When someone is diagnosed with Huntington’s disease, it’s not just a medical label-it’s a life shift. The news doesn’t come with a cure, a quick fix, or even a clear timeline. Instead, it brings a cascade of questions: How did this happen? What will my body do next? How do I plan for a future I can’t control? This isn’t theoretical. For families affected by HD, these are daily realities.
The Genetic Time Bomb
Huntington’s disease isn’t caught like a cold or triggered by lifestyle. It’s inherited. If one parent carries the mutated gene, each child has a 50% chance of getting it. No exceptions. No luck. Just pure, cold genetics. The problem lies in a single gene on chromosome 4 called HTT. Inside that gene, a tiny sequence-CAG-is repeated too many times. Normal people have 10 to 26 repeats. If you have 40 or more, you will develop Huntington’s. There’s no gray area. Between 36 and 39, it’s uncertain-some will, some won’t. But 40 and above? It’s guaranteed.The more repeats, the earlier the symptoms start. Someone with 50 repeats might show signs in their teens. Someone with 45? Maybe their early 30s. And if the mutation comes from the father, it’s more likely to grow bigger during sperm production. That’s why 85% of juvenile cases-those starting before age 20-come from the dad’s side. This is called genetic anticipation. It’s not magic. It’s biology. And it means a father who lived into his 60s with mild symptoms could pass on a version so aggressive that his child is wheelchair-bound by 16.
That’s why genetic testing is so heavy. People who have a parent with HD often wait years-sometimes decades-to get tested. Why? Because knowing doesn’t change the outcome. It just changes how you live. A 2023 survey from the European Huntington’s Disease Network found that 72% of at-risk individuals delay testing until symptoms appear. They’re not avoiding it out of ignorance. They’re protecting their peace.
Chorea: The Unwanted Dance
If you’ve never seen chorea, it’s hard to describe. Imagine your body moving without permission. Fingers twitch. Shoulders shrug. Feet tap. Then your head jerks. Your arm swings out like you’re waving at someone who isn’t there. These movements aren’t rhythmic. They’re random. Wild. Unpredictable. And they disappear when you sleep.This isn’t just embarrassing-it’s exhausting. People with chorea can’t hold a cup without spilling. They can’t walk a straight line. They can’t sit still in a meeting. At first, it’s mild. Maybe just a fidgety hand or an odd facial tic. Doctors call this a UHDRS score of 1 or 2. By the time it hits 3 or 4, the movements are constant. The body doesn’t rest. The person doesn’t rest.
Chorea happens because the brain’s control center-the striatum-is dying. Specifically, the neurons that calm movement are the first to go. Without them, the body overreacts. Every signal becomes noise. And as the disease progresses, chorea doesn’t stay alone. It brings friends: stiffness, slow movements, muscle cramps. What looked like dancing becomes a tangled mess of muscle failure.
The only FDA-approved drugs for chorea are tetrabenazine and deutetrabenazine. Both work by lowering dopamine in the brain. They can reduce movements by about 25-30%. But they come with a cost. One in five people get depressed. Many feel drained, sluggish, or emotionally flat. That’s why some patients refuse them. They’d rather keep the chorea than lose themselves.
There’s a newer option, valbenazine, approved in 2023. It’s slightly less likely to cause depression, but still carries risks. And it doesn’t stop the disease. It just tries to quiet the noise.
Care Planning: The Real Treatment
Here’s the truth most people don’t talk about: There is no cure. But care planning saves lives. A 2021 study from the Enroll-HD global registry showed that patients who had structured care plans lived 2.3 years longer than those who didn’t. Why? Because they avoided hospitalizations. They didn’t choke on food. They didn’t take their own lives. They didn’t get lost in the system.Care planning isn’t a one-time meeting. It’s a roadmap that changes as the disease moves. In the early stage-right after diagnosis-people need to talk about what they want if they can’t speak for themselves. Only 37% of patients in general neurology clinics do this. At specialized HD centers, it’s 82%. That gap is deadly.
By year five, most people need help with daily tasks. Bathing. Dressing. Eating. Occupational therapists teach new ways to hold a toothbrush. Speech therapists help with slurred speech. Nutritionists adjust diets to prevent choking. By year ten, nearly half of patients need speech therapy. By year fifteen, most can’t live alone.
By year twenty, 89% need full-time residential care. The average annual cost? $125,000. And that’s in the U.S. Many families spend over $5,000 out-of-pocket each year on therapies insurance won’t cover. Aquatic therapy helps balance better than land-based exercises. But not everyone can afford a pool.
Coordination is the biggest hurdle. One caregiver told the HDSA forum: “I spend 20 hours a week just scheduling appointments.” Neurologist. Psychiatrist. PT. OT. Social worker. Dietitian. Genetic counselor. Each one has their own office, their own schedule, their own paperwork. Getting them to talk to each other? That’s the real challenge.
Specialized HD centers-there are 53 in the U.S.-do this well. They hold quarterly team meetings. They keep full care plans. They train staff in HD-specific communication. But only 53% of them actually meet quarterly. Insurance doesn’t pay enough. Staff burn out. Patients fall through.
What Works-And What Doesn’t
There’s a lot of hype around new drugs. Wave Life Sciences’ SELECT-HD trial showed a 38% drop in mutant huntingtin protein after 135 weeks. Roche’s tominersen trial was paused, then restarted with lower doses. These are promising. But they’re years away from being widely available. And even if they work, they won’t fix what’s already broken.Right now, the most effective treatment isn’t a pill. It’s a team. A care plan. A conversation.
People who start planning early live longer. They stay out of the ER. They avoid feeding tubes. They die with dignity, not chaos. One doctor at Johns Hopkins put it plainly: “Even with successful gene therapies, comprehensive care planning will remain critical for the 40,000+ Americans currently living with HD.”
That’s the hard truth. Science might one day stop Huntington’s. But today, it’s care planning that keeps people alive.
Where to Start
If you or someone you love has been diagnosed:- Find an HD specialty center. Use the HDSA website to locate one. They know the system. They’ve seen this before.
- Do advance directives now. Not someday. Not when you’re “ready.” Now. A living will. A healthcare proxy. Name who speaks for you.
- Get genetic counseling. Even if you’re not tested, understanding inheritance helps your family.
- Build your team. Neurologist, therapist, social worker. Don’t wait for a crisis to call them.
- Connect with others. The HDSA forum and r/huntington on Reddit are full of people who get it. They share tips, resources, and sometimes just silence.
There’s no perfect path. But there is a better one. And it starts with planning-not waiting.
Is Huntington’s disease always inherited?
Yes. Huntington’s disease is caused by a single mutated gene passed down from parent to child. If a parent has the mutation, each child has a 50% chance of inheriting it. In rare cases (under 1%), a person can have the mutation without a known family history due to a new mutation or misattributed parentage, but this is extremely uncommon.
Can you get Huntington’s disease if neither parent has it?
Almost never. HD is an autosomal dominant disorder, meaning you need only one copy of the mutated gene to develop it. If neither parent carries the mutation, you cannot inherit it. Very rarely, a new mutation can occur during sperm or egg formation, but this is estimated to happen in less than 1% of cases.
What does chorea look like in early-stage Huntington’s?
In early stages, chorea is often subtle. It might look like fidgeting, restlessness, or minor facial grimacing. People may seem clumsy-dropping things, stumbling slightly, or having trouble with fine motor tasks like buttoning a shirt. These movements are involuntary and disappear during sleep. Often, family members notice them first, thinking it’s just nervous energy or stress.
Are there any drugs that stop Huntington’s from progressing?
No. Currently, no medication can slow or stop the underlying brain degeneration caused by Huntington’s disease. Treatments like tetrabenazine, deutetrabenazine, and valbenazine only manage chorea symptoms. Experimental therapies targeting the mutant huntingtin protein are in clinical trials, but none have been proven to halt progression yet.
Why is care planning so important in Huntington’s disease?
Care planning directly impacts survival and quality of life. Studies show that patients with structured, early care plans live 2.3 years longer on average. They’re less likely to be hospitalized for pneumonia, falls, or suicide. Planning ensures someone can speak for the patient when they can’t speak for themselves. It coordinates specialists, prevents gaps in care, and helps families prepare emotionally and financially for the long haul.
How much does Huntington’s disease care cost?
The average annual cost of care in the U.S. is about $125,000 per person by the late stages. This includes medications, therapy, home care, and eventually residential facilities. Out-of-pocket expenses for non-covered services-like specialized therapy or adaptive equipment-can exceed $5,000 a year for many families. Total U.S. healthcare spending on HD is estimated at $1.5 billion annually.
Can genetic testing predict exactly when symptoms will start?
Not precisely. While the number of CAG repeats correlates with likely age of onset-more repeats usually mean earlier symptoms-it’s not exact. Two people with 45 repeats might develop symptoms 10 years apart. Other genetic and environmental factors play a role. Testing gives a range, not a calendar date.
What’s the difference between tetrabenazine and deutetrabenazine?
Both reduce chorea by lowering dopamine levels. Tetrabenazine (Xenazine) was approved in 2008 and requires multiple daily doses. Deutetrabenazine (Austedo), approved in 2017, is chemically modified to last longer, so it’s taken twice daily instead of three or four times. Austedo has a slightly lower risk of depression and sedation, which is why it now holds 65% of the chorea treatment market.
aditya dixit December 4, 2025
It’s wild how genetics doesn’t care about your plans. You can meditate, save money, learn five languages - none of it matters when your DNA has already written the ending. But knowing that doesn’t make it easier. It just makes you more intentional. I’ve watched my uncle go from chess champion to needing help tying his shoes. He never got tested. Said he’d rather live with hope than live with certainty. I get it.
There’s no heroism in waiting. Just humanity.
And yeah - care planning isn’t glamorous. But it’s the quietest form of love there is.