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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
5 Stages of HD

INDEX Page

 
 
There are various papers discussing the "stages" of Huntington's
Disease.  This page reflects a few of them.
_____________________________________________________________
 
Alternate Stages Of Huntington's Disease
by Jim Pollard and Rosemary Best
Stated in positive terms from the person's point-of-view.

Defiance              "I'm not denying the diagnosis, I'm defying
                         the verdict! I'm not refusing to accept it,
                         I'm just boldly resisting the inevitable!"
 
Perseverance     "I'm continuing on...in spite of all the
                         difficulties this damn disease puts in front
                         of me."
 
Compassion       "I'm sorry for the trouble I'm causing my family
                         and everyone else who cares for me. I wish I
                         could do something to help them."
 
Stamina              "I'm not sure just what it is but something
                         keeps me going! It keeps me going through all
                         my fatigue and all the problems and hardship
                         this damn disease presents me."
 
Grace                   "I've quietly resigned myself to needing others
                        to care for me, to sustain me. I can't show them,
                        but I'm more concerned for the welfare of those
                        around me than I am for myself. We know we're
                        there for each other."
April 1996
Toronto
__________________________________________________________________
How Does Huntington's Disease (HD) Progress?

The five stages, as described in "Towards A Fuller Life
- A Guide to Everyday Living With Huntington's Disease",
published by the HDSA, contains the following description.

PROGRESSION:
A general guide toward understanding how Huntington's
Disease progresses was developed by Dr. Ira Shoulson,
Professor of Neurology at the University of Rochester
Medical Center in 1979. 
 
Briefly, these stages are as follows:

The progression of HD after a person is diagnosed
can be divided into five (flexible) stages:

Early Stage - the person is diagnosed as having HD
and can function fully both at home and work.

Early Intermediate Stage - the person remains
employable but at a lower capacity. They are still
able to manage their daily affairs despite some
difficulties.

Late Intermediate Stage - the person can no longer
work and/or manage household responsibilities. They
need considerable help or supervision to handle daily
financial affairs. Other daily activities may be slightly
difficult but usually only require minor help.

Early Advanced Stage - the person is no longer
independent in daily activities but is still able to live
at home supported by their family or professional
carers.

Advanced Stage - the person with HD requires
complete support in daily activities and professional
nursing care is usually needed. People with HD usually
die about 15 to 20 years after their symptoms first
appear. The cause of death is not the disease itself
but complications such as pneumonia, heart failure or
infection developing from the body's weakened
condition.
__________________________________________________________
 
An overview of Huntington disease
May 2002

In addition to the physical symptoms of HD, there are
often subtle cognitive signs as well.
 
Initial stages
 
People with early Huntington's may find they have
difficulty organizing routine matters or coping
effectively with new situations. Difficulty recalling
information may make them appear forgetful. Work
activities may become more time-consuming, and
decision-making and attention to detail may be
impaired.

Early emotional symptoms may be equally subtle.
Individuals at this stage may experience more periods
of depression, apathy, irritability, or impulsiveness, or
perhaps changes in personality.

At this stage, people with Huntington's can function
quite well at work and  home.

Intermediate stages

As the disease progresses, the symptoms become
worse.  The initial motor symptoms will gradually
develop into more obvious involuntary movements
such as jerking and twitching of the head, neck, arms
and legs.  These movements may interfere with
walking, speaking and swallowing.

People at this stage of Huntington's often look as if
they're drunk:  they stagger when they walk and their
speech is slurred. They have increasing difficulty
working or managing a household, but can still deal
with most activities of daily living.

Advanced stages

The advanced stages of Huntington's typically involve
fewer involuntary movements and more rigidity. People
in these stages of HD can no longer manage the activities
of daily living, (Activities of Daily Living-HD) and they
usually require professional nursing.   Difficulties with
swallowing, communication and weight loss are common.

Death usually occurs 15 to 20 years after the onset of
the disease.  People don't  die from Huntington's itself,
but from complications such as choking, heart failure,
or infection.

Juvenile HD

About 10 per cent of Huntington's cases are considered
"juvenile"  -  the symptoms appear in childhood or
adolescence.

The symptoms of juvenile HD are different from the
adult disease.   Children with HD move slowly and
stiffly, they have increasing difficulty learning, and
they can have convulsions or epileptic seizures.
Some children have severe behavioral problems.

The earlier the disease onset, the more likely the child
is to be very rigid and appear very different from the
majority of adult cases. This often makes it difficult to
diagnose. Children with HD are more likely to have an
affected father than an affected mother.

Diagnosis, testing, and treatment

Huntington's is usually diagnosed using neurological
and psychological tests. Sometimes doctors use brain
scans to see whether the caudate and putamen are
working properly, or they use a recently devised
genetic test to confirm diagnosis.

The genetic test uses a blood sample to reveal whether
or not an individual has the gene that causes HD.

Currently, there are no treatments that slow down or
stop HD.   Some drugs will reduce symptoms such as
depression, anxiety, and involuntary movements. These
drugs may have side effects so not every Huntington's
 patient can use them.

Neurologists, psychologists, genetic counselors and
social workers play an important role in helping
individuals and families deal with HD.
 
~~~~
Dr. Alex Goumeniouk, a foremost expert of the disease
and a psychiatrist at UBC's Huntington's Disease Medical
Clinic, says working directly with HD patients is some of
the most rewarding work he does. He says,
 
"I think the reason for this is that the true resilience of the
human spirit is palpably present in these patients. They have
reconciled that, short of being "hit by a truck," they will die
from this illness. While there is an adjustment phase, patients
emerge from this with a new perspective on life."

Source: Huntington Society of Canada and UBC Huntington
Disease Medical Clinic
_________________________________________________________
 
And yet another ;-)  Just keep in mind that everyone is unique
and therefore will not conform to the "standard" stages shown
here! They were written by the authors as guidelines only, to
help families understand the progression of HD.
 
THE STAGES OF HD

Though the pattern and severity of symptoms vary from
person to person, the course of HD can be roughly divided
into three stages.

Early Stages
Early in the disease, manifestations include subtle
changes in coordination, perhaps some involuntary
movements, difficult thinking through problems, and
often, a depressed or irritable mood.

At this stage, medications are often effective in treating
depression and other emotional symptoms. It is a good
time to begin planning for the future. Financial  plans
should be made and legal documents drawn up.

Middle Stages
In the middle stage involuntary movements (chorea)
may become more pronounced. A staggering gait can
sometimes be mistaken for drunkenness.
 
Speech and swallowing will begin to be affected.   It is
important to consult a speech therapist who will be
able to offer suggestions and strategies for improving
communication and swallowing abilities. 
 
Likewise, occupational and physical therapists can
develop programs to help maintain the highest level
of functioning and thereby improve quality of life.
 
Thinking and reasoning skills will also gradually diminish. 
At this stage it may become increasingly difficult to hold
a job and to carry out household responsibilities.
 
Here again, simple strategies may be employed to help
decrease frustration, increase functioning and prolong
independence. For example, disorientation and short
-term memory loss can be addressed by labeling
drawers, maintaining a daily routine and posting a
calendar appointments and events.

Late Stages
People with late stage HD may have severe chorea, but
more often have become rigid. Choking on food becomes
a major concern, as does weight loss. At this stage people
with HD are totally dependent on others for all aspects
of care, can no longer walk, and not able to speak.

Although cognitive abilities are severely impaired, it is
important to remember that the person is generally still
aware of his/her environment, remains able to
comprehend language, and remains an awareness of
loved ones and others.  He/she may continue to enjoy
looking at photographs and hearing stories of family and
friends.

People do not die from HD itself but rather from a
complication of the disease, such as choking or infection.
Death generally occurs about 15 to 20 years after onset.