Co esponding au ho : Akondji Bainako o a Dieudonné
Copy igh © 2025 Au ho (s) e ain he copy igh o his a icle. This a icle is published unde he e ms o he C ea i e Commons A ibu ion License 4.0.
Fac o s he onse o SS anemia and u u e p ospec s in he managemen o sickle cell
pa ien s’ managemen o sickle-cell anemia pa ien s
Bu uga Bhanduni Emmanuel 1, Akondji Bainako o a Dieudonné 2, *, Lilemo losembe Jé ôme 3 and Lokonda
Nome Moïse 4
1 Depa men o Anes hesia, Highe Ins i u e o Medical echniques o Kisangani, RD Congo.
2 Depa men o P i a e and Judicial Law, Uni e si y o Kisangani, Facul y o Law, RD Congo.
3 Depa men o Pedia ics, Highe Ins i u e o Medical echniques o Kisangani, RD Congo.
4 Depa men o Epidemiology, Highe Ins i u e o Medical echniques o Basoko, RD Congo.
Wo ld Jou nal o Ad anced Resea ch and Re iews, 2025, 26(02), 2708-2719
Publica ion his o y: Recei ed on 30 Ma ch 2025; e ised on 16 May 2025; accep ed on 18 May 2025
A icle DOI: h ps://doi.o g/10.30574/wja .2025.26.2.1381
Abs ac
In oduc ion: Sickle cell anemia is a equen and se ious gene ic disease a ec ing immig an popula ions o A ican
and Wes Indian o igin. I is caused by an abno mali y in hemoglobin, he p o ein inside ed blood cells ha anspo s
oxygen h oughou he body, esul ing in he de o ma ion o ed blood cells, which lose hei ounded shape and become
banana-shaped.
The objec i es o his s udy a e o Iden i y he a ious ac o s a o ing sickle cell c isis; P opose he necessa y means
o p e en sickle cell c isis; Raise awa eness o he me hods and echniques o p e en ing sickle cell c isis.
Me hodology: This is a desc ip i e, c oss-sec ional, quan i a i e s udy in ol ing 18 pa en s o sickle-cell child en
ollowed up a he ''G acia onda ion'' cen e in he ci y o Kisangani om 15/10 o 15/11 2024. Da a we e collec ed
using an in e iew echnique based on a p e-es ablished ques ionnai e.
Resul s: A e analysis, we a i ed a he ollowing esul s: 1. In ec ions (mala ia, a h i is, e c.), clima e change and
dus a e ac o s a o ing sickle cell c ises acco ding o 77.7%, 61.1% and 55.5% o he espec i e subjec s; 2. Ea ly
ea men o in ec ions, chemop ophylaxis wi h olic acid and ensu ing a good p o ein die we e ci ed as p e en i e
measu es o sickle cell c ises acco ding o 72. 2%, 66.6% and 50% o he espec i e subjec s; 3. A oiding he ma iage
o AS/AS couples h ough p ema i al examina ions (72.2%), eques ing he a oidance o ma e ni y in he e en o he
inad e en ma iage o AS/AS couples (55.5%) and e ec ing he di o ce o AS/AS, SS/SS couples (27.8%) a e he
me hods used o p e en he ansmission o sickle cell disease.
Keywo ds: Fac o ; Fa o ing; C isis; SS Anemia; Sickle Cell
1. In oduc ion
Following he disco e y o hemoglobin S (HBS) by Pauling in 1949, a cohe en pa hophysiological pa e n eme ged in
he 60s and 70s, de ailed a he molecula le el. This pa e n accoun s o hemoxy-hbs anemia, which o ms la ge ibe s
inside he ed blood cell (RBC) ha de o m and weaken i , and he onse o his c isis is o en linked o ce ain
en i onmen al, social and medical ac o s.
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Sickle cell disease, also known as sickle cell anemia, is a equen and se ious gene ic disease, a ec ing mainly immig an
popula ions o sub-Saha an A ican and Wes Indian o igin. I is caused by an abno mali y in hemoglobin, he p o ein
inside ed blood cells ha anspo s oxygen om he lungs o all he body's o gans. This hemoglobin anomaly esul s
in he de o ma ion o ed blood cells, which lose hei ounded shape (like a disk la ened in he middle) and ake on
he shape o a banana (a peculia i y which gi es he disease i s name). This is known as alci o ma ion [1].
Acco ding o Khella , o su e om sickle cell disease, bo h pa en s mus ha e a leas one copy o he S mu a ion, and
pass i on o hei child en. They canno p esen symp oms i hey a e bo h A/S. In his case, hei child en will ha e a
one in 4 (25%) chance o being S/S and, he e o e, o su e ing om sickle cell disease [2].
Sickle cell disease is a gene ic diso de a ec ing 15,000 pa ien s in F ance (20,000 by 2020). I has become he leading
gene ic disease ahead o cys ic ib osis, wi h mo e han 50% o sickle cell pa ien s li ing in he Île-de-F ance egion, and
ep esen s a eal public heal h p oblem [3].
Elion (2021), cons an ha , in he case whe e one o he wo pa en s su e s om his disease (he/she is he e o e S/S),
he isk o ansmission is 50% (one chance in wo), i one o he pa en s is A/S and he o he is A/A, he child en will
be 50% AA and 50% A/S, ca ie s o he mu a ed gene bu wi hou symp oms [4].
Recen epidemiological da a show ha in he neona al pe iod, 2% o newbo ns a e homozygous o he disease, and
a ound 40,000 sickle cell bi hs a e es ima ed each yea , while he adul popula ion sha ing he ai amoun s o 25%,
wi h he homozygous o m a ec ing a ound 2% o indi iduals [5].
In amilies whe e cases o sickle cell disease ha e been diagnosed, u u e pa en s can bene i om gene ic counseling
and unde go p ema i al examina ions be o e concluding hei ma iage o e en be o e concei ing hei i s child (wi h
analysis o hei genes o ind ou whe he hey a e ca ie s o he S mu a ion) [6].
Physical ac o s ha a o he exp ession o he disease a e: dehyd a ion, s ess and in ec ious synd omes cause an
inc ease in he concen a ion o HbS in ed blood cells, while hypoxemia, acidosis and hype he mia a o
deoxygena ion o ed blood cells. Sickle cell disease o en causes a majo economic imbalance in 79% o amilies whose
membe su e s epea ed c ises and equi es app op ia e ca e. Socio-demog aphic, economic, cul u al, medical-heal h
and en i onmen al ac o s a e all hough o be esponsible o he onse o sickle-cell c ises [7].
In he cou se o deoxygena ion ollowing ce ain espi a o y dys unc ions, he HBS molecule unde goes a
con o ma ional change. The eplacemen o hyd ophilic β6 glu amic acid (a wa e -soluble subs ance) by hyd ophobic
aline causes he la e o es ablish hyd ophobic bonds (a subs ance o po ion o a molecule ha does no dissol e in
wa e ) wi h o he hyd ophobic esidues on he β chain o ano he desoxy-hbs molecule, a polyme is o med and
elonga ed in o helical ibe s ha bundle oge he , s i en and cause he cha ac e is ic sickle cell shape o classic sickle-
shaped ed blood cells on he one hand, and on he o he , a low oxygen p essu e, desoxy HBS polyme izes and o ganizes
in o la ge polyme ibe s ha de o m, s i en and weaken he ed blood cell. This p ocess ep esen s he mechanis ic
basis o hemoly ic anemia and aso-occlusion [8].
In obs e ics, he occu ence o p egnancy in a sickle cell pa ien , wha e e he geno ype (SS, SC, SD, S6 Thalassemia), is
a e y high- isk si ua ion, as i is ma ked by high ma e nal mo bidi y and mo ali y in he pe ina al pe iod. This
associa ion has ecip ocal in luences: (in sickle-cell anemia, p egnancy agg a a es ch onic anemia, he high equency
o in ec ions, and he aso-occlusi e c isis a he end o p egnancy, du ing labo and on he i s day o pos -pa um. In
Re enge, sickle cell disease in luences delayed mena che, hea y mens ua ion and/o me o hagia which can
agg a a e anemia, and o he obs e ic complica ions including p eeclampsia and e oplacen al hema oma can occu
[9].
In i s 2006 epo , he WHO es ima ed ha 500 million people ca y he sickle cell ai , and ha a ound 50 million
people li e wi h he disease. E e y yea , 300,000 child en a e bo n wi h he disease, 2/3 o hem in sub-Saha an A ica.
Sickle cell disease is cha ac e ized by pain ul a acks and hema ological c ises, leading o a high ans usion isk and a
high suscep ibili y o in ec ions. This explains he high mo bidi y and mo ali y eco ded in sickle-cell pa ien s, and he
ac ha 50-80% o child en bo n on he A ican con inen will no each he age o 5 [10].
While aso-occlusi e c isis (VOC) and acu e ches synd ome a e he mos equen mani es a ions, he he apeu ic bases
and o he complica ions such as p iapism and s oke need o be known (B illan DAMUS, 2022) [11].
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Sickle cell disease is one o he mos common diseases in he Democ a ic Republic o Congo, as ecen epidemiological
da a demons a e. They show ha 2% o newbo ns a e homozygous o hemoglobin S and ha an es ima ed 40,000
sickle-cell-a ec ed child en a e bo n each yea , hal o whom die be o e he age o 5... al hough ea ly and app op ia e
ea men enables pa ien s o con ol hei symp oms and a oid se ious c ises. [12]. Acco ding o Shongo e al (2020),
in his s udy a Clinique NGALIEMA, in ec ions, dehyd a ion, hypoxia, ab up change o in ec ions (especially
pneumopa hies), ches pain and low empe a u e can igge sickle cell c isis synd omes in 37% o cases [13].
Sickle cell disease emains a majo public heal h p oblem in he Democ a ic Republic o Congo, he coun y wi h he
highes sickle cell bu den in he wo ld. In pa icula , he ac ha he DRC is he second coun y in A ica wi h he highes
numbe o sickle-cell su e e s, as well as being he i s coun y in he wo ld wi h he highes numbe o sickle-cell
su e e s wi h Ban u haplo ypes, makes his he mos se e e clinical o m o he disease. The main acu e sickle cell
complica ions a e aso-occlusi e bone c isis, acu e ho acic synd ome, s oke, acu e p iapism and acu e anemia [14].
I is a disease o psychological su e ing and isola ion. The bu den o sickle cell disease weighs on he shoulde s o he
whole amily g oup, who sha e he o deal o he ch onic disease and i s inancial consequences. Pa ien s eel excluded
by hei handicap and guil y o he imbalances and ensions induced by hei mis o une.
Wi h his in mind, he ollowing undamen al ques ions we e posed:
• Wha ac o s a o sickle cell c isis?
• Wha can be done o p e en sickle cell c isis?
• How can he ansmission o his gene ic disease wi hin he popula ion be p e en ed?
Resea ch objec i es
Gene al objec i e
The gene al objec i e o his esea ch p ojec is o iden i y he isk ac o s o p e en ing sickle cell c ises by imp o ing
he li ing condi ions o sickle cell pa ien s.
Speci ic objec i es
• Iden i y he a ious ac o s a o ing sickle cell c ises;
• Sugges ways o p e en ing sickle-cell c ises;
• Raise public awa eness o me hods and echniques o p e en ing sickle-cell c ises.
1.1. Resea ch goal
The ul ima e aim o his esea ch is o aise pa en s' awa eness o he ac o s ha con ibu e o sickle cell c ises, and o
p omo e he well-being o sickle cell su e e s. The aim is o secu e he heal h o he whole communi y, a sou ce o
de elopmen .
1.2. In e es o he s udy
Ou wo k is o h ee old in e es :
• I will help us o deepen ou knowledge o he a ious ac o s ha con ibu e o sickle-cell c ises;
• This esea ch is a da a bank o u u e esea che s who wan o unde ake a s udy o aspec s no co e ed in ou
esea ch;
• I is also a means o aising communi y awa eness o e ec i ely comba he ac o s con ibu ing o he onse o
sickle-cell c isis, and also o p e en he onse o his gene ic disease wi hin he communi y.
1.3. Type o s udy
This is a desc ip i e, c oss-sec ional, quan i a i e s udy o ac o s a o ing he occu ence o SS anemia c isis in sickle
cell pa ien s a he ''G acia onda ion'' ea men cen e in he ci y o Kisangani, Democ a ic Republic o Congo om 15
/10 o 15 /11 2024.
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1.4. S udy popula ion
The popula ion o his s udy is composed solely o pa en s o sickle cell child en a ending he ''G acia onda ion'' Sickle
Cell T ea men Cen e in he ci y o Kisangani. As hey o a e, i was di icul o b ing hem oge he , so we d ew a
ep esen a i e sample o acili a e he s udy.
Gi en he impo ance o his s udy, ou sample included 18 pa en s who had shown good ai h in esponding o ou
ques ionnai e.
1.5. Da a collec ion me hods and echniques
In iew o all hese conside a ions, and aking in o accoun he na u e and pu pose o ou s udy, as well as he di e en
le els o educa ion o ou s udy popula ion, we op ed o he in e oga o y me hod, whose in e iew echnique enabled
us o collec he da a. We used a ques ionnai e which we de eloped and adminis e ed o submi ed o he esponden s,
in he o m o a s uc u ed in e iew.
A e inishing wi h he adminis a ion o he ''G acia onda ion'' cen e , we we e accompanied by a nu se o collec da a
om he pa en s. Due o ime cons ain s, da a collec ion ook place e e y Tuesday and F iday om 10am o 2pm.
We he e o e andomly adminis e ed da a o 18 pa en s, making up he size o ou s udy popula ion.
1.6. Da a p ocessing
The da a collec ed we e analysed and encoded using Mic oso O ice Excel 2013 and SPSS 20.1. The esul s ob ained
we e p esen ed in he o m o ables. Da a analysis was made possible by calcula ing equencies and pe cen ages.
2. Resul s
2.1. Socio-demog aphic da a
2.1.1. Gende
Table 1 Gende dis ibu ion o s udy subjec s
Gende
%
Female
12
66.7
Male
6
33.3
To al
18
100
Analysis o Table I shows ha 66.7% o s udy subjec s we e emale, compa ed wi h 33.3% male.
2.1.2. Age
Table 2 Age dis ibu ion o s udy subjec s
Age ange in yea s
%
20 - 29
5
27.8
30 - 39
6
33.3
40 - 49
4
22.2
50 and o e
3
16.7
To al
18
100
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Analysis o Table II shows ha he s udy popula ion is made up mo e o subjec s in he 20 o 39 age b acke , i.e. 61.1%,
while hose aged 50 and o e ep esen only 22.2% o cases.
2.1.3. Le el o educa ion
Table 3 Dis ibu ion o esponden s by le el o educa ion.
Le el o educa ion
%
Uni e si y
10
55.6
Seconda y
8
44.4
To al
18
100
Fo he “le el o educa ion” a iable, analysis o Table III shows ha he majo i y o s udy subjec s we e uni e si y
g adua es, accoun ing o 55.6% o cases, while seconda y school g adua es ep esen ed only 44.4% o subjec s.
2.1.4. Ma i al s a us
Table 4 Dis ibu ion o esponden s by ma i al s a us.
Ma i al s a us
%
Ma ied
12
66.7
Single
6
33.3
To al
18
100
This s udy e ealed ha 12 subjec s (66.7%) we e ma ied, compa ed wi h 33.3% who we e single.
2.1.5. Socio-economic le el
Table 5 Dis ibu ion o esponden s acco ding o socio-economic le el.
Socio-economic le el
%
Medium
10
55.6
Low
7
38.8
High
1
5.6
To al
18
100
The da a in Table V show ha 55.6% o he subjec s in he s udy had an a e age socio-economic s anda d o li ing, while
hose wi h low socio-economic s a us accoun ed o 38.8% o cases. On he o he hand, only one subjec (5.6%) had a
high socio-economic s anda d o li ing.
2.2. Va iables s udied
2.2.1. Knowledge o sickle cell disease
Table 6 Dis ibu ion o subjec s acco ding o hei knowledge o sickle cell disease
Knowledge o sickle cell disease
%
Yes
13
72.2
No
5
27.8
To al
18
100
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The da a in Table VI show ha 72.2% o subjec s had some knowledge o sickle cell disease, compa ed wi h 27.8% o
subjec s wi h insu icien knowledge o sickle cell disease.
2.2.2. Causes o sickle cell disease
Table 7 Dis ibu ion o s udy subjec s by cause o sickle cell disease
Causes
%
Wi chc a o cu se
2
11.1
Cu se
2
11.1
Undecided
5
27.8
Union o couples wi h AS/AS genes
9
50
To al
18
100
The esul s o his able show ha he union o couples wi h AS/AS genes is he main cause o sickle cell disease
acco ding o 50% o he subjec s s udied, whe eas 27.8% o he subjec s knew no hing abou i .
2.2.3. Fac o s a o ing sickle cell c isis
Table 8 Dis ibu ion o esponden s by signi icance o ac o s a o ing sickle cell c isis (N=18)
Fac o s a o ing sickle cell c isis
%
In ec ions (mala ia, a h i is, e c.)
14
77.7
Clima e (season)
11
61.1
Dus
10
55.5
Dehyd a ion
8
44.4
Undecided
5
27.8
Family and cul u al ac o s
4
22.2
In ec ions (mala ia, a h i is, e c.), clima e change and dus a e ac o s a o ing sickle cell c ises acco ding o 77.7%,
61.1% and 55.5% o he espec i e subjec s.
2.2.4. Measu es o p e en a acks
Table 9 Dis ibu ion o subjec s acco ding o p e en i e measu es o sickle cell c ises N=18
Measu es o p e en a acks
%
Ea ly ea men o in ec ions (mala ia, a h i is, yphoid e e , e c.)
13
72.2
Folic acid chemop ophylaxis
12
66.6
Good p o ein die
9
50
A oid exposu e o isk ac o s
7
38.8
Undecided
5
27.8
Table IX shows ha ea ly ea men o in ec ions, olic acid chemop ophylaxis and a good p o ein die we e ci ed as
p e en i e measu es agains sickle cell c ises by 72.2%, 66.6% and 50% o subjec s espec i ely.
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2.2.5. P e en ion o ansmission o sickle cell disease
Table 10 Dis ibu ion o esponden s acco ding o measu es aken o p e en he ansmission o sickle cell disease (N
=18)
P e en ion o ansmission o sickle-cell disease %
%
P e en he ma iage o AS/AS couples h ough p ema i al examina ions
13
72.2
I AS/AS couples ma y inad e en ly, a oid ma e ni y
10
55.5
P ay o God wi h ai h o elimina e S genes in couples
6
33.3
Di o ce o AS/AS couples
5
27.8
Fo he a iable “p e en ion o ansmission o sickle cell disease”, 72.2% o subjec s sugges ed a oiding he ma iage
o AS/AS couples h ough p ema i al examina ions, and 10 esponden s, i.e. 55.5% o cases, had asked o a oid
ma e ni y in he e en o he inad e en ma iage o AS/AS couples. Howe e , 5 esponden s, i.e. 27.8%, had asked o
AS/AS couples o be di o ced.
3. Discussion and commen s
3.1. Gende
The s udy indica ed ha 66.7% o he subjec s we e emale, compa ed wi h 33.3% male.
Yenga, in his s udy conduc ed in 2024 on he phenomenon o p ema u e sexuali y and i s co olla ies among adolescen s
in he Tchai dis ic , epo ed ha young adolescen gi ls we e mo e likely o engage in p ema u e sexuali y, wi h 60%
compa ed wi h 40% o adolescen boys [15].
This may be explained by he ac ha women a e mo e pe manen a home han men, mo e ecep i e making hem
especially accessible o answe esea ch ques ions. They a e he ones who welcome home isi o s and espond o hei
conce ns.
3.2. Age
This esea ch has shown ha he s udy popula ion is made up mo e o subjec s in he 20-39 age b acke , i.e. 61.1%,
while hose aged 50 and o e ep esen only 22.2% o cases.
In he s udy by Moussa Tu é (2009) on he a i udes and p ac ices o couples wi h p ima y in e ili y, 74% we e aged
be ween 33 and 42 [16].
By compa ing hese wo esul s, we can p edic ha mo he hood is an adul ad en u e. In p inciple, you can only gi e
bi h when you' e eached adul hood. Fo his eason, ou s udy popula ion was made up o he pa en s o child en wi h
sickle cell disease.
3.3. Educa ional le el
Fo he a iable “le el o educa ion”, he s udy e ealed ha he majo i y o subjec s in he s udy we e uni e si y
g adua es wi h 55.6% o cases, while hose wi h seconda y educa ion ep esen ed only 44.4% o subjec s.
In he s udy by Moussa Tu é (2019) on he a i ude and p ac ice o a couple wi h p ima y in e ili y, 53.4% we e aged
be ween 33 and 42 [16].
Fo Yenga, 28 young people, o 43.1% we e in seconda y school ollowed by 35.4% in elemen a y school [15].
While Malumbu L, in he s udy o medical ca e o women in p ison, he case o Kisangani cen al p ison, epo ed ha
he majo i y o inma es had lowe o p ima y educa ion, wi h 33.3% in seconda y school and 30.3% illi e a e. Those
wi h seconda y educa ion accoun ed o 27.3% o cases [17].
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3.4. Ma i al s a us
This s udy e ealed ha 12 subjec s, o 66.7%, we e ma ied, compa ed wi h 33.3% who we e single.
These esul s clea ly show ha mo he hood is an ad en u e o ma ied people. In p inciple, you can only gi e bi h
when you li e as a couple. Fo his eason, mo e han hal o ou s udy popula ion we e ma ied.
3.5. Socio-economic le el
The esea ch da a showed ha 55.6% o he subjec s in he s udy had an a e age socio-economic s anda d o li ing,
while hose wi h low socio-economic s a us accoun ed o 38.8% o cases. On he o he hand, only one subjec (5.6%)
had a high socio-economic s anda d o li ing.
In i s Guide de p ise en cha ge la d épanocy ose en A ique (Guide o he managemen o sickle-cell anemia in A ica),
he Socié é A icaine de Pédia ie (A ican Pedia ic Socie y) has shown ha sickle-cell anemia mainly a ec s poo
popula ions in coun ies wi h limi ed heal hca e budge s. Many o he ad ances made in i s managemen a e no ye
a ailable in he a eas whe e i is mos common, bu a e a ailable o pa ien s in ich coun ies o o ich pa ien s in poo
coun ies. These ad ances do exis . I mus be made accessible o as many people as possible [14].
In he Democ a ic Republic o Congo, despi e he absence o a job ma ke , he Congolese ha e become awa e o he need
o ge hei child en in o school, based on Unice 's awa eness- aising slogan: “All child en, gi ls and boys a school”. As
a esul , we ha e a conside able numbe o uni e si y and high school s uden s, mos o whom a e limi ed o he
seconda y le el o a a ie y o easons: ea ly p egnancy, lack o esou ces, olun a y d op-ou , e c.
3.6. Sickle cell disease knowledge
The da a om his esea ch indica e ha 72.2% had knowledge o sickle cell disease, compa ed wi h 27.8% o subjec s
wi h insu icien knowledge o sickle cell disease.
In sickle cell disease, he ed blood cells become igid and s icky, aking on he shape o a C shaped a ming ool called a
“sickle”. The sickle-shaped ed blood cells die o ea ly, causing hei lack in he blood o occu egula ly [18].
This si ua ion can be explained by he ac ha , in he Democ a ic Republic o Congo, sickle-cell anemia is a p esen ,
incu able disease, a ac ing so much a en ion ha i is known by e e yone o i s pain ul aso-occlusi e c ises, and
impo e ishing pa ien s' amilies.
3.7. Cause o sickle cell disease
The esul s o his s udy epo ha he union o couples wi h AS/AS genes is he main cause o sickle cell disease
acco ding o 50% o he subjec s in he s udy, whe eas 27.8% o he subjec s knew no hing abou i .
Sickle cell disease is due o a single poin mu a ion in he DNA o he gene coding o be a-globin, loca ed on ch omosome
11. I con e s a modi ied s uc u e ha enables hemoglobin o o m chains (polyme s) when he concen a ion o oxygen
in he blood is low (hypoxia). These a e he genes esponsible o hemoglobin p oduc ion. The usual gene is called A,
and he sickle-cell gene is called S. A pe son is AA i bo h pa en s ha e passed on he A gene [19].
In ou iew, sickle cell disease is a he edi a y blood diso de caused by he p oduc ion o an abno mal hemoglobin (Hb),
called HbS, which de o ms he ed blood cells, making hem agile and igid, leading o occlusi e seizu es, anemia, and
so on.
3.8. Fac o s a o ing sickle cell c ises
In ec ions (mala ia, a h i is, e c.), clima e change and dus a e ac o s a o ing sickle cell c ises acco ding o 77.7%,
61.1% and 55.5% o he espec i e subjec s.
Acco ding o HAS, i is ecommended o explain o pa en s he ac o s a o ing pain ul aso-occlusi e a acks:
• Hypoxia: excessi e and unusual exe ion, al i ude ( om a ound 1,500 m), igh clo hing, e c. ;
• Cooling: cold-wa e ba hs, e c. ;
• Fe e ;
• Dehyd a ion: omi ing, dia hoea, e c. ;
Wo ld Jou nal o Ad anced Resea ch and Re iews, 2025, 26(02), 2708-2719
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• S ess;
• In ake o s imulan s, alcohol, obacco o illici d ugs (mo e so in han in child en).
I is also ad isable o emind hem o he need o abundan hyd a ion (“child en should keep hei u ine as clea as
possible”). Abundan hyd a ion is essen ial. I mus be un es ic ed and con inuously encou aged. I is ecommended
ha pa en s, and hen he child, be in o med ha he child should d ink un il he u ine is “as clea as possible” [20].
The Socié é A icaine de Pédia ie has iden i ied se e al ac o s likely o a ec he polyme iza ion p ocess: he i s is
any change, howe e sligh , in he in a-e y h ocy e concen a ion o hemoglobin S molecules; he second is he
in e up ion o polyme g ow h by hemoglobin F (HbF) molecules in e cala ed in he ibe . All he ac o s ha ha e an
influence on hese pa ame e s a e likely o be in ol ed in he pa hophysiology o he disease. Such is he case wi h α
halassemia, equen in he same popula ions as hose a isk o sickle cell disease. [14].
Mo eo e , he pa icula suscep ibili y o in ec ion is pa ly he esul o p og essi e au o-splenec omy ( unc ional
exclusion o he spleen) and a educ ion in he phagocy ic capaci y o polynuclea s. De o med ed blood cells a e
dehyd a ed, hype -concen a ed and hype -agg egable. They hus inc ease blood hype iscosi y and s asis, which in
u n agg a a e hypoxia and acidosis. The esul is a icious ci cle. These a ious aso-occlusi e, hemoly ic and in ec ious
phenomena a e o en in e linked. They a e esponsible o he clinical mani es a ions and acu e and ch onic
complica ions o sickle cell disease [14].
In pa ien s wi h majo sickle cell synd omes, mala ia is a ac o in he mo bidi y and le hali y o sickle cell disease o
a leas wo easons: he e e ha accompanies i is, in i sel , a igge o aso-occlusi e c ises. The onse o p egnancy
in a pa ien wi h sickle cell disease, wha e e he geno ype, is a e y high- isk si ua ion, ma ked by high ma e nal and
pe ina al mo bidi y and mo ali y. This associa ion equi es managemen by a mul idisciplina y eam in a sui able
cen e [20].
Acco ding o Khella (2015), cold, al i ude, ai a el, s ess, school o uni e si y exams, e c., can a ec he heme chain
in s ess ul si ua ions (in ec ions, hypoxemia, acidosis), causing ed blood cells o alci o m, leading o occlusion o blood
capilla ies and pa icula ly pain ul bone in a c ions, which explain he aso-occlusi e c ises ha b ing pa ien s o he
ER [2].
Acco ding o Ndikumana (2020), in obs e ics, p egnancy in luences sickle cell c isis by agg a a ing ch onic anemia, he
high equency o aso-occlusi e c ises and he high a e o u ina y ac in ec ions, pneumococcal in ec ions and
su gical si e in ec ions in he case o Caesa ean sec ions. On he o he hand, sickle cell disease is also associa ed wi h
delayed mena che up o he age o 18, p eeclampsia and e oplacen al hema oma [21].
In he p ac ice o anaes hesia in sickle-cell pa ien s, ce ain anaes he ic p oduc s cause espi a o y dis ess which can
educe oxygen le els (hypoxia), esul ing in a consequen sickle-cell c isis [22].
In ou opinion, he e a e se e al ac o s ha a o he exp ession o he disease, no ably: dehyd a ion, which causes an
inc ease in he concen a ion o HbS in he ed blood cells and aso-cons uc ion o he essels; hypoxemia; acidosis;
climen (cold), which a o s aso-cons uc ion o he essels; hype he mia, which a o s deoxygena ion o he ed
blood cells; and in ec ions (in his case mala ia). Pa en s mus he e o e educa e hei child en o a oid hem.
3.9. Seizu e p e en ion measu es
The esul s o his s udy show ha ea ly ea men o in ec ions, chemop ophylaxis wi h olic acid and a good p o ein
die we e ci ed as measu es o p e en sickle cell c ises by 72.2%, 66.6% and 50% o subjec s espec i ely.
Acco ding o he Minis y o Public Heal h and he Figh agains AIDS (2020), p e en ion o sickle cell c ises consis s in
aking abundan be e ages and a oiding : - apid a ia ions in empe a u e, - aking iced d inks, - iolen o sus ained
exe ion, - a elling in non-p essu ized ai c a , - s aying in con ined spaces I is impo an o ge he child in o he
habi o equen ly d inking wa e and o he swee ened be e ages. In addi ion: - Follow he EPI- ecommended
accina ion schedule, and accina e agains he ge ms mos equen ly esponsible o sickle-cell in ec ions:
An ipneumococcal, An imeningo A and Co An i yphoid ( yphimVi), 3 doses o hepa i is B accine a e ecommended, -
Penicillin V 50mg/kg/d un il 5-7 yea s o age, good pe sonal hygiene and sys ema ic dewo ming wi h albendazole o
mebendazole e e y 3 mon hs [22].
