Juvenile-HD

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Physician's Guide To HD
Caring for People with HD
Physical & Occupational Therapy In HD
Understanding Behaviour in HD-Dr. Jane Paulsen
Understanding Behavioral-Dr. Edmond Chiu
Advanced Stages Caregivers Handbook
First Shift-Certified Nursing Assistants
Activities of Daily Living-HD
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
Depression~Physician's Guide

INDEX Page

 

Chapter 5 -HD Physician's Guide To HD

Depression

"Who wouldn't be depressed if they had HD?" Actually, research and clinical experience shows that many HD patients are not depressed, and are able to adapt gradually to having HD.

Nonetheless, even severe depression in someone with HD is often explained away as an "understandable" reaction, therefore not requiring additional treatment. This potential for overinterpretation exists in a variety of other serious medical conditions such as AIDS, stroke, and Aizheimer's disease, which have a high comorbidity with depression. In fact, those patients who have a depressive syndrome, even when the depression is "understandable," and even when there are clear triggers, usually respond to standard treatments, including medications and psychotherapy.

Because depression in HD appears directly related to the brain disease, pharmacotherapy is usually indicated.

TABLE 11: SIGNS AND SYMPTOMS OF DEPRESSION

  • Depressed or irritable mood
  • Loss of interest or pleasure in activities
  • Change in appetite, or weight loss
  • Insomnia or hypersomnia
  • Loss of energy
  • Feelings of worthlessness or guilt
  • Impaired concentration
  • Thoughts of death or suicide
  • Loss of libido
  • Feelings of hopelessness
  • Social withdrawal
  • Psychomotor retardation or agitation
(Based on DSM-IV criteria)

Major Depression is a clinical syndrome, a constellation of signs and symptoms which, taken together, suggest the diagnosis.

Use of diagnostic criteria helps to distinguish major depression from demoralization, transient changes in mood caused by negative life events, such as bereavement, and from some of the symptoms of HD itself, such as weight loss, trouble with concentration, and apathy.

Patients with Major Depression have a sustained low mood, often accompanied by changes in self-attitude, such as feelings of worthlessness or guilt, a loss of interest or pleasure in activities, changes in sleep, particularly early morning awakening, and appetite, loss of energy, and hopelessness. Depressed patients often feel worse in the morning than in the afternoon.

In severe cases of depression, patients may have delusions or hallucinations, which tend to match their depressed mood. A patient may hear voices berating him or urging him to commit suicide, or may have the delusion that he will be going to jail, or that he has killed his family.

Depressed patients often display psychomotor retardation, a slowing of speech and movement as a result of depression. In extreme cases they can appear stuporous or catatonic.

It is important to remember that because depression is a syndrome, with various symptoms and manifestations, the presenting complaint may be something other than a low mood. For example a depressed patient may complain of insomnia, anxiety, or pain, with each problem only a symptom of the depression which is the underlying cause.

It is vital to get the whole story, because symptomatic treatment for any of these complaints, e.g. sleeping pills, tranquilizers, or narcotics, could be worse than no treatment at all.

A specific complaint of depressed mood is not necessary to make the diagnosis if the patient has the other symptoms. In fact patients with HD often have trouble identifying or describing their emotional state. Depression in such a patient may be characterized by changes in sleep or appetite patterns, agitation, tearfulness, or a drop-off in functional abilities. In such circumstances the diagnosis should be considered.

In evaluating an HD patient with depression the physician also needs to consider whether some physical problem, other than HD, might be the cause. The patient's medical history should be reviewed for conditions such as hypothyroidism, stroke, or exposure to certain drugs associated with mood changes, such as steroids, reserpine, beta-blockers, and particularly alcohol.

Pharmacotherapy of Depression

Depressed people with HD can usually be treated with the same agents as any other patient with depression, but certain factors may make some drugs easier to use.

Many new medications have become available since the first edition of the Physician's Guide and the tricyclic antidepressants, while highly effective, should no longer be considered the standard first-line choice.

Instead, the physician should consider the Selective Serotonin Re-uptake Inhibitors (SSRIs), such as sertraline (Zoloft), paroxetine (Paxil), fluoxetine (Prozac), and fluvoxamine (Luvox). These offer the advantages of low side effect profile, once-a-day dosing, and safety in the event of overdose. Of these drugs, fluoxetine has a much longer half-life. If a patient develops an unpleasant side effect it will take longer to wear off. On the other hand this may make it a good choice for patients who sometimes forget to take their medicine.

TABLE 12: KEY POINTS IN THE TREATMENT OF DEPRESSION

  • Avoid overinterpretation of symptoms.
  • Depression is very common in HD. Have a low threshold for diagnosis and treatment.
  • HD patients are sensitive to side effects. Start medications at a low dose and increase gradually.
  • Ask about substance abuse.
  • Ask about suicide.

The SSRIs are sometimes stimulating and most patients should take them in the morning rather than at bedtime.

Initial side effects may be Gl upset or diarrhea, and increased anxiety or insomnia (although, if they are part of a depression, these symptoms will eventually respond to the treatment).

SSRI-induced insomnia may respond to 25-50mg of trazodone (Desyrel) qhs.

A small number of patients will develop sexual problems on SSRIs, particularly anorgasmia or ejaculatory delay. These symptoms are highly dependent on the dose.

Some people have asserted that SSRIs, particularly fluoxetine, cause violence or suicide in psychiatric patients. There is no valid evidence to support this claim.

Patients with HD are sensitive to the potential side effects of CNS drugs. Any new drug should be started carefully, and increased gradually. Sertraline 25-50mg, paroxetine lOmg, or fluoxetine lOmg are appropriate starting doses. If well tolerated, the dose can be increased after a few days or a week to sertraline 50-IOOmg, paroxetine 20mg, or fluoxetine 20mg. Most patients will respond to these doses, but sometimes higher doses will be necessary.

As we will discuss, SSRIs may also be particularly useful for some of the more nonspecific psychiatric symptoms found in patients with HD, such as irritability, apathy, and obsessiveness.

Other, newer antidepressants we have used with success in patients with HD include buproprion (Wellbutrin), venlafaxine (Effexor), and nefazodone (Serzone). These all require dosing several times a day. A new formulation of venlafaxine, Effexor XR, may be given once a day, and nefazodone is sometimes given in a single bedtime dose, despite the short half-life.

It is often difficult for depressed patients, especially those with cognitive impairment, to adhere to a complex medication regimen. Therefore these drugs may not be good first choices if there is no responsible family member who will help make sure that the patient takes his medicine.

Tricyclic antidepressants (TCA's) such as Nortiptyline (Pamelor), Imipramine (Tofranil) or Amitryptiline (Elavil) remain an important class of drugs for depression in HD. They can be given once a day (usually at bedtime because of sedative properties).

Common side effects of TCA's include constipation, dry mouth, tachycardia, and orthostasis. We tend to favor nortriptyline over the others because of the relatively low incidence of these side effects and because of the well-established range of blood levels which have been associated with efficacy. It is not necessary to reach the target blood level if the patient has already responded to a lower dose, but the availability of meaningful blood levels for the TCA's can serve as a useful check of compliance, and a reassurance that a patient's dose is optimal.

Since TCA's can worsen conduction delays, an EKG is indicated prior to treatment if the patient's cardiac status is unknown. TCA's are extremely dangerous in overdose. As little as a week's supply may be fatal if taken at once. They are a poor choice in patients with a history of deliberate overdoses and may have to be dispensed only a few pills at a time if this is a concern.

TABLE 13: MEDICATIONS USED TO TREAT DEPRESSION
CLASS MEDICATION STARTING DOSE MAXIMUM DOSE ADVERSE EFFECTS
SSRIs Fluoxetine IO-ZOmg 60-80mg insomnia, diarrhea, Gl upset, restlessness weight loss
Sertraline 25-50mg 200mg similar
Paroxetine 10-20mg 40-60mg similar, more sedation
Tricyclics Nortriptyline 10-25mg 150-ZOOmg dry mouth, blurry vision, constipation, hypotension, tachycardia, sedation
Other Nefazodone 50-IOOmg 450-600mg sedation, nausea, dry mouth, dizziness, constipation
Buproprion 100-ZOOmg 300-450mg seizures, agitation, dry mouth, insomnia, nausea
Venlafaxine 25-37.5mg 225 mg hypertension, nausea, headache, constipation
 
If the patient's depression is accompanied by delusions, hallucinations, or significant agitation, it may be necessary to add an antipsychotic medication to the regimen, preferably in low doses to minimize the risk of sedation, rigidity, or parkinsonism.
 
If the neuroleptic is being used for a purely psychiatric purpose, and is not required for suppression of chorea, the physician may want to prescribe one of the newer agents such as risperidone (Risperdal), olanzepine (Zyprexa), or quetiapine (Seroquel). These drugs may have a lower incidence of side effects and appear to be just as effective.
 
Among the older neuroleptics, high potency agents such as haloperidol (Haldol) or fluphenazine (Prolixin) tend to be less sedating, but cause more parkinsonism. Lower potency agents such as thioridazine (Mellaril) may aid with overactivity and sleeplessness, but tend to be constipating and can cause orthostasis.

Benzodiazepines, particularly short acting drugs such as lorazepam (Ativan) may be another good choice for the short-term management of agitation. In any case neuroleptics and benzodiazepines used for acute agitation should be tapered as soon as the clinical picture allows.

TABLE 14: SOME ANTIPSYCHQTIC MEDICATIONS USED IN HD
MEDICATION STARTING DOSE MAXIMAL DOSE SIDE EFFFCTS
Fluphenazine 0.5-2.5mg 20-30mg sedation, parkinsonism, dystonia, akathisia, hypotension, constipation, dry mouth, weight gain
Haloperidol 0.5-2.5mg 20-30mg same
Risperidone 0.5-lmg 4-6mg less parkinsonism, less dystonia
Olanzapine 2.5-5mg 15-20mg less parkinsonism, less dystonia
Quetiapine 25-50mg 500-750mg less parkinsonism, less dystonia
 
Electroconvulsive therapy (ECT) has also been found effective in depressed patients with HD. This treatment should be considered if a patient does not respond to several good trials of medication, or if an immediate intervention is needed for reasons of safety. For example a severely depressed patient may be refusing food and fluids, or may be very actively suicidal. ECT may be particularly effective in treating delusional depression.

Depressed patients should always be asked about substance abuse. Substance abuse, particularly of alcohol, can be both a consequence or a cause of depression, makes treatment difficult or impossible if not addressed, and significantly increases the risk of suicide.