Hemophilia
Which of the following disorders is a deficiency of factor VIII?
1. Sickle cell disease
2. Christmas disease
3. Hemophilia A
4. Hemophilia B
3. Hemophilia A
What is Sickle cell disease?
What is Kernicterus?
This is a bilirubin level of 20 or higher when it's able to crowd BBB.
One of the main priority in patient with myelomeningocele is
Prevent from truma
What is cerebral palsy?
Cerebral palsy is a term used to describe a range of nonspecific clinical symptoms characterized by abnormal motor patterns and postures caused by nonprogressive abnormal brain function.
What type of care we should provide for fragile child:
Respite care
Wholistic care
Which measures would the RN know to avoid when planning care for a child with hemophilia? (Select all that apply)
1. Frequent blood pressures
2. Rectal suppositories
3. Rectal temperatures
4. Administering aspirin for pain
5. Carbonated beverages
1. Frequent blood pressures
2. Rectal suppositories
3. Rectal temperatures
4. Administering aspirin for pain
How do we prevent of sickle cell crisis?
Hydration
appropriate oxygenation
Control infection
Jaundice treatment?
Treatment for increased bilirubin levels
Reduce level of Unconjugated Bilirubin
Light energy to convert molecules of Unconjugated Bilirubin so that it can be excreted
Position light 40-50cm (15.5 - 19.5 inches) from infant
A newborn baby is diagnosed with a myelomeningocele. The nurse measures his head circumference daily to assess for the development of what complication?
A. Hydrocele
B. Hordeolum
C. Hypsarrhythmia
D. Hydrocephalus
D. Hydrocephalus
´Prenatal
´Congenital abnormalities
´Genetic
´Hypoxia (bleeding, seizures, drug abuse, prematurity, trauma to abdomen)
´Exposures
´LOW BIRTH WEIGHT & PREMATURITY
´Perinatal
´ASPHYXIA*
´Early delivery
What is the important information about caring baclofen pump
Make sure is attached and working
Do not use bath tub
Which sign/symptom should the nurse expect to assess in the client diagnosed with
hemophilia A?
1. Epistaxis.
2. Petechiae.
3. Subcutaneous emphysema.
4. Intermittent claudication.
1. Epistaxis
During an outpatient well visit with a patient who has sickle cell anemia, you make it PRIORITY to assess the patient's?
A. hemoglobin A1C level
B. heart rate
C. reflexes
D. vaccination history
D. vaccination history
What level of janudice is considered pathologic?
Increase Bilirubin of 5 mg/dl or more in 24 hours
Prior to surgery for a myelomeningocele, the nurse would place the baby in which of the following positions?
A. Prone
B. Right side
C. Left side
D. Dorsal
A. Prone
At some point in the life, the CP patient can be treated.
A. Ture
B.Flase
B.Flase
At what point during a hospital stay should discharge planning be initiated?
A) after surgery and successful recovery
B) after pt is less anxious
C) immediately before discharge
D) on admission to the acute care setting
D) on admission to the acute care setting
Which nursing interventions should the nurse implement when caring for a client diagnosed with hemophilia A? Select all that apply.
1. Instruct the client to use a razor blade to shave.
2. Avoid administering enemas to the client.
3. Encourage participation in noncontact sports.
4. Teach the client how to apply direct pressure if bleeding occurs.
5. Explain the importance of flossing the gums
2. Avoid administering enemas to the client.
3. Encourage participation in noncontact sports.
4. Teach the client how to apply direct pressure if bleeding occurs.
A 25 year-old pregnant female and her partner both have sickle cell trait. What is the percentage that their offspring will develop sickle cell anemia?*
A. 50%
B. 25%
C. 75%
D. 100%
B. 25%
How do we test for Rh incompatibility?
Prenatal testing
Indirect Coombs' test
Neonatal testing
Direct Coombs test
Appropriate nursing interventions for a newborn's myelomeningocele sac prior to surgery include using sterile technique and:
A. Leaving the sac open to air
B. Applying petrolatum to cover the sac
C. Applying moist saline dressings
D. Applying dry dressings
C. Applying moist saline dressings
Not all patients with cerebral palsy have an intelletual disability:
A. True
B. False
A. True
Why do we use baclofen pump?
Baclofen is one of the most effective medications for spasticity (involuntary muscle contractions that cause stiffness) and dystonia (muscle contractions that can result in twisted or abnormal postures).
The client with hemophilia A is experiencing hemarthrosis. Which intervention should the nurse recommend to the client?
1. Alternate aspirin and acetaminophen to help with the pain.
2. Apply cold packs for 24 to 48 hours to the affected area.
3. Perform active range-of-motion exercise on the extremity.
4. Put the affected extremity in the dependent position.
2. Apply cold packs for 24 to 48 hours to the affected area.
A 14 year-old female has sickle cell anemia. Which factors below can incrase the patient's risk for developing sickle cell crisis? Select all that apply:
A. Shellfish
B. Infection
C. Dehydration
D. Low altitudes
E. Hemorrhage
G. Strenuous exercise
B. Infection
C. Dehydration
D. Low altitudes
G. Strenuous exercise
What are the causes of pathologic jaundice?
h & ABO incompatibility
Prematurity
liver not mature yet
Cephalhematoma: more blood.
A baby was born 2 hours ago by Cesarean section. The newborn has a myelomeningocele with the sac intact and has been placed in an incubator. The nurse, when planning care for the baby, should focus on potential for:
A. Disuse syndrome
B. Infection
C. Fluid volume deficit
D. Decreased cardiac output
B. Infection
The mother of an 18-month-old child is concerned about the child not meeting developmental milestones and wants the child tested for cerebral palsy. Which diagnostic approach should the nurse explain to this mother?
A. CT Scan
B. Lab test for certain proteins
C. Urinalysis
D. Observation of symptoms and ruling out other disorders
D. Observation of symptoms and ruling out other disorders
List 3 types of fragile child?
´Premature
´Former premature
´Chronic lung disease (FKA bronchopulmonary dysplasia)
´Technology dependent
´Failure to thrive
´Severe emotional, behavioral, developmental, and cognitive issues
´Sequalae from a traumatic event such as TBI, spinal cord injury,
´Other severe injuries from accidents/ abuse (abusive head trauma)
´Conditions addressed in other parts of this topic
´Congenital heart disease
´Neural tube defects
´Cerebral palsy