Nur 212 Huntington's Disease

Front 1

HUNTINGSTON'S CHOREA
-WHAT IS IT?

Back 1

HD is an inherited autosomal dominant neurologic disease. It is progressive, degenerative and characterized by increasing dementia and chorea.

Front 2

*FYI*
Chorea means dance

Back 2

*FYI*

Front 3

HUNTINGTON'S CHOREA
-PATHOPHYSIOLOGY
A)WHAT CELLS DOES HD EFFECT/WHERE ARE THESE CELLS LOCATED?

Back 3

HD causes localized death of neurons in the basal ganglia (where GABA neurons are)and usually effects the cells in the cortex and cerebellum.
-The cortex and cerebellum is located in the brain.
-HD causes the loss of both the cortex and cerebellum cells

Front 4

-WHAT CAUSES HUNTINGTON'S DISEASE?
-WHAT GENDER (M/F) AND RACE DOES IT EFFECT MORE?

Back 4

-The is no known cause of HD
- HD effects both men and women of all races

Front 5

-WHAT NEURONS ARE DECREASED AS A RESULT OF THE DISEASE AND HOW DOES IT EFFECT OTHER NEURONS?

Back 5

Huntington's Disease decrease the acetycholin levels and gamma-amniobutric acid (GABA) which causes an increase in dopamine (which causes the jerking motions).

Front 6

WHAT AGE RANGE IS A HUNTINGTON'S DISEASE CARRIER (PT. ACTUALLY WITH THE DISEASE BUT WITHOUT SYMPTOMS) REMAIN ASYMPTOMATIC?

Back 6

A pt. who has Huntington's disease is usually asymptomatic between 35-45yrs old before they start showing signs of the disease.

Front 7

HUNTINGTON'S DISEASE
-CAUSE (TRANSMISSION)
-HOW/WHEN CAN A PERSON BE TESTED FOR THE DISEASE?

Back 7

-HD is genetically inherited: autosomal dominant ; either sex can inherit and transmit the disease.
-Testing can be done on an embry (via blood test).

Front 8

HUNTINGTON'S DISEASE
-WHAT'S THE CHANCES (%) THAT A CHILD WITH ONE PARENT WITH HD WILL INHERIT THE DISEASE?
-WHAT'S THE CHANCE (%) THAT A CHILD WHO DOES NOT HAVE THE DISEASE WILL PASS IT DOWN TO THEIR OWN OFFSPRING?

Back 8

-A child born to "a/one" parent with HD has a 50% chance of inheriting the disease.
-HD does not skip generations, therefore a child who has NOT inherited the disease will NOT pass on the disease to their own children

Front 9

HUNTINGTON'S DISEASE
-ARE THERE ANY TIMES WITHOUT THE AID OF PHARMACOLOGY WHEN CHOREA DECREASES?

Back 9

Chorea although still present decreases when the patient is sleeping.

Front 10

HUNTINGTON'S DISEASE
MANIFESTATIONS (Motor effects)
-Early symptoms

Back 10

-Fidgety
-Unsteady gait & Falls
-Slurred speedch & Self care deficit

Front 11

HUNTINGTON'S DISEASE/
MANIFESTATIONS (Motor effects)
-Late symptoms

Back 11

-Facial grimicing
-Dysphagia & Chorea
-Impaired diaphragmatic movement
-Incontinecnce & Confined to bed

Front 12

HUNTINGTON'S DISEASE
-WITH IMPAIRED DIAPHRAGMATIC MOVEMENT WHAT IS THE PT. AT RISK FOR?

Back 12

-The patient is at risk for pneumonia

Front 13

HUNTINGTON'S DISEASE
MANIFESTATIONS (Psychosocial effects)
-Early symptoms

Back 13

-Irritability
-Depression (suicidal)
-Mood swings (could be r/t the illness or the though of knowing they have the disease).

Front 14

HUNTINGTON'S DISEASE
MANIFESTATIONS (Psychosocial effects)
-Late symptoms

Back 14

-Decreasing memory
-Dementia
-Total dependence

Front 15

HUNTINGTON'S DISEASE
-DIAGNOSTIC TESTING; WHAT METHODS ARE USED TO HELP DIAGNOSE THE ILLNESS?

Back 15

-Clinical presentation (S&S)
-Positive family history
-Genetic Testing for Chromosome 4
-Blood and amniotic fluid test
*95% accurate*

Front 16

HUNTINGTON'S DISEASE
-ANTIPSYCHOTICS
A) MEDS
B) HOW DO THEY WORK?

Back 16

-Thiothixene hydrochloride (Navane)
-Haloperidol decanoate (Haldol)
-These antipsychotics work by blocking the dopamine receptors which improves chorea

Front 17

HUNTINGTON'S DISEASE
-TRANQUILIZERS

Back 17

-Klonopin (clonazepam)

Front 18

HUNTINGTON'S DISEASE
-WHAT ARE THE DIFFERENT TYPES OF MEDICATIONS AND IT'S PURPOSE?
-NEWLY APPROVED ANTIPSYCHOTIC DRUG; WHAT ARE IT'S COMMON SIDE EFFECTS?

Back 18

1. Antipsychotics to block the dopamine receptors which improve chorea Haldol (Haloperidol decanoate), Navane (thioxthixene hydrochloride); hallucinations, dilusions anger and violent outburst
2. Tranquilizers to help with anxiety, tics and chorea
3. Antidepressents to help with depression, chorea and OCD
4. Antihypertensive with a off-lable use as a antipsychotic to reduce aggitation and chorea
5.Nitoman (tetrabenazine). The first approved drug in the US for Huntington's disease as treatment of chorea.
a)Side effects: Depression, suicide *akathisia-motor restlessness, usually with overmedication, important to realize because it can be mistaken for the restless fidgity of the illness.

Front 19

*FYI*
DOPAMINE:
It's one of three most abundate catecholamines (the others norepinephrine and epinephrine). All important to the flight or fight response. It's produced in several areas of the brain. Dopamine can be stimulated by meds to increase the heart rate and BP. Dopamine has important roles in behavior, cognition, voluntary movement, motivation, punishment and reward.

Back 19

*FYI*

Front 20

HUNTINTON'S DISEASE
(MEDICATIONS)
-ANTIDEPRESSANTS

Back 20

-Prozac (fluoxetine)
-Zoloft (sertraline)

Front 21

HUNTINGTON'S DISEASE
(MEDICATION)
-NEW ANTIPSYCHOTIC
A)APPROVED BY THE FDA AUG.'08
B) SIDE EFFECTS

Back 21

-Nitoman (tetrabenazine)
-Side effects; suicide and most common depression (decreases the level of seretonin) in addition akathesia (restless pacing inability to keep still).

Front 22

HUNTINGTON'S DISEASE
(NURSING DIAGNOSIS)
1. Altered Nutrition
2. Risk fo fall
3. Hopelessness

Back 22

*fyi*

Front 23

HUNTINGTON'S DISEASE
(NURSING INTERVENTIONS)
-Patients do better with consistancy, having the same nurse
-Reinforce postive behavior and maintain consistency with all caregiving
-Offer emotional support to client and family to relieve anxiety and enhance coping.
-Give the pt. extra time to express themselves (remember the pt has dysarthria-indistinct speech), this decreases frustration
-Important to know the patient's likes and dislikes
a)How they like to be turned/moved
b)What calms the patient, what makes them anxious
-Be alert for S&S of despression to prevent suicide
-Make the environment safe; free from instruments that could permit self-inflicted inury

Back 23

*fyi*

Front 24

HUNTINGTON'S DISEASE
(PATHOPHYSIOLOGY)
Involves a disturbance in neurotransmitter substances, primarily gamma aminobutyric acid (GABA) located in the basal ganglia, along with the cortex and cerebellum are destroyed and replaced with glial. The deficiency of GABA causes an excess of dopamine.(Med-Surg Incredibly Easy).

Back 24

*FYI*

Front 25

HUNTINGTON'S DISEASE
(HOW IS IT TREATED). (Med-Surg Incredibly Easy).

Back 25

-There's no cure so treatment focuses on support and protecting the patient, treating symptoms and providing emotional support to the patient/family.
-Meds help control depression, discomfort and choreic but can't stop mental deterioration

Front 26

HUNTINGTON'S DISEASE
-TEACHING TIPS (Med-Surg Inceredibly Easy)

Back 26

-Talk with the pt.and family about the illness
-Give pt. time (dysarthria)
-Genetic counseling prior to starting a family because a child has a 50% chance of inheriting the disease
-Refer to support groups and organizations to help cope (VNA, long-term facilities,psychiatric counseling).

Front 27

HUNTINGTON'S DISEASE
-JUVENILE HD
A)WHAT IS IT?
B)HOW MANY ARE EFFECTED (%)?

B) WHAT SIGNS & SYMPTOMS DO THESE PATIENT'S HAVE?

Back 27

A) Juvenile HD is when the pt. has the symptoms of the disease prior to 20 years old and can start as early as 14yrs. old
B) 10% can have juvenile HD
C) Pt's have the same symptoms but the earlier the onset of the symptoms the faster it's progression.

Front 28

HUNTINGTON'S DISEASE
(MANIFESTATIONS)
-WHAT NON-PHYSICAL BUT PSYCHOLOGICAL TRAIT IS COMMON AMONGST PATIENTS WITH HD?

Back 28

-Patient's with HD often have obsessive-compulsive traits
*helped with antidepressants

Front 29

HUNTINGTON'S DISEASE
(MEDICATION)
-WHAT'S A COMMONE SIDE EFFECT OF TRANQUILIZERS?

Back 29

-Klonopin (clonazepam) can cause additional stiffness and ridgity.

Front 30

HUNTINGTON'S DISEASE
-Early symptoms may disappear as the disease progress.

Back 30

*FYI*