Sickle cell anemia is a genetic blood disorder caused by a mutation in the beta-globin gene that results in abnormal hemoglobin S. When deoxygenated, hemoglobin S polymerizes and causes red blood cells to take on a sickle shape, which can block small blood vessels and damage tissues. Common complications include painful vasoclusive crises, acute chest syndrome, strokes, and infections. Treatment focuses on prevention of sickling through hydration and pain management of crises with opioids when they occur.

1. Sickle Cell Anemia
 Dr. Philip P. Gevao

2. Sickle Cell Anemia (SCA)
A global disease

3. Definition
 Sickle cell anemia results from the hereditary
presence of abnormal Hgb S in place of Hgb A
 When Hgb S becomes deoxygenated (loses
oxygen), it tends to form polymers that deform the
red blood cells (RBCs) into a sickle shape.
 Sickled RBCs cannot pass freely through capillaries
and cause blockage of small vessels. This can
compromise the blood supply to various organs.

4. Incidence
 Hereditary hemoglobinopathy; autosomal
recessive; 1 African Americans
 Hgb S trait is found in 8 to 10% of the
American black population.
 Sickle Cell Anemia– homozygous form (HbSS)—
most prevalent in US
 In Western African native blacks, up to 40%
of the population carry Hgb S trait.

5. Basic Defect
 A defect in the structure of Hemoglobin A—
substitution of 1 amino acid (valine) for another
(glutamine) @ the 6th position of the beta
polypeptide chain.
 Hgb partially/completely replaced by Hgb S
 When triggered, Hgb S sickles & obstructs cap.
blood flow, s blood viscosity  tissue
ischemia
 Results  hemolysis (RBC destruction) &
anemia;
 Sickling reversible until repeated cycles leads to
irreversible sickling

6. Pathophysiology
 Spleen—enlarged, if untreated, becomes
fibrous & ineffective  susceptibility to
infection.
 Liver—failure & necrosis 2ndary to impaired
circulation. Hepatomegaly and tenderness
are common sx.
 Kidney— perfusion 2ndary to congestion of
glomerular capillaries & tubular arterioles
hematuria, inability to concentrate urine,
enuresis, & possible nephrotic syndrome.

7. Pathophysiology (cont’d)
 Bones—
 Hyperplasia of bone marrow osteoporosis
weakened bones lordosis and kyphosis or
other skeletal deformities
 Chronic hypoxia possible osteomyelitis from
salmonella
 “Hand-foot Syndrome”—occurs in children 6 mos-
2yrs. Caused by infarction of short-tubular bones.
Sx: swelling & pain of soft tissue of hands & feet.
Usually resolves in 2-3 wks.

8. Pathophysiology (cont’d)
 CNS—primarily R/T ischemia
 MAJOR INFARCT—Stroke or CVA paralysis
 MINOR INFARCT—HA, aphasia, learning
disability, weakness, convulsions, visual
changes 2ndary to retinopathy & retinal
detachment.
 Heart—Decompensation & failure 2ndary
to chronic anemia  CHF, pulmonary
hypertension, tachycardia

9. Pathophysiology (cont’d)
 Blood—
 Rate of destruction of RBC’s > rate of
RBC production
 RBC’s lifespan is  to 10-15 days
(compared to 120 days for nml RBC)
  Hgb, Hct & RBC count
  Reticulocyte count, bilirubin & uric
acid

10. Pathphysiology (cont’d)
 Other sx:
 Generalized weakness
 Anorexia
 Joint, back, and abdominal pain
 Fever
 Vomiting
 Possible leg ulcers
 Failure to thrive,  rate of growth &
development

11. Sickle Cell Crisis
 Triggers
 Acute illness
 Dehydration
 Strenous exercise
 Hypoxic conditions
 Trauma
 Respiratory illness
 Results: acute pain, cell destruction, infarcts,
organ damage

12. Types of Crises
 Vaso-occlusive: PAIN, swollen joints,
vomiting, fever, anorexia, dactylitis
 Acute splenic sequestration: liver &
spleen, HR dyspnea, weakness, pallor,
shock
 Aplastic crisis: weakness, pallor, dyspnea,
tachycardia, shock
 Hyperhemolytic— most rare form.
Caused by excess RBC destruction.

13. Other complications
 Acute chest syndrome: chest pain, cough,
fever, abdominal pain.
 Hydroxyurea can help prevent this.
 Megaloblastic anemia
 Hyperhemolytic crisis
 Cerebrovascular injury (stroke)
 Overwhelming infections

14. Early Symptoms
and Complications
 Typically appear during infant's first year
 1st symptom: dactylitis and fever (6 mo-2 yrs)
 Pain in the chest, abdomen, limbs and joints
 Enlargement of the heart, liver and spleen
nosebleeds
 Frequent upper respiratory infections
 Chronic anemia as children grow older
 Over time Sickle Cell sufferers can experience damage
to organs such as liver, kidney, lungs, heart and spleen
 Can result in death

15. Medical Complications
1. pain episodes
2. strokes
3. increased infections
4. leg ulcers
5. bone damage
6. yellow eyes or
jaundice
7. early gallstones
8. lung blockage
9. kidney damage and
loss of body water in urine
10. painful erections in men
(priapism)
11. blood blockage in the spleen
or liver (sequestration)
12. eye damage
13. low red blood cell counts
(anemia)
14. delayed growth

16.  Infectious complications
 Prominent early in life
 Leading cause of morbidity and mortality
 Great improvement in the prognosis related to newborn
screening for sickle cell disease, vaccination for childhood
illnesses, the use of prophylactic antibiotics, and aggressive
diagnosis and treatment of febrile events
 Acute splenic sequestration
 Episodes of rapid increase in splenic size and decrease in
hemoglobin
 Potential source of morbidity and mortality early in life for
children with sickle cell anemia and at any age for those with Hb
SC disease and sickle thalassemia
Serious Complications

17.  Strokes
 Up to 15% of children may have overt or silent strokes during
childhood
 Chronic transfusion therapy reduces the recurrence rate of
overt stroke which may approach 75% without intervention
 Bone disease
 Early risk is primarily from osteomyelitis
 Infectious usually painful inflammatory disease of bone often of
bacterial origin and may result in bone tissue death
 Avascular necrosis of the femur and humerus
 Death of bone tissue due to disrupted blood supply
 Marked by severe pain in the affected region and by
weakened bone that may flatten and collapse
Serious Complications

18. Serious Complications
 Leg ulcers
 Seen in patients older than 10 years of age
 Resistant to therapy and cause significant morbidity
 Ophthalmic complications
 Proliferative retinopathy, vitreous hemorrhage, & retinal detachment
 Priapism
 Distressing complication that occurs at all ages
 Difficult to treat
 Causes a high incidence of impotence
 Chronic Anemia
 Associated with fatigue, irritability, jaundice, pain, delayed puberty, leg
sores, eye problems, gum disease

19. 1. Fever
2. Chest pain
3. Shortness of Breath
4. Increasing tiredness
5. Abdominal swelling
6. Unusual headache
Danger Signs of a Crisis
7. Any sudden weakness or
loss of feeling
8. Pain that will not go away
with home treatment
9. Priapism (painful erection
that will not go down)
10.Sudden vision change
SEEK URGENT HOSPITAL TREATMENT IF IN CRISIS

20. Screening
1. Haemoglobin Electrophoresis
 Simple Blood test
 Routine screening in high risk groups
• During pregnancy
• Before anaesthesia
2. Prenatal Testing
 Amniocentesis
 16 and 18 weeks of the pregnancy
 small risk of causing a miscarriage (1 in 100)
 Chorionic villus sampling (CVS)
 9th or 10th week of pregnancy
 very small amount of material from the developing placenta
 slightly higher chance of miscarriage

21. Diagnostic Tests
 Rarely seen before 4-6 mos.
 Sickledex: sickle turbidity test; finger or heel stick;
results in 3 min
 Sickledex does not distinguish between sickle
cell trait and sickle cell disease. Hemoglobin
electrophoresis is required to distinguish
between these 2 disorders.
 Hgb electrophoresis to “fingerprint” protein
 Hx, , CBC
 CXR if symptoms

22. Diagnostic Tests (cont’d)
 Peripheral Blood Smear
 The routine peripheral blood smear does
not contain sickled red blood cells unless
hypoxemia (inadequate levels of oxygen
in the blood) or a precipitating factor
(such as a red blood cell poison or drug)
is present.

23. Diagnostic Tests (cont’d)
 Electrophoresis
 Both sickle cell disease (homozygous for
hemoglobin S) and sickle cell trait
(heterozygous for Hgb S) can be detected by
this test.
 This test separates different types of
hemoglobin based on electrical charge. Hgb
S can be differentiated from Hgb A and
other types of Hgb.

24. Diagnostic Tests (cont’d)
 Normal Results
 In hemoglobin electrophoresis, no Hgb S
should be present. Normal hemoglobins in
an adult are mostly Hgb A with small
amounts of Hgb A2 and Hgb F.
 What do abnormal results mean?
 sickle cell trait
 sickle cell anemia

25. TREATMENT
 Primary Goals:
1. Prevent Crisis (avoid sickling
phenomenon)—through adequate
oxygenation & hydration in every day life
activities. Hydroxyurea may be used
prophylactically in adolescents and older adults.
Folic Acid 1mg daily is also recommended to
restore folate stores needed for erythropoiesis.
2. Treat crisis as an emergency when it
occurs—determine etiology quickly and
treat appropriately

26. Nursing Management
 Adequate hydration (also any electrolyte
replacement if needed)
 Pain management
 Promote rest
 Treat any infections
 Blood transfusions if needed
 Monitor O2 – supplement if needed
 Oral hygiene
 Maintain nutrition
 Family support and education--

27. NURSING CARE
 Pain Control—strive to prevent pain.
 Opioids as morphine, oxycodone, hydromorphone &
methadone are preferred. Give IV or PO for severe pain
round-the-clock. PCA pumps are great! Avoid Demerol
(Meperidine) - ↑seizure risk
 Tylenol & Ibuprofen may be used after dx is made
 Use non-pharmacologic methods also
 Heat (e.g. K-pad)to affected joints may be soothing by 
O2 with vasodilation.

28. NURSING CARE
 Hydration–
 Accurate I & O essential
  IV/PO fluids up to 150ml/kg/day will promote
hemodilution
 Note color of urine—sometimes brown
 Prevent tissue deoxygenation
 Avoid strenuous activity
 Avoid environments with low O2 concentration
 Avoid extremes in temp- esp. cold environment
 Avoid emotional stress
 Avoid known sources of infection

29. Child/Family Education
 How to manage & minimize crisis: avoid
dehydration, cold, stress, hypoxia,
acute illness, etc.
 Signs of infection
 Importance of immunizations
 Antibiotic administration if ordered
 Education & support re: long term
effects
 Genetic counseling

30. Nursing Considerations
 Numerous blood transfusions
 Assess s/s of hemosiderosis
 Assess s/s infection
 Folic acid supplementation
 Iron chelation tx as ordered
 Family support
 Possible bone marrow transplant

31.  There you have it!