In an interview to Rama Krishnamachari, DEOC, Dr. J.S Arora, National Thalassemia Welfare Society (NTWS), has explained the blood disorders included in The Rights of Persons with Disabilties Act (RPWD), 2016 and how employers and educational institutions can accommodate people with blood disorders. Dr. J.S. Arora has been a strong advocate for the rights of persons with blood disorders and it is due to his efforts that the blood disorders are included as a disability in The RPWD Act, 2016.
What are the blood disorders covered in The RPWD Act, 2016?
The three blood disorders included in The RPWD Act are Thalassemia, Hemophilia and Sickle Cell Disease.
In Thalassemia, body cannot make enough haemoglobin or makes defective red blood cells (RBC) due to imbalance in alpha and beta genes in haemoglobin. RBCs break much early by 10-20 days than normal lifespan of 120 days. This leads to severe anemia. Severe anemia leads to lethargy, loss of appetite, disfigurement of facial bones, enlarged liver and spleen which lead to protruded abdomen and increased destruction of red blood cells.
In Hemophilia, there is a deficiency of one of the factors necessary for coagulation of blood. This leads to excessive bleeding. People with hemophilia bleed easily, and the blood takes a longer time to clot. There are different types of Hemophilia – Types A and B, based on the deficiency of the type of the clotting factor.
Sickle Cell Anaemia/Disease (SCD)
Normal RBC’s (red blood cells) are smooth surfaced, enabling them to change their shape to flow through small blood vessels. Under certain conditions RBC’s containing hemoglobin become rigid, elongated, and sickle shaped. These sickle-shaped cells are not flexible; they stick to vessel walls, causing a blockage of blood flow resulting into reduced supply of oxygen in nearby tissues causing sudden, severe pain attacks, called pain crises. Signs and symptoms of SCD include severe pain, hand-foot syndrome, abdominal pain, infections, leg ulcers, anemia, gallstones, organ damage, hip bone necrosis and stroke.
How common are the blood disorders?
- Thalassemia:. 7% of the world population carries Thalassemia/Hemoglobinopathy gene and 3,00,000-4,00,000 babies with severe hemoglobinopathies are born each year. India, Thailand and Indonesia account for 50% of world’s thalassaemia carriers, thus 50% of thalassaemia majors are born annually in these countries. There are 50 million Thalassemia carriers and over 12,000 new affected children born every year.
- Hemophilia: One in 7500 live male births and approximately 17,000 males in the U.S are suffering from Hemophilia. All races and socioeconomic groups are equally affected. In India approximately 1 in 5000 males have Hemophilia A and 1 in 30,000 have Hemophilia B. India reported cases of Hemophilia were 11586 (year 2014).
- Sickle Cell Disease is common in tribal areas of MP, Chhatisgarh, Maharashtra, Gujarat, Andhra, Karnataka, Odisha and Jharkhand. Carrier rate is as high as 40% in some areas.
What is the treatment and cure for blood disorders?
Thalassemia can be cured by stem cell transplantation but limited availability of matched donors (<30%), huge cost and high risk make this possible only in few cases.
Therefore, most people with Thalassemia have to take repeated blood transfusion every 2-4 weeks throughout their life. Scarcity of blood & transfusion centres, transfusion reactions & infections (Hepatitis B, Hepatitis C, and HIV) and repeated hospital visits make life very difficult for them. Repeated blood transfusion leads to iron overload which damages vital organs resulting into growth retardation, delayed/missed puberty, endocrine complications, bone disease, heart and liver failure and ultimately premature death. As blood transfusion is lifelong chelation therapy (drugs to take out extra iron from body) is also lifelong. Treatment is very costly and may require daily injections for 10-12 hours a day. Inadequately treated person with Thalassemia succumb to death between 12-24 years while adequately treated person live a near normal life.
The recognition of bleeding episodes and treating bleeds as early as possible prevent complications such as loss of range of motion, arthritis & muscle atrophy. Replacement of deficient clotting factor is the single most important step in any intervention. Factor concentrate should be given as close to the time of the bleed as possible.
- Primary – Usually started in young children to reduce or prevent joint disease and it is continued indefinitely.
- Secondary – Short term and is started when a bleed has occurred and continued on a regular schedule for a defined period of time.
Sickle Cell Disease
Prevention and cure options are similar to that of Thalassemia. Treatment includes childhood immunizations, painkillers, antibiotics, lifestyle changes (avoid exhaustion, cold, high altitude, stressful conditions) and sometimes blood transfusions.
What is the cause of the blood disorders?
In Thalassemia major (diseased condition) people have two defective globin genes. If one beta gene is defective it is called thalassemia minor, trait or carrier. If both husband and wife are thalassemia carriers then in each pregnancy there is 25% chance of giving birth to a thalassemia major child. Thalassemia can be prevented in offspring if couple is subjected to thalassemia trait test (HbA2) before conception, followed by antenatal diagnosis if both are thalassemia carriers. Thalassemia carriers are normal & healthy and do not require any treatment.
Like Thalassemia, people with SCD are born with two sickle cell genes, one from each parent.
Hemophilia is X-linked disorder, females carry gene and males are affected. About 30% cases are with new genetic mutations.
How does the blood disorder affect an individual in terms of her/his functioning – daily living, education, employment etc.?
Following snags are common in all three blood disorders Thalassemia, Sickle Cell Anaemia and Hemophilia:
- Repeated blood/factors administration for survival/prevention of complications.
- Repeated pricks for investigations and to infuse medicines or blood.
- Higher incidence of infections, inflammation and painful episodes.
- Higher incidence of transfusion transmitted infections like Hepatitis B, Hepatitis C and HIV.
- Repeated hospital visits for treatment or monitoring. Travel (100 – 500 km) for adequate/proper treatment.
- Even routine activities require micro planning according to health condition, next hospital visit, or sudden crisis. Life style is severely restricted and several activities are curtailed. This affects whole family.
- Mental health could also be affected.
- Loss of work days (education, employment or business).
- Because of anaemia, pain crisis, infections, hospital visits etc. they miss schools (may be exams too) so they have difficulty competing with their peers.
- Repeated hospital visits, anaemia, infections, pains also restrict their extracurricular activities and socializing. This further barricades their life.
- For higher education or better job prospects one has to move 200 – 2000 Km away from home town. Maintaining health with such a challenging treatment at a new place is extremely difficult to manage.
- Treatment is very costly not affordable by even upper middle class family.
- Social stigma a big issue.
- Life expectancy always hovers the minds of affected persons and family.
Some of the government benefits are only for BPL families. There should be no income ceiling. If at all there is need to put income ceiling, it should be minimum Rs. 5,00,000/annum.
What does The RPWD Act, 2016 have for persons with blood disorders?
The RPWD Act 2016 has recognised persons with blood disorders (Thalassemia, Hemophilia and Sickle Cell Disease) as ‘persons with disabilities’ under the Act. Those with 40% and above disability will be given a Disability Certificate.
The first and foremost benefit of The RPWD Act 2016 is non-discrimination. In the past many outstanding persons with blood disorders have been denied employment even though they were selected on merits. With this Act in place, such discrimination cannot happen. Moreover, the Act states denying reasonable accommodation (adjustment) will be also discrimination. Therefore, an organization should not deny any accommodation that a person may need.
The second most important benefit would be “Reservation in Higher Education”. This will enable the persons with blood disorders to pursue higher education of their interest.
The other benefits of the RPWD Act, 2016 are the right to equality, life with dignity, and respect for his/her own integrity equally with others.
Few more measures like appropriate healthcare measures, insurance schemes, rehabilitation programs and incentives to employer in private sector that provide 5% reservation for persons with benchmark disability to be undertaken by the Government will depend on how these are implemented.
What accommodations (adjustments) should employers and educational institutions provide?
Private and government establishments should not discriminate a person with blood disorder. Individualised accommodations should be provided. Some of the common accommodations are listed below (these is not an exhaustive list).
- Time Flexibility: A person may need adjustment in work timings, for treatment and related issues, which should be provided.
- Extra Leave: A person may need extra leave (over and above what is entitled) for anaemia, pain crisis, infections, hospital visits etc.
- Relaxation in Attendance: Schools and colleges should provide relaxation in attendance and should not prevent a student with blood disorders from writing exams if the student does not meet the required attendance. If a student is unable to write an exam on a particular day, she / he should be given reexamination on another day.
- Extra time for exams and assignments (compensatory time): Some people may need to take breaks due to pain or other reasons related to their disability and hence may need extra time to complete their exam paper, which should be provided.
- Financial assistance for the affected person/caregiver to meet the medical expenses.
- Posting and Transfers : Since optimum treatment is available in select cities only, preference in posting should be given to person with blood disorders and their parents.
- Inclusion in extracurricular activities: Some people may have difficulty participating in certain physical activities and they could be exempted. However, this should not lead to the student feeling left out. Some precautions can be taken in consultation with the person/parent, to make the activity safe for them. Inclusive extra curricular activities can also be planned.
- Ensuring no bullying/harassment at school/college/workplace: Organizations/schools/colleges should create adequate awareness so that there is no stigma and there is inclusion in all aspects of school/college/work/ social life. There should be policies to ensure zero tolerance for bullying/harassment/discrimination.
Please tell us about your organization and the work you do?
National Thalassemia Welfare Society (NTWS) was formed by patients, parents, doctors and well-wishers in November 1991 at AIIMS. Since then it is dedicated to the cause of Thalassemia and Sickle Cell Anaemia. Around 3000 persons are members. I am a Thalasssemialogist and the General Secretary of NTWS.
We have two prime objectives: to strive for best possible care for existing patients, and prevent future generation from menace of Thalassemia and SCD.
Our aim is to ensure free treatment to Thalassemics all over India. Our first success was in Delhi around 18-19 years back. Now few more states have started providing free treatment to thalassemia and SCD patients, some are providing partially.
We organize blood donation camps every year to support the blood banks. Those patients who don’t get free medicines from Government, NTWS supports them to some extent. We have been regularly supporting the cost of bone marrow transplant, a permanent cure for thalassemia.
I joined Shri Javed Abidi ji’s cross disability movement in 1996 and campaigned to include Thalassemia, Sickle Cell Anaemia and Hemophilia in the list of disabilities. I am delighted to say that with his continuous, unconditional, untiring support and guidance now we could get these blood disorders in The RPWD Act 2016.
Are there any support groups for people with blood disorders in the country. Please share a few here?
National Thalassemia Welfare Society
KG-I/97, Vikas Puri
New Delhi -110018
Tel (off) +91-11-25511795, 796
E- Mail: email@example.com; firstname.lastname@example.org
Haryana Thalassemia Welfare Society
133/29, Ram Gopal Colony
Near Sagar Villa
Rohtak 124001, Haryana
Thalassemia Children Welfare Society
35-A /1-1A Rampur Garden,
Civil Lines Bareilly, UP
J & K Thalassemia Welfare Society
217/6 Channi Himmat
Thalassemia Society Gwalior
A 106 Samadhiya Colony
Ajmer Region Thalassemia Welfare Society
R.O. 18/2, Mundri Mohalla
Ajmer – 305001
Thalassemia Jagruti Foundation
1,2, Saraswati Co.Op Hous. Society
B/h. Samrathnagar Bus Stand
Indira Bridge Corner, Hansol
Ahmedabad 382475 (Gujarat)
Thalassemia & Sickle Cell Society
22-8-496-501, Cgatta Bazar Ex Road
Near City Civil Court
Hyderabad – 500002
Thalassemia & Sickle CellsSociety of Bangalore
Guru Nanak Niwas
Number 3 Nandidurga Extension
7th Road Opp Corporation Office
Bangalore – 560046