Patient Worthy signed up to cover several sessions at the Huntington’s Disease Society of America’s (HDSA) 36th Annual Convention, which was held virtually this year. This event features informative presentations and info sessions about the latest Huntington’s disease research geared towards the Huntington’s patient community.
We attended one session titled “HD Emergency Communication: How to prepare yourself and loved one for HD emergencies.” Jenna Mannone, whose mother passed from the disease in 2016, was the featured speaker during this session.
About Huntington’s Disease
Huntington’s disease is a heritable disorder that causes brain cells to die. This is a long-term, progressive, and ultimately lethal disease that causes severe debilitation over time. The disease is caused by a genetic mutation that affects the HTT gene. It normally appears between 30 and 50 years, but in rare cases it can occur before age 20. Symptoms of Huntington’s may first appear as subtle mood and behavioral changes and loss of coordination. Other symptoms include random movements called chorea, abnormal posture, sleep issues, trouble chewing, swallowing, and speaking, dementia, anxiety, depression, and impulsivity. Nine percent of deaths are the result of suicide. Treatment for Huntington’s disease is symptomatic, with no cure or disease-altering therapies available. Most patients die around 15 to 20 years after their diagnosis. To learn more about Huntington’s disease, click here.
Huntington’s disease can have a strong, negative effect on a person’s mood and emotional stability. This can lead to serious problems in which the patient either puts themselves or the people around them into potentially harmful or dangerous scenarios.
There are times in which caregivers may need to call emergency services, but in others, it may be more appropriate to de-escalate the situation. Huntington’s disease emergencies can be categorized as medical emergencies or mental health emergencies. Any scenario in which a person is in danger should be considered an emergency in which 911 should be called. Suicide and self-harm are major problems for some Huntington’s disease patients. Spend some time looking up suicide prevention hotlines/text lines, as these can be valuable, life-saving tools.
Aggression and agitation are common problems for patients. However, it is possible to de-escalate and calm the patient down. It may take time to learn what the person will respond to. Unfortunately, an important consideration with Huntington’s disease patients is their access to weapons and potential escape routes if a situation becomes volatile. De-escalation means not responding to hostility with more hostility. Jenna says learning to speak calmly in the face of aggression is critical, and your overall goal in these scenarios is to be safe, not “right.”
Warning signs of an impending conflict may include changes in behavioral patterns. A person may start acting “off” or have changes in their energy level. They may seem depressed or lose interest in hobbies that they used to enjoy.
Adult Protective Services should be contacted in some cases, such as:
- Elder abuse
- Involuntary isolation
- Chemical or physical restraint
- Inability of the person to care for themselves
In cases involving children, Child Protective Services should be contacted as well. Contacting these services should involve detailed explanations of who is involved, when the event occurred, and where the event occurred. It can feel difficult to contact external services, but remember that sometimes it is necessary for the safety of everyone involved.
It can also be useful to explain that Huntington’s disease is involved and to even have information, such as a card or pamphlet, on hand. In a hospital situation, be aware of who has the power to make decisions and of any medications that the patient is taking.
Emergency situations in Huntington’s disease can be difficult and frightening, but these tips can help you be more prepared if you find yourself in one.