Kaitlyn+S

**Kaitlyn Savage **
 * Huntington's Disease **

**__DEFINITION: __** Huntington's Disease is a rare genetic disease that is inherited that causes the progressive degeneration of nerve cells in the brain. Huntington's Disease is an autosomal dominant disorder that has a extensive impact on functional abilities including movement, cognitive and psychiatric. While signs and symptoms don't develop until a person is in their thirties or forties, onset for the disease may be earlier or later in life. If onset of Huntington;s Disease begins before a person's twenties, it is called Juvenile Huntington's Disease. Juvenile Huntington's Disease results in slightly different presentation of symptoms and faster disease progression. While medications can be offered in order to manage symptoms but treatments can't prevent physical, mental and behavioral decline from Huntington's Disease. Comparison: (left) a brain affected by Huntington's Disease and a normal brain (right) **__HUNTINGTON'S DISEASE:__ Inheritance, Which Chromosome and People Affected.** __//Inheritance://__ Huntington's Disease is an autosomal dominant disorder, which means that only one copy of the defective gene is needed to inherit the disease. This happens when a parent passes on the defective copy of the gene instead of the healthy copy. Because of this, a child has a 50-50 chance of inheriting the disease. __//Which// //Chromosome://__ Huntington's Disease is caused by a mutation on a gene (HTT gene) in chromosome 4. The HTT gene provides instructions on making the protein Huntington which plays an important role in nerve cells in the brain. The mutation involves a DNA segment (CAG trinucleotide repeat) that is made up of cytosine, adenine and guanine. Normally this segment is repeated 10-35 times but when the mutation occurs repeated 36 to more than 120 times. People of 36-39 CAG segments might or might not develop signs or symptoms of the disease but people with a CAG segment repeated 40 or more times, almost leads to a development in the disease. __//People// //Affected://__ The disease affects an estimated 3 to 7 per 100,000 people of European ancestry and is less common in people of Japanese, Chinese and African decent.

__**SYMPTOMS:**__ The initial symptoms for Huntington's Disease greatly varies depending on the affected person. Some disorders that are associated with Huntington's are more dominant than others and some disorders have a great impact on the affected person. Impairments in voluntary movements will affect a person's ability to work,communicate, perform daily activities and stay independent. //Movement Disorders Associated with Huntington's:// Movement Disorders in Huntington's are both involuntary movements and impairment in voluntary movements. > 
 * Involuntary jerking or writhing movements
 * Muscle problems - more specifically rigidity or muscle contracture
 * Slow or abnormal eye movements
 * Impaired walk, posture and balance
 * Difficulty with physical production of speech or swallowing

//Cognitive Disorders Associated with Huntington's://
 * Difficult organizing, prioritizing or focusing on tasks
 * Lack of impulse - result in outbursts, acting without thinking
 * Slowness in processing thoughts or communicating words
 * Difficulty in learning new information

//Psychiatric Disorders:// While depression and weight loss are the most common due to injury of the brain and changes in brain function others include... Other disorders associated with Huntington's are obsessive-compulsive disorder( recurrent intrusive thoughts and behaviors), Mania (elevated mood, over-activity, impulsive behavior and inflated self-esteem) and Bipolar disorder (episodes of depression and mania)
 * Feeling irritability, sadness or apathy
 * Social withdrawal
 * Fatigue and loss of energy
 * Frequent thoughts of death and suicide

__**SYMPTOMS OF JUVENILE HUNTINGTON'S DISEASE**:__ Symptoms will slightly differ from onset in thirties or forties.

//Behavioral Changes:// //Physical Changes://
 * Loss of previously learned information academic or physical skills
 * Rapid or significant drop in overall school performance
 * Behavioral problems
 * Contracted and rigid muscles that affect walking
 * Changes in fine motor skills like handwriting
 * Tremors or slight involuntary movements
 * Seizures

__**TREATMENTS AND SIDE** **EFFECTS:**__
__**TREATMENTS:**__ Medicine can be offered in order to manage symptoms but treatments cannot prevent the progression of the disease. While the treatments will be changed based on the progression of the disease some drug will manage symptoms but result in side effects that may worsen other symptoms.

//DRUGS FOR MOVEMENT DISORDERS://
 * Tetrabenazine (Xenazine) suppresses the involuntary jerking and writhing movements
 * Possible side effects include drowsiness, nausea, restlessness and a serious risk of worsening or triggering depression or other psychiatric conditions.
 * Haloperidol (Haldol) and chlorpromazine have a side effect of suppressing movements
 * May worsen involuntary contractions and muscle rigidity
 * Risperidone (Risperdal) and quetiapine (Seroquel) may help suppress movement disorders but should be used cautiously as it can worsen other symptoms
 * Amantadine, levetiracetam (Keppra) and clonazepam (Klonopin) may also help with the movement disorders associated with Huntington.
 * Amantadine can worsen cognitive effects of Huntington's in high doses and may cause leg swelling and skin discoloration
 * Levetiracetam may cause nausea, stomach upset and mood swings
 * Clonazepam may worsen cognitive side effects of Huntington's and cause drowsiness ans well as a high risk of dependence and abuse

//MEDICATIONS FOR PSYCHIATRIC DISORDERS:// Psychotherapy, speech therapy, and physical therapy and occupational therapy can also manage symptoms of Huntington's
 * Citalopram (Celexa, Lexapro), fluoxetine (Prozac, Sarafem) and sertraline (Zoloft) may have side effects in treating obsessive-compulsive disorder such as nausea, diarrhea, drowsiness and low blood pressure
 * Quetiapine (Seroquel), risperidone (Risperdal) and olanzapine (Zyprexa) may suppress violent outbursts, agitation and symptoms of mood disorders or psychosis but may cause different movement disorders
 * Valproate (Depacon), carbamazepine (Carbatrol, Epitol, Equetro) and Iamotrigine (Lamictal) can help prevent the mood swings associated with bipolar disorder
 * Common side effects include weight gain, tremor and gastrointestinal problems

__**MANAGING COGNITIVE AND PSYCHIATRIC DISORDERS:**__ Creating an environment to help avoid stressors and manage cognitive and behavioral challenges...
 * Using calendars and schedules to help keep regular routine[[image:middletownhighschool/Treatment.jpg align="right"]]
 * Initialing task with reminders or assistance
 * Prioritizing or organizing work or activities
 * Breaking down tasks into manageable steps
 * Creating a calm, simple and structures environment (as much as possible)
 * Identifying and avoiding stressors that can trigger outbursts, irritability, depression or other problems
 * Providing opportunities for the person to maintain social interactions and friendships

**__PREVENTION:__**
People with a known family history may consider genetic testing. An at-risk parent who is considering genetic testing should see an genetic counselor who will disuses the risk of a positive test result. Other choices could be whether to have children or consider alternatives

**Work Cited** Genetic Science Learning Center. "Huntington's Disease." __Learn.Genetics__ 9 March 2016  "Huntington Disease." //Genetics Home Reference//. Web. 09 Mar. 2016.  "Huntington's Disease." //- Mayo Clinic//. Web. 09 Mar. 2016.  Pic 2 Fly." //Huntington's Disease Pedigree Chart Submited Images.// Web. 09 Mar. 2016.  Hdroster - About HD The Brain and HD." //Hdroster - About HD The Brain and HD//. Web. 09 Mar. 2016.  Make a Move Against Huntington'sDisease (HD) Chorea." //XENAZINE® (tetrabenazine) Tablets//. Web. 09 Mar. 2016.  Huntington's Disease Advocacy Center - HDAC Article: - A Review of the Treatment Options for HD Symptoms." //Huntington's Disease Advocacy Center - HDAC Article: - A Review of the Treatment Options for HD Symptoms//. Web. 09 Mar. 2016. 