•    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.

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.

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.

•    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

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
               

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

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

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

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

•    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.

•    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.