THINGS TO KNOW ABOUT SICKLE-CELL ANAEMIA
Antigua and Barbuda will join the World Health
Organization (WHO) and the Pan American Health Organization (PAHO) along with
the rest of the region in observance of World Sickle Cell Day on Saturday 19th
June 2021.
Sickle-cell
disease (SCD) is a blood disorder which alters the shape of the red blood cells;
instead of round and flexible they appear half-moon in shape, and are brittle
and sticky. The disease makes the cells shred rapidly, causing severe anaemia along
with the blocking of small blood vessels.
When
blood vessels are blocked, this prevents the flow of blood, oxygen and
nutrients to the tissue which causes unbearable pain and organ damage. This makes
sickle-cell patients predisposed to serious infections.
Some
persons have inherited only the sickle-cell trait; they are referred to as
trait carriers. A person is diagnosed with SCD after inheriting two abnormal
genes but those with the trait obtained one normal and one abnormal gene.
If
both parents have the sickle-cell trait, there is a 25% chance that children
born to them can have SCD and a 25% chance these children can be normal. If one
parent has the trait and the other has SCD, there is a 50% chance the children will
have the disease or and a 50% chance of them being carriers.
A
normal person with a partner who is a trait carrier has a 50% chance of
producing carriers and a 50% chance of producing normal children. Carriers of
the sickle trait may display symptoms of SCD while most are asymptomatic.
Symptoms
of sickle-cell disease include anaemia in which patients can become dizzy,
easily exhausted or experience headaches. Patients sometimes become pale with
yellowish eyes. The most prominent symptom of SCD is pain crises as a result of
blockage of the tiny blood vessels. These crises require emergency medical
attention and hospitalisation.
Pain
intensity differs for those in a sickle-cell crisis with a duration of a few
hours to a few weeks. Some SCD patients have only a few pain crisis annually,
while others find themselves having more than a dozen each year.
SCD
manifests itself in infancy usually between the ages of 5-6 months. In a normal
body, the life span of red blood cells is around 120 days but those with sickled
cells, the cells die within 10-20 days. As a result, the body is then left with
a shortage of red blood cells or anaemia.
People
with sickle cell anaemia have frequent infections due to impairment of their
spleens. Children and infants with sickle cell are generally vaccinated against
specific pathogens and given antibiotics upon infection.
Toddlers
with SCD may experience painful, swollen
fingers or toes with severe anaemia. An exceptionally low blood count will put
patients at risk for devastating complications.
Regular
eye examinations are a necessity in SCD as sickling can cause damage to the
retina and ultimately lead to blindness. Additionally, leg ulcers may occur due
to poor blood flow. Severe, long-term complications of sickling causes damage to the hip joints which
eventually requires hip replacement surgery.
Splenic
sequestration or blood pools in the spleen is a life-threatening condition
associated with SCD. It is commonly found in children and causes the child’s
spleen to expand as it reduces the amount of oxygen- carrying red blood cells
into the body.
Presently, a blood and bone marrow transplant
is the only cure for sickle-cell disease; however, there are treatments that are
effective in reducing symptoms and extending patients’ lives.
Some
treatment options for SCD are: daily folic acid- to produce red blood cells, penicillin
prophylaxis for children, non-routine vaccines (particularly Prevenar-13) for
children and adults along with hydroxyurea: an oral medication to reduce the number of pain crises and
complications.
For
those with SCD their lifestyles are contributing factors. Therefore, they need
to avoid alcohol use, smoking and exertion. Getting enough rest, plenty of
fluids and regular check-ups are compulsory as well.
A
sickle-cell diagnosis increases one’s risk of becoming extremely sick with
Covid-19. Knowing your sickle-cell status is important: get tested.
For
those seeking additional information or help, contact your community clinics or
the Antigua and Barbuda Sickle-cell Association.
Special thanks to Dr. Edda Hadeed for her
wealth of information and willingness to share.