Review of Sickle Cell Disease and its public health significance

1. A REVIEW OF SICKLE CELL DISEASE
AND ITS PUBLIC HEALTH
SIGNIFICANCE
BY
AGATSA DAVID TERNA
BSU/SC/BIO/12/16667
SEMINAR
OCTOBER, 2016

2. INTRODUCTION
Sickle-cell disease (SCD) is a group of
genetically passed down blood disorders. The
most common type is known as sickle-cell
anaemia (SCA).
It results in an abnormality in the oxygen-
carrying protein haemoglobin (Hb) found in red
blood cells. This leads to a rigid, sickle-like shape
under certain circumstances.
Sickle-cell disease occurs when a person
inherits two abnormal copies of the haemoglobin
gene, one from each parent. (Rees et al., 2010).

3. AIM
To review sickle cell disease and its public health
significance.
OBJECTIVES
To describe the effects of Sickle cell disease on
affected persons.
To relate effects of the disease to the public
health impact on affected persons.
To discuss the general public health
significance of sickle cell disease.

4. EFFECTS OF SICKLE CELL DISEASE
Sickle Cell Disease Pain
The hallmark feature of SCD is frequent and
unpredictable pain, termed vaso-occlusive crises
(VOCs) (Shapiro and Ballas, 1994), which account
for approximately 25% of hospital visits in
children with SCD (Rees et al., 2003).
VOCs are the result of “sickling” of the blood
vessels leading to a lack of flexibility.
Specifically, the red blood cells become sickle-
shaped and sticky, blocking blood flow through
the vessels which results in pain, ischemia, and
infarction (Ballas, 1998).

5. Psychological effects of Sickle Cell Disease
Sickle Cell Disease, especially the pain
episodes, can have widespread impact on both
the psychological functioning of the individual
diagnosed with the illness and their families.
Specifically, SCD has been associated with
several indicators of psychological
maladjustment including emotional and
behavioral problems, poor self concept and
interpersonal functioning, limited athletic
abilities (due in part to illness restrictions), and
poor academic performance (Shapiro et al., 1995;
Thompson et al., 1993).

6. Children with SCD are at risk for
maladjustment in almost every area of daily
functioning.
With respect to the family, caregivers of
children with SCD are burdened with missed
work, increased family stress, and increased
disease care demands, which is due in part to the
unpredictability of pain crises care in SCD ( Anie
et al., 2002).

7. Health Related Quality of Life (QOL)of HbS
Patients
Research has demonstrated that there is a
unique interplay between the patient’s
psychosocial adjustment and the
pathophysiology of SCD (Hays, 1995).
Although there are few studies, findings
suggest that QOL outcomes in children with SCD
are generally poor. For example, Kater et al.
(1999) found that a pediatric population with
Sickle Cell Disease had lower daily functioning
and general physical limitations than parents of
healthy children.

8.  Palermo et al. (2002), found that children with
SCD were experiencing more psychosocial
maladjustment compared to the healthy controls.
Moreover, children with SCD had significantly
more limited general health and physical
functioning, more limitations in their academic
functioning and social activities attributed to
their physical health, and more behavior and
emotional problems when compared to a healthy
control group.
Anie et al. (2002) also found that sickle cell pain
epidsodes had a significant impact on the quality
of life of patients involved.

9. Plate 1: Normal and Sickle Red blood Cells
Source:www.yourgenome.org/facts/what-is-sickle-cell-anaemia

10. Plate 2: A Sickle Cell Patient
Source: https://biochemist01.wordpress.com/tag/anemia-cause/

11. Biological Benefits of HbS: Protection from
Malaria
Sickle cell trait, HbAS, produces mild
hematological symptoms but provides protection
against malaria mortality and severe malaria
symptoms (Akide-Ndunge et al., 2003).
In a heterozygous state (HbAS or HbAC), it
does not result in the life shortening condition,
that is, sickle cell disease; instead the subject
benefits from reduced mortality related to
malaria (Serjeant, 2001).

12. RESULTS
Table 1: Prevalence of sickle cell disease in Nigeria
AUTHOR STUDY AREA PREVALENCE
Obisesan et al., 2016 Kogi 1.25%
Adu et al., 2014 Delta state 2.0%
Onuigwe et al., 2014 Sokoto state 1.3%
Adeyemi et al., 2016 Ogun state 1.6%
Nnaji et al., 2013 Enugu 0.9%
Mustafa and Abubakar, 2001 Kano 11.8%
Yakubu et al., 2013 Sokoto State 4.25%
Igbenughu et al., 2015 Oyo 1.60%
Uzeogwu and Onwurah, 2003 Nsukka 3.36%
Babadoko et al., 2014 Borno 0.21%
Onuoha et al., 2015 Bayelsa 1.38%
Adewuyi and Akintunde, 1990 Ilorin 2.8%
Udomah et al., 2015 Sokoto 0.3%

13. RESULTS CONT’D
AUTHOR STUDY AREA PREVALENCE
Ugboma and George, 2015 Port Harcourt 0.22%
Umoh et al., 2010 Uyo 1.54%
Nwafor and Banigo, 2001 Rivers State 4.0%
Mannir Sani, 2014 Katsina 0.02%
Usanga et al., 1996 Akwa Ibom 0.4%
Erhabor et al., 2010 Niger Delta 1.5%
Okpara et al., 2010 Cross River 3.6%
Nubila et al., 2013 Ibadan 6.6%
Akodu et al., 2015 Lagos 9.4%

14. RESULTS CONT’D
0.21%
3.50%
2.03%
2.13%
4.80%
1.83%
Chart 1: Prevalence of Sickle Cell Disease according to Nigeria’s Geopolitical
Zones.
North-East
North-West
North-Central
South-East
South-West
South-South

15. RESULTS CONT’D
0
0.5
1
1.5
2
2.5
3
3.5
2009-2010 2011-2012 2012-2014 2015-2016
3.25
3.08
1.28
1.02
Chart 2: Year Trend of sickle cell disease in Nigeria

16. CONCLUSION
Sickle cell disease is still a noticeable
phenomenon in the world today with Nigeria as
one of the most significant regions affected.
Sickle cell disease results in a number of
physiological disorders in patients with the
disease (those homozygous recessive) and this
results in reduced performance, psychological
traumas, feeling of inferiority and fear of death
among other threats to the sickle cell patient.

17. RECOMMENDATION
 Hospitals should increase the people’s
awareness about SCD and how to reduce the
incidence of the disease via videos, hand-outs
and programs that show the problem and
treatment options available.
Prevention which entails setting up sickle-cell
screening and genetic counselling programmes in
high prevalence states should be ensured.
 Simple, affordable technology that are
accessible to a large proportion of the community
should be made available for management of
sickle-cell disease at different levels of the health-
care system.