Sickle cell disease (SCD) is a genetic blood disorder in high prevalence in sub-Saharan Africa (SSA) that leads to high morbidity and early mortality. Newborn screening (NBS) with evidence-based interventions saves lives of individuals with SCD. SSA accounts for 75% of the global prevalence of SCD, but it has not been able to implement universal NBS for SCD. This article examines policy framework for NBS in SSA; the methods, processes, barriers, and enablers of NBS; and enrollment in comprehensive care to make available the evidence-based interventions that caregivers need to access in order to save the lives of babies with SCD.

1.
Therrell
BL
,
Lloyd-Puryear
MA
,
Ohene-Frempong
K
, et al.
Empowering newborn screening programs in African countries through establishment of an international collaborative effort
.
J Community Genet
.
2020
;
11
(
3
):
253
-
268
.
2.
Vichinsky
E
,
Hurst
D
,
Earles
A
,
Kleman
K
,
Lubin
B.
Newborn screening for sickle cell disease: effect on mortality
.
Pediatrics
.
1988
;
81
(
6
):
749
-
755
.
3.
Charache
S
,
Barton
FB
,
Moore
RD
, et al.
Hydroxyurea and sickle cell anemia. Clinical utility of a myelosuppressive “switching” agent. The multicenter study of hydroxyurea in sickle cell anemia
.
Medicine (Baltimore)
.
1996
;
75
(
6
):
300
-
326
.
4.
Adams
RJ
,
McKie
VC
,
Brambilla
D
, et al.
Stroke Prevention Trial in Sickle Cell Anemia
.
Control Clin Trials
.
1998
;
19
(
1
):
110
-
129
.
5.
Olney
RS
. Newborn screening for sickle cell disease: public health impact and evaluation. In:
Genetics and Public Health in the 21st Century
.
Oxford University Press
;
2000
:
431
-
446
.
6.
Adamkiewicz
TV
,
Silk
BJ
,
Howgate
J
, et al.
Effectiveness of the 7-valent pneumococcal conjugate vaccine in children with sickle cell disease in the first decade of life
.
Pediatrics
.
2008
;
121
(
3
):
562
-
569
.
7.
Piel
FB
,
Rees
DC
,
DeBaun
MR
, et al.
Defining global strategies to improve outcomes in sickle cell disease: a Lancet Haematology Commission
.
Lancet Haematol
.
2023
;
10
(
8
):
e633
-
e686
.
8.
World Health Organization
.
Sickle-cell disease: a strategy for the WHO African Region
. Accessed
2
May
2022
. https://apps.who.int/iris/handle/10665/1682.
9.
World Health Organization Regional Committee for Africa
.
Progress in the implementation of the African Region sickle-cell strategy 2010-2020: information document
. AFR/RC70/INF.DOC/3.
30
July
2020
. https://iris.who.int/handle/10665/334098.
10.
Therrell
BL
,
Padilla
CD
,
Borrajo
GJC
, et al.
Current status of newborn bloodspot screening worldwide 2024: a comprehensive review of recent activities (2020-2023)
.
Int J Neonatal Screen
.
2024
;
10
(
2
):
38
.
11.
Reddy
MN
,
Franciosi
RA
.
Rapid quantitation of hemoglobin S by isoelectric focusing
.
Ann Clin Lab Sci
.
1994
;
24
(
5
):
401
-
406
.
12.
Fisher
SI
,
Haga
JA
,
Castleberry
SM
,
Hall
RB
,
Thompson
WC
.
Validation of an automated HPLC method for quantification of hemoglobin S
.
Clin Chem
.
1997
;
43
(
9
):
1667
-
1669
.
13.
Pant
L
,
Kalita
D
,
Singh
S
, et al.
Detection of abnormal hemoglobin variants by HPLC method: common problems with suggested solutions
.
Int Sch Res Notices
.
2014
;
2014
:
257805
.
14.
Chen
FT
,
Liu
CM
,
Hsieh
YZ
,
Sternberg
JC
.
Capillary electrophoresis–a new clinical tool
.
Clin Chem
.
1991
;
37
(
1
):
14
-
19
.
15.
Cotton
F
,
Wolff
F
,
Gulbis
B.
Automated capillary electrophoresis in the screening for hemoglobinopathies
.
Methods Mol Biol
.
2013
;
984
:
227
-
235
.
16.
Kuhn R
HKS
.
Capillary Electrophoresis: Principles and Practice
.
Springer Science and Business Media
.
2013
Mar
7
.
17.
Arishi
WA
,
Alhadrami
HA
,
Zourob
M.
Techniques for the detection of sickle cell disease: a review
.
Micromachines (Basel)
.
2021
;
12
(
5
).
18.
Kanter
J
,
Telen
MJ
,
Hoppe
C
,
Roberts
CL
,
Kim
JS
,
Yang
X.
Validation of a novel point of care testing device for sickle cell disease
.
BMC Med
.
2015
;
13
:
225
.
19.
Chindima
N
,
Nkhoma
P
,
Sinkala
M
, et al.
The use of dried blood spots: a potential tool for the introduction of a neonatal screening program for sickle cell anemia in Zambia
.
Int J Appl Basic Med Res
.
2018
;
8
(
1
):
30
-
32
.
20.
Nnodu
O
,
Isa
H
,
Nwegbu
M
, et al.
HemoTypeSC, a low-cost point-of-care testing device for sickle cell disease: promises and challenges
.
Blood Cells Mol Dis
.
2019
;
78
:
22
-
28
.
21.
Oluwole
EO
,
Adeyemo
TA
,
Osanyin
GE
,
Odukoya
OO
,
Kanki
PJ
,
Afolabi
BB
.
Feasibility and acceptability of early infant screening for sickle cell disease in Lagos, Nigeria-a pilot study
.
PLoS One
.
2020
;
15
(
12
):
e0242861
.
22.
Okeke
CO
,
Chianumba
RI
,
Isa
H
,
Asala
S
,
Nnodu
OE
. Using dried blood spot on HemoTypeSC™, a new frontier for newborn screening for sickle cell disease in Nigeria.
Front Genet
.
2022
;
13
:
1013858
.
23.
Olaniyan
HS
,
Briscoe
C
,
Muhongo
M
, et al.
Early diagnosis of sickle cell disease at birth hospitals and vaccination centers in Angola using point-of-care tests
.
Blood Adv
.
2023
;
7
(
19
):
5860
-
5867
.
24.
Guindo
A
,
Cisse
Z
,
Keita
I
, et al.
Potential for a large-scale newborn screening strategy for sickle cell disease in Mali: a comparative diagnostic performance study of two rapid diagnostic tests (SickleSCAN ® and HemoTypeSC ®) on cord blood
.
Br J Haematol
.
2024
;
204
(
1
):
337
-
345
.
doi: 10.1111/bjh.19108
.
25.
Hasan
MN
,
Fraiwan
A
,
An
R
, et al.
Paper-based microchip electrophoresis for point-of-care hemoglobin testing
.
Analyst
.
2020
;
145
(
7
):
2525
-
2542
.
26.
Piety
NZ
,
Yang
X
,
Kanter
J
,
Vignes
SM
,
George
A
,
Shevkoplyas
SS
.
Validation of a low-cost paper-based screening test for sickle cell anemia
.
PLoS One
.
2016
;
11
(
1
):
e0144901
.
27.
Therrell
BL
Jr
,
Hannon
WH
,
Bailey
DB
Jr
, et al.
Committee report: considerations and recommendations for national guidance regarding the retention and use of residual dried blood spot specimens after newborn screening
.
Genet Med
.
2011
;
13
(
7
):
621
-
624
.
28.
Chace
DH
,
Hannon
WH
.
Filter paper as a blood sample collection device for newborn screening
.
Clin Chem
.
2016
;
62
(
3
):
423
-
425
.
29.
Cheryl
H
,
Jeffrey
B
,
Michele
C
, et al.
Residual Dried Blood Spot Specimens Educational Toolkit
. Updated APHL DBS Policy Statement A of PHL, editor.
2018
.
30.
Archer
NM
,
Inusa
B
,
Makani
J
, et al.
Enablers and barriers to newborn screening for sickle cell disease in Africa: results from a qualitative study involving programmes in six countries
.
BMJ Open
.
2022
;
12
:
57623
.
31.
Bukini
D
,
Nkya
S
,
McCurdy
S
, et al.
Perspectives on building sustainable newborn screening programs for sickle cell disease: experience from Tanzania
.
Int J Neonatal Screen
.
2021
;
7
(
1
):
12
.
32.
Segbefia
CI
,
Goka
B
,
Welbeck
J
, et al.
Implementing newborn screening for sickle cell disease in Korle Bu Teaching Hospital, Accra: results and lessons learned
.
Pediatr Blood Cancer
.
2021
;
68
(
7
):
e29068
.
doi: 10.1002/pbc.29068
.
33.
Nnodu
OE
,
Adegoke
SA
,
Ezenwosu
OU
, et al.
A multi-centre survey of acceptability of newborn screening for sickle cell disease in Nigeria
.
Cureus
.
2018
;
10
(
3
):
e2354
.
34.
McGann
PT
,
Ferris
MG
,
Ramamurthy
U
, et al.
A prospective newborn screening and treatment program for sickle cell anemia in Luanda, Angola
.
Am J Hematol
.
2013
;
88
(
12
):
984
-
989
.
35.
Hernandez
AG
,
Kiyaga
C
,
Howard
TA
, et al.
,
Operational analysis of the national sickle cell screening programme in the Republic of Uganda
.
Afr J Lab Med
.
2021
;
10
(
1
):
1303
.
36.
Nkya
S
,
Mtei
L
,
Soka
D
, et al.
Newborn screening for sickle cell disease: an innovative pilot program to improve child survival in Dar es Salaam, Tanzania
.
Int Health
.
2019
;
11
(
6
):
589
-
595
.
37.
McGann
PT
.
Time to invest in sickle cell anemia as a global health priority
.
Pediatrics
.
2016
;
137
(
6
):
e20160348
.
You do not currently have access to this content.