1. CURRENT MANAGEMENT OF SCD IN NIGERIA
Oluwatoyin Adeoye

2. BUT ANY ONE WHO DOES NOT LOVE DOES NOT KNOW GOD, FOR GOD IS LOVE.
1 JOHN 4: 8
BUT ANY ONE WHO DOES NOT LOVE DOES NOT KNOW GOD, FOR GOD IS LOVE.

3. OUTLINE INTRODUCTION CLINICAL PRESENTATIONS MANAGEMENT
PATIENT/ PARENTS
DOCTORS/ HEALTH TEAM

4. INTRODUCTION What is sickle cell disease?
Sickle cell disease [SCD] is a genetically inherited disease in which an amino acid glutamic acid in position 6 on the beta globin is replaced by another amino acid called valine; leading to production of defective haemoglobin S.

5. An individual with SCD has inherited 2 abnormal haemoglobin genes, one of which is HbS.
The most common and most severe is SS disease.
Others are SC, Sβ- thal,

6. In SCD, the red blood cells assume an abnormal rigid sickle shape.
It occurs in 1 million Nigerians.
Over 40 million Nigerians are healthy carriers of the S gene.
About 150,000 children are born each year with HbSS.

9. It is an autosomal recessive disorder.
The one in four chance of SS occurs with each pregnancy.
The severity of SCD is multifactorial – type, haplotype, level of care etc

10. CLINICAL PRESENTATIONS
These are usually due to decreased solubility of Hb S. The solubility of deoxy-Hb S is 1% of oxy- Hb S and 10% of deoxy Hb A.
This decrease in solubility leads to rigidity of rbc membrane, sickle shape, and reduced life span.

11. Anaemia
Jaundce
Hepatosplenomegaly
Hand and foot syndrome
Small for age

12. CRISES
Vaso- occlusive
Hyperhaemolytic
Sequestration
Aplastic

13. Stroke
Retinopathy
Blindness
Acute chest syndrome
Chest infection
Cardiomegaly
Splenomegaly

14. Hepatomegaly
Cholilithiasis
Hypostenuria
Haematuria
Priampism
Delayed puberty
Avascular necrosis of femoral head
Ulcer

15. Infections
pneumonia
Osteomyelitis
UTI
Sepsis
Malaria

16. MANAGEMENT Patient/ Parents Regular follow up Balanced diet Water
Healthy habits
Take medications regularly
Immunisation

17. B. Health team
Diagnosis: Antenatal,neonatal,
Investigations: Steady state pcv, Transcranial doppler (TCD), irreversible sickling, Fe studies. Others.
Prevention of infection: immunization, proguanil, oral penicillin.
Folate supplementation

18. Anti – sickling agents: Nicosan, Ciklavit, Fagara zanthoxyloides,
Hydroxyurea
Follow up and counselling
Management of acute illnesses and complications.
Chronic transfusion therapy

19. Bone marrow transplantation:
CVA
Sub arachnoid haemorrhage
Recurrent acute chest syndrome,
Recurrent severe debilitating pain.
Ova and sperm still SS.

20. 1 CORINTHIANS 13:13
THERE ARE THREE THINGS THAT REMAIN – FAITH, HOPE, AND LOVE – AND THE GREATEST IS LOVE.

21. REFERENCES The Living Bible.
Adekile AD, Adeodu AA. Haemoglobinopathies. In: Azubuike JC, Nkanginieme KEO, editors. Paediatrics and child health in a tropical region. 2nd ed. Owerri, Nigeria: African Educational Services p 373 – 386.
Olu Akinyanju OON, The National burden of sickle cell disorder and the way forward. Sickle cell Foundation Nigeria. P 3- 13

22. 4. Sickle cell disease from wikipedia, http://en. wikipedia
4. Sickle cell disease from wikipedia, 5. Aliyu ZY, Tumblin AR, Kato GJ. Current management of sickle cell disease. Haematologica. 2006; 91(1): Oniyangi O. Sickle cell disease: Information for the general public. The sickle cell clinic. National Hospital, Abuja.2007.

23. 7. Muanya C. Scientists validate more ‘cures’ for sickle cell anaemia
7. Muanya C. Scientists validate more ‘cures’ for sickle cell anaemia. Natural health, Guardian