Overview and current management of sickle cell disease in Tanzania

Overview and current management of sickle cell disease in Tanzania

1. SICKLE CELL DISEASE
Dr. Gerald Lumanya

2. SICKLE-CELL DISEASE (SCD)
Definition
Sickle-celldisease(SCD) isa
geneticblooddisorder
characterizedbyredbloodcells
thatassumeanabnormal, rigid,
sickleshape.

4. Sicklingdecreasesthecells' flexibility
duringcirculationandresultsina riskof
variouscomplications.
The sicklingoccursbecauseofa
mutationinthehaemoglobin genewhich
resultsinsubstitutionofglutamicacidby
valineatposition6 oftheβ-chain.

5. Etiology
Sickle cell anaemia is a hereditary disease
inherited as an autosomal recessive trait.
If both parents are both carriers or sickle
cell trait (i.e. both are sickle cell trait),
the chances are that:
1)25% of the children will have sickle
cell anemia
2)50% of the children will be carriers
3)25% will be normal

8. Classification of sickle disease
1.Sickle cell anemia (“HbSS", "SS
disease", or "haemoglobin S" )
2.Sickle cell trait (“HbAS" or "sickle cell
trait“).
3.Sickle-haemoglobin C disease (HbSC),
and
4.Sickle beta-plus-thalassaemia (HbS/β+)
and sickle beta-zero-thalassaemia
(HbS/β0)

10. A WITH BOTH FRONTAL AND BIPARIETAL BOSSING

11. AN INFANT WITH DACTYLITIS DUE SICKLE CELL ANEMIA

12. INVESTIGATIONS

13. Other ancillary laboratory investigations
useful in detection and monitoring of the
disease

14. PERIPHERAL BLOOD SMEAR SHOWING SICKLED RBC

15. Only people with Hb AS are resistant
to malaria, since the infestation of
the malaria plasmodium is halted by
the sickling of the cells which it
infests.
Patients with HB SS are not. For
them malaria is the commonest
cause of sickle cell crisis.

16. Signs and symptoms
Sickle-cell disease may lead to
various acute and chronic
complications, several of which are
potentially lethal.

18. IMMEDIATELY START EMERGENCY
ASSESSMENT AND TREATMENT

20. PAIN RELIEF

21. General Pain relief

22. Morphine for severe relief of Pain

27. Treatment of Morphine overdose

28. FLUID BOLUS(HYDRATION)

30. BLOOD TRANSFUSION
WITHIN 30-60 MINS

31. SECONDARY SURVEY

32. BLOOD CULTURE AND IV ANTIBIOTICS
IMAGING AND RADIOLOGICAL INVESTIGATIONS

33. DOCUMENTATION AND REASSEMENT
CONSULTATION AND DISPOSITION

34. MANAGEMENT OF ACUTE
COMPLICATIONS OF SCD
Full systematic examination should be
done to all patients
FBP should be taken to all patients
presenting with acute complications

35. PAINFUL CRISIS

36. Painful crisis: Limbs/Joints

37. Abdominal crisis and Girdle Syndrome

40. FEBRILE ILLNESS

43. ACUTE CHEST SYNDROME

45. ACUTE SEVERE ANEMIA

47. ACUTE SPLENIC SEQUESTRATION

49. COMPLICATIONS
1.Hepatobiliary
Cholelithiasis
Acute cholecystitis
Intrahepatic cholestasis
2. Neurological complications
Stroke
Seizure/convulsions

50. GENITOURINARY COMPLICATIONS
Priapism

52. Prevention
People who are known carriers of the
disease often undergo genetic
counselling before they have a child.
A test to see if an unborn child has the
disease takes either a blood sample from
the fetus or a sample of amniotic fluid.
Since taking a blood sample from a fetus
has greater risks, the latter test is usually
used.

53. ASSIGNGMENT 1
1.Describe the chronic complications of Sickle cell
disease.
2.Describe the reproductive health choices for patients
with Sickle cell disease.
3.Describe the specific diseases/conditions to be
screened for sickle cell patients.
4.Blood transfusion is a key therapeutic modality in
SCD. Describe three main types of blood transfusion
and rationales of transfusing sickle cell patients