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 Liscense 4.0.
P oblems o pa en al ca e o sickle cell child en a he ''G acia Fonda ion'' ea men
cen e
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), 2720-2732
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 .2025.26.2.1382
Abs ac
In oduc ion: Sickle cell disease is a complex disease, bo h his o ically and medically, a he collec i e and indi idual
le els. I is he wo ld's mos common gene ic disease, a ec ing a ound 50 million people, and i s ea men is
mul idisciplina y, comp ising 2 componen s: analgesic ea men and ea men o ac o s p omo ing sickle cell disease.
The sole objec i e o his s udy is o iden i y he a ious p oblems ela ed o he ca e o sickle cell child en encoun e ed
by pa en s a he ''G acia onda ion'' Cen e .
Me hodology: This is a desc ip i e, c oss-sec ional and quan i a i e s udy whose popula ion is made up o 20 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.
We used an in e iew echnique o collec da a, based on a p e-es ablished ques ionnai e.
Resul s: A e abula ion and analysis o he da a, he ollowing esul was obse ed: insu icien inancial means and
abandonmen o child en by hei a he s a e he main di icul ies expe ienced by pa en s in ca ing o hei sickle-cell-
a ec ed child en, acco ding o 100% and 80% o he espec i e subjec s.
Keywo ds: P oblem; Ca e; Child; Sickle Cell; Pa en .
1. In oduc ion
Acco ding o he In e na ional Co enan on Economic, Social and Cul u al Righ s, S a es Pa ies o he Co enan mus
ake “ he necessa y s eps o ensu e he educ ion o he s illbi h- a e and o in an mo ali y, and he heal hy
de elopmen o he child” (A icle 12). Sickle-cell anemia is a gene ic disease ha can be desc ibed as global, and which
se iously a ec s child en's heal h, e en leading o hei dea h. This anscon inen al and ans-e hnic disease has been
o go en by he in e na ional communi y, ye i was ecognized as a public heal h p io i y by he WHO in 2005 and by
he UN in 2008 (Resolu ion A/63/237) [27].
Sickle cell anemia is a complex disease, bo h his o ically and medically, a he collec i e and indi idual le els. I is he
mos common gene ic disease wo ldwide, a ec ing a ound 50 million people [1].
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Hemoglobin abno mali ies caused by poin mu a ions in a heme chain lead o ed blood cell alci o ma ion in s ess ul
si ua ions (in ec ions, hypoxemia, acidosis), esul ing in 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 eme gency oom [2].
The s uc u al anomaly in hemoglobin is ep esen ed by a DNA poin mu a ion cha ac e ized by he subs i u ion o an
amino acid, glu amic acid, by aline, in posi ion 6 o he ϐ chain. The anomaly is loca ed on ch omosome 11. Gene ic
ansmission is au osomal ecessi e. When only one ch omosome ca ies he HbS gene ( ansmi ed by he mo he o
a he ), he subjec is said o be he e ozygous; a heal hy ca ie . Howe e , when bo h ch omosomes a e ca ie s o he
gene ( ansmi ed by he mo he and a he ), he subjec is said o be homozygous and he ca ie is ill [4].
Depending on he geno ype o he pa en s, he ollowing combina ions a e possible: 1. AA child en bo n o pa en s who
a e bo h AA, who a e no ill and canno ansmi sickle cell disease o hei o sp ing; 2. AA and AS child en, i one pa en
is AA and he o he AS. AS child en a e no sick, bu can ansmi sickle cell disease i hey ma y ano he AS o SS
pe son; 3. AA, AS and/o SS child en, i bo h pa en s a e AS and AS; 4. SS child en i bo h pa en s a e SS and SS [1].
Un il he 1990s, sickle-cell anemia was conside ed a a al disease, wi h s udies epo ing a li e expec ancy o no mo e
han 48 yea s e en in de eloped coun ies. Howe e , majo ad ances in ou unde s anding o he s ill highly complex
pa hophysiology o he disease, and he g owing codi ica ion o i s managemen , ha e made i possible o educe
mo ali y conside ably by adhe ing o ce ain simple ules, and o ans o m sickle cell disease om a “ a al disease”
in o a “ch onic disease” [3].
Sickle cell anemia is a gene ic disease, inhe i ed om bo h pa en s. I is he mos widesp ead gene ic disease in he
wo ld, a ec ing o e 50 million people, wi h a p edominance in A ica, India, he Medi e anean Basin and he Middle
Eas . F om hese egions, i has sp ead o o he coun ies as a esul o popula ion mo emen s mig a ion [5].
In F ance, sickle cell anemia is he mos common gene ic disease, a ec ing 20,000 pa ien s by 2020, making i he
leading gene ic disease ahead o cys ic ib osis. Howe e , o e 50% o sickle cell pa ien s li e in he Île-de-F ance egion
[6].
Sub-Saha an A ica is pa icula ly ha d hi : one in e e y 65 newbo ns he e has sickle cell disease. sickle cell disease. In
Mali, he p e alence o sickle cell disease is es ima ed a 12%, including 1-3% o he homozygous o m, making i a
public heal h p oblem due o i s equency and mul iple complica ions [7].
In Bu undi, he p e alence o sickle cell disease is unknown. In 2016, he WHO es ima ed ha i was a leas 2% and
ha 10% o 40% o he Bu undian popula ion we e ca ie s o he sickle cell gene [1].
Sickle cell disease p esen s as hemoly ic anemia in e spe sed wi h aso-occlusi e c ises, o en complica ed by se e e
bac e ial in ec ions. Pallo and subic e us, indica i e o ch onic hemolysis, a e almos cons an . The clinical signs o
sickle cell disease a e synonymous wi h complica ions. Sickle cell disease is mani es ed bo h by noisy acu e
mani es a ions and silen ch onic complica ions: splenic seques a ion c ises, hype haemoly ic c ises, aplas ic c ises,
os eo-a icula aso-occlusi e c ises o bone in a c s, ex emi y synd ome (hand- oo synd ome), ce eb al c isis due o
ce eb o ascula acciden (CVA), abdominal c isis o di use abdominal pain, p iapism and cli o ism, hype splenism o
splenomegaly, g ow h e a da ion and delayed pube y, se e e in ec ious episodes, e c. [26].
In he Democ a ic Republic o Congo, sickle-cell anemia is a se ious ch onic disease esponsible o ea ly mo ali y and
educing pe sonal, social and amily quali y o li e, and amily li e. Pa ien s may be expe iencing g ea social di icul ies,
amily con lic s, p o essional p oblems (p eca ious wo k and some imes unsui abili y o he job and epea ed
absences) o school p oblems ( epea ed absences, misunde s anding o classma es and he school sys em), and a e
he e o e likely o ha e a g ea e numbe o a acks [8].
The main ac o s igge ing aso-occlusi e a acks a e cold, al i ude (ai a el), s ess (school o uni e si y exams,
social li e, e c.), in ec ions and dehyd a ion.
Sickle cell aso-occlusi e c isis is no usually accompanied by a majo d op in hemoglobin le els. The occu ence o
acu e anemia in he cou se o sickle cell disease should aise he possibili y o cen al complica ions such as bone
ma ow nec osis, pa o i us B19 in ec ion and acu e ola e de iciency, o pe iphe al complica ions such as acu e splenic
seques a ion and immunological ans usion acciden s. This c isis is he mos equen acu e complica ion o sickle cell
disease, wi h an es ima ed p e alence o 95%. The pain ul aso-occlusi e c isis may some imes he ald ano he , mo e
se ious complica ion, o conceal o he di e en ial diagnoses [9].
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Spon aneous mo ali y om sickle cell s oke can be as high as 20%, e lec ing he ex eme se e i y o his complica ion.
The ela i e isk o de eloping a s oke in sickle cell disease is much highe in child en han in adul s, wi h a p edilec ion
o s okes be ween he ages o 2 and 10, and a peak a e he age o 30 [10].
The apeu ic managemen is mul idisciplina y, wi h in e nis s, eme gency physicians and esusci a o s all in ol ed in
he managemen o sickle cell complica ions. I includes symp oma ic ea men o aso-occlusi e c ises, ans usion, o
e en exchange ans usion, and o ganic suppo depending on he se e i y o he c isis. Pa ien s wi h se e e
complica ions a e admi ed o in ensi e ca e [7].
The ea men o a simple aso-occlusi e c isis comp ises 2 componen s: analgesic ea men and ea men o ac o s
a o ing alci o ma ion. The aso-occlusi e c isis is he cause o e y in ense bone pain a a le el supe io o ha o a
bone ac u e. The he apeu ic esponse mus he e o e be a he same le el, and in all cases equi es majo analgesics
such as mo phine [11].
Acco ding o he A ican Socie y o Pedia ics (2018), in sickle cell disease, he e a e only pallia i e ea men s. The bulk
o ea men consis s o managing he symp oms esul ing om c ises. Sickle cell pa ien s need es , good oxygen
he apy in he case o ho acic synd omes, good hyd a ion in ho wea he , and analgesics in he case o pain. Eme gency
ans usion emains he only ea men o p o ound anemia. Folic acid is gi en o li e o acili a e cell enewal [12].
A ound 40% o adul sickle-cell pa ien s p esen wi h in e mi en o acu e p iapism a some s age in he cou se o he
disease. A acks a e mos o en noc u nal and a ou ed by he p esence o sleep apnoea synd ome. The majo unc ional
isk is de ini i e ib osis o he co pus ca e nosum. Sys ema ic anamnes ic sc eening and managemen o in e mi en
p iapism a e essen ial o p e en he onse o acu e p iapism, o en wi h pe manen sequelae [11].
Any sickle cell complica ion should a p io i be ea ed in hospi al, due o he unp edic able isk o agg a a ion o
associa ion wi h o he condi ions ha may ha e been masked a he ou se : any bone pain c isis ha is abno mal in
e ms o du a ion, in ensi y, esis ance o usual ini ial ea men s, agili y o he pa ien 's unde lying condi ion, o he
impossibili y o p o iding adequa e analgesic ea men and hyd a ion on an ou pa ien basis, should be e e ed o
hospi al. Fu he mo e, all ex a-osseous complica ions a e sys ema ic indica ions o hospi aliza ion, wi h
hype he mia leading he way, as well as ho acoabdominal pain and aso-occlusi e synd omes, and se e e anemia.
The e icacy o cu a i e managemen o acu e complica ions o sickle cell disease depends on he quali y o ini ial
managemen o i al dis ess and p ope diagnos ic e alua ion o pa ien s [13].
The c i e ia o e u ning home du ing a aso-occlusi e c isis a e absence o e e , absence o ches pain, Respi a o y
Ra e below 20 mo emen s pe minu e, no mo phine injec ions o mo e han 8 hou s [14].
Sickle-cell anemia can lead o he dea h o he child, especially when he disease is no de ec ed and ea ed om bi h,
as is he case in many A ican and de eloping coun ies such as he Democ a ic Republic o Congo.
In mos coun ies whe e sickle cell disease is a majo public heal h p oblem, he e a e no na ional p og ams o comba
i . The basic s uc u es needed o ca e o pa ien s a e gene ally lacking. Sys ema ic sc eening o sickle cell disease is a
simple blood es , ye his is no common p ac ice. In de eloping coun ies, he disease is usually diagnosed only when
a se ious complica ion occu s. As a esul , o e 50% o child en wi h he mos se e e o m o he disease die be o e he
age o i e, mos o en om in ec ion o se e e anemia. Ea ly de ec ion o he disease is essen ial, so ha couples can
bene i om in o ma ion, educa ion and app op ia e ca e [15].
E ec i e ways o imp o ing he heal h o sickle-cell child en include: 1. s anda d ea men s: an ibio ics, accines,
ola es; 2. ea men o aso-occlusi e c ises: analgesics and oxygen he apy; 3. p e en ion o ac o s igge ing c ises
(cold, al i ude, in ec ions, dehyd a ion); 4. Take ola e- ich medica ion o anemia; 5. Vaccina ion o p e en
pneumococcal and meningococcal in ec ions; 6. Blood ans usion in cases o se e e anemia o in ec ion; 7. Blood
ans usion o educe he p opo ion o hemoglobin S [16].
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 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 [17].
Sickle-cell anemia is linked o he ep esen a ion o dea h in he A ican con ex . Families li e in unce ain y in he ace
o his agedy. Faced wi h his si ua ion, sickle cell disease emains hidden om he amily ci cle; only he pa en s
Wo ld Jou nal o Ad anced Resea ch and Re iews, 2025, 26(02), 2720-2732
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manage he si ua ion, e en du ing he child's aso-occlusi e c isis. Gi en he sensi i i y o he in o ma ion we we e
seeking, a quan i a i e app oach was used o highligh he p oblems associa ed wi h he managemen o sickle cell
disease in ou s udy en i onmen . This app oach is acili a ed by he communi y diagnosis p ocess, which enables a
communi y o iden i y i s own p oblems and needs, and he esou ces a ailable o add ess hem.
In iew o he abo e, we pose he ollowing esea ch ques ion:
Wha a e he a ious p oblems ela ed o he ca e o sickle-cell child en encoun e ed by pa en s a he ''G acia
onda ion'' Cen e ?
1.1. Objec i e
Ou sole objec i e is o iden i y he a ious p oblems encoun e ed by pa en s o sickle-cell-a ec ed child en a he
''G acia onda ion'' cen e .
1.2. Aim o he s udy
Wi h a iew o imp o ing he heal h o sickle-cell-a ec ed child en in he Democ a ic Republic o he Congo, his s udy
has wo in e es s:
Scien i ic and educa ional: his s udy is in ended o make a scien i ic con ibu ion in e ms o new knowledge, as a
lib a y ha can help esea che s in hei u u e s udies.
On a p ac ical le el: his esea ch is in ended o be a ame o e e ence, aising awa eness bo h o adminis a i e and
heal h au ho i ies o hink abou imp o ing he ca e o sickle-cell child en, and o pa en s o b ing hei child en o
specialized cen e s o p ope ca e.
The esul s o his s udy will enable us o o mula e a numbe o conc e e ac ions (possible solu ions) ha could help
decision-make s o d aw up and implemen na ional p og ams o comba sickle cell disease h oughou he Republic.
2. Me hodology
2.1. Type o s udy
This is a desc ip i e, c oss-sec ional and quan i a i e s udy on he p oblem o ca ing o sickle cell child en encoun e ed
by pa en s a he “G acia onda ion” ea men cen e in he own o Kisangani, in he Democ a ic Republic o he Congo,
om 15/10 o 15/11 2024.
2.2. S udy popula ion
The popula ion o his s udy is composed o pa en s o child en wi h sickle cell disease ollowed a he ''G acia
onda ion'' Sickle Cell T ea men Cen e in he ci y o Kisangani du ing he pe iod om 15 /10 o 15 /11 2024.
Gi en he impo ance o his s udy, ou sample included 20 subjec s ep esen ing he pa en s o child en who we e
ollowed up du ing his pe iod.
2.3. 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 pa en s o child en wi h sickle cell disease, we op ed o an in e oga o y me hod based on a
ques ionnai e ha 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 o ob ain in o ma ion on he di icul ies associa ed wi h ca ing o child en wi h sickle cell disease.
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.
2.4. 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.
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3. Resul s
3.1. Socio-demog aphic da a
3.1.1. Gende
Table 1 Gende dis ibu ion o s udy subjec s
Gende
%
Female
13
65
Male
7
35
To al
20
100
Analysis o Table I shows ha 65% o s udy subjec s we e emale, compa ed wi h 35% male.
3.1.2. Age
Table 2 Age dis ibu ion o s udy subjec s
Age ange in yea
%
20 - 29
5
25
30 - 39
8
40
40 - 49
4
20
50 and o e
3
15
To al
20
100
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 15% o cases.
3.1.3. 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
8
40
Seconda y
12
60
To al
20
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 a seconda y
school le el (60%), while hose a uni e si y le el accoun ed o only 40%.
3.1.4. 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
14
70
Single
6
30
To al
20
100
This s udy e ealed ha 14 subjec s, o 70%, we e ma ied, compa ed wi h 30% who we e single.
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3.1.5. Socio-economic le el
Table 5 Dis ibu ion o esponden s acco ding o socio-economic le el.
Socio-economic le el
%
Low
11
55
Medium
7
35
High
2
10
To al
20
100
The da a in Table V show ha 55% o he subjec s in he s udy had a low socio-economic s anda d o li ing, while he
socio-economically a e age ep esen ed 35% o cases. On he o he hand, wo subjec s (10%) we e o high socio-
economic s a us.
3.2. Va iables s udied
3.2.1. Ci cums ances o disco e y o sickle cell disease in child en
Table 6 Dis ibu ion o subjec s acco ding o ci cums ances o disco e y o sickle cell disease
Ci cums ance o disco e y o sickle cell disease
%
A e child's second blood ans usion
8
40
Child's sickly s a e
6
30
Following p onounced pallo
4
20
Following g ow h e a da ion
2
10
To al
20
100
In Kisangani, episodes o blood ans usion and a sickly child a e he ci cums ances in which sickle cell disease is
disco e ed in 40% and 30% o cases espec i ely.
3.2.2. Numbe o aso-occlusi e c ises pe yea
Table 7 Dis ibu ion o subjec s by numbe o aso-occlusi e c ises pe yea
Numbe o a acks pe yea
%
1 o 4 imes
2
10
5 o 8 imes
5
25
Se e al imes
13
65
To al
20
100
The da a in Table VI show ha 65% o subjec s epo se e al aso-occlusi e a acks pe yea , while hose who ha e 1
o 4 a acks ep esen 10% o cases.
3.2.3. Cause o aso-occlusi e a acks
Table 8 Dis ibu ion o subjec s by numbe o aso-occlusi e a acks pe yea n=20
Cause o a acks
%
Mala ia, dia hea and yphoid e e
10
50
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Cold and hea
7
35
P egnancy i adul
4
20
Undecided
2
10
The da a in Table VIII show ha in ec ions (mala ia, dia hoea and yphoid e e ) and clima e (cold and hea ) a e he
main causes o aso-occlusion in 50% and 35% o subjec s espec i ely.
3.2.4. Managemen o child en wi h sickle cell disease
Table 9 Dis ibu ion o subjec s by elemen s o ca e o child en wi h sickle cell disease n=20
Managemen o sickle-cell anemia
%
Pain con ol [ ea men wi h analgesics (mo phine)]
20
100
T ans usion in case o anemia
17
85
Ensu ing good nu i ion
15
75
In ec ion con ol ( ea men wi h an ibio ics)
14
70
Applica ion o li es yle hygiene measu es
9
45
Hyd a ion and oxygen he apy
7
35
T ea men wi h hyd oxyu ea
5
25
T ea men o p iapism
5
25
Pain con ol, ans usion in case o anemia, good nu i ion and in ec ion con ol a e he main componen s o sickle cell
case managemen , acco ding o 100%, 85%, 75% and 70% o cases.
3.2.5. Di icul ies expe ienced by pa en s in ca ing o sickle-cell-a ec ed child en
Table 10 Dis ibu ion o subjec s acco ding o he di e en di icul ies expe ienced by pa en s in ca ing o sickle-cell
child en n=20
Di icul ies expe ienced by pa en s
%
Pa en s' lack o inancial means
20
100
Abandonmen o child en by a he s
16
80
Socio-cul u al p oblems
4
20
Insu icien inancial means and abandonmen o child en by hei a he s a e he main di icul ies expe ienced by
pa en s in ca ing o hei sickle-cell-a ec ed child en, acco ding o 100% and 80% o he espec i e subjec s.
3.2.6. P oposed solu ions o imp o e sickle cell disease managemen
Table 11 Dis ibu ion o subjec s acco ding o p oposed solu ions o imp o e he managemen o sickle cell disease
n=20
P oposed solu ions o imp o ing sickle cell disease
%
Go e nmen suppo o child en wi h sickle cell disease
20
100
Sepa a ion o A/S and A/S o A/S and S/S couples
13
65
S e iliza ion o couples o a oid p oc ea ion
6
30
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Go e nmen ea men o sickle-cell child en by se ing up na ional sickle-cell ea men cen e s h oughou he
Republic (100% o cases) and sepa a ion o A/S and A/S o A/S and S/S couples (65% o cases) a e he solu ions o
imp o e ca e. Howe e , 30% o subjec s epo ed s e iliza ion o couples o a oid p oc ea ion o sickle cell child en.
4. Discussion and commen s
4.1. Gende
Analysis o he s udy da a shows ha 65% o he subjec s we e emale, compa ed wi h 35% male.
This esul can be explained by he ac ha i is women, mo he s in mos cases, who ca e o and accompany child en
o hospi al o ea men . They a e he p ima y amily nu ses, while he men a e he ones who p o ide o hei amilies.
4.2. Age ange
The s udy indica es 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 15% o cases.
Gi en ha he s udy popula ion is made up o pa en s o sickle-cell-a ec ed child en, he 20 o 39 age b acke is
he e o e he age o majo i y. Bo h men and women can ma y, hus ounding hei homes. These a e he ages wi h
which young people wan o iden i y and con i m hemsel es in li e.
4.3. Le el o educa ion
Fo he “le el o educa ion” a iable, he majo i y o subjec s in he s udy we e a seconda y school le el (60%), while
hose a uni e si y le el accoun ed o only 40%.
Con a y o he s udy by Malumbu L, in he s udy o medical ca e o women in p ison, he case o he Kisangani cen al
p ison, epo ed ha he majo i y o inma es had lowe o p ima y educa ion, i.e. 33.3% had seconda y educa ion and
30.3% we e illi e a e. Those wi h seconda y educa ion accoun ed o 27.3% o cases [18].
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 educa e hei child en, 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 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.
4.4. Ma i al s a us
This s udy p esen s he esul s acco ding o which 14 s udy subjec s, i.e. 70% we e ma ied e sus 30% single.
These esul s clea ly show ha mo he hood is an ad en u e o ma ied people, al hough some a he s abandon hei
sickle-cell child en. 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.
4.5. Socio-economic le el
The s udy da a indica e ha 55% o he subjec s in he s udy had a low socio-economic s anda d o li ing, while hose
o socio-economic means ep esen ed 35% o cases. On he o he hand, wo subjec s (10%) we e o high socio-economic
s a us.
The A ican Socie y o Pedia ics, in i s Guide o he managemen o sickle-cell disease in A ica, has shown ha sickle-
cell disease mainly a ec s impo e ished popula ions in coun ies wi h limi ed heal h budge s [19].
In ou opinion, many o he ad ances made in he managemen o sickle-cell disease a e no ye a ailable in he a eas
whe e i is mos p e alen , 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 . We need o make i accessible o as many people as possible.
4.6. Ci cums ances o disco e y o sickle cell disease in child en
In Kisangani, episodes o blood ans usion in child en and sick child en a e he ci cums ances in which sickle cell
disease is disco e ed in 40% and 30% o cases espec i ely.
Wo ld Jou nal o Ad anced Resea ch and Re iews, 2025, 26(02), 2720-2732
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In he Democ a ic Republic o Congo, people ha e no cul u e o medical analysis. They wai o he onse and se e i y o
he disease be o e going o hospi al o ea men . O e 60% o he popula ion sel -medica e. As a esul , ch onic
illnesses such as sickle-cell anemia, cance , HIV/AIDS, e c., in bo h child en and adul s, a e disco e ed la e.
4.7. Numbe o aso-occlusi e a acks pe yea
The da a om his esea ch indica ed ha 65% o subjec s epo se e al aso-occlusi e a acks pe yea , while hose
who ha e 1 o 4 a acks ep esen 10% o cases.
A s udy o sickle cell disease in child en in Beni and Bu embo, Democ a ic Republic o Congo, epo ed ha 197 (1.5%)
o he pa ien s s udied we e sickle cell pa ien s. The e we e 163 cases o se e e aso-occlusi e c ises (82.7% o sickle
cell pa ien s), including 26 no included in his s udy. A o al o 137 cases we e included. The mean numbe o pa ien s
hospi alized pe yea was 15.7 ± 16.5, wi h ex emes o 22 and 30 [19].
In ou opinion, he onse o aso-occlusi e c ises in sickle-cell pa ien s depends on se e al ac o s: hypoxia, cold, e e ,
dehyd a ion, s ess, in ake o s imulan s, in ec ions, e c. Failu e o ake hese ac o s in o accoun can lead o aso-
occlusi e c ises as many imes a yea .
4.8. Causes o aso-occlusi e a acks
This s udy shows ha in ec ions (mala ia, dia hea and yphoid e e ) and clima e (cold and hea ) a e he main causes
o aso-occlusi e a acks o 50% and 35% 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. ;
• e e ;
• dehyd a ion: omi ing, dia hoea, e c. ;
• s ess;
• in ake o s imulan s, alcohol, obacco o illici d ugs (mo e so in han in child en) [25].
The aso-occlusi e c isis is he mos equen ; i is p o oked by he obs uc ion o mic oci cula ions by sickle cell ed
blood cells, esponsible o an acu e hype algesic ischemic lesion o he i iga ed o gan (mono- o mul i ocal
os eoa icula c ises, abdominal c ises, ho acic synd ome, e c.) and he esul ing pain.
4.9. Elemen s o ca e o child en wi h sickle cell disease
Pain con ol, ans usion in case o anemia, good nu i ion and in ec ion con ol a e he main componen s o sickle cell
case managemen , acco ding o 100%, 85%, 75% and 70% o cases.
Acco ding o Keza G.K, Diallo D e al (2023), medical managemen o sickle cell disease consis s o moni o ing anemia,
p e en ing pain ul a acks and in ec ions, and de ec ing complica ions ea ly o ea hem as quickly as possible. Pa ien s
a e always p o ided wi h a p esc ip ion o pain medica ion [20].
The apeu ic managemen o sickle cell disease is mul idisciplina y, including in e nis s, eme gency physicians and
esusci a o s. I includes symp oma ic ea men o aso-occlusi e c ises, ans usion, o e en exchange ans usion,
and o ganic supplemen s depending on he se e i y o he c isis. Pa ien s wi h se e e complica ions a e admi ed o
in ensi e ca e [21].
Acco ding o B illan Damus (2022), he ea men o a simple aso-occlusi e c isis comp ises 2 componen s: analgesic
ea men and ea men o ac o s a o ing alci o ma ion. The aso-occlusi e c isis is he cause o e y in ense bone
pain a a le el supe io o ha o a bone ac u e. The he apeu ic esponse mus he e o e be a he same le el, and in
all cases equi es majo analgesics such as mo phine [22].
Acco ding o he A ican Socie y o Pedia ics (2018), he e a e only pallia i e ea men s in sickle cell disease. The
essence o ea men consis s in managing he symp oms esul ing om c ises. The sickle cell pa ien needs es , good
oxygen he apy in he case o ho acic synd omes, good hyd a ion in ho wea he , and analgesics in he case o pain.
