Stages of HD

Stages of Huntington's Disease print version

There are various papers discussing the "stages" of Huntington's Disease.

This page reflects a few of them.

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Source: Alternate Stages of HD

Alternate Stages Of Huntington's Disease
by Jim Pollard and Rosemary Best

Stated in positive terms from the person's point-of-view.

Defiance "I'm not denying the diagnosis, I'm defying

the verdict! I'm not refusing to accept it,

I'm just boldly resisting the inevitable!"

Perseverance "I'm continuing on...in spite of all the

difficulties this damn disease puts in front

of me."

Compassion "I'm sorry for the trouble I'm causing my family

and everyone else who cares for me. I wish I

could do something to help them."

Stamina "I'm not sure just what it is but something

keeps me going! It keeps me going through all

my fatigue and all the problems and hardship

this damn disease presents me."

Grace "I've quietly resigned myself to needing others

to care for me, to sustain me. I can't show them,

but I'm more concerned for the welfare of those

around me than I am for myself. We know we're

there for each other."

April 1996
Toronto

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How Does Huntington's Disease (HD) Progress?

The five stages, as described in "Towards A Fuller Life - A Guide to Everyday Living With Huntington's Disease", published by the HDSA, contains the following description.

PROGRESSION: A general guide toward understanding how Huntington's Disease progresses was developed by Dr. Ira Shoulson, Professor of Neurology at the University of Rochester Medical Center in 1979. Briefly, these stages are as follows:

The progression of HD after a person is diagnosed can be divided into five (flexible) stages:

Early Stage - the person is diagnosed as having HD and can function fully both at home and work.

Early Intermediate Stage - the person remains employable but at a lower capacity. They are still able to manage their daily affairs despite some difficulties.

Late Intermediate Stage - the person can no longer work and/or manage

household responsibilities. They need considerable help or supervision

to handle daily financial affairs. Other daily activities may be slightly difficult but usually only require minor help.

Early Advanced Stage - the person is no longer independent in daily activities but is still able to live at home supported by their family or

professional carers.

Advanced Stage - the person with HD requires complete support in daily

activities and professional nursing care is usually needed. People with HD usually die about 15 to 20 years after their symptoms first appear. The cause of death is not the disease itself but complications such as pneumonia, heart failure or infection developing from the body's weakened condition.

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An overview of Huntington disease
May 2002

In addition to the physical symptoms of HD, there are often subtle cognitive signs as well.

Initial stages

People with early Huntington's may find they have difficulty organizing routine matters or coping effectively with new situations. Difficulty recalling information may make them appear forgetful. Work activities may become more time-consuming, and decision-making and attention to detail may be impaired.

Early emotional symptoms may be equally subtle. Individuals at this stage may experience more periods of depression, apathy, irritability, or impulsiveness, or perhaps changes in personality.

At this stage, people with Huntington's can function quite well at work and home.

Intermediate stages

As the disease progresses, the symptoms become worse. The initial motor symptoms will gradually develop into more obvious involuntary movements such as jerking and twitching of the head, neck, arms and legs. These movements may interfere with walking, speaking and swallowing.

People at this stage of Huntington's often look as if they're drunk: they stagger when they walk and their speech is slurred. They have increasing difficulty working or managing a household, but can still deal with most activities of daily living.

Advanced stages

The advanced stages of Huntington's typically involve fewer involuntary movements and more rigidity. People in these states of HD can no longer manage the activities of daily living, and they usually require professional nursing. Difficulties with swallowing, communication and weight loss are common.

Death usually occurs 15 to 20 years after the onset of the disease. People don't die from Huntington's itself, but from complications such as choking, heart failure, or infection.

Juvenile HD

About 10 per cent of Huntington's cases are considered "juvenile" - the symptoms appear in childhood or adolescence.

The symptoms of juvenile HD are different from the adult disease. Children with HD move slowly and stiffly, they have increasing difficulty learning, and they can have convulsions or epileptic seizures. Some children have severe behavioral problems.

The earlier the disease onset, the more likely the child is to be very rigid and appear very different from the majority of adult cases. This often makes it difficult to diagnose. Children with HD are more likely to have an affected father than an affected mother.

Diagnosis, testing, and treatment

Huntington's is usually diagnosed using neurological and psychological tests. Sometimes doctors use brain scans to see whether the caudate and putamen are working properly, or they use a recently devised genetic test to confirm diagnosis.

The genetic test uses a blood sample to reveal whether or not an individual has the gene that causes HD.

Currently, there are no treatments that slow down or stop HD. Some drugs will reduce symptoms such as depression, anxiety, and involuntary movements. These drugs may have side effects so not every Huntington's patient can use them.

Neurologists, psychologists, genetic counselors and social workers play an important role in helping individuals and families deal with HD.

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Dr. Alex Goumeniouk, a foremost expert of the disease and a psychiatrist at UBC's Huntington's Disease Medical Clinic, says working directly with HD patients is some of the most rewarding work he does. He says,

"I think the reason for this is that the true resilience of the human spirit is palpably present in these patients. They have reconciled that, short of being "hit by a truck," they will die from this illness. While there is an adjustment phase, patients emerge from this with a new perspective on life."

Source: Huntington Society of Canada and UBC Huntington Disease

Medical Clinic

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And yet another ;-) Just keep in mind that everyone is unique and therefore will not conform to the "standard" stages shown here! They were written by the authors as guidelines only, to help families understand the progression of HD.

THE STAGES OF HD

Though the pattern and severity of symptoms vary from person to person, the course of HD can be roughly divided into three stages.

Early Stages

Early in the disease, manifestations include subtle changes in coordination, perhaps some involuntary movements, difficult thinking through problems, and often, a depressed or irritable mood.

At this stage, medications are often effective in treating depression and other emotional symptoms. It is a good time to begin planning for the future. Financial plans should be made and legal documents drawn up.

Middle Stages

In the middle stage involuntary movements (chorea) may become more pronounced. A staggering gait can sometimes be mistaken for drunkenness.

Speech and swallowing will begin to be affected. It is important to consult a speech therapist who will be able to offer suggestions and strategies for improving communication and swallowing abilities. Likewise, occupational and physical therapists can develop programs to help maintain the highest level of functioning and thereby improve quality of life.

Thinking and reasoning skills will also gradually diminish. At this stage it may become increasingly difficult to hold a job and to carry out household responsibilities.

Here again, simple strategies may be employed to help decrease frustration, increase functioning and prolong independence. For example, disorientation and short-term memory loss can be addressed by labeling drawers, maintaining a daily routine and posting a calendar appointments and events.

Late Stages

People with late stage HD may have severe chorea, but more often have become rigid. Choking on food becomes a major concern, as does weight loss. At this stage people with HD are totally dependent on others for all aspects of care, can no longer walk, and not able to speak.

Although cognitive abilities are severely impaired, it is important to remember that the person is generally still aware of his/her environment, remains able to comprehend language, and remains an awareness of loved ones and others. He/she may continue to enjoy looking at photographs and hearing stories of family and friends.

People do not die from HD itself but rather from a complication of the disease, such as choking or infection. Death generally occurs about 15 to 20 years after onset.

Total Functional Capacity

From Dr. Paulsen's Understanding Behavior in HD

Stage of Illness
The type, frequency, and severity of behavioral problems that occur often vary with stage of illness. One survey suggested that persons with early HD are more concerned about depression, anxiety, and apathy, whereas persons with later-stage HD report agitation, irritability, and disinhibition as the most prominent behavioral concerns.

The ways of responding to the various stages of HD, however, can vary greatly from person to person. It is usually helpful to consider what stage of illness the individual is in to better understand the behaviors that occur.

Based upon the rating of Total Functional Capacity (TFC; Shoulson, 1981), patients are considered to be in one of five stages of the HD illness. In general, descriptions of the stages are as follows:

Stage I:
(0 to 8 years from illness onset) Maintains only marginal engagement in occupation having part-time voluntary or salaried employment potential, and maintains typical pre-disease levels of independence in all other basic functions, such as financial management, domestic responsibilities, and activities of daily living (eating, dressing, bathing, etc.); or performs satisfactorily in typical salaried employment (perhaps at a lower level) and requires slight assistance in only one basic function: finances, domestic chores, or activities of daily living.

Stage II:
(3 to 13 years from illness onset) Typically unable to work but requires only slight assistance in all basic functions: finances, domestic, daily activities, or unable to work and requiring major assistance in one basic function with only slight assistance needed in one other; one basic function is handled independently.

Stage III:
(5 to 16 years from illness onset) Totally unable to engage in employment and requires major assistance in most basic functions: financial affairs, domestic responsibilities, and activities of daily living.

Stage IV:
(9 to 21 years from illness onset) Requires major assistance in financial affairs, domestic responsibilities, and most activities of daily living. For instance, comprehension of the nature and purpose of procedures may

be intact, but major assistance is required to act on them. Care may be provided at home but needs may be better provided for at an extended

care facility.

Stage V:
(11 to 26 years from illness onset) Requires major assistance in financial affairs, domestic responsibilities, and all activities of daily living. Full-time skilled nursing care is required.

These stages are very broad guidelines and individuals can vary a great deal in the course of the illness. For instance, one of our patients has had chorea for 15 years yet he is still employed (in a reduced capacity) at his regular job.