Implementing comprehensive health care management for sickle cell disease in an African setting

Niger J Paed 2015; 42 (4): 298 – 302

PERSPECTIVE

Odunvbun ME

Implementing comprehensive health

Okolo AA

care management for sickle cell

disease in an African setting

DOI:http://dx.doi.org/10.4314/njp.v42i4.3

Accepted: 28th May 2015

Abstract : Sickle cell disease is

pital. The mortality rate among

the commonest single gene dis-

children with sickle cell disease

Odunvbun ME

(

)

ease in Africa. Morbidity and

has reduced to 1.3%, requirement

Okolo AA

mortality from this disease has

for recurrent blood transfusion has

Department of Child Health

University of Benin Teaching Hospital,

remained unacceptably high in

reduced to about 2%, and their

Benin-City, Nigeria

Africa whereas there has been a

nutritional status has improved:

Email: magodunvbun@yahoo.com

marked reduction in the burden of

75.9% have normal nutritional

maggieodunvbun@gmail.com

this disease in the developed

status while 7% are actually over-

countries. This reduction was not

weight. The frequency of bone

achieved through the use of so-

pain crisis has reduced to about

phisticated care such as bone mar-

one in every two years and some

row transplant, but through the

of the patients have been crisis-

adoption of a Comprehensive

free for as long as five years. Hy-

Health Care Management proto-

droxyurea is not routinely used for

col for sickle cell disease. This

our patients so this cannot explain

protocol of care emphasizes pre-

the marked improvement recorded.

vention of crises through effective

In conclusion,

comprehensive

management of the disease. In

health care, adapted to our setting

Africa, where sickle cell disease is

is a very cheap and effective way

prevalent, this strategy of care is

of managing sickle cell disease. It

yet to be globally adopted.

can be utilized in all health facili-

2003, this protocol of care was

ties for the care of children with

adopted at the University of Benin

sickle cell disease and is capable

Teaching Hospital, Nigeria and

of reducing the morbidity and mor-

this has contributed to the im-

tality associated with the disease as

proved clinical status of children

well as improving their quality of

with sickle cell disease in the hos-

life.

Introduction

children with SCD. Therefore, alternative treatment mo-

dalities which are effective in reducing the morbidity

Sickle cell disease (SCD) is the commonest single gene

and mortality associated with SCD as well as improving

disease in Africa. About 40 countries in Africa and at

the quality of life in SCD are highly desirable.

least 23 countries of West and Central Africa carry the β

gene. In Nigeria, 20-25% of the population are carri-

The management of SCD continues to pose a challenge

ers of the genetic abnormality, and about 3% of babies

in Africa. Not only does the populace have poor knowl-

edge of the disease, the healthcare providers may also

are born with the disease . The basic pathology in SCD

is the polymerization of haemoglobin following deoxy-

not be familiar with the current concepts in the manage-

genation, a process that results in vaso-occlusion, which

ment of the disease. The management of SCD in most

is the hallmark of the disease . This process results in

health facilities in Africa largely addresses the complica-

various forms of complications such as bone pains, ab-

tions of SCD rather than the disease itself hence, mor-

dominal pain, cerebrovascular accidents and priapism,

bidity and mortality in SCD has remained high. In some

which characterize the disease. In addition, children with

developed countries, comprehensive health care was

SCD are prone to various forms of infections as a result

adopted as the strategy for the management of SCD and

of defective immunity arising from defective splenic

this has resulted in the reduction of mortality from 16-

30% to less than 1% in that setting.

15-18

functions,

6,7

functional abnormalities of white cells

This method of

well as abnormalities of the complement pathway. The

care, as practised in the developed countries, requires

mortality rate in SCD has remained high in Africa.

10-13

the involvement of several professionals such as the

Bone marrow and stem cells transplant offers the poten-

genetic counsellor, paediatric haematologist and the

tial for clinical cure in SCD. This modality of treatment

social worker among others. This multi-disciplinary

is very expensive and thus, not available to a majority of

approach offers a holistic form of care to children with

299

SCD and ideally should be the model of care. In Africa

Management of sickle cell disease

and other developing countries where SCD is prevalent,

the form of care described above is not within reach due

It is important to stress that the management of SCDis

to dearth of most of the required health professionals.

not synonymous with the management of the complica-

Therefore, there is a need to adapt this form of care for

tions of the disease. Patients with SCD only have

the low-resource settings in Africa where SCD is preva-

chronic anaemia as a constant feature of the disease.

lent. This model of care has been successfully adopted

While chronic anaemia is the only constant feature of

in Cotonou, the Republic of Benin. This resulted in a

SCD, other morbidities associated with the disease are

drastic reduction of morbidity and mortality associated

actually complications of the disease.The aim of manag-

with SCD in the country. In recognition of this adapta-

ing SCDincludes the prevention and early recognition of

tion, the World Health Organisation, at the meeting of

the complications of the disease. These complications

the Regional Committee for Africain June 2010, pro-

are, usually, the causes of death in SCD.

posed that by 2020, half of the 23-Member States with

The concept of Comprehensive Health Care manage-

high prevalence of SCD should have developed and

ment of SCDis hinged on the following constituent ser-

commenced the implementation of a clearly designed

vices which are provided by the clinician during clinic

visit:

National Sickle Cell Control programme within the con-

text of a National Health Strategic Plan. It is also ex-



Parental counselling and education.

pected that 25% of the countries in the African Region



Education on the need for adequate nutrition.

should have adopted the concept of comprehensive



Education of the need for adequate hydration.

health care management by the year 2020.

This strat-



Early identification of fever and its urgent treatment

egy was adopted by the Sickle Cell Centre at the Repub-



Early identification of a large spleen.

lic of Benin in 1993

and the University of Benin, Be-



Use of prophylactic medications like Penicillin V,

nin City, Nigeria in 2003. In Cotonou, the Republic of

anti-malarial drugs, Folic acid and Vitamin C.

Benin where this strategy of care is being utilized, the



Immunization againstinfections which are common

mortality of SCD dropped to 15.5/ 10000, a value ten

to children with SCD such as Salmonella , Hemophi-

times less than the overall Under-Five mortality ratein

lus influenza type b, Pneumococcus and Hepatitis B

that country.

In Italy where this strategy was also

virus infections.

adopted, the morbidities and mortality associated with

SCD have reduced.



Need for regular hospital follow-up.

In the last 13 years, the Paediatric Haematology Unit of

Parental counselling and education

the University of Benin Teaching Hospital has man-

aged547 children with sickle cell anaemia (SCA) using

The knowledge of how sickle cell disease is acquired

the new protocol. Twenty children with SCA died over

has remained very poor amongst Africans despite the

high prevalence of the disease in this area. Therefore,

this period; the majority of the deaths occurred among

13 children referredfrom other facilities on account of

marriage between carrier individuals persists without

inhibition. Usually, it is the presence of the disease in

complications of SCA. Seven children in the new proto-

col died; twofrom acute chest syndrome, one from liver

an affected child that brings the carrier status of the par-

failure, one from sequestration crisis (died in a private

ents to fore. Only very few intending couples practice

facility) and the rest from sepsis. In the last five years,

pre-marital screening for SCD particularly where

all the deaths among children in the programme oc-

churches insist on screening before marriages are con-

curred at the Children Emergency Room before the Pae-

tracted.In situations where couples co-habit and where

diatric Haematology Unit could have an input in their

marriages are contracted according to traditional prac-

care. The admission rate for sickle cell crises has re-

tices,screening for SCD is usually not done. In counsel-

duced to an average of one in about two years with some

ling couples with a child who has sickle cell disease,

childrenbeing crises-free for fiveyears. The transfusion

they are taught how the child acquired the disease and

rate has also reduced drastically to less than 2% and the

the effects of the disease on the child are explained to

mortality rate amongst our cohort is 1.3% (7/547). Using

them to enhance a thorough understanding of the patho-

the Body Mass Index (BMI)-Z scores in a recent nutri-

physiology of the disease. The aspect of education is

tional survey of 187 children with SCD, the nutritional

very important in an African setting where mothers are

status of the children in this programme was similar to

usually blamed for the production of “bad children” or

that of their peers, with a reduction in the prevalence

witchcraft when families experience childhood deaths

rate of severe malnutrition to less than 18%. Indeed,

attributed to SCD. In addition, the knowledge of the

75.9% had normal nutritional status while 7% were

patho-physiology of the disease helps parents to manage

overweight. With these achievements, it became neces-

the child with SCD better and comply with the protocol

of care. The care of the children enrolled into this pro-

sary to share this method of care so healthcare providers

in other parts of the country and beyond, can adopt same

gramme is accomplished in the homes of the affected

and more children with SCD may benefit from its nu-

families rather than in the hospitals, hence, the need for

merous advantages.

parental education.

Adequate nutrition

Malnutrition is a common problem among African chil-

300

dren. A lot of African children are stunted due to poor

usually poor.

Caregivers should be taught how to de-

nutritional intake. In Nigeria, 41% of the general popu-

tect fever in children using a thermometer or by feel and

lation of children are stunted.

It is this same setting

report to the hospital once the child with SCD is noticed

that has the bulk of children withSCD.Specifically, SCD

to the febrile. In most parts of Africa, this advice is very

poses a high nutritional burden on the affected children.

pertinent as most caregivers patronize drug vendors for

The disease is associated with a high metabolic rate be-

medications when their children are ill. This contributes

cause of the bone marrow hyperactivity.

25,26

The life

to delay in seeking appropriate care for the child with

span of the sickled red cell is 10-20 days instead of the

SCD. It is important to note that pneumococcal infec-

tions have a doubling rate of 30 minutes.

100-120 days for normal haemoglobin. This places a

high nutritional demand on the child with SCD. In addi-

tion, a child with SCD also requires the same nutrients

Early identification of a large spleen

for his growth and development. This implies that chil-

dren with SCD on diet similar to that of healthy children

Splenic function among children with SCD is defective

are at risk of failure to thrive in early childhood and

as early as the sixth month of life. However, the most

stunted grow with delayed secondary sexual characteris-

life threatening splenic event in SCD is the sequestration

crisis. This event results in the pooling of sickled red

tics later in life. These children are usually thin with

asthenic build. With adequate nutrition, children with

cells in the spleen with a resultant severe anaemia and

SCD are able to achieve normal growth and develop-

severe hypovolaemia or shock. This event can be fatal if

ment as their peers with normal haemoglobin. The stig-

it is not promptly identified and appropriately managed

with blood transfusion. Caregivers should be taught

mata of the disease such as prognatism, skull bossing

and long thin extremities have been observed to disap-

how to identify a large spleen in a child. In Africa, iden-

pear with adequate nutrition. Therefore, caregivers are

tifying a large spleen by caregivers is important asits

advised to feed their children frequently with as much as

presence has been associated with anaemia and frequent

six to eight meals daily using nutritious diets. Non-

illness. In traditional parlance, scarification marks are

nutritious meals or snacks should be discouraged and the

made over the abdomen to “treat the spleen”. A large

parents are advised to ensure children go to school with

number of children with SCDin Nigeria have these

food instead of snacks.

scarification marks on their anterior abdominal wall.

The caregivers should be taught how to detect a large

Adequate hydration

spleen in their children during bath. The caregivers are

advised to take the child to the hospital if they notice an

Vaso-occlusive events occur frequently among children

enlarged spleen for thorough evaluation especially to

with SCDbecause the sickled red cells cause hypervis-

check the packed cell volume.

cosity of the blood which results in sluggish blood flow.

Dehydration worsens this hyperviscosity and the latter

Use of prophylactic medications

reduces the flow velocity and thus, increases the delay

time that is critical in polymer formation. Polymers are

Drugs routinely given to children with SCD include Fo-

responsible for vaso-occlusion which is the hallmark of

lic acid 5 mg daily, Vitamin C and malaria prophylaxis.

SCD. Most of the morbidities and mortality which char-

Folic acid therapy prevents megaloblastic crises as a

acterize SCD result from multiple organ damage follow-

result of high red cell turnover, Vitamin C functions as

ing recurrent vaso-occlusive events. Therefore, the pre-

anti-oxidant because of the oxidant stress imposed by

the red cell hemolysis while malarial prophylaxis is

vention of vaso-occlusive events is important in ensur-

important for children in malaria-endemic region. Ma-

ing not only the wellbeing of the SCD child but also the

prevention of chronic organ damage and early death.

laria not only worsens the anaemia but is also a cause of

Children with SCD are advised to drink 2-3L /m of

frequent morbidity. Penicillin V tablet is administered

for the prevention of pneumococcal infections.

31, 32

water daily. The child is advised to go about with water

For

in order to facilitate adequate water intake and good

children less than three years, Penicillin V 125 000 IU is

hydration. Adequate hydration also enhances the urinary

administered twice daily while the dose is doubled for

excretion of bilirubin resulting from the chronic haemo-

children aged between three and five years. The drug is

lytic state in SCD. Children with SCD in our programme

used along with the pneumococcal vaccine.

remain crisis-freefor as long as two to five years follow-

ing adequate water intake. Hydroxyurea, an anti-sickling

Immunization for the child with SCD

drug, is not routinely used for the children with SCD

enrolled in the programme hence the use of this medica-

Infection is the commonest cause of death in SCD chil-

tion may not explain the observed improvements among

dren, especially infections due to encapsulated organ-

isms.

27,28,31,33-35

our cohorts.

Therefore, vaccinations against Haemo-

37,38

philus influenza type b, pneumoccocus

and Salmo-

Early identification and treatment of fever

nella are essential in the care of children with

SCD.However, the routine immunization schedules in

Fever is regarded as an emergency in a child with SCD

most African countries do not include these vaccines.

as fever may be a flag sign for infections. Infections are

Nigeria, for example, has just implemented the use of

common causes of morbidities and mortality among

the Haemophilus influenza type b and pneumococcal

children with SCD since their immune functions are

vaccines for routine immunization. Salmonella vaccine

301

is not included in the routine immunization schedule for

Regular hospital follow up

children in Nigeria.

These bacterial organisms have

been proven to be the major causes of infections in chil-

For a disease that has lifelong implications, there is a

dren with SCD, hence, there is an urgent need to vacci-

need for regular hospital follow up. In Africa, most pa-

nate these immune-compromised children to prevent

tients with SCD do not attend follow-up care in the hos-

morbidities and mortality associated with infections.

pital setting. Children are only brought for care when

These vaccines are routinely used for the children with

they have crises. This attitude may be is detrimental to

SCD enrolled into the programme at the University of

the health of children with SCD because regular follow-

Benin Teaching Hospital, Benin City. Hepatitis B vac-

up hospital visits may ensure adequate growth and de-

cine was only introduced 12 years ago in Nigeria, hence,

velopment through growth monitoring and frequent edu-

children with SCD who are older than 12 years must

cation. This allows for early identification and prompt

have missed the vaccines. Therefore, children who are

management of complications. This allows the child

older than 12 years and those aged less than 12 years but

with SCD to maintain good health.

who were not vaccinated should be tested for Hepatitis

B virus infection and the vaccine should be administered

if they are sero-negative.

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