Chapter 32: Sickle Cell Disease (Children)

Question 1

Sickle Cell Disease (SCD)

Answer 1

common genetic hematological condition
-transmitted via an autosomal recessive pattern of inheritance; both parents must have the sickle cell gene for the child to have SCD
-the globin chain in normal hemoglobin A (HbA) is partially or completely replaced by hemoglobin S (HbS)
>when a patient has a large amount of HbS and a decrease in oxygen levels, these abnormal hemoglobins clump together within the cell and change the shape from donut-like to a sickled shape

Question 2

The two most significant pathophysiological features of this disease include

Answer 2

• tissue ischemia as a result of the occlusions

- the inherited fragility of the sickled cells

Question 3

What happens when The RBCs Sickle?

Answer 3

they are more fragile and easily destroyed

• the surfaces of these sickled cells are sticky and adhere to blood vessel walls
• the shape of the sickled cell does not promote good oxygenation or movement throughout the circulatory system, hence clinical symptomatology results
• if the RBCs cannot circulate through the vascular system and there are occlusions, hypoxemia may result and can lead to ischemia, infarcts, and possible tissue death

Question 4

Life Span of a Sickled RBC

Answer 4

8 to 21 days
-as a result of the short life span, children with SCD have an increased amount of hemolysis as evidenced by their chronic anemia

Question 5

Signs and Symptoms

Answer 5

b/c it is a blood disorder, all organs of the body may be affected
>symptoms most evident is the result of the vaso-occlusion of the blood vessels from the sickled RBCs and hemolysis, which causes pain; the pain can be found anywhere in the body
>other symptoms:
-weakness
-pallor
-fatigue 
-tissue hypoxia
-jaundice as a result of RBC hemolysis 

> Resulting Conditions include:

• chronic hemolytic anemia
• increased susceptibility to infections
• hand-foot syndrome (dactylitis)
• enuresis and nocturia
• stroke
• avascular necrosis of the shoulder or hip
• acute chest syndrome (severe pneumonia)
• priapism (prolonged and unwanted erection of the penis that occurs without sexual stimulation)
• cholelithiasis
• leg ulcers
• delayed physical growth and sexual maturation

Question 6

Complications

Answer 6

• chronic hemolytic anemia
• increased susceptibility to infections
• hand-foot syndrome (dactylitis)
• enuresis (nighttime incontinence) and nocturia (wake up in the middle of the night to urinate)
• stroke
• avascular necrosis of the shoulder or hip
• acute chest syndrome (severe pneumonia)
• priapism (prolonged and unwanted erection of the penis that occurs without sexual stimulation)
• cholelithiasis
• leg ulcers
• delayed physical growth and sexual maturation

Question 7

Diagnosis

Answer 7

-in utero, chorionic villus biopsy is done on the fetus when both parents are aware they carry the sickle cell trait; this allows early identification and appropriate diagnosis and prophylactic
-newborn screening is standard; not a definitive diagnosis
-tests: thin-layer isoelectric focusing (IEF), high performance liquid chromatography (HPLC), and hemoglobin electrophoresis
>hemoglobin electrophoresis assay separates the various types of hemoglobin and quantifies the percentage of various hemoglobins present
-CBC and reticulocyte count
-expected findings: decreased hemoglobin and hematocrit and an elevated reticulocyte count (immature RBCs)

Question 8

Prevention

Answer 8

• raising awareness
• genetic counseling and follow-up of genetic screening at birth may assist parents in being aware of susceptibility and proper care of a child with SCD
• family members who are carriers of sickle cell trait should take precautions when planning pregnancies and participating in high-risk behaviors such as becoming dehydrated with excessive physical activity, high altitudes, and/or the need for anesthetics or surgical procedures

Question 9

Nursing Care

Answer 9

• supportive and specific care based on type of crisis present
• focuses on rest, hydration, pain control, and adequate oxygenation
• coordinate nursing care to allow adequate rest periods and to minimize unnecessary interruptions
• administering and monitoring adequate hydration (oral or IV)
• monitoring respiratory status and oxygenation of sickle cell patients; child may be at risk for an acute pulmonary event that may be a result of the disease itself or the use of opiates; complete respiratory assessment includes s/s of respiratory distress, including auscultation of lung sounds to detect decreased and abnormal breath sounds, respiratory effort and quality, continuous monitoring of oxygen saturations, color and perfusion, and any necessary laboratory or radiographic studies

Question 10

Bone Marrow Transplant

Answer 10

treatment modality to cure sickle cell patients with numerous complications
-can come with risks

Question 11

Medical Care

Answer 11

• early identification and treatment of hypoxic episodes
• hydration
• pain management

Question 12

Management for Home

Answer 12

oral pain management consisting of:
-acetaminophen (children's Tylenol)
-ibuprofen (children's Advil)
-acetaminophen with codeine (children's Tylenol-Codeine)
for mild to moderate pain

Question 13

Management for Hospitalized child with vaso-occlusive crisis

Answer 13

Opioids such as:
-immediate and sustained release morphine (Duramorph)
-oxycodone (OxyContin)
-hydromorphone (Dilaudid)
-methadone (Methadose)
>may be administered around the clock
>may be administered by patient-controlled analgesia (PCA)
-another med useful for pain management that does not have common side effects of the opiate drugs: ketorolac (Toradol) (do not exceed 5 days of use)

Question 14

Hyper-transfusion Therapy

Answer 14

children who have experienced numerous hospitalizations, CVA, or acute chest syndromes may be candidates

• receive blood transfusions every 3 to 4 weeks to increase their amount of hemoglobin A
• tx regimen not without potential risks, which may include the transmission of various infectious diseases, an acute transfusion reaction, or iron overload from multiple blood transfusions
• when blood transfusions are administered, often the quantity of the iron contained in the blood transfusion is greater than the body’s ability to excrete, resulting in an iron overload
• sickle cell patients also advised to receive chelation therapy to prevent organ damage from iron accumulation

Question 15

Chelation Therapy

Answer 15

prevention and treatment of iron overload

Question 16

When a Child is Asplenic

Answer 16

absence of normal spleen function
-a child with a temperature greater than 101.5 requires immediate attention b/c most children with SCD are “functionally asplenic”
-when a child is asplenic, he/she does not have the ability to filter certain encapsulated bacteria; this allows bacteria to multiply in the bloodstream, causing a low-grade bacterial infection or serious sepsis
-if a child with SCD has a fever, immediate treatment involves blood cultures and the implementation of broad-spectrum antibiotics; children with SCD often receive prophylactic oral penicillin at home to prevent overwhelming sepsis
>ensure child receives the pneumococcal and H influenza type b vaccine and other routine vaccinations

Question 17

Education about SCD

Answer 17

• learn to cope with a lifelong chronic illness
• knowledgeable of the s/s of sickle cell crisis so they can report to primary care provider
• educate family about goals of ongoing care including the prevention of complications associated with infections, hypoxemia, and vaso-occlusive crisis
• strenuous activity should be avoided
• knowledgeable about complications such as management of pain and fever, splenic sequestration, priapism, acute chest syndrome, and stroke; all of these can permanent disability or death
• avoid sickle cell crisis by providing rest and hydration
• adherence to mediations such as prophylactic penicillin to prevent overwhelming sepsis
• have all required immunizations

Question 18

What to do in the event a patient experiences a mild sickle cell crisis at home?

Answer 18

-stop what he/ she is doing
-rest
-drink fluids
-take prescribed pain medication
>if improvement is not observed, notify physician, anticipate need to see a health0care provider or go to nearest ED

Question 19

Medication: Toradol (Ketorolac)

Answer 19

• Indications: short-term (less than 5 days) management of moderate to severe pain, including post-op pain
• Therapeutic Effects: pain relief
• Contraindicated: hypersensitivity to Toradol, acetylsalicylic acid (children’s Aspirin), or other nonsteroidal anti-inflammatory drugs (NSAIDs); patients with peptic ulcer disease or anyone with bleeding tendencies
• Side Effects: dizziness, headache, rash, diarrhea, GI pain, bleeding, prolonged bleeding times, anaphylaxis, hypersensitivity reactions
• Nursing Implications: instruct on medication administration; do not exceed 5 days of total use; may cause drowsiness and impair ability to perform activities requiring mental alertness