Sickle Cell Anemia ***

Front 1

Sickle cell disease is an;

inherited autosomal recessive genetic condition. A specific mutation in the hemoglobin gene alleles on chromosome 11 leads to the formation of HbS instead of HbA.

Back 1

When both parents are carriers of the sickle cell trait, there is a 25% chance with each pregnancy of producing an off spring with SCA.

Front 2

Newborn screening;

Sickledex test and Hemoglobin Electrophoresis test.

Back 2

Early diagnosis facilitates parental education

regarding the importance of

immunizations,

penicillin prophylaxis,

detection of splenomegaly, and

the need to report fever and increasing pallor

Front 3

SCA Patho;

conditions that cause an increased demand for oxygen (dehydration, vomiting, diarrhea, trauma,hypoxia, fever, exposures to extreme hot and cold and emotional/physical stress) ,

Back 3

the amino acid of the β chain changes.

This then distorts the cell membrane so that the cell changes from a pliable disk to a crescent- or sickle-shaped RBC.

Front 4

The sickled forms of the RBC are associated with much greater viscosity.

In most instances the sickling response is

reversible with adequate oxygenation and

hydration.

Back 4

RBCs with HgbS can sickle and unsickle under appropriate conditions.

After repeated cycles of sickling and unsickling, the RBCs become irreversibly sickled

Front 5

A patient experiencing a Vaso-occlusive crisis will

Back 5

Head; CVA, HA , Seizures, Blindness.

Lungs; Pneumonia, atelectasis, A.C.S., pulmonary hypertension.

Heart; H.F., Cardiomegaly, Murmur, M.I.

have localized or generalized pain to muscles, arthralgia,

acute abdominal pain from visceral hypoxia,

Front 6

A patient experiencing a Vaso-occlusive crisis will have

Back 6

a priapism which is an unwanted painful penile erection.

Front 7

Children under the age of 2 years often develop dactylitis, also known as hand-foot syndrome.

Back 7

It is caused by infarction of short tubular bones and is characterized by pain and swelling of the soft tissue over the hands and feet. .

It usually resolves spontaneously within a couple of days to weeks. During this crisis , the pain is often migratory, with the presence of a low-grade fever.

Front 8

Due to the sickling, the patient will develop

jaundice and possible hematuria.

Back 8

Jaundice results from RBC destruction and release of bilirubin.

Front 9

To assess for jaundice in patients with darker skin,

Back 9

inspect the roof of the mouth for a yellow appearance and

examine the sclera closest to the cornea.

Front 10

Due to the obstruction, the patient can develop the life threatening signs associated with stroke and chest pain, known as

Back 10

chest syndrome, that is associated with a MI.

Front 11

Medical management of a crisis is directed at supportive,

symptomatic, and

specific treatments.

bed rest to minimize energy expenditure

Back 11

& to improve oxygen utilization,--hydration,

electrolyte replacement (since hypoxia results in metabolic acidosis, which also promotes sickling),--

analgesia for pain from vasoocclusion,

blood transfusion to treat anemia & to reduce the viscosity of the sickled blood, & antibiotics

Front 12

Oxygen administration is usually not effective in reversing sickling or reducing pain because

Back 12

the oxygen is notable to reach the enmeshed sickled RBCs through the clogged vessels.

In addition, prolonged administration of oxygen can depress bone marrow activity,which further aggravates the anemia.

Front 13

Once a SCD patient begins to experience a

Vasoocclusive crisis episode, they are told to Rest,

Begin drinking water and take the prescribed pain medication.

Repeat process a second time before coming to hospital.

Back 13

if patient is;

feverish,

pale or experiencing cerebral vascular accident symptoms or chest syndrome pain; then

they are to seek immediate medical help.

Front 14

Hydroxyurea (HU) is the only effective drug to treat adult SCD patients

Back 14

to reduce the incidence of recurrent severe

painful episodes and acute chest syndrome by increasing the concentration of HgbF and ultimately to reduce complications.

Front 15

Hydroxyurea is teratogenic (can cause birth defects).

Back 15

Teach sexually active women of childbearing age using this drug to adhere to strict contraceptive measures while taking hydroxyurea and for 1 month after the drug is discontinued.

Front 16

Vassoocclusive crises and sickle cell anemia lead to multiple complications

Back 16

cerebrovascular accident,

Sickled cells block the major blood vessels in the brain,which results in cerebral infarction causing variable degrees of neurologic impairment.

Front 17

Vassoocclusive crises and sickle cell anemia lead to complications

Back 17

Repeat strokes causing progressively greater brain damage in children who have already experienced one stroke. neurologic symptoms can indicate a minor cerebral insult, such as headache, seizures, or visual disturbances. Blindness is usually the result of progressive retinopathy and retinal detachment.

Front 18

Vassoocclusive crises and sickle cell anemia lead to complications

Back 18

Complications in the lung include frequent

pneumonia with atelectasis.

Another seriouscomplication is acute chest syndrome which is clinically similar to pneumonia.

Front 19

Vassoocclusive crises and sickle cell anemia lead to complications. Acute chest syndrome is defined as

Back 19

a new pulmonary infiltrate on chest x-ray of a SCD patient that may be accompanied by

chest pain,

fever,

cough,

tachypnea,

wheezing, and hypoxia.

Front 20

Researchers believe that a VOC or infection

Back 20

results in sickling in the small blood vessels of the lungs, with ensuing occlusion, stasis, and anemia.

Repeated episodes of chest syndrome may cause restrictive lung disease and pulmonary

hypertension.

Front 21

Heart problems are mainly attributable to

the stress of chronic anemia, which can eventually result indecompensation and heart failure.

Back 21

Cardiomegaly is visualized on a chest X-ray and a systolic flow murmur is frequently present as a consequence of the anemia.

A serious cardiac issue resulting from occlusion is the potential for a myocardial infarction.

Front 22

Bone changes include

hyperplasia and congestion of the bone marrow, which result in osteoporosis.

As a result of the weakening of bone, skeletal

deformities, particularly lordosis and kyphosis, may occur. Because of chronic hypoxia, the bone becomes susceptible to osteomyelitis.

Back 22

Avascular necrosis of the femoral head from chronic ischemia is an occasional problem.

Front 23

Kidney abnormalities are probably the result of the same cycle of congestion of glomerular

capillaries and tubular arterioles with

sickled cells,

tissue necrosis, and

eventual scarring.

Back 23

The principal results of kidney ischemia are

hematuria,

frequent urinary tract infections,

pyelonephritis, and

nephrotic syndrome.

Kidney failure can result.

Front 24

The liver is also altered in form and function.

Liver failure and necrosis are the result of

Back 24

severe impairment of hepatic blood flow from anemia and capillary obstruction.

The rapid destruction of RBCs often results in the development of obstructive jaundice and gallstones.

Front 25

If recurrent episodes of right upper abdominal pain occur,

Back 25

cholecystectomy may be indicated.

Initially the spleen may become enlarged (called splenomegaly) from congestion and engorgement with sickled cells.

Life-threatening

Front 26

Splenic sequestration crisis:

After numerous crisis events, the functioning cells are gradually replaced by fibrotic tissue

until, by the age of 5 years, the spleen has

decreased in size and has been totally replaced by a fibrous mass.

Back 26

Without the spleen to filter bacteria and to

promote the release of large numbers of

phagocytic cells, these individuals are highly

susceptible to infection.

Front 27

Many adults with SCD have skin ulcers on the lower legs that are caused by poor perfusion, especially on the outer sides and inner aspect of the ankle or the shin.

Back 27

These lesions often become necrotic or infected, requiring débridement and antibiotic therapy. Inspect the legs and feet for ulcers or darkened areas that may indicate necrotic tissue.