Haemoglobin disorders are inherited
blood diseases that affect how oxygen is carried in the body.
Haemoglobin disorders fall into two main categories: sickle-cell
disease and thalassaemias.
Sickle-cell disease is characterized by a mutation
in the shape of the red blood cell from a smooth, donut-shape to a
crescent or half moon shape. The misshapen cells lack plasticity
and can block small blood vessels, impairing blood flow. This
condition leads to shortened red blood cell survival, and
subsequent anaemia, often called sickle-cell anaemia. Poor blood
oxygen levels and blood vessel blockages in people with
sickle-cell disease can lead to chronic acute pain syndromes,
severe bacterial infections, and necrosis (tissue death).
Thalassaemias are also inherited blood disorders. People with
thalassemia are not able to make enough haemoglobin, which is
found in red blood cells and carries oxygen to all parts of the
body. When there is not enough haemoglobin in the red blood cells,
oxygen cannot get to all parts of the body. Organs then become
starved for oxygen and are unable to function properly. There are
two major types of thalassaemia, alpha and beta, which are named
for the two protein chains that make up normal hemoglobin. Alpha
and beta thalassaemias have both mild and severe forms.
FACTS ABOUT HAEMOGLOBIN
DISORDERS
It is estimated that each year
over 300,000 babies with severe forms of these diseases are born worldwide;
the majority in low and middle income countries.
Approximately 5% of the
world’s population are carriers of a trait gene for sickle-cell
disease or thalassaemia; The percentage of people who are
carriers of the gene is as high as 25% in some regions.
These conditions are most
prevalent in tropical regions; however population migration has
spread these diseases to most countries.
Thalassaemias are the most
common in Asia, the Mediterranean basin, and the Middle East.
Sickle-cell disease
predominates in Africa.
WHAT CAUSES HAEMOGLOBIN
DISORDERS?
Haemoglobin disorders are
inherited from parents in much the same way as blood type, hair
color and texture, eye colour and other physical traits.
Sickle-cell disease and thalaessemias can occur only when both
parents have trait genes for the particular condition. A child who
inherits two of the same trait genes - one from each parent - will
be born with the disease. However this is not inevitable: a child
of two carriers has only a 25% chance of receiving two trait genes
and developing the disease, and a 50% chance of being a carrier.
Most carriers lead completely normal, healthy lives.
HOW CAN HAEMOGLOBIN DISORDERS BE
REDUCED?
Haemoglobin disorders can be effectively
reduced through a strategic balance of disease management and
prevention programmes.
Sickle-cell disease cannot be cured; however the condition can be
managed by:
High fluid intake
Healthy diet
Folic acid supplementation
Pain medication
Antibiotics for infections
Thalassaemias require regular
blood transfusions to maintain a healthy supply of haemoglobin and
sustain life. As a result of multiple transfusions, organs become
severely overloaded with iron and secondary treatment is needed to
manage this condition. Thalassaemias can be cured by successful
bone-marrow transplantation, however this procedure is expensive
and not readily available in most settings.
The most cost-effective strategy for reducing the burden of
haemoglobin disorders is to complement disease management with
prevention programmes. Inexpensive and reliable blood tests can
identify couples at risk for having affected children. This
genetic screening is especially opportune before marriage or
pregnancy, allowing couples to discuss the health of their family.
Subsequent genetic counselling informs trait carriers of risks
that may be passed along to their children and the treatment
needed, if affected by a haemoglobin disorder. Prenatal screening
of genetic diseases raises specific ethical, legal and social
issues that require appropriate consideration.
WHO'S ACTIVITIES FOR PREVENTING
AND CONTROLLING HAEMOGLOBIN DISORDERS
The governing bodies of the World Health
Organization have adopted two resolutions on haemoglobin
disorders. The resolution on Sickle-Cell Disease from the 59th
World Health Assembly in May 2006 and the Resolution on
Thalassaemia from the 118th meeting of the WHO Executive Board
call upon affected countries and the secretariat of WHO to
strengthen their response to these conditions.
Specifically, WHO will:
Increase awareness of the
international community of the global burden of these disorders;
Promote equitable access to
health services;
Provide technical support to
countries for the prevention and management of these disorders;
and
Promote and support research
to improve quality of life for those affected.
WHO'S STRATEGY FOR PREVENTION
AND CONTROL OF CHRONIC DISEASES
WHO’s work on haemoglobin
disorders is integrated into the overall WHO chronic disease
prevention and control framework of the Department of Chronic
Disease and Health Promotion. The strategic objectives of the
Department are to raise awareness about the global epidemic of
chronic diseases; create healthy environments, especially for poor
and disadvantaged populations; slow and reverse trends in common
chronic disease risk factors such as unhealthy diet and physical
inactivity; and prevent premature deaths and avoidable disability
due to major chronic diseases.
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