Thalassemia disease causing severe anemia in children

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DHAKA, June 19, 2018 (BSS/UNICEF FEATURE) – Thalassemia, a hereditary
blood disorder that affects the body’s ability to produce hemoglobin and red
blood cells, is one of the worst type of diseases causing severe anemia among
children in Bangladesh and elsewhere across the world.

According to Bangladesh Thalassemia Foundation (BTF), a Thalassemia patient
needs 1-2 bags of blood every month to survive and the disease is very common
in Bangladesh. At least 7% of the Bangladesh population is thalassemia
carriers, it said.

The BTF also said that every year 7,000 new babies are born with
thalassemia in Bangladesh and the disease can be easily preventable with
proper knowledge and awareness. A person with thalassemia has too few red
blood cells and too little hemoglobin. The impact can range from mild to
severe and life-threatening, expert said.

Eminent physicians often suggest conducting blood tests of both male and
female before getting married only to ensure that a baby is not born with
thalassemia, a genetically inherited deadly disease transmitted to babies by
parents. They also suggest avoiding intra-family marriage to keep them away
from giving birth to a Thalassemia trait infant.

“Both male and female should go for a detailed blood test before getting
married to find out the symptoms of possible emergence of the chronic disease
Thalassemia,” Secretary General at Bangladesh Thalassemia Foundation Dr M
Abdur Rahim said.

A simple health check up for Thalassaemia carrier status before starting a
family can save any future child from becoming a patient of Thalassaemia
Major, unending cycle of injections, suffering and misery, says the eminent
haematologist.

“The ‘Thalassemia major’ occurs when a person inherits two Thalassemia
genes, one from each parent. Both parents may have ‘Thalassemia minor’ but
when two individuals who have ‘Thalassemia minor’ get married, there is a 25
percent possibility that any pregnancy can result in a child with Thalassemia
major,” Dr Rahim said.

Director of Dhaka Shishu Hospital Prof Dr Manzoor Hussain said: “If we can
introduce the anti-natal screening system in our country, we can decrease the
rate of the disease by terminating the Thalassemia positive unborn.”

 

Around 100,000 newborns are delivered each year with severe form of
thalassemia, most common with Mediterranean, South Asian, and African
ancestry. Symptoms will not show until the age of 6 months in most infants
with beta thalassemia and some types of alpha thalassemia.

The physicians say prevention is the only measure that can drastically
reduce the incidences of severe blood disorders. In affluent areas, the
prevention of thalassemia is largely dependent on prenatal care. If newly
pregnant couples come in for prenatal care, tests for blood disorders like
thalassemia are conducted, amongst many other crucial exams to ensure the
growth of a healthy embryo.

According to them, if the new pregnancy is tested positive for
thalassemia parents are counseled on their options, including early
termination. This form of screening and prevention has been very effective so
that no more than 20-30 new births with thalassemia occur as opposed to the
expected several hundreds.

After 6 months “normal” hemoglobin starts replacing the fetal type, and
symptoms may begin to appear with jaundice and pale skin, drowsiness and
fatigue, chest pain, cold hands and feet, shortness of breath, leg cramps,
rapid heartbeat, poor feeding, delayed growth, headaches, dizziness and
faintness and greater susceptibility to infections.

Skeletal deformities may result as the body tries to produce more bone
marrow. If there is too much iron, the body will try to absorb more iron to
compensate. Iron may also accumulate from blood transfusions. Excessive iron
can harm the spleen, heart, and liver.

According to the physicians, patients with hemoglobin H are more likely
to develop gallstones and an enlarged spleen. Untreated, the complications of
thalassemia can lead to organ failure. Thalassemia is likely to emerge as a
major health concern in South Asia and Bangladesh as it lies in the world’s
thalassemia belt.

In a comprehensive review paper on Thalassemias in South Asia: clinical
lessons learnt from Bangladesh, a group of physicians have depicted the
epidemiological aspects of thalassemias, mutation profile and current
treatment and management practices in the country by sharing the experience
of dealing with 1,178 cases over the 2009-2014 period in a specialized
thalassemia treatment centre.

They have also discussed the preventative strategies of thalassemias
from the context of Bangladesh which could be effective for other developing
countries. Having a thalassemia trait means that one may not have any
symptoms, but might pass that trait on to the children and increase their
risk for having thalassemia.

 

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