MALARIA IN SCD

Dr. Meshack Liru

Consultant Paediatrician
Homabay County Teaching and Referral Hospital
Malaria and Sickle Cell
Disease
Dr Liru
Sickle-cell disorders

• Haemoglobin disorders, which occur due to mutations in

one of the globin subunits of Hb, resulting in a change in
amino acid sequence
• Examples are SCA(HbSS), sickle cell trait (HbAS),
HbSC disease, sickle β-thalassemia disease (S/β thal),
HbS with other Hb variants (like D, O-Arab etc.) and
HbS/Hereditary persistence of fetal haemoglobin
HbS/HPFH (HbSF)
Hallmark of SCD

• Polymerization of deoxygenated RBC, distorting into

sickle shaped that results in the obstruction or occlusion
of the blood vessels
• Manifestations include chronic anaemia,VOCs,
Infections,CVAs, ACS, poor growth, fatigue and
jaundice and cognitive impairment.
• 75% of SCD patients are in SSA.
• Disorder follows a more severe clinical course in Africa
than in other parts of the world and that infectious
diseases (like malaria) have a role in causing this
increased severity
• Local prevalence rates is about 2% of general
population in high burden zones
• SCT rate above 15%
• 50% deaths by the age of 5 years.
Malaria

• Holoendemic in some regions in Kenya

• Causes asymptomatic, sub- microscopic parasitemia,
Uncomplicated and complicated syndromes
• High mortality among children U-5 yrs and gravid women
• Relationship with haemoglobinopathies
(Malaria/SCD/Burkitt’s belt)
• Balanced polymorhism
• Robust public health control programmes and case
management guidelines
PFPR Kenya 2015
Is malaria a big problem in the sickle cell community

• There is irrefutable evidence that Hb AS protects

against severe malaria(90%), Non-severe
malaria(50%).
• Variable protection by Hb C, thallasaemia
• HB SS- majority of studies suggest it’s protective
• Marginal Hb reserve and susceptibility to bacterial
sepsis is the big risk
Kilifi Genetic Birth Cohort Study (KGBCS) 2019

• Studies have shown that individuals with HbSS or HbAS variants have reduced
risk of high-density parasitaemia. However, HbSS individuals have an increased
mortality rate if they become parasitaemic compared to HbAS [9, 13]. Those with
HbAS have been shown to have asymptomatic infections and are best described
as malaria-protective [8, 14, 15]. The mechanisms proposed involves reduced
parasite growth in infected erythrocytes, decreased cytoadherence of parasitized
RBCs to endothelium, which enhances the removal of parasitized cells by the
immune system [16].
• HbSC disease is associated with significant clinical manifestations, but milder
than those of HbSS [1, 8]. Individuals with HbSC disease have a higher mean Hb
than those with HbSS [17]. Although HbSF has ~ 70% HbS and ~ 30% HbF, it is
clinically asymptomatic compared to HbSS because every RBC contains ~ 30%
HbF. Since HbF does not participate at all in polymer formation, a situation where
hydrophobic amino acid interacts with another haemoglobin to aggregate into
large polymers, thus prevents sickling of all RBCs under physiological conditions
[1]. HbSS has more severe clinical manifestations, thus it is of great public health
concern, compared to HbSC and HbSF that has mild clinical symptoms [10].
Kilifi Genetic Birth Cohort Study (KGBCS) 2019
TRACT Trial- Uganda

• Malaria infections are both less frequent and less severe

among children with SCA than they are in HbAA children.
• PFHRP2 lower among HBSS than Hb AA in cerebral
Malaria
• Only 4% of children recruited to TRACT had HbAS, in
comparison to 16% of children within the same community,
reflecting very high levels of malaria protection afforded by
this condition
Lancet Child Adolesc health. 2022 Sep. Sickle cell anaemia and
severe Plasmodium falciparum malaria: a secondary analysis of the
Transfusion and Treatment of African Children Trial (TRACT). Sophie
Uyoga PhD et al
• There is big vulnerability of children with SCA to severe
anaemia and bacterial sepsis with Malaria infections
• Low parastemia levels still trigger severe anaemia crisis
• The reason why in Africa, SCA should be diagnosed
early in life and affected children offered malaria
protection.
Malaria and Bacteremia

• Malaria infection strongly predisposes individuals to

bacteraemia and can account for more than half of all
cases of bacteraemia in malaria-endemic areas.
• HBb AS protects against this.
• Interventions to control malaria will have a major
additional benefit by reducing the burden of invasive
bacterial disease
Lancet 2011 Oct 8;Relation between falciparum malaria and bacteraemia in
Kenyan children: a population-based, case-control study and a longitudinal study,
,J Anthony G Scott et al
Main SCD and Malaria Pathogenetic factors

• Anaemia
• Hypersplenism
• Invasive bacterial infections
Strategies

• Early detection of SCD and Malaria

• Standard care for SCD
• Appropriate case management of Malaria
• Responsive blood transfusion service
• Appropriate antibiotic cover
• Malaria prevention –Public and Individual
actions(Mosquito protection, chemoprophylaxis and
vaccination)
Malaria Chemoprophylaxis

• In LIC-MIC, provision of prophylaxis for malaria should

be considered.
• SCD patients traveling to or living malaria endemic
areas would benefit from use of maximal mosquito
protection techniques and malaria chemoprophylaxis
• Long-term malaria chemoprophylaxis shown to lower
the incidence of crisis and to reduce mortality
Malaria Chemoprophylaxis

• Few studies have evaluated its benefit, particularly in SCD

• Malaria prophylaxis reduces the frequency of sickle cell
crisis, hospital admission, blood transfusion, and anemia
severity
• Further studies to compare antimalarial prophylaxis
medications needed
• It is not known how antimalarial drug resistance affects its
efficacy
Published online 2020 Feb 20. Life-Threatening Infectious Complications in Sickle
Cell Disease: A Concise Narrative Review. Dominik Ochocinski et al
Conclusion

• Malaria infection in SCD can lead to severe anaemia

and other complications
• Prompt diagnosis and treatment is essential
• Check out for bacterial sepsis and treat urgently
• Malaria protection in endemic regions is key
Questions?