Thalassemia & Sickle Cell Society

Thalassemia & Sickle Cell Society

Registration No. 5359

Tax exempted under Section 80G of Income Tax

Strive For Prevention

Administrative Office & Transfusion Centre

Door No: 22-8-496 to 501

Chatta Bazaar “X” Road

                                                   Near City Civil Court

                                                   Purani Haveli

                                                   Hyderabad - 500 002

                     Phone No: 040-24560011 & 64610610

    E-mail: tscsap@gmail.com

                                                   Web-site: www.tscs.in

Patrons

Smt. Haripriya Rangarajan		Mr. Naresh Rathi
Dr. K. Rajyalakshmi		Mr. Shivcharan Agarwal
Mr. Pradeep Uppala		Mr. G. Dhasarath

Board Members

President

Mr Chandrakant Agarwal

Vice President

Mr.Manoj Rupani &  Mrs.K.Ratnavali

Secretary                        Jt.Secretary

Dr.Suman Jain                Mr.Aleem Baig

Treasurer                         Jt.Treasurer

Mr. J Rajeshwar           Mrs. Rama Vuppala

Executive Members

Dr. Dandamudi Ramana

Mohammed Amin

Mr.Surender Agarwal

                                      

                                                                                                                

                              

                              

About Society

The Thalassemia and Sickle Cell Society was founded in 1998 by patients, parents, doctors, and well wishers and is the only society in Andhra Pradesh committed for the care and control of Thalassemia.

Mission

To promote the provision of appropriate treatment and to achieve a good quality of life for every patient with Haemoglobinopathies, and to encourage prevention policies with the aim of reducing the number of newly affected births

Aims & Objectives

F  To campaign for safe blood transfusion.

F  To counsel & educate thalassaemics, their families & doctors about the management of thalassaemia.

F  To create better facilities for transfusion, treatment & prevention.

F  To collect funds, equipments, medicines for needy children and families.

F  To create awareness among public & promote detection / prevention programmes for the total control of thalassaemia with the help of Government and other Institutions.

F  Arranging blood donation camps for thalassemics, with the help of other voluntary organizations and the media.

F  To provide medicines and equipment required for Thalassemic patients at subsidized rates throughout the State.

F  To carry out screening programmes, besides giving Genetic counseling at District centers across the state.

F  Seek assistance from philanthropic persons / organizations for adopting a Thalassemic child for his / her treatment, and for promoting our activities.

F  Promote education, employment and the integration of Thalassaemia patients into society, improving quality of life and the chance to fulfill their dreams and objectives, including marriage and having children of their own

F  To promote collaboration with State & Central Governments, World Health Organization (WHO), Thalassaemia International Federation, Hospitals  and  other NGO’s.

What is Thalassemia?

HAEMOGLOBINOPTHIES (Thalassemic Syndromes) are a group of inherited disorders in which the production of normal haemoglobin is partly or completely suppressed because of a defective synthesis of one or more of its component globin chains.

Basic Inheritance

¨       Thalassaemia is an inherited disorder of the red blood cells. These cells contain the haemoglobin molecule, which is responsible for binding oxygen from the air we breathe and carrying it to the tissues where energy is released.

¨       In Thalassaemia one of the components of the haemoglobin molecule is inadequately produced or not produced at all. If there is lack of α- chain production then the result is known as α-thalassemia. If the component that is lacking is the β-chain, then the resulting condition is β- thalassemia.

¨       The reason for the inadequate or non- production of these components is a change in the genetic code (mutation), in that part of the DNA, which is the template for the production of the protein. The mutation or altered gene cannot initiate the process, which hamper the production of necessary amount of protein.

¨       Genes, sections of DNA responsible for a protein, are carried on chromosomes and each individual has a pair: one chromosome from each parent.

¨       A mutation may exist on one chromosome of a pair, but not on the other. The protein produced by the one, “healthy”, chromosome is enough to keep the individual well, even though his/her red cells are smaller than normal. Such an individual is known as a carrier (or heterozygote) who can only be detected by special blood tests. A carrier may give his/her offspring either the healthy   chromosome or the one bearing the mutation.

¨       Severe thalassaemia (Thalassemia Major) will result if a child inherits the abnormal (mutation bearing) chromosome from both parents. In other words both parents must be carriers if a major Thalassaemia disorder is present in the child. This situation is known as homozygous thalassaemia.

Types of Thalassaemia

There are three types of thalassemia that are of global importance:

1. Alpha (α) - thalassaemia
2. Beta   (β) - thalassaemia
3. Haemoglobin E (HbE)  thalassaemia

β-thalassaemia:

β thalassaemia results from inadequate or lack of production of β-Chains. Homozygous β-thalassaemia has two forms: major, in whom the patient can survive only with regular transfusions of blood and intermedia, in which the patient can survive with occasional or even with no transfusions at all.

This form of Thalassaemia is the most important and constitutes a major public health problem in many parts of the world, because of the high frequency of carriers and the demanding treatment that must be followed

Epidemiology of Thalassaemia

It is estimated that there are 80-90 million carriers of Thalassaemia worldwide and 60-70000 births of affected children every year. Most of these die in early life, often without a diagnosis or because of inadequate treatment

Carriers are found in all parts of the world:

l  People from the North Mediterranean (South Europe) coast are 1-19% carriers.

l  People of Arab origin are over 3% carriers. In Central Asia 4-10% and in South East Asia, the Indian subcontinent and China 1-40% carriers (the very high rates in this part of the world are due to HbE).

l  In the Americas, North Europe, Australia and South Africa the local population has very low carrier rates but Thalassaemia is still present because of the significant immigration from high prevalence areas.

In India:

It is estimated that there are 35 million carries of Thalassemia i.e. 1 in 25. Around 10 – 15,000 babies with Haemoglobinopathies are born in India every year. Few of the ethnic group like Sindhis, Gujarathis, Punjabis, Jains, Marwadis, etc is high risk communities for this disease

Signs and Symptoms

The child is normal at birth but between the age of 6 weeks and 2 years, parent or doctors can identify important signs like pale skin, restlessness, poor appetite and a mass (spleen) felt on the left side of the tummy.

Diagnosis

Patient subjected to special tests like Hb Electrophoresis (estimation of HbA2 and Hb F levels) and complete blood picture (CBP) will clinch the diagnosis. 

Treatment

ü  Saline washed packed red cell blood transfusions every 3 – 4 weeks to maintain hemoglobin above 10 gm/dl.

ü  Iron Chelation therapy after 20 blood transfusions.

ü  Periodic medical checkup for serum ferritin level, liver function test, hepatitis B and C, HIV screening, renal function test, serum calcium & phosphorus test, dental checkup, cardiac checkup and endocrine function test.

ü  Bone marrow transplantation can cure the disease but in only 30% of siblings can Histocompatibility Linked Antigen (HLA) be matched. Besides it is very expensive and there are chances of rejection.

Cost of the Treatment

	Particulars	Cost
1	Blood transfusion	Free*
2	Leucodepletion filters (per transfusion)	Rs. 1,000/-
3	Iron Chelation
a)	Desferal (Parental Chelation
	i.  Infusion pump (once in life time)	Rs. 14,040/-
	ii. Drugs (life long)	Rs. 8,000 to 10,000 / month
b)	Kelfer (Oral Chelation)
	i. Drugs (life long)	Rs. 2,000 to 3,000 / month
c)	Tab. Asunra 400mg & 100mg	Rs.2300 to 9000 per month
4	Other drugs and disposables (life long)	Rs. 500 to 1,000 / month
5	Periodic medical check-up (life long)	Rs. 2,000 / year
6	Bone marrow transplantation (permanent cure)	Rs. 10,00,000/-

           

Prevention

It is said that “Prevention is Better than Cure”. It is suggested the following prevention policies can be adopted to reduce affected births by doing simple blood test (HbA2 estimation) and save resources so that the best possible treatment can available for already existing cases .

1. Epidemiological studies: To determine local carrier rates, birth rates etc.
2. Health Education:  All about Thalassaemia, the risk of having an affected child. Health Education is directed school pupils, the public and   health providers.
3. Population Screening: To identify carriers, directed usually to the relatives of patients and couples before marriage.
4. Genetic Counseling:   For couples at risk.
5. Prenatal diagnosis: Carried out at 10-12 weeks of pregnancy and is usually followed by the offer of termination of pregnancy of affected fetuses.
6. Pre-implantation diagnosis: The use of in-vitro fertlisation (IVF) to either select healthy (non-carrier) ova for fertilization or after fertilization to select disease-free early embryos (blastocysts) for implantation in the uterus

What is Sickle Cell Anemia?

The most common Haemoglobin variant in the world is Haemoglobin S (HbS). This causes a serious condition called Sickle cell Disease (SCD), which is characterized by anaemia, and episodes of severe pain and blocking of blood vessels. It may also be co-inherited with β-thalassaemia, causing a condition know as Sickle Cell Thalassaemia. The severity of this condition mostly depends on how much β-globin is being produced, Β-globin abrormality is caused by substitution of valine for glutamic acid in position 6 in the β chain (GAG to GTG) Due to this, Sickle shaped RBC’s are generated. Deoxygenated hemoglobin S polymerizes and distorts the shape of red blood cells. Most patients with sickle cell disease have painful vaso-occlusive crises. Which is dependent on the type of thalassaemia mutation. If no β-chains are produced then the condition is identical to SCD. The more β-globin the less severe the condition.

Parameters for detection:

Clinical : Anemia.

Hematological : MCV, MCH, and NESTROFT TEST.

Laboratory :

Hb%, HbF, Hb variant analysis, electrophoresis.

Treatment:

If severe anemia, blood transfusion and symptomatic management of pain.

Activities: Thalassemia & Sickle Cell Society

•          Care of Thalassemic and Sickle cell anemia patients.

•          500 to 600 children are getting regular blood transfusion.

•          Patients on chelating agents.

                     a) Oral chelating agents :  478

                     b) Oral + Injection Desferal       :      6

                     d) Tab.Asunra                            :  147

                     e) Kelfer                                     :  325              

Nearly 88 blood donation camps organized by our society with the help of various organizations.

•          Screening for school children – 1000 (10 Schools)

•          Continued Medical Education (CME) programs for professionals conducted by society.

•          In 1999 at Green Park Hotel “Pediatric Hematology  Update”

•          In 2002 at Global Hospital    “Update on Thalassemia”

•          In 2006 at National Institute for Mentally Handicapped “Role of Prenatal Diagnosis in Preventive Pediatrics “ on 10^th Sept 2006

•          CME’s / Updates for every 2 months for parents and patients.

•          Screening for Thalassemia carrier by estimating HbA2 level by HPLC at Institute Genetics since 2001.

Facilities

•          Facilities for blood transfusion.

•          Pre-transfusion Hemoglobin (Hb).

•          Free consultation and genetic counseling.

•          Growth monitoring.

•          Hepatitis-B vaccination at subsidized rates.

•          Serum ferritin level at subsidized rates.

•          Periodic health check-up at subsidized rates.

•          A2 level screening for Thalassemic traits with Bio-Rad Column/HPLC.

•          Chelation therapy at a concessional price.

•          Psycho-social support for parents and patients.

•          Parent group meeting at society once in two months for group discussion and mutual support.

                                                        Statistics

In the society 1623 patients are registered since the inception in 1998 the details of the patients are given in following tables.

D e m o g r a p h i c   D e t a i l s

Dist		Total		Dist	Total
ADILABAD	154			MEDAK	44
ANANTHAPUR		14		NALGONDA	93
CUDDUPAH		19		NELLORE	6
CHITOOR		9		NIZAMABAD	37
EAST GODAVARI		42		PRAKASHAM	31
GUNTUR		52		RANGA REDDY	64
HYDERABAD		460		VISHAKHAPATNAM	23
KARIMNAGAR		93		WARANGAL	88
KAKINADA		6		WEST GODAVARI	49
KHAMMAM		108		KARNATAKA	19
MAHABOOBNAGAR		53		MAHARASTRA	11
KRISHNA		32		ONGOLE	7
KURNOOL		42		VIJAYAWADA	8
VIJAYANAGARAM		7		NOT AVILABLE	43
SRIKAKULAM		9		GRAND TOTAL	1623

Classifications of Disease
Type of Disease	No.
Thalassemia Major	1222
Thalassemia Intermedia	32
Sickle Thalassemia	61
Sickle Cell Anemia	252
E – Thalassemia	10
Others	35
Not Available	11
Grand Total	1623

Economic Status
Monthly Income	No.
<1000	96
1000 – 2000	594
2001 – 5000	525
5001 – 8000	115
8000 – 10000	83
10001-15000	35
>15000	40
Total	1488
Data Not Available	135
Net	1623

                  D e t a i l s   o f   t h e   P a t i e n t ’ s   s t a t u s

                     

Age  and  Sex wise Details
Age	Sex
	Female	Male	Total
00 - 02	216	283	499
03 – 04	112	155	267
05 – 06	73	120	193
07 – 08	66	94	160
09 – 10	52	70	122
11 – 15	52	76	128
16 – 20	42	67	109
21 - 25	21	38	59
> 25	11	14	25
Total	645	917	1562
Not available			61
Total Patients			1623

                                                                       

Blood Group Details
Blood Group	No.
A –ve	12
A +ve	314
B –ve	31
B +ve	464
AB +ve AB-ve	97 5
O –ve	28
O +ve	635
Oh (Bombay Phenotype)	1
Total	1587
Not available	36
Total Patients	1623

  

Consanguinity Details
Consanguinity	No.	%
Present	922	56.80
Not present	701	43.20
Total	1623	100

Patients from Various Religions (Caste) in Andhra Pradesh

HINDU
Caste	Total		Caste	Total
Arya Katika	4		Medari	4
Balija	10		Mrugaraj	2
Banjara	56		Mocha	5
Bestha	10		Mudiraj	20
Boi – Oby	5		Munura	7
Brahmin	35		Naidu	13
Chakali	2		Naya Brahmin	11
Chowdary	6		Netakani	32
Devanga	4		Oddi	2
Dhobi	10		Padmasali	14
Gangaputhra	4		Parna	4
Gavara	2		Rajeka	6
Gold Smith	2		Rajput	3
Goud	38		Ranga Raju	1
Goulia	2		Reddy	19
Gujrathi	1		Sindhi	5
Harijana	8		Sista Karanam	1
Jalary	3		Settibalija	22
Jangam	2		Soni (MP)	1
Kamma	21		Telaga	2
Kammari	19		Telagalu	8
Kappu	71		Uppar	1
Katika	6		Uppari	1
Kayalta Bengali)	9		Vaddera	16
Kshtriya	6		Vadabalija	5
Kummari	11		Valmiki	2
Lambadi	90		Velama	16
Lodi	4		Virabadra	2
Madiga	150		Visya	2
Maher	6		Vodiraju	8
Mala	141		Vyathakar	2
Manepu	5		Weapers	2
Marwadi	17		Yadau (Goua)	3
Manapallam	2		Yadavulu	1
Meduru	3		Yadav	22
Bengali	3		Not Available	130
Buddhist	3		Total	1164

CHRISTIAN
Caste	Total
Kummari	2
Lambadi	30
Madiga	30
Mala	16
Protestant	7
Catholics	2
Not Available	12
Total	99

SIKH
Sikh	3
Total	3

MUSLIM
Caste	Total
Arabs	2
Arif	3
Biag	4
Bin	1
Dawoodi	1
Ismail	2
Khan	8
Menon	2
Mohamad	89
Patan	5
Sayed	23
Shaik	98
Shia	1
Sunni	26
Syed	8
Share	1
Khojja	2
Not Available	81
Total	357

Grand Total

1623

FUNDING OPTIONS FOR DONORS

In the following note we have provided some of the ways in which support can be extended to provide financial viability for the efforts made by the dedicated team of society.

O P T I O N  - 1

Support a Child Programme

      T h a l a s s e m i a:-

      Transfusion facility (blood provided free of charge from Red-cross and other similar       licensed Blood Banks)

     Chelation & Transfusion:

Blood Transfusion (per Unit)	Rs.   1,200/- (per month)
Infusion Pump	Rs. 17,000/- (one time)
Desferal   Tab.Asunra	Rs.   8,000 to 10,000/- (per month) Rs.2300 to 9000 per month
Kelfer	Rs.   2,000 to 4,000/- (per month)
Bone marrow transplantation	Rs. 10,,00,000/-

O P T I O N  - 2

Committed and persistent efforts are required to progressively diminish the burden of inherited and preventable Blood Disorders.

Awareness programmes aimed at propagating relevant and appropriate information are planned at regular intervals. Such event requires funds for their execution.

¨          Material for community and general awareness

a.     Printed bulletins, posters, etc;        Rs. 30,000/-

b.    Reach-out programmes                  Rs. 20,000/-

c.     Short films                                                 Rs. 60,000/- (one Film)

¨          Continuing Medical/ Professional Education Programmes directed towards Pediatricians, Obstetricians, and Pathologist & Allied Groups. 

Three sessions a year – Rs 1,50,0000/-.

To include   150   to    200 participants for a    One day schedule requires an estimated Rs 50,000/-.

The Thalassemia & Sickle Cell Society depends on the benevolence of it’s donors in order to provide the wherewithal essential to care well for patients afflicted by thalassemia and sickle cell.

O P T I O N - 3

The third way of supporting the society could be to give us a Corpus Fund which supports the activities and administration for running the society services. With the help of voluntary contributions of private and concerned donors society is able to run its activities.

The following table gives an idea of our present society’s expenditure position to run the activities.

Thalassemia & Sickle Cell Society  & Blood Bank
Expenditures per month
EXPENDITURES	Rupees
Salaries	300000
House Keeping	50000
Telephone Bill	8000
G.J. Multiclave	6000
Blood Donation Camp Rs.5000/- per camp	75000
Medicines	30000
Awareness Camp	30000
Electricity Charges	35000
Consumables	200000
Printing and Stationery	10000
Totals	734000

The success of a programme of this nature depends to a large extent on the team supported by adequate funding.

Hope is what drives everyone in life and it is the hub of the moving forces everywhere. It is re-enforced to us every time we look in to the children affected by Thalassemia. Their immense strength to endure their today’s so that their tomorrows would bring them a ray of hope. We with all our heart hope that your support and contribution in this war against the genetic disease will help to prevent it.