Sickle cell disease awareness observed

The
Cayman Islands is joining the United Nations on Saturday to observe Sickle Cell
Disease World Day.

This
year is the 100th anniversary of sickle cell disease being officially recognised
as a separate disorder.

In
December 2008, the United Nations General Assembly adopted a resolution that
recognised sickle cell disease as a public health problem, and the Sickle Cell
Disease World Day was first observed last year in a bid to raise public
awareness of the disease.

The
Public Health Department is aware of 36 people in the Cayman Islands with
sickle cell disease, and the islands’ hospitals have facilities to diagnose and
manage it.

Sickle
cell disease is a chronic disease for which there is no cure, but medical
professionals can treat symptoms. Sickle cell testing is offered to all newborn
babies in Cayman.

Cayman
has a peer support group called the Sickle Cell Support Group, where family and
patients share experiences in coping with the disease, and educational sessions
are organised for awareness and management of the disease. The group meets
three to four times a year.

“If
someone has sickle cell trait, it is important to know if one’s partner is also
a carrier. If both parents are sickle cell carriers, then with each pregnancy,
there is a 25 per cent risk of having a child with sickle cell disease,” said
Joy Merren, genetics coordinator at the Cayman Islands Health Services
Authority.

“Knowing
ahead of time can help couples make informed reproductive choices. While sickle
cell trait is mild, sickle cell disease is serious and can potentially affect
every organ of the body,” she added.

Sickle
cell disease is an inherited chronic disorder that affects red blood cells. It
is seen worldwide and is one of the most common genetic disorders.

Sickle
cell disease is an inherited blood disorder that affects red blood cells.
People with sickle cell disease have red blood cells that contain mostly haemoglobin*
S, an abnormal type of haemoglobin. Sometimes these red blood cells become
sickle-shaped (crescent shaped) and have difficulty passing through small blood
vessels.

When
sickle-shaped cells block small blood vessels, less blood can reach that part
of the body and tissue that does not receive a normal blood flow eventually becomes
damaged. 

A
normal red blood cell lives approximately 120 days but a sickle cell may only
live 11 or 12 days. 

Sickle cell trait?

When
only one gene is affected, it is called sickle cell trait, or persons are
called sickle cell carriers. Having sickle cell trait means that the person
normally stays healthy and the main significance is that it can be passed on to
one’s children. However, under certain extreme circumstances, a person with the
trait may experience complications as if having sickle cell disease. People
with just the trait cannot later develop the disease.

Inherited sickle cell disorder

If
a man and a woman are both sickle cell carriers, with each pregnancy, there is
a 25 per cent chance of the child having the disease; 25 per cent chance of the
child being completely free from sickle cell; and a 50 per cent chance of the
child having the sickle cell trait, i.e., being a carrier.

If
only one parent is a carrier, there is a 50 per cent chance of the child having
the trait; 50 per cent chance of the child being completely free of sickle; and
no chance of the child having the disease.

Sickle cell disease symptoms

Anaemia,
jaundice and gallstones due to rapid breakdown of the red blood cells

Painful
swelling of fingers and toes in babies

Painful
attacks of joints, back and abdomen as there may be damage to the bone marrow

Infections,
such as pneumonia, may develop

Leg
ulcers may develop due to less oxygen to lower legs

Management of sickle cell disease

Full
recommended immunisations plus pneumococcal vaccines

Penicillin,
starting from about two months of age to five years of age to help prevent
serious infection

Management
of symptoms by using medications as needed

Folic
acid daily to help make new red cells

For
further information on sickle cell disease and on upcoming meetings of the
Sickle Cell Support Group, contact Joy Merren on 244-2630 at the Public Health
Department.