Sickle cell anemia and nursing diagnosis and intervention

Sickle cell anemia
Structure of RBC
Erythropoiesis


•    Formation of blood
–    Adults: In red bone marrow of sternum, ribs, vertebrae and pelvic bone.
–    Fetus: In spleen and liver.
•    Normal life span of RBC is 120 days
•    Hemoglobin
–    Iron containing pigment of RBC, transports Oxygen from lungs to tissues and Carbon dioxide from tissues back to lungs.
•    Bilirubin: Results from destruction of RBC. A product of decomposition of Hb.

Types of Hemoglobin (Normal)

Hb A    Normal adult hemoglobin
Hb F    Fetal Hb up to 6 months after birth
Hb A1C    Glycosylated Hb. Shows if the glucose level of a diabetic patient on insulin has been regulated for a period of up to 90 days.

Hemoglobin Structure

Abnormal Hb
Hb S    Abnormal Hb found in Sickle cell anemia. Shape becomes sickled in adverse conditions. In the β-chain glutamic acid is replaced with valine.
Hb C    Abnormal hemoglobin which result in chronic hemolytic anemia. In the β-chain glutamic acid is replaced with lysine.
Hb E    Abnormal hemoglobin which result in a mild form of hemolytic anemia
Hb M    Abnormal hemoglobin with poor Oxygen carrying capacity, which result in congenital cyanosis.

Sickle Cell Anemia

•    Due to the presence of abnormally shaped RBCs. Autosomal recessive inherited disorder of Hb associated with  chain of Hb. It is a defect in the amino acid sequence where glutamic acid is replaced with valine. RBCs become sickled (crescent shaped) in adverse conditions – hypoxia, dehydration, infection
•    Sickled RBCs can not move freely through the small capillaries leading to capillary occlusion and blood stasis leading to further hypoxia and sickling.
•    A sickle cell crisis occurs.
•    The disease is not present in infants below 6 months due to the presence of fetal Hb


Normal and Sickled RBCsSickle cell anemia

Pattern of inheritance of sickle cell anemia

Pattern of inheritance of sickle cell anemia
Types of SCA
•    Sickle cell disease ——- Homozygous (Hb SS)
•    Sickle cell trait ———- Heterozygous (mild)  (Hb AS)
•    Sickle thalassemia disease  —— A combination of sickle cell and β-                        thalassemia trait

Pathophysiology

Pathophysiology sickle cell
assessment
•    Heart : Cardiomegaly from chronic anemia, murmurs, cardiac failure
•    Lungs: Pulm. vessels occluded by sickled RBC or embolus, resp distress, pneumonia
•   Spleen: Enlarged spleen from engorged sickled RBC, splenic rupture with massive hemorrhage, eventually fibrosis and atrophy
•    Liver: Progressive impairment, hepatomegaly, tenderness, jaundice, gall stones, cirrhosis
•    Kidneys: Impaired renal function from glomerular tubular necrosis and fibrosis, hematuria, nephrotic syndrome
•    CNS: Infarction of CNS, stroke, peripheral vascular occlusion of retina, retinal detachment, meningitis
•    Skeletal: Infarction of bone and bone marrow, Hand and foot syndrome, delayed closure of epiphyses, osteomyelitis, chronic leg ulcers, Lordosis, kyphosis

Stressors

•    Heredity
•    Homogenous form of the disease
•    Sickle cell trait for parents
•    Bone aches
•    Abdominal Pain
•    Sickle cell crisis
•    Infection
•    Anemia

Prognosis

•    Sickle cell trait      —  good
•    Sickle cell disease    — Infection is a common cause of death. Also from cardiac,                     hepatic or renal failure

TREATMENT

–    Pain relief using narcotics
–    Hydration
–    Oxygen administration
–    Treatment of infections
–    Blood transfusion
•    Prevention
–    Genetic counseling

Nursing diagnoses

•    High risk for ineffective tissue perfusion R/T infarcts due to sickling.
•    Altered health maintenance R/T lack of knowledge regarding long term care.
•    Altered family process R/T having a child with potentially life threatening disease.

Health teaching

•    Regular follow up visits.
•    Avoid strenuous activities.
•    Protect from hypoxia – avoid mountain climbing, travel in unpressurized plains, under water swimming.
•    Promote hydration – oral intake, observe for signs of dehydration, avoid extremes of climates.
•    Prevent infections – adequate nutrition, rest, good hygiene, immunization.
•    Avoid exposure to infections
•    Pain management – inform doctor about child’s condition, liberal use of drugs round the clock, Use hot compresses and avoid cold compresses.

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