End of Life Issues

It is important to discuss issues related to the end of life before someone with HD loses the ability to communicate. By discussing the expected changes in advance patients can plan for the support that they and their families will need, and can have a discussion with their family and physicians about which medical treatments and interventions they think they would like to undergo, and which they would prefer to have withheld when they reach the late stages of the disease.

By the late stages of HD affected individuals will have little control over voluntary movements and may not be able to walk, talk, or eat. Chorea may be suppressed, or may be severe. Death, when it comes, is usually due to the consequences of the immobility, general debilitation, and malnutrition. Pneumonia, and heart failure are typical immediate causes of death.

TABLE 18: END OF LIFE ISSUES In-home versus outside care Gastrostomy tube feeding Life sustaining emergency measures (e.g. CPR, intubation) Use of antibiotics to treat infections Other specific care issues (e.g. treatment of other ongoing health problems) Guardianship, substituted consent, and "living wills" Autopsy/ brain donation for research

Huntington's disease patients and their families have a number of important decisions to make about this phase of the illness.

The first concerns where the patient will be cared for. Some people wish to spend their last months at home, and receive terminal care in this setting, but others require the services of a nursing home for the final phase of their illness. This may make the patient more comfortable and relieve stress on the family.

Patients and their families must decide which treatments they want if they become acutely ill, such as antibiotics for pneumonia, or CPR for a cardiac arrest.

Patients who are unable to swallow will die if not given food and fluids by other means, but with a gastrostomy tube they may live for years. Improved caloric intake can increase resistance to infections, improve physical appearance, and is sometimes associated with a decrease in chorea. Others may not desire such an intervention, depending on their view of the quality of life at that time and their individual spiritual beliefs.

There are different legal mechanisms in every state by which patients can make their wishes known in advance, but it must be stressed that there is no substitute for good communication directly between patients, their families, and their doctors.

The process should start early, so that difficult topics can be introduced gradually, in an unhurried manner, and so that the conversation can take place while the patient retains the ability to communicate.

It is also important to readdress these issues periodically. An advance directive reflects a person's ideas at one discreet interval, often several years in the past.  For example a blanket statement such as "I would never want a feeding tube," made shortly after the diagnosis of HD, may be revised as the patient and family gradually adapt to increasing disability.

One must avoid overgeneralizations about "end-stage HD."  An intervention that is right for one person may not be right for another.

For example, many patients who can no longer eat safely are still able to talk and are fully aware of their surroundings. In one instance a man was told that placement of a gastrostomy tube would reduce the number of aspiration pneumonias from which he suffered. He replied that eating was one of his few pleasures and he preferred to take this risk, knowing that it might shorten his life.

In another instance, a teenaged girl with juvenile onset HD had become very rigid and was unable to eat. A ward of the state, she was initially denied a gastrostomy tube by her official guardian who believed that such interventions were "futile" and "only prolong suffering." This decision was reversed when her foster mother strenuously pointed out that the girl was in no pain, was enjoying activities and family life, could still talk, and in fact had been asking for the tube all along.

For other individuals, the issue of a gastrostomy tube does not arise until the patient no longer seems aware of his surroundings. In this circumstance, it often seems best to a family not to prolong the process artificially, but to support the patient's comfort and let him die a natural death.

It is our hope that when death does come to a person with HD, that this person's family will consider making a gift of brain tissue to one of the projects  that study such  material,  which are listed in Appendix 1  http://hdlighthouse.org/see/pg2/a1.htm

We hope that, where possible, patients and families will discuss this decision with each other in advance and will also inform the staff of nursing homes and hospices of their intentions ahead of time.

The cost of autopsy and transportation to and from the funeral home are usually born by the institution receiving the donation, and the brain can be removed quickly so as not to delay burial and in such a way that it does not show and will not interfere with viewing.

These generous gifts, made at a sad time, may give the person's death great meaning. Each one moves us closer to the day when no one will have to die from Huntington's disease.