Juvenile-HD

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Physical & Occupational Therapy In HD
Understanding Behaviour in HD-Dr. Jane Paulsen
Understanding Behavioral-Dr. Edmond Chiu
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Unified HD Rating Scale (UHDRS) Motor Section
Westphal Variant
SECTION 1 - AT RISK
Age & Probability Chart
At Risk For HD-What Next?
At-Risk Checklist
Best Interest of Child?
Crystal Ball?
Food For Thought
Parent Hasn't Tested?
Q&A On Risk of Inheriting JHD
Testing Children
SECTION 2 - GENETIC TESTING
Genetic Disorders & Birth Defects
Genetic Testing for HD
Genetic Counseling-In General
Psychological Impact
Intro: Genetics/Genetic Testing
Prenatal & Preimplanation
Prenatal Testing-In General
o Genetic Testing Resources
o Personal Stories
SECTION 3 - JHD
Coping With The Early Years
Age of HD Appearance
Age of Onset-Historical
Family-HD Underestimated
Children of Parents With HD
Child~Parent Ill
Clinical Description JHD
HD - What Kids Are Saying
HD & Me
JHD-Duration of Illness
JHD-Clinical and Research
JHD Symptoms
Parenting With HD
Patients/Families Coping
Talking With Children About HD
5 Stages of HD
JHD Resources
SECTION 4 - SYMPTOM RECOGNITION
Parent Resources
8 Fears of A Chronic Illness
Anxiety/Apathy/Irritability~HD
Anxiety, Fears & Phobias
Apathy-Physician's Guide
Ataxia
Attention-Perceptual/Unawareness Physician's Guide
Bed/Pressure Sores
Bed/Pressure Ulcer Guideline
Behavior Management
Bi-Polar Disorders
Botulinum toxin therapy
Bradykinesia
Caring Tips
Child Abuse-Reconizing Signs
Chorea-Physician's Guide
Chorea
Cognitive/Decision Making/Impulsivity
Cognitive-Short Tips
Contractures~Joints Locking
Dehydration-Physician's Guide
Dehydration
Delirium
Denial of HD
Depression~Physician's Guide
Depression-Understanding It
Depression-How To Help
Depression - Treatment Resistant Patient
Depression-Other Resources
-Read If Your Child Is On Antidepressant
Disgust - Impaired Recognition in HD
Dissociative disorders
Driving - Physician's Guide
Dyslexia
Dyslexia Resources
Dystonia
Dystonia/Rigidity & Spasticity Physician's Guide
Dystonia-Predominant Adult-Onset HD
Epileptic Seizures and Epilepsy
Epilepsy-Seizures~PG
-Seizures ~Special Populations
Falling~Safety
Falling - Subdural Hematoma Risk
Fevers - Unexplained
Fevers, sweating & menstural cycles in HD
GERD (Stomach)
HD Principle Treatments
Hallucinations/Psychosis~PGHD
Hand muscle reflexes in HD
Hypothalamus - A Personal Theory
Insomia ~Physician's Guide
Irritability~Temper Outburst Physician's Guide
Learning Disability
Mania/OCD~Physician's Guide
Mood Disorder Rate In HD
Myoclonus (Movements)
Nails-What To Look For
Night Terrors
Obsessive Compulsive OCD
Panic Disorder
Personality disorders
Pneumonia
Pneumonia-Advanced Stages
Pneumonia - Aspirated (Inhaled)
Prosody - Social Impairment
Sexuality~Physician's Guide
Skins Sensitivity
Sleep Disorders
Smoking-Physician's Guide
Spasticity
Stress
Tremors
Why Certain Symptoms Occur
Symptom & Treatment Resources
SECTION 5 - COMMUNICATION
Communication Resources
Communication Problems
Communication Strategies For HD~Jeff Searle
SECTION 6 - EATING/SWALLOWING/NUITRITION
Hints For Weight Loss in HD
HD & Diet~HSA Fact Sheet 7
Nutrients: Some Possible Deficiency Symptoms
Nutrition and HD~Anna Gaba (Recipes)
Nutrition Information In HD~Naomi Lundeen
Speech & Swallowing~Lynn Rhodes
Swallowing & Nutrition Physician's Guide To HD
Swallowing & Nuitrition Resources
Swallowing Warning Signs
5 Swallowing Problems
Taste changes in HD
Weight Gain
Resources-Drinks/Shakes
-Feeding Tubes~Advanced Stages of HD
-Feeding Tube~Jean Miller
-Feeding Tubes: One More Word ~Jean Miller
-Feeding Tubes & Baby Foods
-Feeding Tube~Dental Care
-Feeding Tube Instructions~Jean Miller
-Feeding Tube Resources
SECTION 7 - THERAPIES
Finding a Therapist - Behavoir
What Is A Physiotherapist?
Physical Therapy In HD
Speech-Language Therapy
Therapy Descriptions
Therapy Resources- Easter Seal
Therapy Resources
SECTION 8 - MEDICATIONS
HD Treatments
Medications-Movement Disorders
Medication/Emergency Info Forms
Cutting Prescriptions
Drugs-Look 'Em Up
-Adolescents Under 25
-Antidepressant Adverse Effects
-Anti-psychotic
-Anxiety-Antidepressant
A-Z Mental Health Drugs
-Creatine
-EPA~Fish Oil
-Haldol/Haloperidol - Clinical Sheet
-Haldol~Clinician Description
-Haldol & HD
-Haldol/HD Patient Experiences
-Haldol~ Patient Handout
-Mood Stabilizers: ASK 3 Questions
-Neuroleptic Malignant Synd WARNING
-Olanzipine-Risperidone/blood tests
-Celexa/Luvox/Paxil/Prozac/Zoloft
-Psychiatric Drugs & Children
Sertraline ~Zoloft
-Spasticity Meds/Treatments
-SSRI Medications
-Tardive Dyskinesia WARNING
-Weight Gain Medications
-Sites/Help the Medicine Go Down
-Vitamin & Mineral Deficiencies
SECTION 9 - SURGERIES
Surgery-Movement Disorders
o Surgery Resources
SECTION 10 - PROCEDURES
Clinic Visits-How To Prepare
CT Scans, MRI's etc.
Swallowing Tests
Tests Commonly Used
o Procedures Resources
SECTION 11- ALCOHOL/DRUGS
Alcohol-Parent's Guide
Alcohol-Talking To Your Child
Drugs-What To Do?
Drugs-Talking To Your Child
Disciplining-Ages 0-13 & Up
SECTION 12- SUICIDE
Straight Talk On Suicide
Teen Suicide-You Need To Know
o Suicide Resources
SECTION 13 - DIVORCE
Divorce & Child Stress
Tips For Divorcing Parents
SECTION 14 - DISABILITY ISSUES
Guides To Disability Issues
Caring-Child & Medical Technology
Caring for a Seriously Ill Child
Child Long Term Illness
Disability-Special Education Plan
IFSP Early Intervention Process
Disability Resources
Financial Planning
Wishes Can Come True-Children's Wish Foundations
Special Needs Resources
Special Needs Camp - About
Special Needs Camp - Finding One
SECTION 15 - ASSISTIVE TECHNOLOGY
Child Assistive Technology
Adaptive Equipment Resources
Products
SECTION 16 - EMOTIONAL ISSUES
Signs of Unhealthy Self-Esteem
Emotional Behavior Links
o Emotional Support Resources
SECTION 17 - GRIEF
Helping Child Deal With Death
o Grief Addtional Resources
SECTION 18 - ADD/ADHD
ADD & Teens
Conduct Disorders
FAQS & Related Info
Understanding AD/HD
What Is AD/HD?
Research Articles
Resources
SECTION 19 - HD SUPPORT GROUPS
HD Support Groups
National Youth Association
SECTION 20 - HD LINKS
HD Links
Related Resources
Tips For Friends
SECTION 21 - BENEFITS/INSURNACE
HD Disability
Benefits Check UP - See What You Can Get
Medical Insurance Bureau's Facts On You!
Medicare-Medicaid
Medicare Rights-Home Health & Hospice
Medicare Rights Center Resources
No Insurance? Try This!
Prescription Drug Cards Part I
Prescription Drug Cards Part II
Social Security-Children With Disabilities
SECTION 22 - ARTICLES/JHD
JHD and ADD
SECTION 23 - CAREGIVING
Articles-Resources
Caregiver Self-Assessment
Caregiver's Handbook
"First Shift With A Person With HD"
Getting Respite Care/Help At Home
Helpful Forms-Info
Home Emergency Preparations
Symptom Management
Ten Tips
Useful Tools
SECTION 24 - BIO
Our Personal Experience
Coping At The End
Kelly E. Miller
Song & Verse
Letter From My Heart
GUESTBOOK
Mania/OCD~Physician's Guide

INDEX Page

 
 

Chapter 5-Physician's Guide to Mangement of HD

Mania

While depression is the most common psychiatric problem in HD, a smaller number of patients will become manic, displaying elevated or irritable mood, overactivity, decreased need for sleep, impulsiveness, and grandiosity.

Some may alternate between spells of depression and spells of mania with times of normal mood in between, a condition known as bipolar disorder. Patients with these conditions are usually treated with a mood stabilizer. Lithium is probably still the most popular mood stabilizer for people with idiopathic bipolar disorder, but we have not found it to be as helpful in patients with HD.

It is not known why this is the case. Lithium has a narrow therapeutic range, particularly in patients whose food and fluid intake may be spotty, but there may be some other aspect to the mood disorders found in HD patients which make them poor lithium responders.

We recommend beginning with the anticonvulsant divalproex sodium (Depakote) or valproic acid (Depekene) at a low dose such as 125 to 250mg po bid and gradually increasing to efficacy, or to reach a blood level of 50-150mcg/ml. A dose of 500mg po bid is fairly typical, but some patients will require as much as several grams per day.

Another anticonvulsant, carbamazepine (Tegretol), is also an effective mood stabilizer. This can be started at 100-ZOOmg per day, and gradually increased by lOOmg/day to reach an effect or a therapeutic level of 5-IZmcg/ml, which may require a dose of 800-1 ZOOmg/day.

Therapeutic ranges for these drugs were established on the basis of their anticonvulsant properties, so it is important to remember that a patient may show a good psychiatric response below the minimum "therapeutic" level (but generally should not exceed the maximum level in any case).

Both drugs carry a small risk of liver function abnormalities (particularly divalproex sodium) and blood dyscrasias (particularly carbamazepine), and so LFT's, and CBC should be routinely monitored every few months and clinicians should be alert for suggestive symptoms.

Valproic acid may cause thrombocytopenia, and both drugs are associated with neural tube defects when used during pregnancy.

Manic patients with HD who have delusions and hallucinations may require a neuroleptic, and patients who are very agitated may need a neuroleptic or a benzodiazepine for immediate control of these symptoms.

As discussed for depression, the doctor may wish to prescribe one of the newer antipsychotics which have fewer parkinsonian side effects, such as risperidone, olanzepine, or quetiapine.

In cases of extreme agitation, a rapidly acting injectable agent, such as droperidol (Inapsine) or lorazepam may be necessary.

Finally, ECT is known to be a very effective treatment for idiopathic mania and should be considered when other treatments fail, or when the individual is extremely dangerous.

TABLE 15: MEDICATIONS USED FOR MANIA IN HD
MEDICATION STARTING DOSE MAXIMAL DOSE SIDE EFFECTS
Neuroleptics (see table 14) see table see table see table
Divalproex sodium 250mg 500-2000mg G.I. upset, sedation, tremor,
liver toxicity, throbocytopenia
Carbamazepine 100-200mg 1200-1600mg sedation, dizziness, ataxia, rash,
bone marrow suppression

Obsessive-Compulsive Disorders

Obsessions are recurrent, intrusive thoughts or impulses which are experienced as being senseless, at least initially.

A compulsion is a repetitive performance of the same activity, a stereotyped routine which must be followed, often in response to an obsession, such as handwashing because of an obsessive concern with germs.

Obsessions are usually a source of anxiety and the patient may struggle to put them aside, whereas the acting out of compulsions generally relieves anxiety and may not be as strongly resisted.

True Obsessive-Compulsive Disorder (OCD) is rare in HD, but HD patients often display an obsessive preoccupation with particular ideas. Patients may worry about germs or contamination, or engage in excessive checking of switches or locks. Sometimes patients will become fixated on an episode of being wronged in the past (e.g. fired from a job, divorced, driver's license revoked), and then bring it up constantly, or become preoccupied with some perceived need, such as a desire to go shopping, or to eat a certain food.

Serotonergic antidepressants are used to treat OCD and may ameliorate obsessions and compulsions in HD patients that do not meet the criteria for the full syndrome. The use of the tricyclic antidepressant clomipramine (Anafranil) has largely been superceded by the SSRIs fluoxetine, sertraline, paroxetine and fluvoxamine (Luvox) which have milder side effects and lower lethality in overdose.

Patients may require higher doses than those needed for depression, e.g. 40-60mg of fluoxetine. For relentless perseverative behavior unresponsive to these agents, one might consider neuroleptics, keeping in mind that the newer, atypical drugs may be better tolerated.

Schizophrenia-Like Disorders

Schizophrenia and schizophrenia-like conditions are much less common than affective disorder in HD.

The new onset of delusions and hallucinations should prompt a search for specific causes or precipitating factors, including mood disorders, delirium related to metabolic or neurologic derangements and intoxication with or withdrawal from illicit or prescription drugs.

Once these possibilities of mood disorder, drug intoxication, and delirium have been considered, neuroleptics may be employed for HD patients with schizophrenia-like syndromes. The doses used for treatment of psychosis may be somewhat higher than those used for treatment of chorea.

As mentioned before, if neuroleptics are not needed for the control of involuntary movements, patients may do better on newer agents such as risperidone, olanzepine or quetiapine which do not cause as many extrapyramidal side effects.

Some patients will respond completely and others only partly, reporting that "voices" have been reduced to a mumble, or becoming less preoccupied with delusional concerns.

Patients with delusions will rarely respond to being argued with, but a clinician may certainly express skepticism regarding a delusional belief and explain to the patient that it may be the product of a mental illness.

Caregivers should be encouraged to respond diplomatically, to appreciate that the delusions are symptoms of a disease, and to avoid direct confrontation if the issue is not crucial

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The National Alliance for the Mentally Ill (NAMI) is a nonprofit, grassroots, self-help, support and advocacy organization of consumers, families, and friends of people with severe mental illnesses, such  as schizophrenia, major depression, bipolar disorder, obsessive-compulsive disorder, and anxiety disorders.  URL: http://www.nami.org/illness/
 
  • List of Commonly Prescribed Psychotropic Medications - A list of commonly prescribed medications categorized by the mental illnesses they are used to treat as well as alphabetically by generic name.
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