Juvenile-HD

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INDEX Page
Tools For Viewing
10 The Most Commonly Asked Questions
Clinical Trials & Research
Huntington's Disease~WeMove Info
Advocacy/Donations/Press Info
Clinical Definition & Search
Facing HD~Family Handbook
JHD Handbook-Chapter 1
JHD Info-Stanford Univ.
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
JHD Symptoms

INDEX Page

 
HD-Martha Nance, M.D
Juvenile Huntington's Disease

--------------------------------------------------
Diagnosis
Treatment And Services
Support
Research And Advocacy
About The Author
 

While Huntington's Disease (HD) primarily affects adults, in approximately
10 percent of the cases, onset of symptoms occurs before age 20.
Juvenile-onset HD presents unique challenges to families and caregivers.
Understanding the stages of juvenile HD will help caregivers and doctors
support and guide the children through the progression of the disease.
 
Diagnosis
 
Many physicians are slow to suspect or diagnose juvenile-onset HD.
The US HD Genetic Testing Group found that several factors were
characteristic of juvenile HD:
 
  • An affected father
  • Drooling or changes in speech or swallowing
  • Seizures
  • Severe behavior problems
  • Rigidity (stiffness)
  • Chorea (very uncommon in young children, but may be
    present in teenagers)
While a child with Juvenile HD may not exhibit every symptom, the more
symptoms shown, the more likely that the child has the condition.  A gene
test will confirm that the HD gene is present, but it cannot predict the time
of symptom onset nor can it prove the symptoms are caused by HD.
 
Treatment And Services
 
Children with HD usually need a range of services, which no single provider
can offer. HDSA Centers of Excellence for Family Services are comprehensive
medical centers that bring together multidisciplinary teams of professionals
who are skilled in assisting families facing HD. These may include: pediatric
neurologists; movement disorder specialists; physical, speech, and
occupational therapists; psychologists or psychiatrists; dentists and
pediatricians.
 
Although nothing can slow or stop the disease, some medications and therapies
may alleviate some symptoms. As symptoms increase, caregivers must become
familiar with local services for handicapped children-both at home and in school.
 
Support
 
Though Juvenile HD is rare, support is available through telephone and e-mail
networks, where caregivers can share resources and experiences. HDSA is an
excellent source for references and referrals to healthcare professionals,
educational specialists and Centers of Excellence.
 
NOTE: Find the following at http://clix.to/HDSupportInfo
 
(a) HD Center of Excellence
(b) HDSA Chapter near you
(c) HD Support peer-lead group
(d) Find an on-line HD support group
 
Research And Advocacy
 
Because of the rarity of Juvenile HD and fears about toxicity of drugs, only a
few clinical trials, such as the CARE-HD trial, have targeted Juvenile HD.
Research has not ignored Juvenile HD, though. It appears that the new
transgenic mouse models may actually be more representative of juvenile
than adult HD, due to the very large CAG repeats.
 
This may lead to breakthroughs in care or a cure. But until a cure is found,
caregivers and their health and social care providers must be at the child's
advocate at school, at home and at the clinic.
 
NOTE: To find out more about the HD Study Group and on-going clinical trials,
 
About The Author
Martha Nance, M.D., is the Director of the HDSA Center of Excellence for
Family Services at the Hennepin County Medical Center. She is an HDSA
Scientific Editorial Board Member and recognized expert on Juvenile HD.
 
NOTE:
Dr. M.A. Nance,
Department of Neurosciences,
Park Nicollet Clinic,
6490 Excelsior Blvd., Suite E 500,
St. Louis Park, MN 55426
612-993-3200/3360

~~~~~~~~~
 
The Physician's Guide to The Management of HD
under Overview & Principles of Treatment sections:
 
HD with onset in childhood has somewhat different features. Chorea is a much
less prominent feature, and may be absent altogether. Initial symptoms usually
include
  • attentional deficits
  •  behavioral disorders
  • school failure
  • dystonia
  • bradykinesia and sometimes
  • tremor
  • Seizures, rarely found in adults, may occur in this juvenile form.
Juvenile-onset HD tends to follow a more rapid course, with survival
less than 15 years. The vast majority of patients with juvenile onset
have inherited their HD gene from an affected father.  The reason for
this tendency is now understood in genetic terms and will be
 
~~~~~~~~~~~~~
Juvenile HD

Some affected individuals develop symptoms before age 20. Initial
signs of HD have been reported before puberty, or, in rare cases,
in children as young as four years of age or younger.

In those with juvenile HD, progressive loss of cognitive skills occurs
along with increasing muscle stiffness and resistance to movement
(rigidity) and relative slowness of movement (bradykinesia). This
form of the disease is sometimes referred to as "akinetic-rigid" or
"Westphal variant" HD. Although it most commonly occurs in juvenile
cases, the rigid form of the disease has also been reported in some
adults.

Typical findings associated with juvenile HD include a sudden decline
in academic performance, changes in handwriting, behavioral problems,
and seizures (epileptic convulsions). In addition, rather than the
chorea  typically seen in adult-onset disease, the movement abnormalities
of juvenile HD tend to resemble those observed in Parkinson's  disease.
Individuals with juvenile HD may experience muscle rigidity and
bradykinesia as well as tremors; brief, "shock-like" spasms of certain
muscles or muscle groups (myoclonus); progressively impaired control
of voluntary movement (ataxia); and dystonia. Juvenile HD typically
has a relatively rapid disease progression as compared with that observed
in adult-onset disease.