sickle cell anemia by rowell dotollo power point

8/6/2019 Sickle Cell Anemia by Rowell Dotollo Power Point

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Sickle cell anemia

Congenital hemolytic anemia that occursprimarily, but not exclusively, in black.An inherited, autosomal recessive disorder that

result in chronic hemolytic anemia characterize bysickle shape red blood cells.Result from a defective hemoglobin molecule(hemoglobin S) that cause red blood cell to roughenand become sickle cell shaped.

Such cells impair circulation, resulting in chronicill health, periodic crises, long term complication,and premature death.


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Causes

homozygous inheritance of the Hb S gene, which causes

substitution of the amino acid valine for glutamic acid in theB Hb chain

Heterozygous inheritance of this gene result in sickle cell

trait ( sickle cell carrier), usually an asymptomatic condition


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Pathophysiology

The erythrocytes of individual with sickle cell diseasecontain more hemoglobin (Hgb) S than Hgb A.Consequently; the erythrocytes become rigid, rough,

elongated, and crescent shaped when oxygen tensiondecreases. Hypoxic condition or elevated blood viscositycauses a decrease in oxygen tension. These sickled cellare easily destroyed as, they enter the smaller blood vessel

in the body.T

hey accumulate in the capillary, impairingcirculation and causing pain, tissue and organ infarction,and hypoxia, which in turn causes more sickling.


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Sign and symptoms

TachyracdiaCardiomegallySystolic and diastolic murmursPulmonary infarctionChronic fatigue

Unexplain dyspnea on exersionHepatomegalyJaundicePallorJoint swellingAching bonesChest painsIschemic leg ulcer ( especially around the ankles)Increase susceptibility to infection


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Such symptoms usually dont developed untilafter age 6 months, because large amount offetal Hb protect infant s for the first fewmonths after birth. Low socioeconomic status

and related health problems, such as poornutrition and education, may delay thediagnosis and supportive treatment.Infection, stress, dehydration, and condition

that provoke hypoxia like strenuous exercise,high altitude unpressurized aircraft, cold, andvaso constrictive drug may all provoke periodiccrises.


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Periodic Crisis

Painful Crisis( Vaso-Occlusive Crisis )

result from blood vessel; obstruction by rigid,

tangled sickle cells, which causes tissue anoxia

and possible necrosis that is characterized by

severe abdominal, thoracic, muscular, or bone

pain and possibly increase jaundice, dark urine,

or a low grade fever.


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Aplastic Crisis ( Megaloblastic Crisis )

result from bone marrow depression and isassociated with infection, usually viral that is

characterized by pallor, lethargy, sleepiness,

dyspnea, possible coma, markedly decrease

bone marrow activity, and RBC hemolysis.

Acute Sequestration Crisis

In infants between 8 months and 2 years

old, an acute sequestration crisis may cause

sudden massive entrapment of RBCs in the

spleen and liver. This rare crisis causes

lethargy and pallor; if untreated, it commonly

progresses to hypovolemic shock and death.


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Hemolytic crisis

Quite rare, hemolytic crisis usuallyoccurs in patients who have a glucose-6-

phosphate dehydrogenase deficiency

with sickle cell anemia. Probably result

from complication of sickle cell anemia,such as infection, rather than from the

disorder itself


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Indication of crisis

Suspect any of these crisis in a sickle cell

anemia patients with pale lips; toungue,

palms, or nail beds; lethargy, listlessness;sleepiness, with difficulty awakening;

irritability; severe pain; temperature over 40

C or a fever of 37.8 C that persis in two

days.


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Long term Complication

Child smaller of his age

Puberty delayed

In adult the body builds spider like- narrow

shoulders and hips, long extremities, curvedspine, barrel chest and elongated skull.

Organ infarction such as retinophaty and

nephropathy.

Premature death due to infection or repeatedocclusion of small blood vessels and consequent

infarction or necrosis of major organs.


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Diagnostic Test

Positive family history and typical clinical suggest

sickle cell anemia

Hb electrophoresis showing Hb S or otherhemoglobinopathies can confirm it.

Electrophoresis should be done on umbilical

cord blood samples at birth to provide sickle cell

disease screening for all neonates at risk..


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Treatment

Prophylactic penicillin is given before age 4

months.If the patients Hb drops suddenly or if hiscondition deteriorates rapidly, a transfusion of

packed RBCs is needed.

In a sequestration crisis, treatment may include

sedation, administration of analgesics, bloodtransfusion, oxygen administration, and large

amounts of oral orI.V. fluids.


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Special ConsiderationDuring painful crisis:

Apply warm water to painful area and

cover the child with a blanketAdminister an analgesic-antipyretic such

as aspirin or acetaminophen

Encourage bed rest on a sitting position

Give antibiotics when appropriateSuggest biofeedback techniques


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During remission

Avoid tight clothes that will restrict the circulation

Warn again st strenuous exercise, vasoconstrictingmedication, cold temperature, high altitude, and other

condition that provoke hypoxia

Avoid stress, wound care, good oral hygiene, balance

diet as safe guard against infection

Increase fluid intake to avoid dehydration but not coldwater

Refer parents to genetic counseling for the risk future

offspring.

During pregnancy avoid use of contraceptives because

it is risky for patient having sickle cell anemia

During surgery, make sure that patient has optimal

ventilation to prevent hypoxic crisis and provide a pre-

operative of packed RBCs as needed.

sickle cell anemia by rowell dotollo power point

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