Huntington’s disease is a key medical-surgical topic from the nervous system section that every nurse should master.
It’s also something every aspiring nurse should know about, since it’s a big part of the NCLEX.
This is why, today, I share with you my complete guide to Huntington’s disease, from what it is to what causes it to how to treat it and everything else in between.
And that’s not all!
I’ll also be sharing with you ten practice questions and answers so you can ace this part of the NCLEX when you take the test.
Are you ready?
Let’s dive in!
Everything You Need to Know About Huntington’s Disease
Huntington’s disease, also known as Huntington’s chorea, is a progressive brain disorder and an autosomal dominant genetic disorder. The disease presents with uncontrolled movement, emotional problems, and dementia.
What Is an Autosomal Dominant Genetic Disorder?
Someone with an autosomal dominant genetic disorder will inherit an abnormal gene from one parent. The other parent’s genes could be normal but the abnormal gene is dominant, so the child will have a 50% chance of being affected by the condition.
The Signs and Symptoms of Huntington’s Disease
The symptoms of Huntington’s disease include chorea, which is an involuntary movement that affects the arms, legs, and facial muscles. It gives the appearance that the affected patient is dancing. Chorea is not considered a life-threatening symptom, but it is the classic sign of Huntington’s disease. It can affect the arms and the face more than the legs. It usually starts out unilaterally, and can then progress from there.
Dementia is another symptom of Huntington’s disease. It starts out mild and then becomes more severe and affects the personality. Memory loss, poor impulse control, depression, mania, and personality changes are all cognitive and psychiatric issues that we note over time.
Usually, the signs of Huntington’s disease appear in patients between their mid-thirties and mid-forties. Still, the onset may occur earlier or later in life. Speech and swallowing difficulties may be seen later on, as the disease progresses.
Juvenile Huntington’s Disease
Juvenile Huntington’s disease is when the onset of Huntington’s disease begins before the age of twenty.
The first signs usually are behavioral changes, epilepsy, and learning difficulties. The average life expectancy for juvenile Huntington’s disease is ten to fifteen years after diagnosis.
What Causes Huntington’s Disease?
Huntington’s disease is due to the excess repeats of CAG, aka cytosine-adenine-guanine. The cerebral cortex is where the brain learns and stores different motor patterns.
Basal ganglia is responsible for motor control, behaviors, and emotions and these are switched off by the indirect pathway. This prevents unwanted muscle contractions from competing with voluntary movements.
To initiate a wanted movement, the motor cortex sends the signal to the basal ganglia to activate the specific motor pattern through the direct pathway.
Huntington’s disease affects the neurons in the indirect pathway. This damages the ability to switch off the motor pattern, so uncontrollable movements occur. Understanding this will help you remember what Huntington’s disease is and how it occurs.
How to Diagnose Huntington’s Disease
Someone with signs and symptoms of Huntington’s disease plus a family member diagnosed with this genetic disorder will have a very high chance of having it, so we perform genetic testing for the CAG repeats for confirmation.
PET scans can also be performed to detect the disease. We can also perform CT scans and MRIs to look for brain atrophy, which would also confirm Huntington’s disease.
How to Treat Huntington’s Disease
Do not be fooled on your exams when you see a choice for curing Huntington’s disease, because there is no cure. However, Huntington’s disease can be treated by managing the symptoms and providing emotional support for the patient and family.
Tetrabenazine (xenazine) is commonly used to treat the chorea. Antipsychotics and antidepressants may also be prescribed for symptom management. Remember that treatment will not slow down mental deterioration.
Nursing Management
As a nurse to a patient with Huntington’s disease, you need to:
- Support the patient’s activity of daily living with hygiene, bowel and bladder care, and nutrition.
- Assess and evaluate the patient’s mobility and functional abilities. Nursing interventions must be done to assist the patient with daily living while maintaining the patient’s independence.
- Create a plan of care to maintain the patient’s safety and keep the patient free from injuries. Examples include ensuring the wheelchair is locked in place or having handrails in the shower.
- Observe for potential depression or suicidal ideation. Being diagnosed with Huntington’s disease is overwhelming and challenging, so make sure that the patient and their family are being heard. Be alert for signs and symptoms of suicidal ideations and ensure that the environment is free of potential tools for self-harm.
Nursing Considerations
As a nurse to a patient with Huntington’s disease, you should also:
- Educate the patient and family on the genetic disorder and provide additional time to answer questions. Due to the patient’s mental deterioration, they will require time to process information.
- Encourage genetic counseling if the patient wants to start a family and educate that their offspring will have a 50% chance of inheriting Huntington’s disease.
- Refer the patient and family to resources, such as organizations or groups for Huntington’s disease or counseling.
Huntington’s Disease Questions and Answers
Question #1: The nurse is reviewing the dietary recommendation for a 44-year-old patient in the late stage of Huntington’s disease. Which of the following foods should the nurse question?
- Walnuts
- Ice cream
- Soup
- Mashed potatoes
Answer: Walnuts.
Rationale: Those with Huntington’s disease should eat small, frequent meals that are soft and easy to chew. The patient is in the late stage of Huntington’s disease, so the ability to swallow may be affected. Maintaining patient safety is done by preventing potential patient harm, such as choking hazards. Walnuts are hard and may be difficult to chew, which should not be given to someone with Huntington’s disease.
Question #2: What type of genetic disorder is Huntington’s disease?
- Autosomal recessive disorder
- Autosomal dominant disorder
- X-linked dominant
- X-linked recessive
Answer: Autosomal dominant disorder.
Rationale: Huntington’s disease is an autosomal dominant disorder. An example of an autosomal recessive disorder is cystic fibrosis. Fragile X syndrome is an example of x-linked dominant disorder. Hemophilia is an example of x-linked recessive disorder.
Question #3: The nurse is providing education to a 35-year-old female patient with a family history of Huntington’s disease. Which of the following statements indicates that further education is required?
- “I should consider genetic testing.”
- “I am a carrier for Huntington’s disease so I should look into IVF options.”
- “My husband does not have Huntington’s disease so my child should be fine.”
- “I can still have children.”
Answer: “My husband does not have Huntington’s disease so my child should be fine.”
Rationale: Huntington’s disease is an autosomal dominant genetic disorder. The offspring will inherit an abnormal gene from one parent. The other parent’s genes could be normal but the abnormal gene is dominant, so the child will have a 50% chance of being affected by the condition. Genetic testing should be considered. The patient can look at IVF options, which can play a role in screening embryos for Huntington’s disease and transferring embryos that do not have it. Those with Huntington’s disease can still have children, but should be encouraged for further education and be given emotional support.
Question #4: The nurse is providing education for a 45-year-old patient newly diagnosed with Huntington’s disease. Which of the following statements indicates that the patient requires further education?
- “There is a cure for Huntington’s disease.”
- “I will inform the staff if I am getting suicidal ideations.”
- “I should tell my daughter to get genetic testing for Huntington’s disease.”
- “There is no cure for Huntington’s disease.”
Answer: “There is a cure for Huntington’s disease.”
Rationale: Unfortunately, there is no cure for Huntington’s disease. The other statements are correct. Suicidal ideations should be reported and genetic testing for family members should be encouraged.
Question #5: Nursing student Logan is studying about nervous system disorders and is learning about Huntington’s disease. Which of the age groups does Huntington’s disease usually first appear?
- Childhood
- Teens
- 20s and 30s
- 30s and 40s
Answer: 30s and 40s.
Rationale: Huntington’s disease usually first appears in the 30s and 40s. Juvenile Huntington’s disease is when the onset of Huntington’s disease begins before the age of 20.
Huntington’s Disease True or False Statements
Statement #1: Huntington’s disease is a hereditary disease.
This statement is true.
Statement #2: Patient safety should be prioritized by keeping the patient free from injury.
This statement is true.
Statement #3: Huntington’s disease is an autosomal dominant genetic disease.
This statement is true.
Statement #4: There is a cure for Huntington’s disease.
This statement is false.
Statement #5: Children of someone with Huntington’s disease have a 25% chance of inheriting the disease.
This statement is false. Children of someone with Huntington’s disease have a 50% chance of inheriting the disease.
