Khai Ho International University of Nursing Professor Geri Mitchell Huntington   Running Head: Huntington Chorea
Table of Contents <ul><li>Introduction of Huntington Chorea (HD) </li></ul><ul><li>History </li></ul><ul><li>Prevalence </...
Introduction <ul><li>HD is an inherited, degenerative brain disorder which results in loss of both mental and physical con...
The Discovery of HD <ul><li>HD was first described by Dr. George Huntington in 1872 as a chronic progressive neurodegenera...
Prevalence <ul><li>It is rare but deadly, common among white European 7/100,000 </li></ul><ul><li>America: 30,000 people a...
Biochemistry/Pathophysiology <ul><li>HD is a chromosome disorder that occurs on 4p16.3 </li></ul><ul><li>At 4p16.3 junctur...
Biochemistry/Pathophysiology Continued Number of CAG repeating determine onset  Repeated count Classification Disease Stat...
Pathophysiology Continued <ul><li>mHtt will cause decaying of neurons in various regions of the brain such as the basal ga...
Huntington’s chorea <ul><li>Note loss of caudate and putamen </li></ul>
Symptoms <ul><li>Motor </li></ul><ul><li>Cognitive </li></ul><ul><li>psychosocial </li></ul><ul><li>Chorea, muscle rigidit...
Prognosis/Progression/Longevity <ul><li>Once affected, disease will progress </li></ul><ul><li>Prognosis, chronic disabili...
Genetic Association <ul><li>Single gene & chromosome related disorder </li></ul><ul><li>Is autosomal dominant disorder </l...
Genetic Testing <ul><li>Research is ongoing </li></ul><ul><li>Direct gene testing (predictive or pre-symptomatic testing) ...
Nursing Dynamic <ul><li>Nursing theory: Jean Watson theory of caring </li></ul><ul><li>Nursing Diagnosis: </li></ul><ul><l...
Nursing Dynamic continued <ul><li>Expected outcome </li></ul><ul><li>The pt will: </li></ul><ul><li>not injure himself </l...
Treatment & Intervention <ul><li>Monitor pt for self-harm (sleeping, home safety) </li></ul><ul><li>Keep pt away from shar...
Drugs Treatment <ul><li>1. Antipsychotics: hallucinations, delusions, violent outbursts </li></ul><ul><li>2. Antidepressan...
Nutritional  <ul><li>The pt requires more time to eat or be fed </li></ul><ul><li>Cut food in small pieces (choking) </li>...
Ethical/Legal/Political <ul><li>Ethical dilemmas  </li></ul><ul><li>Political issue </li></ul><ul><li>Individuals who are ...
Legal Issue <ul><li>Draw up pt will during early stage of disease </li></ul><ul><li>Legal assistance may be necessary if t...
Future Research and Treatment <ul><li>Intrabody therapy:  genetically engineered intracellular antibody: inhibit mHtt  agg...
Cost of Care <ul><li>Drugs </li></ul><ul><li>Caregivers  </li></ul><ul><li>Therapy </li></ul><ul><li>Dr. visit </li></ul><...
Reference <ul><li>Famiy Caregiver Alliance: Huntington Disease. Retrieved December 5, 2008 from FCA. Website:  http:// www...
Questions/ Quiz <ul><li>Which type of Huntington disease progresses faster and is more severe </li></ul><ul><li>Juvenile H...
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huntington chorea for nursing

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huntington chorea for nursing

  1. 1. Khai Ho International University of Nursing Professor Geri Mitchell Huntington Running Head: Huntington Chorea
  2. 2. Table of Contents <ul><li>Introduction of Huntington Chorea (HD) </li></ul><ul><li>History </li></ul><ul><li>Prevalence </li></ul><ul><li>Biochemistry/Pathophysiology </li></ul><ul><li>Genetic Association and Testing </li></ul><ul><li>Nursing dynamic </li></ul><ul><li>Ethical/legal/political issue </li></ul><ul><li>Reference </li></ul>
  3. 3. Introduction <ul><li>HD is an inherited, degenerative brain disorder which results in loss of both mental and physical control. </li></ul><ul><li>HD also known as Huntington Chorea (chorea mean to dance-like movement that is uncontrollable) </li></ul><ul><li>Onset before the age of 20y/o, the condition is call Juvenile HD </li></ul>
  4. 4. The Discovery of HD <ul><li>HD was first described by Dr. George Huntington in 1872 as a chronic progressive neurodegenerative disorder affecting movement, cognitive function, and personality. </li></ul><ul><li>In 1983 scientists discovered the Huntington's gene was associated with chromosome 4 . </li></ul><ul><li>After 10 more years of research, scientists have pinpointed the exact location of Huntington gene to be at 4p16.3 gene site on chromosome 4. The gene was known as IT15 (HTT). </li></ul>
  5. 5. Prevalence <ul><li>It is rare but deadly, common among white European 7/100,000 </li></ul><ul><li>America: 30,000 people are affected and 200,000 are at risk of inheriting the disease </li></ul><ul><li>Onset usually occurs between the age of 30-50, death usually follow 12 years after onset </li></ul>
  6. 6. Biochemistry/Pathophysiology <ul><li>HD is a chromosome disorder that occurs on 4p16.3 </li></ul><ul><li>At 4p16.3 juncture, the Huntington gene (HTT) contain a sequence of DNA bases (cytosine-adenine-guanine, CAG) </li></ul><ul><li>This sequence repeat many times (CAG-CAG-CAG-CAG-CAG-CAG-CAG-CAG) </li></ul><ul><li>Repeating of the CAG bases create a protein called huntingtin (Htt) </li></ul><ul><li>< 36 repeat is Htt but > 36 is mHtt </li></ul>
  7. 7. Biochemistry/Pathophysiology Continued Number of CAG repeating determine onset Repeated count Classification Disease Status < 27 Normal Unaffected 27-35 intermediate unaffected 36-39 Reduce penetrance +/- affected >39 Full penetrance affected
  8. 8. Pathophysiology Continued <ul><li>mHtt will cause decaying of neurons in various regions of the brain such as the basal ganglia, frontal and temporal lobe </li></ul><ul><li>Basal ganglia is responsible for motor function control, cognition, emotion, and learning </li></ul>
  9. 9. Huntington’s chorea <ul><li>Note loss of caudate and putamen </li></ul>
  10. 10. Symptoms <ul><li>Motor </li></ul><ul><li>Cognitive </li></ul><ul><li>psychosocial </li></ul><ul><li>Chorea, muscle rigidity, unbalanced gait, tremor, dysphasia and choking </li></ul><ul><li>Difficulty processing information and concentration, loss of memory, poor judgment, aphasia </li></ul><ul><li>Aggressive antisocial behavior, outburst of yelling and aggression toward others, depression </li></ul>
  11. 11. Prognosis/Progression/Longevity <ul><li>Once affected, disease will progress </li></ul><ul><li>Prognosis, chronic disability and death </li></ul><ul><li>Life expectancy is 15-20 years after symptoms occur </li></ul><ul><li>Rate of progression depend on number of repeated CAG </li></ul><ul><li>Progression usually begins with lack of concentration, short-term memory, uncontrollable muscular movements, depression, changes of mood (aggressive or antisocial behavior) and stumbling and clumsiness. It get more severe with times. </li></ul>
  12. 12. Genetic Association <ul><li>Single gene & chromosome related disorder </li></ul><ul><li>Is autosomal dominant disorder </li></ul><ul><li>HD is inheritance/ sporadic mutation due to (radiation, chemical, and viral infection) </li></ul>
  13. 13. Genetic Testing <ul><li>Research is ongoing </li></ul><ul><li>Direct gene testing (predictive or pre-symptomatic testing) </li></ul><ul><li>Pre-implantation Genetic Diagnosis (PGD) </li></ul><ul><li>Confirmatory testing </li></ul><ul><li>Pre-natal testing </li></ul><ul><li>Testing can cost $600-$1500 </li></ul>
  14. 14. Nursing Dynamic <ul><li>Nursing theory: Jean Watson theory of caring </li></ul><ul><li>Nursing Diagnosis: </li></ul><ul><li>Risk for injury </li></ul><ul><li>Risk for self-mutilation (suicide) </li></ul><ul><li>Risk for imbalance nutrition less the body requirement </li></ul><ul><li>Mobility impair </li></ul><ul><li>Risk for ineffective airway </li></ul><ul><li>Chronic confusion </li></ul><ul><li>Powerlessness </li></ul><ul><li>Hopelessness </li></ul><ul><li>Memory impairment </li></ul><ul><li>Impaired communication </li></ul>
  15. 15. Nursing Dynamic continued <ul><li>Expected outcome </li></ul><ul><li>The pt will: </li></ul><ul><li>not injure himself </li></ul><ul><li>Maintain normal wt </li></ul><ul><li>Not have skin break down </li></ul><ul><li>Interact socially with others </li></ul><ul><li>Maintain independence as possible </li></ul><ul><li>Maintain LOC </li></ul><ul><li>Able to communicate with care giver </li></ul><ul><li>Family Dynamic </li></ul><ul><li>Patience </li></ul><ul><li>Assist in meal </li></ul><ul><li>Monitor for self-injury </li></ul><ul><li>Support/comfort </li></ul><ul><li>Assist in legal issues </li></ul><ul><li>Knowledge deficit </li></ul><ul><li>Self-care deficit </li></ul>
  16. 16. Treatment & Intervention <ul><li>Monitor pt for self-harm (sleeping, home safety) </li></ul><ul><li>Keep pt away from sharp objects </li></ul><ul><li>Feed pt slowly </li></ul><ul><li>Use communication strategies </li></ul><ul><li>Encourage pt to be socially active </li></ul><ul><li>Group homes or nursing home </li></ul><ul><li>Outpatient therapy (physical/speech) </li></ul><ul><li>Educate family </li></ul><ul><li>Respite Care if at home </li></ul><ul><li>Hospice/palliative care </li></ul>
  17. 17. Drugs Treatment <ul><li>1. Antipsychotics: hallucinations, delusions, violent outbursts </li></ul><ul><li>2. Antidepressants: depression </li></ul><ul><li>3. Tranquilizers: anxiety, chorea </li></ul><ul><li>1. haloperidol, chlorpromazine, olanzapine (contraindicated if patient has dystonia) </li></ul><ul><li>2. fluoxetine, sertraline hydrochloride, nortriptyline </li></ul><ul><li>3. benzodiazepines, paroxetine, venlafaxin, beta-blockers </li></ul><ul><li>4. Tetrabenazine: chorea </li></ul>
  18. 18. Nutritional <ul><li>The pt requires more time to eat or be fed </li></ul><ul><li>Cut food in small pieces (choking) </li></ul><ul><li>Thick liquid in later stage (reduce aspiration) </li></ul><ul><li>Increased caloric intake </li></ul><ul><li>Insertion of PEG tube </li></ul>
  19. 19. Ethical/Legal/Political <ul><li>Ethical dilemmas </li></ul><ul><li>Political issue </li></ul><ul><li>Individuals who are carriers of HD want to have children </li></ul><ul><li>Psychological burden of genetic testing </li></ul><ul><li>Personal eugenic: abortion, Pre-implantation Genetic Diagnosis (PGD) </li></ul><ul><li>Stem cell Research </li></ul>
  20. 20. Legal Issue <ul><li>Draw up pt will during early stage of disease </li></ul><ul><li>Legal assistance may be necessary if the patient encounters discrimination over insurance or employment. </li></ul><ul><li>Insurance/employment can drop client if they found out the client is positive or carrier of HD </li></ul><ul><li>Abuse of the patient (sexual, physical, verbal. exploitation) </li></ul>
  21. 21. Future Research and Treatment <ul><li>Intrabody therapy: genetically engineered intracellular antibody: inhibit mHtt aggregation to delay neurodegeneration </li></ul><ul><li>Gene silencing: epigenetic process of switching off the mHTT gene </li></ul><ul><li>Stem cell implant: neural stem cells - a type of somatic (adult) stem cell or embryonic stem cell </li></ul>
  22. 22. Cost of Care <ul><li>Drugs </li></ul><ul><li>Caregivers </li></ul><ul><li>Therapy </li></ul><ul><li>Dr. visit </li></ul><ul><li>Genetic test </li></ul><ul><li>Genetic counseling </li></ul><ul><li>Hospital bill </li></ul><ul><li>2.5 billion a year </li></ul>Woody Guthrie
  23. 23. Reference <ul><li>Famiy Caregiver Alliance: Huntington Disease. Retrieved December 5, 2008 from FCA. Website: http:// www.caregiver.org/caregiver/jsp/content_node.jsp?nodeid =574 </li></ul><ul><li>Huntington Disease Society of America. The High Cost of Care. Retrieved December 5, 2008 from HDSA. Website: http:// www.lkwdpl.org/hdsa/dmb / </li></ul><ul><li>Millers Jean. The Physician Guide to Management of HD. Retrieved December 5, 2008 from HuntDiseaseFAQS. Website: http://endoflifecare.tripod.com/huntdiseasefaqs/id46.html </li></ul><ul><li>Neurology Channel. (January 15, 2008). Huntington Disease “Treatment and Prognosis”. Retrieved December 5, 2008 from Healthcommunities.com. Website: http:// www.neurologychannel.com/huntingtons/treatment.shtml </li></ul><ul><li>Kansas Medical Center. (June 13, 2003). Caring for People with Huntington Disease. Retrieved December 5, 2008 from Kansas Medical Center. Website: http:// www.kumc.edu/hospital/huntingtons / </li></ul><ul><li>Thulin, Perla Cassayre. (September 16, 2008). The Neurochemistry of Huntington Chorea. Retrieved December 5, 2008 from Docstoc. Website: http:// www.docstoc.com/search/huntingtons -chorea/ </li></ul><ul><li>University of Iowa. (2005). Huntington Chorea. Retrieved December 5, 2008 from University of Iowa Hospital and Clinic. Website: http://www.uihealthcare.com/topics/neurologicalhealth/neur3530.html </li></ul>
  24. 24. Questions/ Quiz <ul><li>Which type of Huntington disease progresses faster and is more severe </li></ul><ul><li>Juvenile Huntington </li></ul><ul><li>Adult Huntington </li></ul><ul><li>Nursing priority for a pt experiencing tremor during meal should be: </li></ul><ul><li>Do nothing, and wait for tremor stop </li></ul><ul><li>Remove sharp objects near pt </li></ul><ul><li>Prevent pt from choking on food </li></ul><ul><li>Administer a dose of Tetrabenazine to stop chorea </li></ul>
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