Sickle cell anemia

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1. SICKLE CELL ANEMIA
Sickle cell anemia is
a serious disease in
which the body
makes sickle-
shaped red blood
cells. “Sickle-
shaped” means that
the red blood cells
are shaped like a
"C."

3. Normal red blood cells are disc-shaped
and look like doughnuts without holes in
the center.
They move easily through your blood
vessels. Red blood cells contain the
protein hemoglobin .
This iron-rich protein gives blood its red
color and carries oxygen from the lungs
to the rest of the body.

4. Sickle cells contain abnormal hemoglobin
that causes the cells to have a sickle
shape.

Sickle-shaped cells don’t move easily
through your blood vessels.

They’re stiff and sticky and tend to form
clumps and get stuck in the blood vessels.

5. Classificaton
Sickle-cell anaemia is the name of a
specific form of sickle-cell disease in
which there is homozygosity for the
mutation that causes HbS.
Sickle-cell anaemia is also referred to
as "HbSS", "SS disease", "haemoglobin
S“.

6. It is referred to as "HbAS" or "sickle cell
trait".
sickle-haemoglobin C disease (HbSC)
including both Hbs and Hbc.
Sickle thalassaemia disease a
combination of sickle cell trait and beta
thalassaemia trait.

7. Cont…
β+ refers to the ability to still produce
some normal Hba.
sickle beta-zero-thalassaemia (HbS/β
0
)
indicates that there is no ability to
produce Hba

8. Causes

9. Patho…
Sickle-cell anaemia is caused by
point mutation

in β-globin chain of haemoglobin
causing the amino acid glutamic acid

to be replaced with the hydrophobic
amino acid valine at the sixth position

10. β-globin gene is found on the short arm of
chromosome 11
association of two wild-type α-globin subunits
with two mutant β-globin subunits forms
haemoglobin S (HbS)

11. Under low-oxygen conditions
the absence of a polar amino acid at
position six of the β-globin chain
promotes the non-covalent polymerisation
of haemoglobin (aggregation)

12. which distorts red blood cells into a sickle
shape and decreases their elasticity
loss of red blood cell elasticity is central
to the pathophysiology of sickle-cell
disease

13. Normal red blood cells are quite elastic,
which allows the cells to deform to pass
through capillaries
sickle-cell disease, low-oxygen tension
promotes red blood cell sickling

14. repeated episodes of sickling damage
the cell membrane
and decrease the cell's elasticity
These cells fail to return to normal
shape when normal oxygen tension is
restored

15. As a consequence, these rigid blood cells
are unable to deform as they pass
through narrow capillaries
leading to vessel occlusion and
ischaemia

16. point mutation
A type of mutation that causes the
replacement of a single base nucleotide
with another nucleotide of the genetic
material, DNA or RNA.
The term point mutation also includes
insertions or deletions of a single base
pair

17. Signs and Symptoms
Common symptoms include:
Attacks of abdominal pain
Bone pain
Breathlessness
Delayed growth and puberty
Fatigue

18. Cont..
Fever
Jaundice
Paleness
Rapid heart rate
Ulcers on the lower legs

19. Cont..
Other symptoms include:
Chest pain
Excessive thirst
Frequent urination
Poor eyesight/blindness
Strokes
Skin ulcers

20. Precipitating Factors of Crisis
Dehydration
Infection
Trauma
Strenous physical exertion
Extreme fatigue

21. Cont..
Cold exposure
Hypoxia
Acidosis
Surgery
pregnancy

22. Vaso-occlusive crisis
Vaso-occlusive
crisis- is caused
by sickle-shaped
red blood cells
that obstruct
capillaries and
restrict blood flow
to an organ

23. Signs and Symptoms

24. Cont….

25. Diagnosis
Complete blood count
Hemoglobin electrophoresis
Sickle cell test
Bilirubin
Blood oxygen
CT scan or MRI
Peripheral smear

26. Cont..
Serum creatinine
Serum hemoglobi
Serum potassium
Urinary casts or blood in the urine
White blood cell count

27. Diagnosis
In HbSS, the full blood count reveals
haemoglobin levels in the range of 6–8 g/dL
with a high reticulocyte count
(as the bone marrow compensates for the
destruction of sickle cells by producing more
red blood cells). In other forms of sickle-cell
disease, Hb levels tend to be higher.

28. A blood film may show features of
hyposplenism
The presence of sickle haemoglobin can also
be demonstrated with the "sickle solubility
test".
A mixture of Hb S in a reducing solution (such
as sodium dithionite) gives a turbid
appearance, whereas normal Hb gives a
clear solution.

29. Abnormal Hb can be detected on Hb electrophoresis
on which the various types of haemoglobin move at
varying speeds
HgbS and haemoglobin C with sickling (HgbSC)—
the two most common forms—can be identified
A urinalysis to detect an occult urinary tract infection,
and chest X-ray to look for occult pneumonia should
be routinely performed

30. Prevention of sickling
Promote adequate oxygenation and
hemodilution
Encourage increased intake of fluids
150ml/kg/day
Avoid high altitude and other low
oxygen environment
Avoid strenuous physical exertion
Maintain oxygen saturation at 90%

31. Avoid extreme heat or cold
Routine blood transfusion

32. Treatment
Dietary cyanate, from foods containing
cyanide derivatives, has been used as a
treatment for sickle- cell anemia
Cyanate and thiocyanate irreversibly
inhibit sickling of red blood cells drawn
from sickle cell anemia patients

33. Vaso-occlusive crisis
Most people with sickle-cell disease have
intensely painful episodes called vaso-
occlusive crises.
Painful crises are treated symptomatically
with analgesics; pain management requires
opioid administration at regular intervals until
the crisis has settled.

34. For milder crises, a subgroup of
patients manage on NSAIDs (such as
diclofenac or naproxen)
For more severe crises, most patients
require inpatient management for
intravenous opioids; patient-controlled
analgesia (PCA) devices are commonly
used

35. Diphenhydramine is also an effective
agent that is frequently prescribed by
doctors in order to help control any
itching associated with the use of
opioids.
Folic acid and penicillin
Children born with sickle-cell disease
will undergo close observation by the
pediatrician.

36. These patients will take a 1 mg dose of
folic acid daily for life. From birth to five
years of age,
they will also have to take penicillin
daily due to the immature immune
system that makes them more prone to
early childhood illnesses

37. Acute chest crisis
Management is similar to vaso-
occlusive crisis, with the addition of
antibiotics

Oxygen supplementation for hypoxia,
and close observation
If pulmonary infiltrate worsen or the
oxygen requirements increase, simple
blood transfusion or exchange
transfusion is indicated

38. It involves the exchange of a significant
portion of the patients red cell mass for
normal red cells
Which decreases the percent of
haemoglobin S in the patient's blood
Hydroxyurea, was shown to decrease
the number and severity of sickle cell
disease

39. This is achieved, by reactivating fetal
haemoglobin production in place of the
haemoglobin S that causes sickle-cell
anaemia.
Bone marrow transplants have proven
to be effective in children

40. Dialysis or kidney transplant for kidney
disease
Drug rehabilitation and counseling for
psychological complications
Gallbladder removal (if you have
gallstone disease)

41. Hip replacement for avascular necrosis
of the hip
Irrigation or surgery for persistent,
painful erections (priapism)
Surgery for eye problems
Wound care, zinc oxide, or surgery for
leg ulcers

42. Nursing Management
Edu. of the family and child
Early intervention for problems such as
fever
Administer antibiotics acc. To
prescription
Adequate hydration to prevent sickling
Early recognition of resp. problems

43. Alternative pain management
technique, behavior modification
program ,relaxation therapy,hypnosis,
music therapy, message and
transcutaneous electrical nerve
stimulation
Give primary immunizations as well as
pneumococcal, HIB, hepatitis and
trivalent influenza vaccine

44. Complications
Chronic hemolytic anemia
Risk of death in children younger than 5
yrs mainly from sepsis
Splenic sequestration
Hemolysis
Aplasia
Vaso-occlusion