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
Swallowing & Nutrition Physician's Guide To HD

INDEX Page

Chapter 3 - Physician's Guide To The Management of HD
 
The Movement Disorder

Swallowing Difficulties

Dysphagia is, directly or indirectly, the most common cause of death in people with late stage HD, whether through choking, aspiration, or malnutrition.

Dysphagia results from impaired voluntary control of the mouth and tongue, impaired respiratory control due to chorea, and impaired judgment, resulting in eating too rapidly, or taking overly large bites of food and gulps of liquid.

Dry mouth, which can be brought on by neuroleptics, antidepressants, and anticholinergics, may worsen the problem.

TABLE 4: SWALLOWING TIPS

  • Eat slowley and without distractions.
  • Prepare foods with appropriate size
    and texture.
  • Eating may need to be supervised.
  • Caretakers should know the
    Heimlich maneuver.

 

 

 

 

 

No medications are known to improve swllowing directly.

Early referral to a speech-language pathologist will help identify swallowing difficulties, and periodic reassessment can identify changes in swallowing ability and suggest appropriate non-pharmacologic interventions such as a change in food consistency.

Devices such as enlarged grips tor silverware and nonslip plates with raised edges to prevent spilling may prolong independent eating.

HD affected individuals should be instructed early in the disease, before the onset of dysphagia, to eat slowly and deliberately, to sit in an upright position during and after meals, to take small bites, and to clear the mouth of food after each bite by taking sips of liquid.

Individuals with dysphagia should avoid doing other activities while eating, in order to concentrate on chewing and swallowing. For instance, patients should not talk while eating, nor be distracted by television or ambient noise.

Those who tend to hyperextend the neck due to chorea or dystonia should be encouraged and reminded to use a "chin-tuck" position. Drinking fluid through a straw may be easier than drinking directly from a cup, and the use of a covered cup or mug, like a "sippy cup" used by young children, may prevent spillage due to chorea.

Grainy items, such as ground beef or rice, may irritate the pharynx and cause choking. Foods such as steak, which are hard to chew, should also be avoided, or ground to a puree. Patients may have difficulty adjusting to different textures of food, and may do better if they finish each item on the plate in turn.

In late HD, when even liquids may be difficult to swallow, the texture of food should be soft and smooth, and liquids may be thickened with an additive (see Appendix 3). For those patients who may be unable to follow instructions reliably, a caregiver can cut the food in advance, and ensure that each mouthful has been completely chewed and swallowed before the next bite is begun. Supervision throughout the meal may be necessary, and the family or caregiver should be taught to perform the Heimlich maneuver.

In some cases, eating eventually requires so much energy and concentration that the patient becomes tired and frustrated before consuming adequate amounts of food.

Weight loss, very prolonged mealtimes or an inability to handle utensils may be the signal that he will need to be fed for at least part of the meal. Self-feeding may be prolonged by having the patient eat more frequent, but smaller meals, and by using "finger foods." The transition to assisted feeding does not have to be all or nothing, as patients may still be able to eat unassisted at certain times and be fed at other times.

Choking may decrease once self-feeding is stopped, because the caregiver will have greater control over the size and frequency of the bites. The caregiver should still promote eating slowly, and not talking while eating, and should make sure the mouth is empty before each bite.

With supervision, most patients are able to assist with feeding and to take adequate amounts of food by mouth quite far into the illness. However, before dysphagia and communication difficulties become severe, the issue of feeding tubes should be discussed with the patient and family, to ensure that appropriate nutrition can be maintained throughout the illness.

A gastrostomy tube can clearly improve nutritional status in a debilitated person with severe dysphagia, and may prolong life. However, patients and families may not desire this intervention late in the course of HD. The question of whether to use a gastrostomy tube, and other end of life issues are discussed in the final section of chapter 6.

Nutrition

Weight loss is a common problem in Huntington's disease. This is probably due in part to diminished food intake because of dysphagia, fatigue, and depression. However many HD patients also require a large caloric intake to maintain their body weight. This may be simply due to the expenditure of energy through involuntary movements, but there may be other metabolic reasons not fully understood.

Two strategies can be employed to increase the caloric intake of someone with HD: increase the number of meals, or increase the calorie content of the food.

The first goal can be achieved by eating five small meals a day or by adding high calorie snacks such as milkshakes. The caloric content of the food can be increased by measures such as adding oil to soups, drinking cream instead of skim milk, adding margarine liberally as a condiment, and focusing on easily eaten, high-calorie foods such as pasta with cream based sauce.

Consultation with a nutritionist can help in selecting the most appropriate foods and supplements to meet the patient's needs. Regaining lost weight sometimes results in improved alertness and responsiveness, and often appears to reduce chorea as well.

Maintaining hydration is also very important, particularly in the summertime in patients who may not be able to request fluids.

Cyproheptadine, an antihistamine, given as 4mg at bedtime, may help increase weight by stimulating appetite in some patients.