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THALASSAEMIA

 

Nepal Thalassaemia  Society

G.P.O . Box No . 13446

Kathamandu, Nepal

Email : thalassaemia @wlink.com.np

 

Introduction

Realizing the growing threat posed by the thalassaemia , an incurable disease , we the parents and guardians of thalasseamic patients made a decision to work for their benefit . we have formed Nepal thalassaemia society to raise awareness and lobby for the cause.

 

Our Objectives

·        Maintain a roster of all thalassaemic families in the organization and provide up – to –date information .

·        Provide free of cost or economical safe blood transfusions.

·        Arrange and manage a safe blood transfusion center.

·        Provide free or low cost medication .

·        Find national and international sponsors or donors to support the thalassaemic.

 

Long term initiatives

Preventing thalassaemia is the only way of getting rid of the disease and is of primary importance in the overall management of the disease. Nepal thalassaemia society's long –term programme include :

 

·        Raising public awareness and encouraging screening .

·        Raising funds to establish a genetic lab in Kathmandu to carry out various tests to diagnose the disease ,screen the disease ( prenatal diagnosis ) and identify carrier status, in close supervision and cooperation with national and international organizations , related professionals and donors.

·        Updating treatment and technology procedures that will be useful in managing the disease .

 

 

What is thalassaemia?

Thalassaemia are a heterogeneous group of genetic disorders in which the production of normal hemoglobin is partly of completely suppressed because of defective synthesis of one or more globin chains . it is hereditary ,passing from parents to children . it was first diagnosed in 1925 and named thalassaemia from the greek world .since this disease was initially observed around the Mediterranean sea , it is also called Mediterranean anemia . some know it as cooley's anemia . in nepali it is called "APUSTA RAKTAKOSH"  , meaning "not well developed blood cells " . each year 60.000 to 70.000 children in the world are born with thalassaemia major .

 

Thalassaemia major

 

Depending on the defect on the globin chain, several types of thalassaemias have been described, amongst which homozygous B – thalassaemia and thalassaemia is one of the most common ,severe  and clinically important types . in homozygous B – thalassaemia ,although the child is born normally , chinical features appear in the first few years of life .the called will need to continue treatment with regular blood transfusions and various drugs to remain alive .if a thalassaemia patient is not on regular blood transfusion ,haemoglobin level may drop to a very level . and death may occur due to severe anemia or cardiac failure .

 

Treatment of thalassaemia

Treatment is extremely laborious and expensive . to date,this disease is incurable .as for all chronic and incurable disease , prevention is of primary importance .it is evident that appropriate and extensive screening ,accurate detection and counseling of at risk couples and antenatal diagnosis help reduce mortality and morbidity significantly . bone marrow transplantation offers cure but to find a compatible donor is almost impossible and the prognosis is also not incorrigible .newer modes of  treatment such as steam – cell transplantation , preimplantation  HLA typing and gene therapy are beyond a common man's reach . so far regular blood transfusion is the only treatment that is available for thalassaemics .but regular blood transfusion also cause iron own complications .to overcome them regular iron chelation  therepy is needed .

 

 

Quality of life of a thalassaemic

 

If a patient of  thalassaemia is being effectively treated with regular blood transfusion using WBC filter with regular iron chelation and is on a regular clinical follow up , he /she will go on to live a normal life . a well treated thalassaemic can have a normal life .they can do official work , get married and have normal children but they must be careful not choose a thalassaemic or a carrier as a life partner .

 

Thalassaemia in Nepal

 

Nepal , being one of the least development countries in the world , struggles to deal with even the most common disease such as acute respiratory tract infections , diarrhea and measles . in such a situation , we must regrettably acknowledge that treatment of rare diseases such as thalassaemia is extremely difficult ,if not impossible .about 4% of total population has born every year . eversince this society has been registered 29 thalassaemic are in touch .there are 16 thalassaemics are scattered all over Nepal and do not belong to a particular area and ethnicity . there is no suitable facility for the diagnosis of the disease outside Kathmandu .

 

BLOOD SAVES LIFE

Hari maya upreti – 7 years , thalassaemic , before blood transfusion

 

Hari maya upreti – 7 years , thalassaemic . after blood transfusion

 

 

Our appeal

We would like to request to you to support us and provide us with the opportunity to find out the number of scattered thalassaemics , bring them into the organization , save their lives by providing regular treatment and prevent the birth of children carrying this dreadful disease . for further details ,please contact us in the following address .

 

GPO BOX NO : 13446 ,Kathmandu

E-mail : thalassaemia@wlink.com.np

                   mrdurga@wlink.com.np