– Sarah Dawson, LCSW
People often call and ask what stage they or their loved one is in. Though it may be somewhat comforting to see a listing of symptoms and issues that are associated with the various stages of HD, the most important thing to consider is how well someone is coping with their illness regardless of its progression. Age of symptom onset and timing of progression from one stage to another may vary even within the same family, i.e. the way one’s mother was affected by HD is not necessarily the way in which her children’s disease will progress. Length of CAG repeat is related to age of onset of symptoms but doesn’t explain all of the variation in disease progression or in symptoms in any individual. Talking with a genetic counselor or neurologist can shed more light on this subject.
Here is a basic outline of the characteristics of Early Stage, Mid Stage, and Late Stage HD, once known as stages 1-5. Suggested ways to cope follow a description of each stage.
Early HD (formerly “Stages 1 and 2”): A Great Time to Prepare
- Normal or mildly reduced work ability
- Need some help with finances
- Normal or mildly impaired ability to help with chores at home
- Minimal impairment in activities of daily living
- Can live independently
- Psychiatric symptoms are intermittent and manageable
- Emerging motor symptoms
How to Cope with Stages 1-2:
The early stages of HD can be a time of productive conversations and decision-making about the rest of your life. Consider getting support from a therapist or psychiatrist who can help you adjust to the diagnosis and help you talk openly about symptoms, especially depression, which is very common. Attending support groups is a great way to meet other families and to learn how they are coping with HD. Meeting with an attorney and financial advisor at this stage is a great idea as they can help with advanced directives and estate planning. Necessary conversations with family members about what you want your future to be like are best discussed at this stage. Topics to consider may include how you want to be taken care of in the future, what is your plan for driving in the future, and where do you want to live if you can’t be taken care of at home. Consider touring some assisted living and skilled nursing facilities near you so that you can have an idea of what things you like and don’t like and also what facilities you could afford in the future. It cannot be emphasized enough (from a planning perspective) that the research you do into long term care options at this stage will help you significantly later on. We encourage families not to wait until their loved one with HD is very sick before beginning to look for long term care options.
Learning about all available emergency resources is recommended. We advise patients to program emergency resource phone numbers right into their cell phones so that they will have them at the ready if they ever need them in the future. We encourage patients to learn about available technology that can help keep their life organized (tablet and smartphone applications, using a simple whiteboard at home, etc). Occupational therapists and speech pathologists can be very helpful with ideas.
Paying attention to any minor problems one may be experiencing in the workplace is important at this stage as one may be able to seek accommodations from an employer that will allow a longer working period. A helpful resource for job accommodations is the Job Accommodation Network at www.askjan.org.
Consider becoming part of a clinical trial if there is a site near you. In general patients who are part of clinical trials tend to do better over time; the reasons for this include closer monitoring of symptom progression due to more frequent visits with the treatment team. Patients also gain a sense of having control over some aspects of their illness. People also report that participation in clinical trials makes them feel proud that they have contributed valuable information that will benefit future generations.
Mid-Stage HD (“Stage 3”): Time to Implement Plans
- Walking, communicating and eating become affected
- Noticeable impairment in many activities of daily living
- Some impairment in managing money
- Cognitive symptoms are less treatable than in Stage 1-2
- Frustrations can be great– patients are at high risk of suicide
- Driving may terminate
Coping During Mid-Stage HD:
Mid-stage HD can be a very stressful time for families though there are a lot of things that you can do to improve the quality of life of someone in this stage. Physical safety needs must be monitored at this stage: people often begin to lose weight and problems with choking/swallowing may begin. Consider meeting with a speech pathologist and/or dietician for assistance with these problems. Falls often become more frequent; physical therapy may be helpful to strengthen and tone muscles so that falls happen less often, and a physical therapist may have recommendations about equipment that would make walking easier and safer. At-home safety assessments by an occupational therapist may be ordered through your doctor or pursued independently; these can give you ideas about ways to set up your living space as safe as possible.
Doctors may advise patients to stop driving during this stage; families can work with their treatment team to strategize and make this difficult transition as smooth as possible. (Please see our driving section for more information.) Due to the cognitive changes common in mid- stage HD, it may be helpful to patients to follow a structured daily routine that can be written down on a whiteboard at home. Mid-stage HD is when people typically apply for Social Security Disability (SSDI). Make sure you meet with your treatment team as frequently as necessary so that your records are up to date and ready to go when you need them for a SSDI claim; always remember to tell your doctors what symptoms you are experiencing and how they affect your performance at work.
Depression, anxiety, irritability, and obsessive thoughts and behaviors frequently occur during this stage; find a psychiatrist and therapist to work with you and your family if you have not done so before. Make plans for emergencies and communicate those plans to all family members. Give yourself permission to utilize home health care services if you can afford it; often people are amazed at how much relief they experience when they have just a little bit of outside help during the week. Ask for help! Consider using a program like “Share the Care” to involve your friends and family to help manage the care-giving demands of HD (www.sharethecare.org)
Late Stage HD (“Stages 4-5”) Time to Ask For and Receive More Help:
- All areas of functioning become severely impaired so total care is necessary; patients tend to enter long term care facilities
- Chorea can be severe or movements become rigid or dystonic (stiff)
Verbal expression is impaired but language comprehension is not.
How to Cope During Stages 4-5:
Obtaining high quality long term care is the key to healthy coping during the last stages of HD. (This of course requires a lot of research during earlier stages.) If your loved one enters a long term care facility, you may need to help educate the facility about HD. There are many key differences between HD and Alzheimer’s Disease; facilities will know a lot more about Alzheimer’s Disease. Social workers and HDSA publications can be helpful with educational efforts. Many families find that preparing a scrapbook featuring photos and meaningful mementoes that belong to patients that describe their life story to be very helpful in engaging long term care facility staff in the care of their loved one. Caregivers will need respite and emotional support during this stage more than ever. Some families find that the last stages of HD can actually be a time of gratitude in the midst of stress as families sometimes come together as never before.
Decisions about what type of long term care is appropriate for people are best made with the support of a person’s medical team and with available resources in mind. Some families may opt to keep their loved one at home as long as possible with the support of home health care agency workers and other caregivers. This can become very expensive, as home health care is typically paid for out of pocket unless a person has long term health care coverage for these services. Caregivers sometimes feel their loved one’s needs have exceeded their ability to take care of them properly and this is when they seek out a facility where they could receive care from multiple people around the clock.
Hospice care is another option for people in the late stages of HD. Hospice is a specialized team approach to care for individuals with life-limiting illnesses and their families. This care includes symptom control during the last days of life as well as family and bereavement support. Hospice services can be provided at home, in nursing facilities and in medical as well as psychiatric hospitals.