Juvenile-HD
Q&A On Risk of Inheriting JHD

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SECTION 1 - AT RISK
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Q&A On Risk of Inheriting JHD
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SECTION 2 - GENETIC TESTING
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SECTION 3 - JHD
Coping With The Early Years
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SECTION 4 - SYMPTOM RECOGNITION
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Anxiety/Apathy/Irritability~HD
Anxiety, Fears & Phobias
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Dehydration
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Denial of HD
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-Read If Your Child Is On Antidepressant
Disgust - Impaired Recognition in HD
Dissociative disorders
Driving - Physician's Guide
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Dyslexia Resources
Dystonia
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Epileptic Seizures and Epilepsy
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-Seizures ~Special Populations
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Fevers, sweating & menstural cycles in HD
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Hand muscle reflexes in HD
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Mood Disorder Rate In HD
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Nails-What To Look For
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Panic Disorder
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Smoking-Physician's Guide
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Tremors
Why Certain Symptoms Occur
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SECTION 5 - COMMUNICATION
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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
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What Is A Physiotherapist?
Physical Therapy In HD
Speech-Language Therapy
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SECTION 8 - MEDICATIONS
HD Treatments
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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
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SECTION 14 - DISABILITY ISSUES
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Child Long Term Illness
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Special Needs Camp - About
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SECTION 15 - ASSISTIVE TECHNOLOGY
Child Assistive Technology
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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
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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

INDEX Page

Q&A On Risk of Inheriting JHD
 
Question:
I do have a question though........some genetic abnormalities
(not necessarily HD) are passed down more readily to a child of the opposite sex; like father to daughter or mother to son.  I am wondering if that factor plays into inheriting HD.

Response
To the best of my knowledge, no research has ever shown any
sex-linked relationship in the inheritance of HD except for the change in the number of repeats from generation to generation.

When HD is inherited from the father, the repeats are likely to be
higher than those of the father; when inherited from the mother, about the same.There is a lot of variability involved, though, especially when the father has HD.  Thus, it is possible for the offspring to have fewer repeats than the father; and it is also possible, if the mother has HD, for the offspring to have somewhat different repeats.

Age of onset is associated with the number of repeats, so that there
is a tendency among offspring of affected fathers for the age of onset to be earlier than that of the father; offspring of affected mothers tend to have onset at about the same age she did.

Question
I have written to the group about my grandson, who my husband
and I are raising, previously - but it has been quite a while, so let me give a little background.   
 
My grandson's mother developed symptoms at age 15-16 and is now 24 (he is 4).  His mother mom has a CAG repeat of 66.  I know that HD sometimes is dramatically worse when passed from a father to a child; but might that also be true of a mother passing it to a child, when the mother has a high CAG; and could age of onset then be earlier?

Response
"Worse" is a term which HD families and researchers might have
trouble agreeing on.  If Mr. A has more behavioral problems than Mr. B, but Mr. B has more chorea, who is worse?  Furthermore, HD may give Mr. A more severe symptoms at onset but Mr. B more severe ones as the Disease progresses.  Who has "worse" HD?

What most of us can agree on, though, is how fast the disease
progresses (and most of us would say that if the disease progresses faster in Mr. A than in Mr. B, Mr. A has "worse" HD).  So, if the father  has HD, then the odds of passing more repeats on to his offspring are greater than those of passing on fewer repeats, and that means that earlier onset is more likely than later onset.

I believe that it is generally accepted that juvenile HD progresses
faster than adult HD, so that the odds are that the offspring of a young HD father, if they inherit HD at all, will have it "worse" than the father.

In your daughter's case, if she passed the HD gene to her son, she
most likely passed the same number of repeats to him that she has, so he will probably have about the same age of onset as she did, and the Disease will probably progress as fast in him as it has in her.

Question
And in both cases (mother to child or father to child), does the child
being the opposing sex than the parent have anything to do with severity of transmission to the child?  (I know ANY transmission of the defective gene will be severe at some point in the child's life, but what I really mean is, with a mom who has higher than usual CAG repeats, would it be reasonable to expect a male child to have an earlier and more severe onset of symptoms, and would the answer to that question be any different if the child were a female?)

Response
No.  The number of repeats the child inherits has nothing to do with the
sex of the child.

Question
My grandson's grandfather - who was the parent passing HD on to my
grandson's mother - is his late 40's and now is symptomatic, but it only began within the last couple of years.  We have just recently learned that his brother - my grandson's great uncle (age unknown) is becoming symptomatic. 
 
The dilemma we are dealing with is this:   My grandson shows marked delayed development in every area - psychologically, cognitively, physically (coordination-dexterity).  He is in a pre-K class for delayed children.  Lately, he has been telling me his leg is 'shaking'.  On one occasion, when I felt the calf muscle of his leg, it was indeed tightening and relaxing repeatedly.  We had decided to not have him tested for HD, but now it seems more reasonable to do it, since the approach for helping him with his problem areas would be completely different if the reason behind them is HD, rather than some form of retardation or something.

Response
I think the best thing would be to have him  examined by a competent
neurologist, one who has considerable HD expertise.  If he thinks it's HD, he probably will recommend testing.  None of us are neurologists, to the best of my knowledge, and you really needs an expert.

Question
I think I am still just trying to sort it out in my own mind, and trying to
make a wise decision about testing.  I guess the biggest questions in my mind are: is there less of a chance that my grandson  would have HD since he will have inherited it from his mother, than if he had inherited it from a father?

Response:  No.

Question
And, if he did inherit the gene for HD, would the time and severity of onset
be expected to be later in life since it came from his mom, than it might have been if it had come from his dad?

Response
Yes, on the average (meaning that out of 100 cases of paternal transmission,
maybe 55-60 of them would result in more repeats and/or earlier onset).

Question
With his mom having such a high CAG, could we realistically expect that his
count will be at least as high, if not higher than hers?  (I know that the higher the CAG, symptoms would occur earlier and more intensively).

Response:
The odds of higher repeats would be about the same as those of lower repeats.


Thanks for bringing up these excellent questions.  You and your family are in a difficult situation, and I wish you the best.  "The best" includes an early treatment for HD, and I firmly believe that will happen.

Tom Caldwell
HDSA Tucson, Arizona Chapter
mailto:TECaldwell@msn.com