse, social worker, psychologist, etc worked with the care-partner and local doctor on an ongoing basis. If you have no such team in your community, identify resources that you can access.
- Take some time for yourself. Burn-out helps no one.
In summary: Very early on, HD families need to be educated about a potential agnosognosia minefield, how to avoid the bombs, how to call in or develop the best bomb-squad and to "keep them on the line". Equally important is to know that -- as in many HD situations -- there aren't any perfect answers.
Snowden JS, Craufurd D, Griffiths HL, Neary D. Awareness of involuntary movements in Huntington disease. Arch Neurol. 1998 Jun;55(6):801-5. PubMed abstract
Hoth KF, Paulsen JS, Moser DJ, Tranel D, Clark LA, Bechara A. Patients with Huntington's disease have impaired awareness of cognitive, emotional, and functional abilities. J Clin Exp Neuropsychol. 2007 May;29(4):365-76. PubMed abstract
Villars H, Dupuy C, Soler P, Gardette V, Soto ME, Gillette S, Nourhashemi F, Vellas B. A follow-up intervention in severely demented patients after discharge from a special Alzheimer acute care unit: impact on early emergency room re-hospitalization rate. Int J Geriatr Psychiatry. 2013 Nov;28(11):1131-40. doi: 10.1002/gps.3932. Epub 2013 Jan 24. PubMed abstract