Thalassemia Screening and Management (TSM)
“Saving lives by early diagnoses of blood disorder diseases using an effective screening programme for pregnant women and newborn babies; and management of Thalassemia for the suffering children” at Al-Hayat Hospital
The need for Establishing a Centre for Thalassemia Patients in Afghanistan
Afghanistan has experienced nearly four decades of civil war and instability. During this period the social sectors including the health sector has been severely damaged. As health indicators reveal there are still a consistently high mortality from preventable and curable diseases including, communicable disease, malnutrition, and mental health problems.
Furthermore as majority of Afghan population are illiterate, the practice of marriage between relatives and getting married at a very young age are still very common. By challenging these practices and educating people to avoid under age marriage and marriage between relatives, could reduce blood disorder diseases in their offsprings.
Among congenital or inherited problems present in Afghanistan B-Thalassemia takes the first place because Afghanistan is one of the southeast Asian countries where Thalassemia gene is carried in about 2-3% population and marriage between close relatives causes the recessive forms to become dominant or be expressed phenotypically or clinically and carriers of the Thalassemia to give birth to offspring with obvious or clinical Thalassemia disease.
Afghanistan being a country with limited resources, many children still die daily from infections (pneumonia, diarrhea, measles, malaria etc.) and malnutrion especially in their first 5 years of life.
The Ministry of public health of Afghanistan (MoPH) and other stakeholders (WHO, UNICEF etc.,) give priority to invest in prevention of the above mentioned causes of morbidity and mortality and their limited resources unable them in combating long-term chronic diseases and their consequences on quality of life e.g. Thalassemia, anemia, growth retardation, learning disabilities etc.
We undertook a survey study on children affected by thalassemia and available current facilities to manage their chronic diseases. The Central government hospitals are extremely overcrowded with immeasurable flow of patients from rural areas as well as the cities. Our study shows that many children suffering from diseases like Thalassemia die due to the lack of healthcare system specified for this disease. There are no specific management programmes to fulfill the needs of thousands of children living with Thalassemia in Afghanistan. The parents of Thalassemic children are not getting any help in how to manage their affected children. They give them only blood transfusion sheets, and nowadays many families transfuse blood to their children in their own homes, without receiving any training concerning Blood transfusion.
Families are not aware of other therapies that exist and are needed for their children in addition to routine monthly transfusions (iron chelation, folic acid therapy, and splenectomy, vaccination (hepatitis B, pneumococcal, meningococcal etc.), or stem cell transplantation (if available).
Al-Hayat Hospital is establishing a center for the screening and detection of the new Thalassemia patients and for effective management of the previously diagnosed patients in west of Kabul city, and this will be the first model center for research about Thalassemia.
Normal course of the disease
Thalassemias are now known as Thalassemia syndromes and needs careful attention to every aspect of the disease, the main problem in this illness is the severe anemia which halts the normal growth and development of the growing children and also causes cardiorespiratory decomposition, which can be managed only with repeated blood transfusions.
Secondly, as these patients are anemic, their Gastrointestinal system tries to absorb more iron from foods, this coupled with transfusion related iron accumulation (200mg/ transfusion) causes the iron to accumulate in different body parts e.g. liver, heart, glands etc.(hemochromatosis).
So these patients need regular chelation therapy with deferoxamine (desferal) injected subcutaneously or deferasirox (Exjade) given orally to wash out or chelate the stored iron from their bodies.
The 4th problem of these patients is transfusion related risks, especially blood born infections (Hepatitis B, Hepatitis C, HIV, CMV, Malaria etc.) which can be prevented by proper testing of donated blood and administering some vaccinations.
The 5th problem is spleen enlargement known as splenomegaly which occurs despite proper management of the Thalassemia, and sometimes predispose the patient to mechanical or traumatic rupture risks.
So they should undergo splenectomy provided they are more than 5 years old, and they needed more than 250ml/kg of packed cell/year and they are given the 3 vaccines (pneumococcal, meningococcal, H.influenzae B).
The Hospital technical care of the diagnosed Thalassemia patients will include the following.
- Regular packed cell (R.B.C) transfusion to keep their hemoglobin not less than 9.5 to 10g/dl.
- Regular chelation with deferoxamine to keep their ferritin level in normal limits and to prevent them from bad effect of iron accumulation.
- Vaccination of hepatitis B, and other available vaccines.
- Splenectomy; if they need it, and giving them 3 vaccines 3 weeks prior to surgery and oral penicillin V lifelong.
- Offering them and informing them about the availability of Bone marrow transplantation in advanced centers.
- Giving the child and family emotional support.
- Detection of thalassemia in severely anemic children (with Hb electrophoresis or HbF detection).
- Providing blood transfusion facilities, performing screening tests on donated blood, separation of RBC and plasma, and solving blood group mismatch problems in our blood bank.
- Providing regular chelation with desferal and help of specific machines to inject the drug subcutaneously.
- Testing of blood annually for serum ferritin level.
- Providing hepatitis B vaccines to all patients.
- Providing all 3 (meningococcal, pneumococcal, H.ifluenza B) vaccines to patients needing surgery (splenectomy).
- Regular checkups of growth and development of the patient and monitoring of anthropometric values.
- Training of family members to provide some of the care at their own homes.
- Advocacy for blood donation and making pair blood donors (one donor-one recipient).
- Supporting children suffering from Thalassemia and their families emotionally.
Location of the Thalassemia Centre will be in the Pediatric Department of Al-Hayat Hospital, Kabul, Afghanistan
Ashram responded to the appeal to support the refugee camp in Kabul. Many families retuning from Pakistan found their homes ruined by war and then had to live in camps in Kabul. The sever winter affected them badly.
We contributed towards cost of providing quilts for the families living in the camps. The quilts will be made by the girls who work at our partner's carpet weaving and tailoring workshop in Kabul.
Training young girls in traditional carpet weaving skills and income generation in Kabul
We have invested in ten girl (initially) to train them in carpet weaving.
While the girls were on six months training they also benefited from stipend ~$30, numeracy and literacy classes, midday meal and medical check up run by our partner organisation Afghan Action.
After the training the girls have been supported to set up their own carpet weaving business working from home and earn income. Our partner organisation - Afghan Action is continuing to support the girls with design, marketing and supply of raw materials. The girls are encouraged to continute their education whilest running thier new businesses.
The girls regular income, eduaction and carpet weaving skill has improved their self-esteem and confidence. They manage better in life. All the girls dreams to be able to support thier families income, as you see few case studies below. With this project some are able to help their families.
A Case study of one of the trainees
from the carpet weaving workshop
Zakia is 15 years old. She was born in Ghazni province. During the Taliban regime her family was in kabul and her brother was killed by the Taliban. Her father was ill and died. Now her second brotheris working as a mechanic and he is respondible for feeding family. Zakia came to the center to learn carpet weaving skill in order to be able to bring income to the family after the training. Zakia very much wants to help the family's economic problem and help her brother.
The workshop has been expanded for tailoring and tweleve girls are learning tailoring skill and some are earning income through tailoring.