Thalassemia Awareness Program by OSF

A Brief Overview of Thalassemia

Omair Sana Foundation: Tackling Thalassemia in Pakistan

Omair Sana Foundation (OSF) started off as a diversified NGO, operating in the three verticals.  Blood Diseases , Medical Relief  and General Welfare.

In Thalassaemic sphere their mission is to make free treatment available to Thalassemic patients who cannot afford it on their own.   Provide Thalassemic patients with Educational and Social rehabilitation to make them productive and confident members of the society

Dr. Saqib Ansari - Omair Sana Foundation

They help Thalassemics not just EXIST but rather LIVE and lead a healthy life.  While in emergency and general relief we serve the humanity in their medical and social concerns.

Their area of work is medical relief, advocacy, rehabilitation, reconstruction & education in the time of any natural or man-made calamity.  They believe in the collective and collaborative approach for accomplishment of goals that seem hard to achieve   The name of Omair Sana Foundation as the name suggests was derived from Omair & Sana, the two children of Dr. Kashif Hussain Ansari , himself an Oncologist, Hematologist serving in America. Both were suffering from a genetically oriented blood disease. Even after a strong and vigilant health management and having all required resources he couldn’t make their survival possible.

Omair & Sana died in the age of 5&3 respectively. After recovery from the trauma of his children’s demise, Dr. Ansari, Thought that even having all resources in a modern, technically equipped and developed United States, the chance of survival for those who have blood disorders is limited.

What would be happening in a third world country like Pakistan where more than 40% people are living below poverty line, average income level is 2$, medical institution are ill equipped, access to the proper facility center is difficult expensive.

This mishap and insight provoked him to do something for those who deserve back in the motherland, Pakistan.

Bone Marrow Transplant - An Expansive Treatement

Bone marrow transplant

 

It is a very much expansive treatment for the prevention of all types of Thalassemia. Bone marrow transplantation may offer the possibility of a cure in young people who have an HLA-matched donor. Success rates have been in the 80–90% ra

nge. Mortality from the procedure is about 3%. There are no randomized controlled trials which have tested the safety and efficacy of non-identical donor bone marrow transplantation in persons with β- Thalassemia who are dependent on blood transfusion.

If the person does not have an HLA-matched compatible donor, another method called bone marrow transplantation (BMT) from haploidentical mother to child (mismatched donor) may be used. In a study of 31 people, the thalassemia-free survival rate 70%, rejection 23%, and mortality 7%. The best results are with very young people.

Delta Thalassemia is the Reason of Hemoglobins Abnormality

Delta-Thalassemia

As well as alpha and beta chains present in hemoglobin, about 3% of adult hemoglobin is made of alpha and delta chains. Just as with beta Thalassemia, mutations that affect the ability of this gene to produce delta chains can occur.

HBB Gene Must Hurt by Beta Thalassemia

Beta-Thalassemia

Beta Thalassemia is actually due to mutations in the HBB gene on chromosome 11,also inherited in an autosomal, recessive fashion. The severity of the disease depends on the nature of the mutation. Mutations are characterized as either βo or β Thalassemia major if they prevent any formation of β chains, the most severe form of β-Thalassemia; as either β+ or β Thalassemia intermedia if they allow some β chain formation to occur; or as β Thalassemia minor if only one of the two β globin alleles contains a mutation, so that β chain production is not terribly compromised and patients may be relatively asymptomatic.

Alpha Thalassemia - Severe Genetical Blood Disorder

Alpha-Thalassemia

The α-Thalassemia involves the genes HBA1 and HBA2, inherited in a Mendelian recessive fashion. Two gene loci and so four alleles exist. It is also connected to the deletion of the 16p chromosome. α Thalassemia result in decreased alpha-globin pro

duction, therefore fewer alpha-globin chains are produced, resulting in an excess of β chains in adults and excess γ chains in newborns. The excess β chains form unstable tetramers (called hemoglobin H or HbH of 4 beta chains), which have abnormal oxygen dissociation curves.

Thalassemia: Signs and symptoms

Abnormality Seems Clear in Thalassemics

Iron overload: People with Thalassemia can get an overload of iron in their bodies, either from the disease itself or from frequent blood transfusions. Too much iron can result in damage to the heart, liver, and endocrine system, which includes glands that produce hormones that regulate processes throughout the body. The damage is characterized by excessive deposits of iron. Without adequate iron chelation therapy, almost all patients with beta-Thalassemia accumulate potentially fatal iron levels.

Infection: People with Thalassemia have an increased risk of infection. This is especially true if the spleen has been removed.

Bone deformities: Thalassemia can make the bone marrow expand, which causes bones to widen. This can result in abnormal bone structure, especially in the face and skull. Bone marrow expansion also makes bones thin and brittle, increasing the risk of broken bones.

Enlarged spleen: The spleen aids in fighting infection and filters unwanted material, such as old or damaged blood cells. Thalassemia is often accompanied by the destruction of a large number of red blood cells and the task of removing these cells causes the spleen to enlarge. Splenomegaly can make anemia worse, and it can reduce the life of transfused red blood cells. Severe enlargement of the spleen may necessitate its removal.

Slowed growth rates: Anemia can cause a child's growth to slow. Puberty also may be delayed in children with Thalassemia.

Heart problems: Diseases, such as congestive heart failure and abnormal heart rhythms, may be associated with severe Thalassemia.