Who has Sickle Cell Disease, SCD, and why are
some people born with this disease? In the view
of Professor of Medicine, Department of Medicine,
College of Medicine, University of Lagos, CMUL,
Professor Michael Kehinde, not less than 300,000
children are born with one form of SCD every year
mostly, in sub-Saharan Africa, where malaria is
common. Kehinde, a Specialist Physician and
Clinical Haematologist who for more than two
decades, headed the Clinical Haematology Unit at
the Lagos University teaching Hospital, LUTH, says
parents who fail to verify their genotype before
they get married, are to be blamed when they
produce children with SCD.
A child should know his or her genotype right from
primary school. The genotype is the set of genes
in the cells of every living organism which is
responsible for a particular trait or
characteristics. Last week, while presenting his
Inaugural Lecture entitled: “Human Unique Blood
Cells and Consequences”, at the Main Bowl of the
University of Lagos, Akoka, Lagos, Kehinde
observed that, Nigeria being the most populous
black nation in the world, unfortunately, has the
highest incidence of SCD in the world.
“It is estimated that around 2 percent of new
borns in Nigeria are affected by sickle cell
anaemia giving a total of at least 150,000 affected
children born every year in Nigeria alone. “Sickle
Cell Disease causes approximately 8 percent of all
infant deaths per year. The carrier of haemoglobin
S frequency is between 10 to 40 percent with an
average of 25 percent in Nigeria. That means the
sickle cell trait carrier population is over 50
million. Thus, one person out of every four persons
has HbS.”
Overlooked epidemic: Further, Kehinde said if an
inherited disorder can be described as an
epidemic, then sickle cell anaemia in Nigerian
would eminently qualify for that description.
“So it is an epidemic that has been over looked.
People should know their genotype long before
they get married and not just before or after
marriage, because once they are about to settle
down, there is little that they can be told again to
change their perception. They will run away from
you and settle down somewhere else.
Explaining that SCD is inherited and not acquired,
the physician noted: “When AA marries AA, all
their children will be AA. If AA marries AS, there is
50 percent chance that a child will be AA and 50
percent chance that a child will be AS.Fpr children
that inherit the sickle haemoglobin gene from one
parent and a normal gene from the other parent
have sickle cell trait. People who have sickle cell
trait usually have few, if any, symptoms and live
normal lives.”
Selection and consequences
“When AA marries AA, all their children will be AA.
If AA marries AS, there is 50 percent chance that
a child will be AA and 50 percent chance that a
child will be AS. When AS marries AS, there is 50
percent chance the child will be AS, 25 percent
chance the child will be AA and 25 percent
chance the child will be SS.”
“If a couple with AA and SS genotypes marry, all
of their children will be AS. When AS marries SS, it
is 50 percent chance their child will be AS and 50
percent chance the child will be SS. When SS
marries SS, all their children will be SS.”
SCD body functioning
The life journey of a person with SCD can be
troublesome at times with occurrence of bone
pains due to sickling and haemolysis so that the
red cell lives only for about 30 days or less rather
than for about 120 days (which is normal) as a
result of prevailing unfavourable environment such
as dehydration, infection, emotional stress,
strenuous physical exercise, very cold weather,
etc.
The red blood cells are more fragile and more
readily scavenged from the circulation,
contributing to the chronic anaemia. Sometimes
sickle cell crises occur spontaneously due to no
identifiable risk factor. They have acute
vasoocclusion events, chronic haemolytic anaemia
and organ dysfunction due to repeated sickling
episodes. Reduced flow and oxygen supply cause
pain and lead to rapid destruction of blood cells.
Wrong management: Sickle cell pain crisis is a
medical emergency in Nigeria and when we
assessed the management of SCD pain crises in
adults by medical practitioners in Nigeria, in a
cross sectional survey of 174 medical
practitioners, we found that 70 to 80 percent of
these doctors were giving appropriate strength of
analgesics for appropriate severity in pain.
“Thirty two of 163 would however not prescribe
narcotic analgesics even in severe pain rises, for
various reasons. However, 38 of 174 would give
inadequate quantity of fluid and 18 of 124 will not
give antibiotic even in the presence of markedly
elevated white blood cell count. 45 of 90 will give
antimalaria drugs routinely.
Others will give antimalaria drugs only if there is
fever. “None of the doctors will insist on a
laboratory menstruation of malaria parasitaemia
before giving anti-malaria drugs. Although, 70 to
80 percent of doctors manage pain crises
appropriately, it would be desired that all doctors
manage sickle cell pain crises appropriately.
Management protocol
To achieve this, a pain management protocol may
be introduced in order to ensure that every sickle
cell pain is properly and constantly
managed. Survival: When we looked at our
outpatient record in 1984, there were no patients
older than 30 years of age. 31 years later, in 2015,
30 percent of patients with sickle cell disease
were over 30 years. The oldest patient was over
60 years. This is as a result o the increased
medical care and support instituted in our
haematology unit.
The main cause of poor survival of children with
sickle disorder is lack of consistent coordinated
care and follows up. On his part, the Chairman,
Sickle Cell Foundation of Nigeria, Professor Olu
Akinyanju, said with more funding for research, life
can be more interesting for people living with
SCD. He said as far back as 1972, American
Congress passed the Sickle Cell Anaemia Act into
law and put $250 million down to research on it.
Every year they are putting almost about that
same amount into it which has been helping in
making life easier for sickle cell patients. But here
in Nigeria what we allocated to the whole health
budget is not even up to that amount. The level of
awareness is still low. It is something that should
be taught in schools.
“We have a 65-year old in our Centre who donated
a generator to the Centre recently. Our hope is to
get more people to reach that age of 65, whether
you are diabetic, hypertension, you should be able
to have normal life expectancy. That is why we
need research, regrettably, in Nigeria, research is
not our culture, the reason is that Nigeria believes
in immediate result, but research takes a long
process.”
