Sickle Cell Disease Among Children in Africa: An Integrative Literature Review and Global Recommendations

Abstract

Sickle cell disease (SCD) is a genetic blood disorder affecting red blood cells, with high morbidity and mortality rates. The United Nations has recognized SCD as a global public health concern, and the World Health Organization (WHO) recommends that 50% of member states will have established SCD control programs by 2020 (World Health Organization, 2006).
This paper presents an integrative review of 63 references related to SCD among children less than 18 years of age in Africa, published between 2000 and 2015. The review focuses on the incidence, prevalence, morbidity, and mortality; current practices and challenges related to screening, diagnosis, and treatment; and recommendations for practice, policy, and research to improve health outcomes of children with SCD in Africa.
There have been significant improvements in the morbidity and mortality rates for children with SCD in high resource countries such as the United States due to factors such as early diagnosis through newborn screening programs, prophylactic therapy, comprehensive care programs including hydroxyurea therapy, and bone marrow transplant. Many of these interventions can confer the same benefits to SCD patients in Africa. Newborn screening for SCD, developing partnerships between high resource countries and countries in Africa to support training of healthcare workers, research, and sharing of knowledge can help to reduce the SCD burden in Africa.

Full text

Sickle cell disease among children in Africa: An integrative literature
review and global recommendations
Lucky L. Mulumba
a
, Lynda Wilson
b,
⇑
a
Intensive Care Unit, 60th Medical Group, Travis Air Force Base, CA, USA
b
The University of Alabama at Birmingham School of Nursing, Birmingham, AL 35294-1210 USA
article info
Article history:
Received 26 December 2014
Received in revised form 8 August 2015
Accepted 23 August 2015
Available online 2 September 2015
Keywords:
Sickle cell disease
Sickle cell anemia
Children
Africa
abstract
Sickle cell disease (SCD) is a genetic blood disorder affecting red blood cells, with high morbidity and
mortality rates. The United Nations has recognized SCD as a global public health concern, and the
World Health Organization (WHO) recommends that 50% of member states will have established SCD
control programs by 2020 (World Health Organization, 2006).
This paper presents an integrative review of 63 references related to SCD among children less than
18 years of age in Africa, published between 2000 and 2015. The review focuses on the incidence, preva-
lence, morbidity, and mortality; current practices and challenges related to screening, diagnosis, and
treatment; and recommendations for practice, policy, and research to improve health outcomes of chil-
dren with SCD in Africa.
There have been significant improvements in the morbidity and mortality rates for children with SCD
in high resource countries such as the United States due to factors such as early diagnosis through new-
born screening programs, prophylactic therapy, comprehensive care programs including hydroxyurea
therapy, and bone marrow transplant. Many of these interventions can confer the same benefits to
SCD patients in Africa. Newborn screening for SCD, developing partnerships between high resource coun-
tries and countries in Africa to support training of healthcare workers, research, and sharing of knowledge
can help to reduce the SCD burden in Africa.
Ó2015 The Authors. Published by Elsevier Ltd. This is an open accessarticle under the CC BY-NC-ND license
(http://creativecommons.org/licenses/by-nc-nd/4.0/).
Contents
1. Introduction .......................................................................................................... 57
2. Epidemiology . . . . . . . . . . . . . . . .......................................................................................... 57
3. Mortality rates . . . . . . . . . . . . . . .......................................................................................... 57
4. Morbidity . . .......................................................................................................... 58
5. Prevention, neonatal screening, and treatment strategies . . . . . . . . . . . . . . . . . . .................................................... 59
6. Genetic screening and counselling . . . . . . . . . . . . . . . . . ....................................................................... 59
7. Neonatal screening. . . . . . . . . . . .......................................................................................... 59
8. Prophylaxis treatments . . . . . . . .......................................................................................... 59
9. Malaria prophylaxis . . . . . . . . . . .......................................................................................... 60
10. Hydroxyurea treatment . . . . . . . .......................................................................................... 60
11. Traditional Herbal Remedies . . . .......................................................................................... 60
12. Treatment of anemia . . . . . . . . . .......................................................................................... 60
13. Treatment of vaso-occlusive crises . . . . . . . . . . . . . . . . . ....................................................................... 61
14. Prevention of stroke . . . . . . . . . . .......................................................................................... 61
15. Treatment of infection . . . . . . . . .......................................................................................... 61
16. Management of spleen dysfunction . . . . . . . . . . . . . . . . ....................................................................... 61
http://dx.doi.org/10.1016/j.ijans.2015.08.002
2214-1391/Ó2015 The Authors. Published by Elsevier Ltd.
This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
⇑
Corresponding author. Tel.: +1 205 934 6787.
E-mail addresses: nlukiah@yahoo.com (L.L. Mulumba), lyndawilson@uab.edu
(L. Wilson).
International Journal of Africa Nursing Sciences 3 (2015) 56–64
Contents lists available at ScienceDirect
International Journal of Africa Nursing Sciences
journal homepage: www.elsevier.com/locate/ijans

17. Recommendations for comprehensive SCD treatment programs . . . . . . . . . . . . . . . . . . .............................................. 61
18. Practice, policy, and research recommendation . . . . . . . . . . . . . ................................................................. 61
19. Recommendations for future research . .................................................................................... 62
20. Developing partnerships to address SCD in Africa . . . . . . . . . . . ................................................................. 62
21. Summary and conclusions . . . . . . . . . . . .................................................................................... 62
Conflict of interest . . . . . . . . . . . . . . . . . .................................................................................... 62
Acknowledgement . . . . . . . . . . . . . . . . . .................................................................................... 62
References . . . . ....................................................................................................... 62
1. Introduction
Sickle cell disease (SCD) is a genetic blood disorder affecting red
blood cells, with high morbidity and mortality rates. Sickle haemo-
globin (HbS) is a structural variant of normal adult haemoglobin
(HbA) (Chakravorty & Williams, 2015). SCD includes a series of
pathological genotypes resulting from the inheritance of HbS.
SCD affects 20–25 million people globally, and 50–80% of infants
born with SCD in Africa die before the age of 5 years (Aygun &
Odame, 2012). It is estimated that 240,000 children are born with
SCD annually in sub-Saharan Africa (Makani et al., 2011).
The United Nations General Assembly has recognized SCD as a
global public health concern due to the morbidity and mortality
caused by the disease and the significant social and economic
impact that results (United Nations General Assembly, 2009). The
purpose of this paper is to review the literature related to SCD
among children less than 18 years of age in Africa. The review
focuses on the incidence, prevalence, morbidity, and mortality of
SCD among children in Africa; current practices and challenges
related to screening, diagnosis, and treatment; and recommenda-
tions for practice, policy, and research to improve health outcomes
of children with SCD in Africa based on the literature and on global
guidelines.
The integrative review method proposed by Whittemore and
Knafl (2005) guided the process used for this review. This method
consists of five steps: (a) identification of the problem; (b) identi-
fication of search strategies; (c) evaluation of data quality; (d) data
analysis; and (e) synthesis and presentation of the data. The inclu-
sion criteria included full-text reports, web pages, or articles
(research-based or descriptions of existing programs or policies),
published between 2000 and 2015, and focused on SCD prevalence,
morbidity, mortality, screening, treatment, and/or policy guideli-
nes for children less than 18 years of age in Africa. Data-bases
included in the initial search included Medline, CINAHL, PubMed,
and Google Scholar, using the key words: ‘‘Sickle Cell Disease
(SCD)”, ‘‘Sickle Cell Anemia (SCA)”, ‘‘SCA/SCD Screening, Treatment,
and Policies,” cross-listed with keywords ‘‘Children” and ‘‘Africa.”
Additional sources were identified by examining reference lists of
each paper that was reviewed. A total of 63 references were iden-
tified that met inclusion criteria.
After the articles were retrieved, their strengths and limitations
were assessed by both co-authors, and key concepts were identified.
This paper presents a synthesis of the articles reviewed, and a discus-
sion of implications of the findings for practice, policy, and future
research. The articles were analyzed and grouped according to the
following categories to facilitate the final synthesis: epidemiology,
prevalence, and global health significance; mortality rates; morbid-
ity; genetic counseling and newborn screening; management; and
recommendations for policy, research, and global partnerships.
2. Epidemiology
Of the 330,000 babies born with a major hemoglobinopathy
worldwide, 275,000 have SCD, making it the major global
hemoglobinopathy (Aygun & Odame, 2012; Modell & Darlison,
2008; Weatherall, 2011). SCD patients in the developed world
account for only 10% of the world’s SCD patient population
(Aygun & Odame, 2012). In 2008, Aliyu et al. (2008) reported Uni-
ted Nations estimates that there are between 20 and 25 million
people worldwide living with SCD, of which 12–15 million live in
Africa. It is estimated that 75–85% of children born with SCD are
born in Africa, where mortality rates for those under age 5 range
from 50% to 80% (Aygun & Odame, 2012; Makani et al., 2011).
The highest prevalence of sickle-cell trait (SCT) in Africa occurs
between the latitudes of 15°North and 20°south, where the preva-
lence ranges between 10% and 40% of the population (Agasa et al.,
2010). In 2010, Rawezula reported results of a study of records of
over 2000 newborns at a hospital in Tanzania (Rwezaula, 2010).
Findings indicated that 18.2% of the neonates had abnormal hemo-
globin levels and that the incidence of abnormal hemoglobin levels
differed based on the geographical regions of the newborns’ par-
ents. The incidence of SCT among infants whose parents were from
the coastal areas was 35.6%, compared with 6.7% for infants whose
parents were from the northern region.
The incidence of the SCT in Cameroon, the Democratic Republic
of Congo, Gabon, Ghana, and Nigeria ranges from 20% to 30%, and
in some parts of Uganda, the prevalence is 45% (Afolayan &
Jolayemi, 2011; Agasa et al., 2010; Anie, Egunjobi, & Akinyanju,
2010; Serjeant & Ndugwa, 2004; World Health Organization,
2006). Chakravorty and Williams (2015) suggested that there are
few places where the carrier rate for SCD is greater than 25%
because of the disadvantages conferred by homozygosity.
Because many births occur outside of hospitals and many chil-
dren die before diagnosis with SCD, there are limited statistical
data on the incidence of SCD in Africa (Serjeant, 2010; Serjeant &
Ndugwa, 2004). There is a need for additional research to gather
accurate data about SCD prevalence in order to direct appropriate
health care policies (Munyanganizi, Cotton, Vertongen, & Gulbis,
2006; Odunvbun, Okolo, & Rahimy, 2008). One approach to gener-
ating more accurate data about the incidence and prevalence of
SCT and SCD was reported in 2010 by Piel et al. (2010), who devel-
oped a geo-statistical mapping model based on the frequency of
the hemoglobin S (HBS) allele and population data.
It has been suggested that one factor associated with the high
incidence of SCD in tropical Africa is the protection against Plas-
modium malaria associated with having the SCT (Aygun &
Odame, 2012). However, that protection seems not to extend to
people with SCD (Komba et al., 2009; Rahimy et al., 2003). The the-
ory that the SCT offers some immunity against the malaria parasite
was found to be strong in tropical Africa through a geostatistical
mapping study, but could not be explained in other parts of the
world (Aidoo et al., 2002; Piel et al., 2010). Migration from Africa
and other regions with high rates of SCT and SCD has contributed
to the global spread of SCD to areas such as North America and
the Caribbean (Weatherall, 2011; Wonkam et al., 2011).
3. Mortality rates
Mortality rates associated with SCD vary widely across the
globe. Children born in high-resource countries with major
L.L. Mulumba, L. Wilson/ International Journal of Africa Nursing Sciences 3 (2015) 56–64 57

hemoglobinopathies (including SCD) have higher chances of sur-
vival and lower mortality rates than those born in poor resourced
countries (Aygun & Odame, 2012). The higher life expectancies for
SCD patients in high resource countries may be due to earlier diag-
nosis, greater access to care, education of caregivers, and better
disease management (Aygun & Odame, 2012; Modell & Darlison,
2008). The life expectancy for people with SCD in the United States
(U.S.) is 42 and 48 years for men and women, respectively. Quinn
and colleagues (Quinn, Rogers, McCavit, & Buchanan, 2010)
reported a 93.9% survival to adulthood of children diagnosed with
SCD who were followed in Dallas, Texas (Quinn et al., 2010). In
Jamaica, life expectancy stands at 53 and 58.5 years for men and
women, respectively (Wierenga, Hambleton, & Lewis, 2001).
In contrast, in 2010 Aygun and Odame reported estimates that
50–80% of children born with SCD in Africa die before the age of
5 years (Aygun & Odame, 2012), although accurate statistics are
often not available (Grosse et al., 2011; Serjeant & Ndugwa,
2003). The high mortality rates in Sub-Saharan Africa are influ-
enced by multiple factors including limited resources leading to
poor access to care, and lack of comprehensive SCD management
programs. Interventions that have been effective in reducing mor-
tality among SCD patients in high resource countries such as new-
born screening, and prophylactic penicillin administration are not
available in most low resource countries (Odame, 2010).
4. Morbidity
The disease process in SCD causes complications in multiple
body organs. Some of the most common morbidities associated
with SCD include chronic pain and intermittent painful episodes,
musculoskeletal problems, stroke, pulmonary hypertension, and
septicemia. These complications often co-exist, affecting the qual-
ity of life for patients, and if untreated, they may lead to death.
Pain associated with vaso-occlusive crises is due to microvascu-
lar occlusions triggering the activation of nociceptive afferent
nerve fibers (Stuart & Nagel, 2004). Children younger than 3 years
are prone to painful swelling of the hands, known as hand-foot
syndrome or dactylitis. Long bones and joints are often areas of
necrosis leading to pain. Micro vaso-occlusion in the mesenteric
blood vessels also causes intense abdominal pain (Stuart & Nagel,
2004). In 2003, Komba and colleagues published findings from a
study of 236 SCD children (age 8 months–2 years) in Benin, and
reported that that 77.5% experienced painful vaso-occlusive crises,
with an average of 3.3 episodes per patient.
Many children with SCD experience musculoskeletal complica-
tions due to avascular necrosis, osteomyelitis, and septic arthritis.
Balogun et al. (2010) published findings in 2004 from a study of
318 SCD patients in Nigeria. Study participants ranged from 1 to
45 years of age, and 46% below age 10. These authors noted that
children below age 10 years were more likely to have multiple
musculoskeletal problems, and reported that among the children
younger than 10 years in this study, 95% had septic arthritis,
63.3% had osteomyelitis, and 7.2% had avascular necrosis of the
femoral head. Among children between 11 and 20 years of age,
46.4% had avascular necrosis of the femoral head, 30.6% had
osteomyelitis, and 5% had septic arthritis.
Stroke due to vascular occlusion is one of the most serious com-
plications of SCD (Kolapo & Vento, 2011). In high resource coun-
tries, it is estimated that the risk of developing a stroke among
SCD patients is 250 times higher than for those without SCD
(Makani, Williams, & Marsh, 2007). In 2009, (Verduzco & Nathan,
2009) reported findings from a study in the U.S. indicating that
24% of patients with SCD suffer a stroke by the age of 45 years.
In 2001, Makani, Williams and Marsh estimated that the rate
may even be higher in Africa due to the high prevalence of several
risk factors including low hemoglobin, leukocytosis, and the Bantu
haplotype (Makani et al., 2007). Recommended stroke prevention
interventions include screening using the Trans-cranial Doppler
assessments and chronic blood transfusions for higher risk patients
(Stuart & Nagel, 2004), but these interventions may not be avail-
able to patients in Africa due to limited care accessibility. Makani
et al. (2007) identified the need for more studies to document
the scope and risk factors for stroke among children with SCD in
Africa.
Pulmonary hypertension (PH) results from the chronic hemoly-
sis that occurs with SCD. This complication affects 30% of patients
with SCD in the U.S. with a mortality rate of 40% within 40 months
of diagnosis (Aliyu et al., 2008). Although most information about
PH in SCD patients is derived from studies on adults, new evidence
suggests that PH is also a problem in the pediatric population
(Aliyu et al., 2008). Although data about the incidence of PH among
children in Africa is limited, the high incidence of known infectious
risk factors for PH (HIV/AIDS, Hepatitis B and C, and malaria), may
contribute to high rates of this complication in Africa (Battersby,
Knox-Macaulay, & Carrol, 2010; Serjeant, 2005; Williams et al.,
2009).
Findings reported in articles published from 2005 to 2010 indi-
cate that bacterial infections leading to septicemia are also major
causes of morbidity and mortality for children with SCD in Africa,
particularly those below age 2 years (Battersby et al., 2010; Obaro,
2010; Serjeant, 2005; Williams et al., 2009). Children with SCD are
more likely to suffer from pneumococcal disease than those who
do not have SCD (Battersby et al., 2010; Obaro, 2010; Williams
et al., 2009). Even though Streptococcus pneumoniae is the widely
causative agent of infections in SCD children, findings from studies
published in 2005 and 2010 suggest that bacteremia in African
children with SCD may be caused by other bacteria (Battersby
et al., 2010; Obaro, 2010; Serjeant, 2005). In 2007, Kizito,
Mworozi, Ndugwa, and Serjeant (2007) reported findings from a
study of 155 children with SCD in Uganda, indicating that Staphy-
lococcus aureus accounted for 60% of the 47 positive blood cultures.
Other organisms identified included Haemophilus influenza,Staphy-
lococcus epidermis,S. pneumonia,Streptococcus viridans, and Escher-
ichia coli. In 2010, Battersby et al. reported that septicemia due to
nontyphi Salmonella species and Klebsiella species was the most
common cause of death in children below 5 years of age with
SCD in Nigeria (Battersby et al., 2010). In 2009, Williams et al.
reported findings from a study of 1749 children with SCD younger
than 14 years in Kenya (Williams et al., 2009). Findings from this
study indicated that the most common causes of bacterial infection
were S. pneumonia (41%), non-Typhus Salmonella (18%), and Hae-
mophilus influenza (12%). Other causative organisms included
Acinetobacter species (7%) and E. coli (7%). The findings from these
studies suggest that the types of bacteria causing infections in chil-
dren with SCD differ from country to country. In 2007, Kizito and
colleagues emphasized the importance of identifying the organism
causing bacterial infections in order to develop effective manage-
ment strategies (Kizito et al., 2007). In 2005, Sergeant questioned
the effectiveness of pneumococcal prophylaxis in SCD children in
Africa where there is infrequent isolation of S. pneumonia
(Serjeant, 2005).
A final complication of SCA and SCD that may contribute to both
morbidity and mortality is malaria. In 2009, Komba and colleagues
published findings from a study conducted in Kenya to examine
the prevalence and outcome of malarial infections among children
(ages 0–11 years) admitted to a hospital in Kenya from 1998 to
2005 (Komba et al., 2009). The authors noted that although it has
long been assumed that malarial infection is a major cause of mor-
bidity and mortality among SCD patients, findings from this study
indicated that the incidence of malarial parasitemia was lower
among children among children with SCD compared to children
58 L.L. Mulumba, L. Wilson/ International Journal of Africa Nursing Sciences 3 (2015) 56–64

without SCD. In 2010, Makani and colleagues reported similar find-
ings from a prospective surveillance study conducted in Tanzania
between 2004 and 2009 (Makani et al., 2010), Findings from this
study indicated that the prevalence of malarial parasitemia was
lower in patients with SCA than patients without SCA. However,
among patients who were hospitalized, parasitemia was associated
with higher mortality rates. Makani and colleagues stressed the
importance of effective treatment for malaria during hospitaliza-
tions among SCA patients, and both groups of researchers identi-
fied the need for further studies to guide policy about malarial
prophylaxis for SCD patients in Africa (Komba et al., 2009;
Makani et al., 2010).
5. Prevention, neonatal screening, and treatment strategies
Effective management of SCD revolves around genetic coun-
selling, neonatal screening and early diagnosis; prophylaxis with
immunizations; providing anti-malarial medications, antibiotics,
and hydroxyurea; and prompt management of complications. Bone
marrow transplantation in a selected segment of patients is the
only proven cure for SCD to date (Walters et al., 2001), but this is
an expensive treatment and in 2004 Sergeant and Ndugwa noted
that this option is not feasible as a public health approach in low
resource countries (Serjeant & Ndugwa, 2004).
6. Genetic screening and counselling
Several authors have suggested that prenatal screening and
diagnosis could reduce the burden of haemoglobinopathies in poor
resource countries (Kafando, Sawadogo, Cotton, Vertongen, &
Gulbis, 2005; Weatherall, 2011). In 2008, Modell and Darlison sug-
gested that the use of genetic epidemiological data can help to
assess SCD health care needs (Modell & Darlison, 2008). In 2006,
the World Health Organization (WHO) identified a variety of med-
ical genetic screening programs that are appropriate for low- and
middle-income countries and that could help to reduce the inci-
dence of SCD (World Health Organization, 2006). These programs
include carrier identification using family pedigrees and screening
tests, and postnatal screening for sickle cell disorders. The success
of SCD prevention through heterozygote detection and premarital
screening is influenced by the knowledge and attitudes of health
care providers and community members about SCD and its treat-
ment (Abioye-Kuteyi, Oyegbade, Bello, & Osakwe, 2009). In 2009,
Abioye-Kuteyi et al., 2009 reported results of a survey of 320 gov-
ernment workers in Nigeria. Findings indicated that 69% had poor
knowledge about SCD, although 95% had positive attitudes towards
premarital screening. These researchers also reported that 86.7% of
respondents and 74% of their partners had had sickle cell screen-
ing. Although 25% of the married and engaged respondents did
not know their partner’s sickle cell status, 33–66% indicated that
they would continue the relationship with their partner if either
or both had haemoglobinopathy (Abioye-Kuteyi et al., 2009).
Prenatal genetic testing as a means of prevention and control of
genetic diseases including SCD has been suggested as one of the
effective ways of eradicating genetic haemoglobinopathies. How-
ever, in 2006, Ahmed, Atkin, Hewison and Green noted that such
testing may result in ethical and moral challenges because positive
results may suggest termination of the pregnancy (Ahmed, Atkin,
Hewison, & Green, 2006). In 2005, Animasahun, Akitoye and Njo-
kanma reported findings from a cross-sectional survey of health
workers at a teaching hospital in Nigeria. Although 91.3% had
heard about SCD prenatal screening, only 75.3% knew that SCD
could be prevented by prenatal screening. A total of 48.2% of these
health workers were not aware that prenatal screening was avail-
able in Nigeria; and 42.1% would not allow preventive termination
of pregnancy for positive screening results (Animasahun, Akitoye,
& Njokanma, 2009).
In 2011, Wonkam et al. (2011) reported a high level of accept-
ability of pre-natal diagnosis and pregnancy termination for SCD
among Cameroonians parents, even though Cameroon had not
implemented a national SCD control program (Wonkam et al.,
2011).
7. Neonatal screening
In 2008, Tshilolo and colleagues noted that in high resource
countries where newborn screening has been introduced, patients
are enrolled into comprehensive care programs resulting in better
outcomes than in lower resource countries without comprehensive
screening programs (Tshilolo et al., 2008). In 2003, Rahimy and col-
leagues suggested that the introduction of newborn screening in
the developed world 20 years ago has cut down the mortality rate
for SCD from 16% to >1% (Rahimy et al., 2003). Early neonatal
screening for SCD enables the implementation of a comprehensive
care approach including prophylactic treatment, parental educa-
tion, and initiation of a tracking and follow-up program for identi-
fied patients (Grosse et al., 2011; Ohene-Frempong, Bonney,
Tetteh, & Nkrumah, 2005; Ohene-Frempong, Oduro, Tetteh, &
Nkrumah, 2008).
In low resource countries, access to newborn screening for SCD
or SCT is limited because of economic constraints. In 2008,
Odunvbun, Okolo, and Rahimy (2008) reported that despite the
huge SCD burden in Africa and the advantages associated with
newborn screening in the management of SCD, Benin and Ghana
are the only two countries in Africa that have comprehensive new-
born screening programs. The program in Benin is however tar-
geted at mothers with the SCT (Odunvbun et al., 2008).
A number of studies have been conducted to assess the efficacy
of newborn screening in Africa including studies in Nigeria
(Odunvbun et al., 2008); the Democratic Republic of Congo
(Tshilolo et al., 2009), and Ghana (Bosu, 2012). In 2008, Odunvbun,
Okolo and Rahimy suggested that newborn screening would be
widely accepted by parents in Nigeria (Odunvbun et al., 2008). Bet-
ter management protocols including early screening and compre-
hensive care may reduce mortality rate among children with SCD
in Africa. In 2012, Piel and colleagues recommended utilization
of global mapping to track the findings from neonatal screening
programs may provide important data on the incidence and preva-
lence of the disease in low-resource settings, thus promoting tar-
geted interventions and services and more effective use of scarce
resources (Piel et al., 2012).
8. Prophylaxis treatments
In 2009, Obaro noted that the high rate of child mortality due to
invasive bacteria in SCD children in Sub-Saharan Africa has not
received global international attention, however use of childhood
immunizations could reduce the incidence of this problem
(Obaro, 2009). Children with SCD need to receive routine immu-
nizations based on the country-specific guidelines. In 2007,
Makani, Williams and Marsh noted that pneumococcal vaccina-
tions and prophylaxis using penicillin has increased survival rates
among children in the developed world (Makani et al., 2007). In
2012, Aygun and Odame (2012) noted that improved immuniza-
tion and better nutrition programs in many countries has
improved survival among children with SCD. In 2010, Obaro sug-
gested that development of vaccines against non-Typhi Salmonellae
may reduce childhood mortality in SCD (Obaro, 2010).
Cox and colleagues (in 2011) reported that introduction of tar-
geted interventions like penicillin prophylaxis and Pneumococcal
L.L. Mulumba, L. Wilson/ International Journal of Africa Nursing Sciences 3 (2015) 56–64 59

vaccinations in the developed world has increased survival to
18 years of age to 94% in the U.S. and 99% in the United Kingdom
(Cox et al., 2011). There is disagreement among experts, however,
as to whether penicillin prophylaxis and pneumococcal vaccina-
tion are effective in Africa since some studies have shown that bac-
teremia in most African countries is not caused by S. pneumonia.In
2005, Serjeant (2005) described results from studies conducted in
Nigeria and Uganda, which indicated that S. pneumonia was
responsible for only 10% of all septicemias, with Staphylococci,
E. coli and Klebsiella responsible for the majority of septicemias.
Children with SCD below 2 years are 600 times more susceptible
to invasive Pneumococcal disease than children without SCD. In
2009, Battersby and colleagues reported that bacteria-related sep-
ticemia is responsible for many SCD-related deaths. Pneumococcal
vaccine and antibiotic prophylaxis forms the backbone of sep-
ticemia prevention (Battersby et al., 2010). These authors sug-
gested that febrile SCD patients suspected of bacteremia should
have blood cultures to identify the causative organism, and prompt
initiation of appropriate antibiotic therapy (Battersby et al., 2010).
9. Malaria prophylaxis
In 2007, Makani et al. (2007) noted that although it was widely
believed that malaria was a major cause of hospital admission and
mortality among SCD patients in sub-Saharan Africa, there was a
need to review the evidence about which drugs should be recom-
mended for long-term prophylaxis among SCD patients. In 2012,
Aygun and Odame acknowledged that further research is needed
to clarify the role of malaria as a cause of morbidity and mortality
in SCD patients, but advised that the use of insecticide-treated bed-
nets along with anti-malarial prophylaxis during high risk seasons
may be useful (Aygun & Odame, 2012).
10. Hydroxyurea treatment
Aygun and Odame reported in 2012 that Hydroxyurea therapy
has been used for the treatment of SCD in the United States and
Europe for over 25 years; with proven effectiveness in the reduc-
tion of acute painful episodes (Aygun & Odame, 2012). Hydrox-
yurea works by increasing fetal hemoglobin, and increasing
water content of red blood cells resulting in less cell deformity
and adhesion to the endothelium. It is also believed to have anti-
hemolytic properties (Aliyu et al., 2008; Aneni, Hamer, & Gill,
2013; Stuart & Nagel, 2004). Hydroxyurea also reduces hepatic
sequestration and priapism reducing the need for blood transfu-
sion; and lowers mortality from SCD related complications by
40% (Aliyu et al., 2008). In 2013, Aneni, Hamer, and Gill published
a systematic review of strategies for reducing morbidity from
malaria in SCD, including the use of hydroxyurea to reduce
malaria-associated morbidity and mortality in SCD patients. These
authors noted that most studies of hydroxyurea have been con-
ducted in non-malarious regions, and there is little information
on its use in malaria endemic areas. These authors noted that
hydroxyurea actually upregulates the intercellular molecule recep-
tor for adhesion of malarial-infected red blood cells and thus the-
oretically its use could enhance replication of malaria cells.
However hydroxyurea also increases fetal hemoglobin levels
which protect against malaria. In 2004, Stuart and Nagel (2004)
suggested that infants and children with SCD who are at greatest
risk for negative outcomes (e.g. those with hand and foot syn-
drome, severe anemia, or a history of a stroke) should be consid-
ered for hydroxyurea therapy. In 2015, Chakravoty and Williams
reported that although hydroxyurea is the only agent that has been
found to reduce the number of painful sickle cell crises in infants,
children, and adults with SCD, it is not widely used due to patient
and provider uncertainties about its risks and benefits
(Chakravorty & Williams, 2015). There is clearly a need for further
research to evaluate the use of hydroxyurea for children in Africa,
particularly those who live in areas with a high prevalence of
malaria.
11. Traditional Herbal Remedies
In 2005, Akinyanju, Otaigbe and Ibidapo reported that the use of
traditional herbal remedies in the management of SCD is common
practice in poor resourced countries such as Nigeria, where access
to care is limited and the population is predominantly rural
(Akinyanju, Otaigbe, & Ibidapo, 2005). In 2008, Okpuzor,
Adebesin, Ogbunugafor, and Amadi (2008) reviewed medicinal
plants with potential anti-sickling properties that have been used
in low resource countries and described research that has been
conducted to identify their beneficial effects. There is a need for
further studies on efficacy and effectiveness of these remedies
(Akinyanju et al., 2005). Such studies can be facilitated through
partnerships between healers with knowledge of herbal remedies,
and research organizations. One example of such a partnership is
the development of NIPRISAN by collaboration between the
National Institute for Pharmaceutical Research and Development
(NIPRD) in Nigeria and members of the local communities with
knowledge of herbal remedies. NIPRISAN is a phytomedicine that
was developed to manage SCD by NIPRD from local plants includ-
ing Piper Guineese and Eugenia Caryophllate that have been used
by the Yoruba people to treat SCD. In a 2001 report of a double
blind, placebo controlled, randomized cross over clinical and labo-
ratory study, NIPRISAN was found to be safe and effective in less-
ening the frequency of severely painful episodes (Wambebe
et al., 2001). In 2012, Ameh, Tarfa and Ebeshi reported that other
medicines were under development at NIPRD (Ameh, Tarfa, &
Ebeshi, 2012).
12. Treatment of anemia
Anemia is a major cause of morbidity and mortality in SCD, and
many patients die in hospital emergency rooms and wards before
blood transfusions can be initiated. In 2007 Ikefuna and Emodi
reported results from a study of 71 children hospitalized for SCD
in Nigeria (Ikefuna & Emodi, 2007). Findings indicated that 39.4%
had severe anemia, and that most cases were related to aplastic,
acute sequestration, hyper-hemolytic and vasculo-occlusive crises.
The authors suggested, however, that it was important to assess for
other causes of anemia in SCD patients including malaria para-
sitemia and septicemia.
In 2004, Stewart and Nagel reported that blood transfusions in
SCD are routinely done either as an interim intervention to correct
anemia or hypovolemia, or as a chronic therapy to prevent stroke
(Stuart & Nagel, 2004). Indications for transfusion therapy include:
chronic splenic sequestration; severe or lasting aplastic crises;
acute stroke; acute chest syndrome; and hemolytic episodes asso-
ciated with malaria. Indications for chronic transfusions are stroke
prevention and chronic pulmonary hypertension (Aliyu et al.,
2008; Kolapo & Vento, 2011; Stuart & Nagel, 2004). In order to pre-
vent iron overload due to chronic transfusions, chelation may be
indicated. In 2007, Makani, Williams and Marsh noted that the
use of blood transfusions in Sub-Saharan Africa is restricted due
to the limited supply of blood, and potential HIV infection due to
poor blood screening (Makani et al., 2007). Sergeant cautioned that
limited human and diagnostic resources in many African settings
result in failure to properly investigate the causes of anemia, and
use of tansfusions when these may not be indicated. Sergeant fur-
ther suggested that reticulocyte counts and red blood cell indices
60 L.L. Mulumba, L. Wilson/ International Journal of Africa Nursing Sciences 3 (2015) 56–64

can help to diagnose iron or folate deficiency which can be treated
more appropriately by iron or folic acid, instead of by blood trans-
fusion (G. Sergeant, Personal Communication, December 1, 2014).
13. Treatment of vaso-occlusive crises
Vaso-occlusive crises are also known as pain crises, and these
crises are the major reason for visits to the emergency rooms by
children with SCD. These painful episodes arise as a result of
microvascular occlusions in bone marrow leading to necrosis
(Stuart & Nagel, 2004). Common sites of pain include long bones,
ribs, sternum spine and pelvis (Sadarangani et al., 2009; Stuart &
Nagel, 2004). In children younger than 3 years, acute hand-foot
syndrome which involves painful swelling of the hands and feet
is common. There are also abdominal painful episodes due to the
micro-vascular occlusion in the mesenteric vessels coupled with
decreased intestinal motility (Stuart & Nagel, 2004). Mild to mod-
erate painful episodes may be treated at home using nonsteroidal
anti-inflammatory drugs (NSAIDs), or opioids. Severe pain episodes
requiring hospitalization are treated with continuous parental opi-
oids. In 2004, Stewart and Nagel cautioned that although hydrox-
yurea therapy has been found to be effective in patients with
frequent severe occlusive episodes, further research was needed
and that the drug should only be given in an environment where
medical care was available and compliance could be monitored
(Stuart & Nagel, 2004).
14. Prevention of stroke
Stroke is the occlusion in the cerebral microvasculation and is
another common cause of morbidity among SCD patients. In
2011, Kolapo and Vento reported that stroke is an increasing prob-
lem in sub-Saharan Africa in both adults and children, and noted
that one of the factors contributing to this problem is SCD
(Kolapo & Vento, 2011). In 2011, Weather all reported that preven-
tion strategies of using regular screening and blood transfusion
have brought down complications related to stroke (Weatherall,
2011). In 2004, Stewart and Nagel suggested the use of transcranial
Doppler screening every year for children from 2 to 16 years of age
who have SCD as a strategy to identify those at risk for stroke
(Stuart & Nagel, 2004).
15. Treatment of infection
In a 2010 review of studies related to bacterial infections in
children with SCD, Battersby, Knox-Macaulay and Carrol noted that
a major reason for the increased susceptibility is due to splenic
dysfunction (Battersby et al., 2010). These authors acknowledged
the need for further research to study the benefits of treatments
such as penicillin prophylaxis and pneumococcal vaccines in
Africa. Nevertheless, these authors recommended that when chil-
dren with SCD present to the emergency room with a fever, a broad
spectrum antibiotic should be started as soon as blood and urine
specimen have been obtained for culture purposes.
16. Management of spleen dysfunction
Battersby and colleagues (2010) noted that functional asplenia
is present in about 50% of SCD children below the age of 2 years,
and is due to sluggish splenic blood flow which leads to shunting
of the splenic blood flow, bypassing the spleen’s normal filtration
function. In older children, asplenia is usually caused by repeated
splenic infarction (Battersby et al., 2010). Hypovolemia caused by
spleen sequestration requires prompt blood transfusion. In 2004,
Stewart and Nagel recommended splenectomy following the first
episode of acute spleen sequestration for children over the ages
of 2–3 years (Stuart & Nagel, 2004). In 2008, Hankins and col-
leagues reported findings from a study of 43 children with SCA
who were treated with hydroxyurea. The study was conducted in
the U.S., and the authors concluded that use of hydroxyurea at
the maximum tolerated dose may preserve spleen and brain func-
tion in children with SCA.
17. Recommendations for comprehensive SCD treatment
programs
The decrease in morbidity and mortality among SCD children in
low-resource countries is partly attributed to the presence of com-
prehensive care programs that include immunizations and vacci-
nations, prophylaxis therapy, vitamin supplements and patient
and caregiver empowerment through education (Rahimy et al.,
2003). In 2011, Cox and colleagues suggested that because SCD is
associated with a high prevalence of malnutrition and stunting
particularly among adolescents (Cox et al., 2011), comprehensive
programs should also include nutrition education.
In 2003, Rahimy et al. (2003) reported findings from an evalua-
tion of a comprehensive program in Benin that included provision
of parent education about the SCD disease process and factors that
may lead to acute SCD events. The education addressed topics such
as the importance of hydration, providing adequate nutrition,
spleen palpation, and other strategies to identify symptoms of
acute SCD complications. Other interventions included providing
recommended vaccinations and anti-pneumococcal and antimalar-
ial prophylaxis. Findings indicated a 78% reduction in the fre-
quency and severity of SCD-related acute events, as well as
improvement in general status and physical growth.
In 2005, Akinyanju and colleagues reported findings from eval-
uation of a holistic program provided to 1223 SCD patients in Nige-
ria that included malaria prophylaxis, health and nutrition
education, provision of folic acid and vitamin C, prompt attention
for acute illnesses, and a support club for patients and their fami-
lies. These authors reported a decrease in hospital admissions,
mortality rate, and number of patients requiring blood transfusions
over a 7 year period (Akinyanju et al., 2005).
18. Practice, policy, and research recommendation
In 2009, the members of the United Nations General Assembly
passed a resolution declaring SCD as a major public health concern
(United Nations General Assembly, 2009). In 2012, Bosu (2012)
suggested the need for governmental policies and standards to
address SCD that focus on health system strengthening. The
WHO Regional Office for Africa has recommended the need for
developing national SCD control programs that include advocacy,
prevention and counseling, early detection, treatment, surveil-
lance, research, and community education and partnerships
(World Health Organization Regional Office for Africa, 2015).
Others have suggested that policies should also provide for screen-
ing and genetic counseling for hemoglobin disorders (Modell &
Darlison, 2008; Ohene-Frempong et al., 2005; Tshilolo et al.,
2008). In 2008, Modell and Darlison recommended that policies
should address strategies to ensure that members of the public
and healthcare workers in Africa are informed about the impor-
tance of genetic testing, newborn screening, and management of
children SCD (Modell & Darlison, 2008).
In 2010, Odame reported that participants at an international
symposium and workshop on SCD in Benin noted that Hydrox-
yurea therapy is provided infrequently in Africa, yet its effective-
ness in the management of SCD has been demonstrated in high
resource countries (Odame, 2010). Odame also noted that other
L.L. Mulumba, L. Wilson/ International Journal of Africa Nursing Sciences 3 (2015) 56–64 61

interventions such as bone marrow transplantation and chronic
blood transfusion are either limited or not used at all, leaving
hydroxyurea to be a more viable option. For this therapy to succeed
however, certain barriers may have to be addressed including ade-
quate funding, and training of healthcare staff (Odame, 2010).
In 2015, Chakravorty and Williams (2014) recommended advo-
cacy by influential groups such as celebrities and politicians, and
the development of inexpensive and reliable point of care diagnos-
tic methods. In addition, these authors recommended increased
education about SCD in schools and colleges.
19. Recommendations for future research
There is a need for ongoing research related to SCD among chil-
dren in Africa. Primary research priorities that have been identified
in the literature include research to identify strategies to provide
SCD education and screening (Abioye-Kuteyi et al., 2009), provide
reliable data on SCD prevalence in Africa (Odame, 2010), identify
the relationship between malaria and SCD mortality, and evaluate
the use of antimalarial prophylaxis (Makani et al., 2007), pneumo-
coccal vaccines, and penicillin prophylaxis (Komba et al., 2009).
Although these vaccines have improved outcomes in developed
countries where S. pneumonia is the major causative agent for bac-
terial infection, there is conflicting data in Sub-Saharan Africa
about the organisms responsible for bacterial infections
(Williams et al., 2009).
20. Developing partnerships to address SCD in Africa
In 2012, Aygun and Odame (2012) identified the need for
greater international cooperation and partnerships to facilitate
access to education, management, surveillance and treatment of
SCD. These authors identified the potential role of partnerships
between industry and academic health centers, and acknowledged
that each country should develop their own programs based on
their unique needs. Participants in the 2009 Sickle Cell Symposium
in Benin agreed that global partnerships were essential for better
clinical care and study of the disease, and recommended establish-
ment of a global SCD network to foster global community and
advance the clinical care and study of patients with SCD (Odame,
2010).
21. Summary and conclusions
It has been over a hundred years since the first SCD formal diag-
nosis from a dental student from Granada was made by Dr. James
Herrick in Chicago (Serjeant, 2010). SCD has since ravaged the
world in particular amongst people of African and Mediterranean
heritage. The management and treatment of SCD in high resource
countries has greatly improved. Life expectancy for people with
SCD in Jamaica was reported at 53 years for men and slightly over
58 for women back in 2001 (Aygun & Odame, 2012). In 2012,
Aygun and Odame reported that mortality from SCD among chil-
dren in the U.S. has also greatly decreased with reports of patients
reaching 18 years of age at 93.9% (Aygun & Odame, 2012). These
results have been realized due to a number of advances in the man-
agement and treatment of SCD including early diagnosis through
newborn screening programs, prophylactic therapy for encapsu-
lated bacteria, comprehensive care programs including education
to caregivers, hydroxyurea therapy, and bone marrow transplant.
Although it is important to be careful in transplanting wholly
some of the interventions that have worked in high resource coun-
tries, many of these interventions can confer the same benefits to
SCD patients in Africa. Newborn screening for SCD, developing
partnerships between high resource countries and countries in
Africa to support training of healthcare workers, research, and
sharing of knowledge can help to reduce the SCD burden in Africa.
Improvements in health policies are also needed, including
increased funding for SCD programs, and establishment of compre-
hensive care programs encompassing nutrition, caregiver educa-
tion, prevention and management of complications, use of
hydroxyurea, and eventually, availability of bone marrow trans-
plantation will assist in reducing morbidity and mortality related
to SCD among children in Africa. As noted by Serjeant in 2005
‘‘We cannot yet cure sickle cell disease, but we have learnt that
simple interventions significantly improve morbidity and mortal-
ity. We need to extend these benefits to African patients urgently”
(Serjeant, 2005).
Conflict of interest
None declared.
Acknowledgement
The authors acknowledge the contributions of Dr. Graham Ser-
jeant who reviewed a draft of this paper and provided suggestions
and comments for revision.
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