In the last stages of Huntington’s disease (HD), patients have difficulty thinking and communicating clearly, so decisions about their end-of-life medical care often fall to doctors and relatives. However, many people with HD have strong opinions as to how they would like their last years to unfold. Advance directives are instructions written by mentally and physically competent patients to convey their preferences about end-of-life medical care ahead of time. They are powerful expressions of a person’s wishes about their future health care. Anyone over the age of 18 is legally qualified to make these decisions for himself or herself. Health care professionals who are caring for a patient will refer to advance directives if the patient is unable to make or communicate their decisions later in life.
Advance Directives and HD^
Why are advance directives important for patients with HD? Because the age of onset and severity of HD symptoms are relatively unpredictable, sudden changes can quickly change the course of patients’ lives. During the end stages of HD, many patients are nonverbal and unable to communicate their wishes. Advance directives allow patients to decide their end-of-life care ahead of time so that their treatment fits their unique mental and emotional needs.
While it may seem like a simple task to address advance directives before late-stage symptoms occur, there are numerous obstacles that make it difficult for patients to make such decisions earlier in their life. These obstacles mostly fall into three categories: the doctor, the patient, and the patient’s family. Regarding doctors, there are currently no guidelines requiring doctors to discuss advance directives with patients early in the course of the disease. Thus, many doctors do not discuss end-of-life care with their patients when they are in earlier stages of chronic diseases. This greatly contributes to the difficulty of the decision process for the patient.
Patients themselves may exhibit cognitive dysfunction, denial, poor judgment, or psychiatric symptoms and thus may not be able to make an informed decision even at early stages of the disease. In early stages of the disease, patients may want to avoid the topic of advance directives, as they have difficulty confronting their declining ability to function as parents, employees, and other roles they fill in their lives. In late stage HD, communication becomes the predominant issue, as patients cannot make their wishes clearly known.
Advance directive decision-making may also be delayed by the dynamics of the family. First of all, once a patient becomes mentally incompetent or is unable to communicate his or her wishes, deciding on who should make these decisions becomes a source of controversy. Furthermore, the family not only has to deal with the shock of a newly diagnosed relative, but also may become increasingly worried about other at-risk individuals. In order to cope, families may be experiencing what medical professionals have deemed denial —a common, short-term protective mechanism for dealing with the shock of diagnosis. If end-of-life care is not discussed early enough, family members may not agree with the patient’s decisions.
Despite the obstacles in addressing advance directives at an early stage of disease, the value of deciding between options for end-of-life care early-on is an important means of ensuring autonomy for HD patients.
Elements of Advance Directives^
Advance directives have several important elements: a health care proxy, a living will, do-not-hospitalize and do-not-resuscitate orders; decisions about tube feeding; and decisions about brain donation for diagnosis and research purposes.
A healthcare proxy is a person who makes decisions for the patient once the patient is deemed unable to make those decisions for himself or herself, and is usually a family member of the patient. The healthcare proxy should be an adult over 18 who is mentally, physically and emotionally equipped to make decisions. The healthcare proxy form is approved by law in all states and is a written document. It is very important that a patient’s doctor knows who is appointed health care proxy, and that a healthcare proxy is chosen earlier rather than later.
A living will is a set of instructions to a doctor about treatment and life-sustaining procedures. It is helpful for the patient to discuss living wills with a medical professional to stay informed about the advantages and disadvantages of all types of treatments and procedures. These instructions do not become effective until two doctors certify in writing that the patient is unable to make a healthcare decision. Lastly, the patient can change or revoke advance directive instructions anytime, as long as s/he has the mental capacity/communication abilities to do so.
The wishes of the patient in regards to use of a feeding tube can be stipulated in the advance directive. Feeding tubes are usually used when the patient has no other way to swallow food. They may also be used to provide supplements when the patient can eat but needs to receive more nutrition through the tube. Tube feeding is common in late-stage HD treatment. The tube is made of soft plastic and is usually inserted into the stomach. It can be used to avoid problems such as aspiration, which is when food goes into the lungs instead of the stomach. Aspiration can increase the likelihood of pneumonia, which can cause serious medical complications in HD patients. The advantages of the feeding tube include: 1) ensuring that the body receives essential nutrients, 2) allowing patients to eat foods that they enjoy, 3) ameliorate weight loss, which is a serious health risk for late-stage HD. Disadvantages include: 1) medical risks associated with feeding tube insertion, such as infection or bleeding, 2) not all patients gain weight and feel full while on the feeding tube, 3) vomiting and aspiration can still occur occasionally, 4) potential re-hospitalization if the feeding tube is dislodged. In the end, it is the patient’s decision whether or not he/she would like a feeding tube if he/she is deemed capable of making healthcare decisions—but again, the decision should be discussed early and included in the advance directive if desired, as the patient may not continue to be able to choose for him/herself.
Do Not Hospitalize Order^
A “Do Not Hospitalize Order” (DNO) is one way for patients and families to influence the type of care patients and their families receive, and where the care takes place. During late-stage HD, people may prefer the warmth and comfort of a home, or they may wish to be hospitalized. There are numerous advantages and disadvantages to each choice. Hospitalization allows patients to have more advanced medical care. Physicians can more quickly diagnose and treat patients and have access to specialized equipment that might be needed to treat certain conditions. However, hospitalization is undesirable for some patients because hospital staff is less familiar with the patient and their individual needs. Patients sometimes become anxious in the unfamiliar surroundings, and may dislike the repeated diagnostic tests, such as blood pressure measurements.
Do Not Resuscitate Order^
A “Do Not Resuscitate” (DNR) order is written by the patient’s doctor in consultation with the health care proxy and other family members. This order states that cardio-pulmonary resuscitation (CPR) will be withheld when a person’s breathing or heart stops. When anyone is admitted to a hospital or nursing home, the staff is required by law to perform CPR if and when breathing or the heart stops unless there is a DNR order in effect (3). Like the “do not hospitalize” order, the DNR can be changed at anytime provided that the patient is mentally capable.
Brain donation involves donating brain tissue after death so researchers can use it to study HD and other neurodegenerative diseases. Researchers can use brain tissue to directly analyze nerve cells and brain changes due to HD in humans, which is an important step on the path towards better understanding HD and finding treatments that work.
There are several challenges that make brain donation challenging for some individuals.
First, the patient or his/her relatives must agree to donate the brain before the time of death. Second, not all institutions have the resources to pay for brain donation, as it costs between 10,000-30,000 US dollars to collect each brain.
Thinking Towards the Future^
While it may seem a bit overwhelming to face end-of-life care issues at a young age or during early stages of Huntington’s disease, it is extremely important to do so in order to ensure that patients’ true wishes are met even if patients are unable to make or communicate their decisions in the future. There is a lot of information available regarding each of these decisions, and speaking about each of them early on can potentially reduce stress during late-stage HD when family members and health care workers need to be aware of a patient’s desires. Finally, it is important to remember that the advance directive is a “living” legal document and any instructions specified in the document can be changed or revoked at the patient’s discretion at any time, as long as s/he has the mental capacity/communication abilities to do so.
Further Reading (GREAT LINKS!):^
Klager J., Duckett A., et al. Huntington’s Disease, A Caring Approach to the End of Life, Care Management Journals. 9.2 (2008).
This medium-difficulty article describes challenges in end-of-life care, identified in a long-term care facility specializing in Huntington’s disease
Kretzschmar H. Brain banking: opportunities, challenges and meaning for the future. Nat Rev Neurosci. 2009 Jan;10(1):70-8. Epub 2008 Dec 3.
This easy-to-read article describes how brain banking is important for science, and the challenges that stand in the way
Sheila A. Simpson, Late Stage Care in Huntington’s disease, Brain Research Bulletin. 72.2-72.3 (2007): 179-181.
This easy-to-read article describes issues that should be addressed in late stage care for HD.
P. Bakhai, 9.19.11