Huntington’s Disease and a self-controlled death

I have been interested in Huntington’s Disease (also called Huntington’s Chorea) since the early 1960s, when I learned that this disease was killing Woody Guthrie, who died from its effects in 1967.

In his early published writing, Woody described how his mother Mary had been treated at a time before much was known about Huntington’s disease, from which she is believed to have suffered, also.  Her behavior had become wild, erratic, and inexplicable.  One day, without any notice to her children, Mary was sent to the “insane asylum” in Norman, Oklahoma, and the family was split up among relatives and others.

Around 1952, Woody was diagnosed with the disease, whose symptoms had become better known than they were in the 1920s.  The Mayo Clinic offers this analysis and description of the disease: 

Huntington’s disease is an inherited disease that causes the progressive breakdown (degeneration) of nerve cells in the brain. Huntington’s disease has a broad impact on a person’s functional abilities and usually results in movement, thinking (cognitive) and psychiatric disorders.  Most people with Huntington’s disease develop signs and symptoms in their 30s or 40s. But the disease may emerge earlier or later in life. . . . Medications are available to help manage the symptoms of Huntington’s disease, but treatments can’t prevent the physical, mental and behavioral decline associated with the condition.

The symptoms are potentially so devastating that they have been described by the Huntington’s Disease Society of America (HDSA), a group founded by Woody Guthrie’s second wife, Marjorie, as like having a combination of ALS, Parkinson’s, and Alzheimer’s simultaneously.

The Mayo Clinic provides these descriptions of Huntington symptoms divided into three categories:

The movement disorders associated with Huntington’s disease can include both involuntary movement problems and impairments in voluntary movements, such as:

• Involuntary jerking or writhing movements (chorea)
• Muscle problems, such as rigidity or muscle contracture (dystonia)
• Slow or abnormal eye movements
• Impaired gait, posture and balance
• Difficulty with the physical production of speech or swallowing

Impairments in voluntary movements — rather than the involuntary movements — may have a greater impact on a person’s ability to work, perform daily activities, communicate and remain independent.

Cognitive impairments often associated with Huntington’s disease include:

• Difficulty organizing, prioritizing or focusing on tasks
• Lack of flexibility or the tendency to get stuck on a thought, behavior or action (perseveration)
• Lack of impulse control that can result in outbursts, acting without thinking and sexual promiscuity
• Lack of awareness of one’s own behaviors and abilities
• Slowness in processing thoughts or ”finding” words
• Difficulty in learning new information

The most common psychiatric disorder associated with Huntington’s disease is depression. This isn’t simply a reaction to receiving a diagnosis of Huntington’s disease. Instead, depression appears to occur because of injury to the brain and subsequent changes in brain function. Signs and symptoms may include:

• Feelings of irritability, sadness or apathy
• Social withdrawal
• Insomnia
• Fatigue and loss of energy
• Frequent thoughts of death, dying or suicide

Other common psychiatric disorders include:

• Obsessive-compulsive disorder — a condition marked by recurrent, intrusive thoughts and repetitive behaviors
• Mania, which can cause elevated mood, overactivity, impulsive behavior and inflated self-esteem
• Bipolar disorder — a condition with alternating episodes of depression and mania

In addition to the above symptoms, weight loss is common in people with Huntington’s disease, especially as the disease progresses.

No one with Huntington’s is likely to develop all of these symptoms.  Each set of symptoms can be treated with drugs, but often with side effects that make other symptoms worse.  

It does not surprise me that someone with Huntington’s might want to control the manner and timing of his own death.  Such has been the case with several people whose lives have touched mine over the years.  One such person I will call Matthew.  He was a college graduate who, while still in college, was diagnosed with Huntington’s.  Several members of his family also had inherited the gene that causes Huntington’s, which is typical of many families once the gene is introduced into their genome, and he knew how the disease would progress.

By the time Matthew was in his mid-30s, Huntington’s was beginning to dominate his life, and he sought help.  His needs had surpassed the ability of his closest family members to continue caring for him.  He had moved in with a friend, in a city where he could receive good medical, social, and assisted living resources.  He accepted prescription medications to try to manage his anxiety, depression, and chorea (a movement disorder that causes involuntary, unpredictable body movements).  A service dog helped him with anxiety and depression.  A large group of supportive friends grew up around him and helped with the activities of daily living–cooking, transportation, daily hygiene, caring for his service dog, preventing falls, keeping appointments, avoiding hassles with the police who often thought he was drunk because of the way he walked and talked, a common problem for many Huntington’s sufferers.

Matthew had a medical support team that included a primary care physician, a physician’s assistant, a movement disorder specialist, a neurologist, a neurological specialist in Huntington’s, a therapist, and a psychiatrist.

In spite of his personal and medical support groups, Matthew’s condition continued to deteriorate.  In less than two years, Matthew was chronically hungry because of his swallowing problems.  His speech problems began to significantly interfere with his ability to communicate with others.  His symptoms were becoming more pronounced.  Despite constant encouragement from his friends, Matthew knew that he was losing the functional abilities he would need to control both his life and his death on his terms.

He learned how to use inert gas to hasten his death from research he did on the internet.  Matthew’s reasons for wanting to die at his relatively young age were based entirely on his Huntington’s symptoms.  He could no longer work.  Where he could live was limited.  Everything was getting harder:  standing up without falling; eating; talking; shaving; clipping his nails; taking care of his house, his hygiene, his dog.  His entire life had become increasingly difficult. 

Matthew wanted to control when he died.  He did not want to lose what small bit of independence he had left.  He had witnessed the final stages of Huntington’s for other family members.  He knew that living to the end of the disease was not something he wanted for himself.  He wanted a peaceful death that was not preceded by still more agony.

Matthew’s closest family members supported his wishes with both understanding and grief.  They remembered how he had managed the care of his father, and watched the prolonged deterioration of an uncle (who died around the same age Matthew was), both stricken with Huntington’s.  They had supported Matthew in every way possible, but accepted that it was becoming more difficult for him to continue to be the caring, funny, and vibrant person he had always been.  The treatments offered were not improving his life.  He was getting progressively worse with each week that passed.  Their sadness and discomfort at the thought of losing him did not overcome their respect for his right to decide when and how he would die.  They saw his decision as both legitimate and appropriate.

Volunteers from the Final Exit Network confirmed Matthew’s knowledge about using inert gas as a means to a peaceful, self-controlled death.  With the compassionate presence of two FEN volunteers and his beloved service dog at his side, Matthew overcame Huntington’s in the way he chose.