HEHI Exam 3 Set 6-CP and Down Syndrome

What is the diagnostic criteria for an Intellectual Disability?
1. Intellectual functioning (IQ)
2. Functional strengths/weaknesses Impairement in at least 2/10 adaptive skill domains.
3. Must have onset <18 years

What are the 10 adaptive skills domains used in diagnoses of Intellectual Disability?
Communication
Self -Care
Home living
Social
Community
Self direction
Health/Safety
Functional academics
Leisure
Work

What standardized tests are used to diagnose Intellectual Disability? Which one can be done by nurse?
1. Bayley Scale of Infant Dev
2. Denver Dev. Screening Test-can be done by the nurse

What Standardized Test examines personal, social, fine motor, language & gross motor skills for Diagnoses of Intellectual Disability?
Denver Developemental Screening Test

True/False-Routine developmental screening assists in early ID of Intellectual Disability?
TRUE!

What are 6 etiologies for and Intellectual Disability?
1. Infection/Intoxication
2. Trauma or Physical Agent
3. Metabolism or nutrition
4. Chromosome Abnormalities
5. Gestational Disorders
6. Environmental influence

A patient has an IQ in the 50-70 range. What is the level of Intellectual Disability and how might the individual present?
Mild level Disability
Person may be slow to talk & walk but disabilities are not noticed by observers.

What are the IQ & level of impairment of patient with a Moderate Intellectual Disability?
IQ: 40-60
Person has noticeable delays in motor & skills development.

Which level of Intellectual Disability requires total care?
Profound-IQ is <20

What other neuromuscular disorder is often seen with Intellectual Disability?
CP

What is the primary GOAL in nursing care of a patient with an Intellectual Disability?
Promote optimum developement

How will a client with a severe level Intellectual Disability present? What is their IQ?
IQ 20-40
Client has pronounced delays with few communication skills.

What 5 CONSIDERATIONS are important in providing nursing care to clients with Intellectual Disabilities?
1. Education
2. Hospitalization
3. Discipline (training)
4. Social Skills
5. Play/Exercise
6. Sexuality

Why is sexuality an important consideration in Nursing Care of Intellectual Disability and what needs to be included in the care plan?
The client is vulnerable because they can be easily persuaded and may lack judgement.
A code of conduct MUST be included in the care plan that communicated in a way the client can understand.

True/False-Cerebral Palsy is a cognitive diagnoses.
FALSE! NO!

What type of disorder is Cerebral Palsy and what is it caused by?
-Group of nonprogressive disorders of movement & posture.
-Caused by abnormal development of or damage to motor control centers of the brain.

What causes impaired movement in CP?
Abnormal functioning of cerebral cortex

What neuromuscular disorder is the leading cause of disability children?
CP

What is the single most important risk factor for Cerebral Palsy?
Prematurity

What are postnatal risk factors/etiology for CP?
infection
kernicterus
toxins
traumatic brain injury

True/False-most prenatal cases of CP have a known etiology.
FALSE-70-80% cases have unknown cause

True/False-birth trauma attributes to CP in perinatal cases.
TRUE-but it is a VERY low % of overall cases

What type of CP is most common?
SPASTIC

What characterizes hypertonicity in Spastic CP?
Persistant primitive reflexes
Increased muscle tone
Development of contracture

What does a nurse or provider need to know about reflexes when they are assessing a patient for possible CP?
They MUST know when the primitive reflexes disapear.

What are the early manifestations of Spastic CP and how long must they last?
Generalized Hypotonicity MUST LAST up to 1 year.

What is the UNIVERSAL impairement of CP?
Delayed gross motor developement.

What changes in posture are seen in Cerebral Palsy?
1. Abnormal posturing at rest or with position changes.
2. Poor control of balance coordination

What are the manifestations of of Dyskinetic-Nonspasitc CP? What term is used to describe these manifestations?
Term is Athetoid:
1. Worm-like writhing movements involving extremities, trunk, neck, facial muscles & tongue.

What is caused by involvement of Oral muscles in Dyskinetic Nonspastic CP?
Drooling
Dysarthria-Impaired Speech

What is the common cause of Nonspastic CP?
Kernicterus

What is the primary modality for diagnoses of CP?
Careful complete history and neuro exam

What must the nurse understand about appearance of motor dysfunction in pediatric patients with CP?
Motor dysfunction may not present until 2-4 months of age

What are the 5 GOALS in Therapeutic Management, Early Diagnoses & Early Intervention of Cerebral Palsy?
1. Early locomotion, communication & self help skills
2. Gain integration of motor function
3. Correct associated defects
4. Provide educational opportunities
5. Promote socialization experiences

What types of interventions are part of a multidisciplinary approach?
PT, OT and Speech therapy

When should speech therapy be started for a patient with CP?
Start before the child begins to talk for eating and communication.

What is the FIRST therapeutic intervention that nurses should implement for children with CP?
Why is this intervention beneficial?
Feeding therapy-SELF FEEDING

It is beneficial because it requires it requires integration of fine & gross motor skills and visual perceptions.

What is main complication associated with CP?
aspiration

True/False-Education and Recreation can be therapeutic interventions for CP?
YES! They are just as important as nutritin etc.

What drugs are used for PHARM therapy management of Cerebral Palsy? (4)
1. Baclofen-may be used as pump
2. Dantrolene
3. Botox
4.Anticonvulsants-Dilantin & Phenobarbital

What PHARM agent is a skeletal muscle relaxant?
Dantrolene

What role does a Baclofen pump play in management of CP?
Surgically implanted, externally loaded & releases at slow rate.

What must the nurse understand about PO Baclofen dosage for patients with CP?
High doses of PO Baclofen have significant side effects & poor relief of symptoms.

Which PHARM agent is a local nerve block used to decrease spasticity in children with CP?
Botox

What are the steps of Diagnoses of Down Syndrome? (2 parts)
1. Suspected with physical manifestations
2. Confirmed with karyotype

The majority of Down Syndrome children are born to women in what age group?
<35 years AND older women

What must the nurse understand about the intellectual capacity of a child with Down Syndrome?
Intellectual capacity can’t be determined at birth BUT cognitive disability IS expected.

A child with Down Syndrome exhibits inadequate drainage of mucus and is at risk for nasal obstruction. Why does the nurse know leads to this problem?
Underdeveloped nasal bone

What change makes feeding difficult for an infant/child with Down Syndrome?
Large tongue & hypotonia

What should the nurse teach the parent about tongue-thrusting during feeding of a child with Down Syndrome?
Tongue thrusting is NOT a sign of refusal.
Parents can learn to refeed with assistance of OT and speech therapy.

What is Atlantioaxial Instability in a child with Down Syndrome?
Lax muscles & ligaments in cervical spine can lead to spinal chord injury & damage with forcible flexing or stretching or the neck.

What problem contributes to conductive hearing loss in children with Down Syndrome?
otitis media
narrow canals & impacted cerumen

What are the leading causes of death in the first year of life in children with Down Syndrome?
Respiratory & Cardiac Anomalies since they are most prevalent.

When must a child with Down Syndrome be screened for Atlantioaxial Instability?
Must be done by 2nd birthday.

Why must children with Down Syndrome avoid activities that stress head & neck?
Atlantioaxial Instability

What are the DISTINCTIVE Physical Characteristics present in patients with Down Syndrome? (4)
1. Brushfield spots
2. Epicanthial Fold
3. Simian Crease
4. Hypotonia & Hyperflexibility

What is the current lifespan of patient with Down Syndrome?
55 years; lower than general population

By what age are guaranteed educational services available under the Disabilities Education act?
3 years

What 7 PHYSICAL Characteristics support a SUSPECT Diagnoses of Down Syndrome?
1. Flattened hypolastic midface
2. Depressed nasal bridge
3. Inner epicanthal folds
4. Upward obliquity of palepbral fissues
5. Small external ears.
6. Small mouth & narrow palate.
7. Prominent Palantine ridges.

What methods of prenatal screening can be performed to screen for Down Syndrome? When? (4)
1. Nuchal Translucency Test-11-14 wks
*2. Chorionic Villus Sampling-8-12 wks
*3. Amniocentesis-15-20 wks
4. Percutaneous Umbilical blood sample-20 wks

What is the MOST important consideration for General health maintenance of a child with Down Syndrome?
1. Accurate Information

After accurate information, what are other concepts involved with general health maintenance of Down Syndrome?
2. Frequent well child visits
3. Immunization as scheduled
4. Monitor physical growth with special charts
5. Consider cognitive vs. social intelligence

What is the most common cardiac problem in children with Down Syndrome?
septal defects

Hypospadias in males
Alteration in GU system r/t altered urinary elimination
*Abnormal opening of male urethra upon undersurface of penia.

What is the concern with Leukemia in patients with Down Syndrome?
Low remission & High mortality rate

What alterations in visual acuity/perception are associated with Down Syndrome?
Refractive errors and Stabismus

What type of hearing loss is most common in patients with Down Syndrome? When should the initial screen be done?
Conductive
-initial screen by 6 months

What is a main concern related to nutrition in post natal cases of Down Syndrome?
Feeding Intolerance & vomiting

True/False-Obesity and Constipation are both common problems in patients with Down Syndrome
TRUE!

What is the major respiratory concern in patients with Down Syndrome r/t to impaired gas exchange?
Risk of Upper Respiratory Infection, & Obstruction.

What are the orthopedic problems in patients with Down Syndrome?
1. Subluxation & dislocation of hips
2. Atlantoaxial Instability

What must the nurse understand about reproductive effects in male patients with Down Syndrome?
Fertility in males is rare because genitalia is underdeveloped.

What type of thyroid dysfunction is common with Down Syndrome? How is this managed?
Hypothyroidism-managed with periodic testing

True/False-females with Down Syndrome are also infertile.
FALSE!-they even have menstruation at an average age.

What neurological conditions at have increased risk with Down Syndrome? What is the main risk associated with the neuro system?
A. Seizures
Alzheimer’s
Cognitive Impairments

B. Risk of injury r/t Diminished muscle tone.

How might a patient with Down Syndrome present with a Cognitive Impairment? What is the expected IQ?
A. Expressive language difficulties
B. Mild to Moderate IQ (40-70)

In which adaptive skill area of function do patients with Down Syndrome exhibit the most strength?
Social interaction

True/False-Family Issues, Developmental level and Physiological observation are ALL considerations critical in enhancing optimal development for a patient with Down Syndrome?
TRUE!- The nurse must be involved in all of these areas during management of care.

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