Presentation for clinical Pharmacy Obstetrics and Gynaecology rotation

1. SICKLE CELL DISEASE
BY MR BWALYA FERNANDO
COMPUTER NUMBER: 14115867
OBSTETRICS AND GYNAECOLOGY

2. INTRODUCTION
• Is an autosomal recessive disease
• HbS caused by mutation in β globin gene in which 17th
nucleotide is changed fromA toT and the 6th AA in
the β globin chain becomes valine instead of glutamic
acid .

3. PATHOPHYSIOLOGY
• HbS polymerises when deoxygenated, making it less soluble.
• These Hb polymers interact with the RBC membrane causing the characteristic
sickle shaped blood cell.
• The pathological features of SCD relate to the shortened life span of the sickled
blood cells (16-20 days in contrast to a lifespan of 120 days in normal red cells)
causing a haemolytic anaemia and adhesion of HbS containing cells and white cells
to the lining (endothelium) of the microvascular vessels.

4. CLINICAL PRESENTATION
• Clinical features are of a severe haemolytic anaemia punctuated by crises
• Some patients may have crisis free lives while others may have severe ones
and die in early childhood or as young adults.
• Crises may be vaso - occlusive, visceral, aplastic or haemolytic

5. PREGNANCY
• Painful episodes become more common in the last trimester
• The incidence of pre-eclampsia is higher than normal in SCD patients and
there is a slight increase in maternal mortality
• Risk to the fetus from abortion, stillbirth, low birth weight and neonatal
death is also increased.

6. CONTRACEPTIVE USE
• Due to pregnancy complications they are encouraged to use contraceptives
• It is safe to use oral contraceptives for birth control in
SCD.
• Research has shown that injectable are more advantageous than oral in
terms of pain episodes.

7. Contraceptive Use
• Combined Oral Contraceptives should be avoided due to risk of thrombosis.

8. OTHER COMPLICATIONS
•Hepatobilliary complications
•Priapism
•Leg ulcers

9. DIAGNOSIS
•Typical clinical picture of chronic hemolytic
anemia and vaso-occlusive crisis.
•Newborn blood spot screen
•Blood Smear
•Blood Electrophoresis

10. Management
GOALS
improve survival
reduce the frequency, duration and severity of painful
crises and other complications
improve quality of life, which includes early detection
and management of organ damage.

11. Treatment
•Improved with Oxygen and Fluids
Hypoxia
Dehydration
Acidosis

12. Treatment for Life
•Folic acid 5mg OD for life
•Deltaprim (Dapsone and Pyrimethamine)
•Enoxaparin

13. CONT
• Analgesics e.g. Aspirin 600mgTDS, Ibuprofen 400mgTDS if severe Opioids e.g.
Tramadol 50-100mg in 4-6 hours, Morphine to manage Pain
• Morphine most preferred in sickle cell crisis.
• Antibiotics e.g. cefuroxime, amoxicillin/clavulanate, penicillin VK, ceftriaxone,
azithromycin
To treat underlying bacterial infection from acute chest syndrome caused by
encapsulated bacteria mostly due to spleen damage.
Prevented with hygiene, vaccine and penicillin therapy especially to children

14. Non Pharmacological
• Blood transfusion – Monitor for risk of iron overload and
immune intolerance. Indicated in severe anaemia.
Also helps splenic sequestration, this can be prevented by
splenectomy.
• Bone MarrowTransplant

15. PresumptiveTreatment
•Children: Penicillin and Polysaccharide vaccine against
Streptococci pneumoniae to prevent sepsis and
meningitis
•Hydroxyurea- increases amount of α globin thus fetal
hemoglobin

16. Oral Iron Preparations used:
Preparation Tablet Size Elemental Iron Per
Tablet
Usual Ault Dosage
Ferrous sulfate
(hydrated))
325mg 65mg 3- 4 tabs /day
Ferrous sulfate
((desiccated)
200mg 65mg 3- 4tabs/day
Ferrous gluconate 325mg 36mg 3- 4 tabs/day
Ferrous fumarate 100mg 33mg 6- 8tabs /day
325mg 106mg 2- 3 tabs/day

17. Iron Use in SCD
• Taking iron supplements will not help people with sickle cell disease.This
type of anemia is not caused by too little iron in the blood;
• It’s caused by not having enough red blood cells.
• In fact, taking iron supplements could harm a person with sickle cell disease
because the extra iron builds up in the body and can cause damage to the
organs

18. Iron use in SCD in Pregnancy
• Only when you have done Iron studies

19. REFERENCES