Sickle-Cell Anemia

Chloe Agnes Regina L. Aranas

 Blood Cells
RBC
Platelet
[thrombocyte]

Basophil
[WBC]

B-lymphocyte
[WBC]

Neutrophil
[WBC]

Monocyte
[WBC]

Eosinophil
[WBC]
 Definition

• a severe
hemolytic anemia
that results from
inheritance of the
sickle hemoglobin
gene.
 Pathophysiology
• sickle hemoglobin gene
• hemoglobin molecules
become defective
• low oxygen level in venous
blood
• (HbS) acquires a crystal-like
formation
 Pathophysiology
• RBC containing (HbS) loses its
round, very pliable, biconcave
disk shape and becomes
deformed, rigid, and sickle-
shaped
• these RBCs can adhere to the
endothelium of small vessels
• when they pile up against each
other, blood flow to a region
or an organ may be reduced
 Causes
• genetics
• temperature
Clinical Manifestations
• hemoglobin values of 7 to 10 g/dL
• jaundice [sclerae]
• enlargement of the bones of the face and
skull [children]
• tachycardia
• cardiac murmurs
• cardiomegaly
• dysrhythmias
• heart failure
Clinical Manifestations
Sickle Cell Crisis
• sickle crisis
– most common and very painful
– tissue hypoxia and necrosis

• aplastic crisis
– human parvovirus
– hemoglobin level falls rapidly and the marrow
cannot compensate
– absence of reticulocytes
Clinical Manifestations

Sickle Cell Crisis

• sequestration crisis
– organs pool the sickled cells
– has to do with the spleen

Acute Chest Syndrome

– rapidly falling HgB level
– tachycardia
– fever
– bilateral infiltrates on the chest-ray
 Complications
organ mechanisms assessment symptoms
involved findings
Spleen •Primary site of sickling •Autosplenectomy; ↑ •Abdominal pain;
→infarctions→↓ infection (esp. fever, signs of
phagocytic function of pneumonia, infection
macrophages osteomyelitis)
Lungs •Infection •Pulmonary •Chest pain;
•Infarction→↑pulmonary infiltrate dyspnea
pressure →pulmonary •↑ sPLA2+
hypertension
CNS •Infarction •CVA (cerebral •Weakness (if
vascular accident, severe); learning
brain attack) difficulties (if
mild)
 Complications
Kidney •Sickling→damage to •Hematuria; inability to •Dehydration
renal medulla concentrate urine; renal
failure
Heart •Anemia •Tachycardia; •Weakness,
cardiomegaly →heart fatigue, dyspnea
failure
Bone •↑ Erythroid production •Widening of medullary •Ache
spaces and cortical
•Infarction of bone thinning
•Osteosclerosis→avascul
•Bone pain,
ar necrosis especially hips
CNS •Infarction •CVA (cerebral vascular •Weakness (if
accident, brain attack) severe); learning
difficulties (if
mild)
 Complications
Liver •Hemolysis •Jaundice and gallstone •Abdominal pain
formation;
hepatomegaly
Skin •↑ Viscosity /stasis •Skin ulcers; ↓ wound •Pain
→infarction healing
→skin ulcers
Eye •Infarction •Scarring, hemorrhage, •↓ Vision;
retinal detachment blindness

Penis •Sickling •Priapism→impotence •Pain, impotence

 Medical Management

Pharmacologic Therapy
• Hydroxyurea [Hydrea]
• Cyanate
• Penicillin
 Medical Management

Transfusion Therapy
- chronic transfusions with RBCs
- folic acid replacements
- antibiotic therapy
- corticosteroids
- intravenous immunoglobulin
- erythropoietin
- bone marrow transplant
 Medical Management

Supportive Therapy
- oral hydration
- intravenous hydration
- supplemental oxygen
- pain reliever
Nursing Interventions
Assessment
- pain
- joints
- abdomen
- respiratory system
- cardiac failure
- hydration
- neurologic examination
- bones
Nursing Interventions
- managing pain
- preventing and managing infection
- promoting coping skills
- minimizing deficient knowledge
- monitoring and managing potential
complications
- promoting home and community-based care