Advance Directives Can Help Clarify End-of-Life Care for HD Patients
The importance of discussing your wishes regarding death and dying
Thinking about death isn’t pleasant, and neither is talking about it. But my wife, Jill, who is gene-positive for Huntington’s disease (HD), asked me to write about the topic.
In particular, she wanted me to discuss the importance of making a living will. Being proactive in this manner makes sense to her because of what happened while we were living in Florida years ago.
In the Tampa area, a media circus erupted around the plight of a young woman named Terri Schiavo. In early 1990, Schiavo was resuscitated after going into cardiac arrest, but she’d suffered massive brain damage. She became comatose and was later declared to be in a persistent vegetative state.
Several years later, her husband requested that she no longer be kept alive by artificial means. Her parents disagreed, saying she would have wanted to live, and a protracted legal battle ensued. The saga become a national drama, as the case involved numerous lawsuits, court appeals, and extensive political interventions at the state and federal levels.
In short, it was a hot mess.
I covered this story as a freelance journalist as the wrangling neared its conclusion. Eventually, Schiavo’s husband won, and her feeding tube was removed. She died in the spring of 2005.
The importance of advance directives
When I interviewed people about the case, I was struck by how many felt so strongly about Schiavo’s plight. I can’t imagine being in her family’s shoes, but to me, the saddest part was that all of the legal fighting could have been avoided if Schiavo had executed an advance directive before her collapse.
Most states recognize two main components of advance directives. One is commonly referred to as a living will, which allows you to make end-of-life healthcare decisions now. For example, you can declare whether you wish to be kept alive through the use of artificial breathing machines or feeding methods.
The other main element is a durable power of attorney, which allows you to appoint a healthcare proxy. This person can make medical decisions for you in the event that you’re unable to do so.
When properly drafted and executed, an advance directive is legally binding and must be honored. Had Schiavo executed a directive, her wishes would have been clear and honored without the financial and emotional toll of litigation.
The Schiavo saga stuck with Jill. She decided that it’s important for everyone to talk about their wishes regarding death and dying.
She’s told me that she doesn’t want to be put on artificial life support if she ever becomes brain-dead, nor does she want a feeding tube when she’s no longer able to eat. We’ve both created living wills so that no one can go against our wishes.
Jill knows that her ability to think and make decisions will one day be impaired due to Huntington’s disease, so she’s expressed how she wants to live and die. I promise to honor her wishes.
I realize this is not an easy topic to discuss or follow through on. Denial about HD or one’s mortality can be great deterrents to this difficult conversation. But most HD families have a pretty good idea of what’s in store for those who are gene-positive, so after witnessing the bitterness that surrounded the Schiavo case, I realize we must be as relentless as the disease in preparing legally for the end.
Note: Huntington’s Disease News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website. The opinions expressed in this column are not those of Huntington’s Disease News or its parent company, Bionews, and are intended to spark discussion about issues pertaining to Huntington’s disease.
About the Author
Peter Ellis
Hi, i live in the uk. On August 1st of this year, 2022, my wife died. She was gene positive with Huntingtons disease. Around 8 years ago my wife told me what she wanted at the end of her life, and then around 3 years ago we went to see our GP and completed a 'respect' form. The respect form details what her end of life wishes are in the uk, it must be signed by the GP to become 'live'
In Late July of 2022, my wife got an infection, the gp was called, she explained that they needed to take my wife to hospital. I discussed with the GP what was on the form, these were: not to go into a care home, hospital or hospice, but to stay at home for the remainder of her life. Not to be given CPR.
Not to have a PEG fitted, for feeding and drug treatment. Not to be given any drugs that were to keep her alive, without the hope of any quality of life.
My wife, who was called Linda, could only speak one or two words at a time.
She could not answer a question, her brain cognition was vertually gone.
she could not lift her arms or legs.
she was double incontinent.
she could not open her eye lids but could focus her eyes if i held them open.
In discussion with her doctor and two children, it was decided not to treat her but let her eventually pass away.
It was very painfull for me to let, and watch, this happen. I dearly loved her, and still do, i miss her every day. But it is what she had asked me to do, and the completed respect form supported this. I hope this helps.
Carlos Briceño
Thank you, Peter, for sharing your journey with us. I can feel your heartbreak and can tell you loved your wife very much. It is never easy to take care of anyone with HD, but the late stages of this disease are particularly cruel to the patient and their loved ones. As hard as it is to follow the wishes of the person suffering from HD, it takes great love and courage to witness what happens to them before they pass on. I hope that you have some support with what you are going through. Jill always describes losing a loved one that you spend so much time taking care of as odd. You are happy they aren’t suffering anymore, and you are sad they are gone, but the caregiver part of you feels a little lost. Being a caregiver is 24 hour-7- days a week job. It becomes your life and when that ends there’s a gap that needs to be filled. Hopefully, you are filling it with your support network. Jill and I will keep you in our prayers.
A.
Hi! I have a question. I know this isn't the most appropriate place to ask, but I will still try my luck, so here it goes: If a child has the same CAG repeats as their mother (who has HD), will the child start showing symptoms at nearly the same age? (or do they show earlier in each progressive generation) And, will the disease progression be similar?
Also, thank you so much for writing on HD so regularly! All your columns hit very close to home.
Carlos Briceño
Hello,
Thank you for reaching out and for reading our columns. I am sorry that the stories hit home for you. I think your question is a very valid one. I can say that HD affects everyone differently and there are a lot of different factors that go into when symptoms appear. As I am not an expert, I cannot give you a clearer answer. If you have questions, you can reach out to the place that the test was performed and they may be able to help you with any questions you may have. You can also reach out to one of the social workers at your local HDSA center of excellence. If you click on the link below you can locate many resources.
Locate resources - Huntington's Disease Society of America hdsa.org.