Vital Information
These are common symptoms of sickle cell disease
Acute/chronic pain, fatigue, infections, painful swelling of hands and feet (Dactylitis)
Sickle cell crisis could best be defined as a ______occlusive crisis.
What is vaso-occlusive?
How is sickle cell disease diagnosed?
Newborn screening, sickle cell turbidity test (Sickledex)
Sickled Red Blood Cells become rigid and take on this elongated 2-sided shape.
What is a sickle/crescent?
A nurse instructs the parents of a child with sickle cell disease about factors that might precipitate a pain crisis in the child. Which of the following factors identified by the parent as being able to cause a pain crisis indicates a need for further instruction?
A. Infection
B. Overhydration
C. Stress at school
D. Cold environment
B. Overhydration does not cause a crisis
What are two common laboratory tests for a patient with sickle cell disease?
CBC, reticulocyte count, blood culture, liver and kidney function tests.
This is the primary symptom associated with Sickle Cell Crisis.
Pain
This method of prenatal genetic testing can be used to assist diagnosis of sickle cell disease in an unborn infant?
Amniocentesis or fetal blood sampling
The organ most commonly infarcted by Sickle Cell Anemia, usually occurring by 2 to 4 years of age.
What is the spleen?
Which of the following factors need(s) to be included in a teaching plan for a child with sickle cell disease? Select all that apply
A. The child needs to be taken to a physician when sick.
B. The parent should make sure the child sleeps in an air-conditioned room.
C. Emotional stress should be avoided.
D. It is important to keep the child well hydrated.
E. It is important to make sure the child gets adequate nutrition.
A, C, D, E
Patients with sickle cell disease should avoid…
Strenuous exercise, dehydration, swimming in cold water, high altitudes, smoking
This crisis develops when large amounts of blood pool in an organ, leading to decreased blood volume and shock.
Splenic sequestration crisis/hypersplenism
Why are children with sickle cell disease more likely to get infections?
Sickled cells damage the spleen, making it harder to fight infection.
This antibiotic is commonly prescribed as prophylactically to prevent pneumonia for children under 5 with sickle cell.
What is penicillin
A nurse is caring for a 5-year-old with sickle cell vaso-occlusive crisis. Which of the following orders should the nurse question? Select all that apply.
A. Position the child for comfort
B. Apply hot packs to painful areas
C. Give meperidine (Demerol) 25mg intravenously every 4 hours as needed for pain.
D. Restrict oral fluids
E. Apply oxygen per nasal cannula to keep oxygen saturations above 94%
200
C, D, E
C. Tissue hypoxia is very painful. Narcotics such as morphine as usually given for the pain while the child is in a crisis. Meperidine (Demerol) should be avoided because of the risk of Demerol-induced seizures.
D. The child should receive hydration because when the child is in crisis, the abnormal S-shaped red blood cells clump, causing tissue hypoxia and pain.
E. Oxygen is of little value unless the tissue is hypoxic. The objective of treatment is to minimize hypoxia.
Why is meperidine (Demerol) avoided in patients with sickle cell disease?
It can increase the risk of seizures in patients with renal dysfunction
This complication results from pulmonary infiltrates and is often accompanied by chest pain, fever, cough, tachypnea, wheezing, and hypoxia.
Acute chest syndrome
What are reticulocytes and what is the significance of a high reticulocyte count?
Reticulocytes are immature red blood cells produced in the bone marrow. A high reticulocyte count can indicate anemia due to red blood cells being destroyed sooner than normal
Increased blood viscosity and lack of oxygen can ultimately lead to the failure of this pair of organs.
What are the Kidneys? Sickled cells can reduce blood flow to the kidneys
The nurse is taking care of a child with sickle cell disease. The nurse is aware that which of the following problems is (are) associated with sickle cell disease?
A. Polycythemia
B. Hemarthrosis
C. Aplastic Crisis
D. Thrombocytopenia
E. Vaso-occlusive crisis
C & E
Aplastic crisis, temporary cessation of red blood cell production, is associated with sickle cell disease.
Vaso-occlusive crisis is the most common problem in children with sickle cell disease.
Patients with sickle cell disease are at risk for stroke. What symptoms would be concerning for stroke?
Headache, nausea & vomiting, focal neurological deficits, seizures, altered level of consciousness
This is a major complication resulting from occlusion, ischemia, and infarction affecting 11% of children with sickle cell disease between ages 18-20 who present with focal neurologic findings lasting more than 24 hours
Stroke/cerebrovascular accident
Sickle Cell Disease is classified as an autosomal recessive disorder. This would be the percentage a child would have of having Sickle Cell Disease if both parents were CARRIERS of the disease.
What is 25%?
(Both parents have sickle cell trait)
Children with sickle cell anemia may begin taking the antibiotic penicillin when they're about this age to help prevent infection.
What is 2 months of age?
The nurse is caring for a child with sickle cell disease who is scheduled to have a splenectomy. What information should the nurse explain to the parents regarding the reason for the splenectomy?
A. To decrease potential for infection
B. To prevent splenic sequestration
C. To prevent sickling of red blood cells
D. To prevent sickle cell crisis
B. Splenic sequestration is a life-threatening situation in children with sickle cell disease. Once a child is considered to be at high risk of splenic sequestration or has had this in the past, the spleen with be removed.