Maldives is an island nation in the Indian ocean with a population of 328,536 (2012). In this

small nation thalassemia has become one of the major health concerns. Thalassemia is an inherited

blood disorder which weakens and destroys red blood cells which results in less hemoglobin or abnormal

hemoglobin made.So people with thalassemia suffers from mild or severe anemia .Maldives has the

highest concentration of carriers ( 18%

to 20% ) in the world . This indicates that every one in

five person from the population is a carrier. If both the parents are carriers they have a 25%

chance of getting a baby who have thalassemia.In Maldives every 1 in 120 births is of a baby

having thalassemia.This frequent occurrence in such a small population is of paramount

importance to be considered as an issue .

Total registered patients in NTC 772

Total No of patients living 573

Total patients belonging to Islands 422

Thalassemia in Maldives-Problem

Inheritance of Alpha thalassemia Inheritance of Beta thalassemia

http://www.nhlbi.nih.gov/health//dci/Diseases/Thalassemia/Thalassemia_Causes.html

Thalassemia is a very common genetic disorder. Worldwide, several million people have thalassemia

disorders and about 300 million people carry the genetic trait for thalassemia.-Ellis Neufeld, MD, PhD,

Fig.1

Fig .2

SOLUTION

*Diagnosis

The no of genetic screenings done has increased compared to the past in Maldives. One of the

main reason to this is that certain laws implemented strictly requires the couple getting married

of the service in islands, it is not mandatory for those getting married in islands to have done the

test before marriage.Every one out of five screened for Thalassemia is a carrier and one out

every 30 weddings is a union among 2 carriers.( statistics obtained

fromhttp://beautifulmaldives.wordpress.com/2009/05/11/highest-number-of-thalassemia-

172

40 40 39 38 35 34 29

20 20 19 15 15 12 12 11 11 6 5

0 0

20

40

60

80

100

120

140

160

180

200

K N HDh R L Ha Th Sh S Lh F Ga GDh Gn B Adh Aa Dh M V

No of thalassemia patients in atolls

1 6

477

1 75

3 1 9

Patient Diagnosis type ( living)

Data from 1992-2013(3rd march)

(NTC)

Data obtained on 3rd March 2013 from NTC

Fig 3

Fig 4

patients-registered-in-2008-ntc/) Screening can be done free of charge at the National

Thalassemia society. Also it can be done at Society for Health Education after paying.

2232 2002

2509

3189

3722

2496

1100

0

500

1000

1500

2000

2500

3000

3500

4000

2005 2006 2007 2008 2009 2010 2011

Thalassemia screenings done at NTC

Prenatal and postnatal diagnosis of

thalassemia enables the parents to make

an informed decision whether to bring a

child having thalassemia into this world

or do abortion.some of the tests done

include chorionic villus sampling,

amniocentesis, and percutaneous

umbilical blood sampling. All these tests

have the chances of causing miscarriages .

This procedure is yet to be available in

Maldives. So couple at risk or those

wanting to do the procedure has to travel

abroad.

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Fig 5

Fig 6

http://www.slideshare.net/Thalassaemia_Intl_Fed/overview-of-the-national-thalassaemia-programme-in-the-maldives#btnNext

Treatment for thalassemia varies from one patient to another depending on the type and severity.

The standard treatments used for thalassemia majors include blood transfusions, iron chelation

therapy.

Regular blood transfusions are done to provide normal red blood cells from donor to patient.

The process takes up to 1 to 4 hours. Since red blood cells has a life span of 120 days, blood

transfusions should be done within every 2 to 4 weeks to keep the red blood cells at a normal

level.

In Maldives, blood transfusions can be done in National thalassemia center (male),Indira

Gandhi Memorial Hospital(male), six regional hospitals,69 atoll hospitals and health centers.

TREATMENT LOCATION

patients taking regular treatment from NTC

patients taking treatment from different islands

3220 3224 3457 3536

4144 4301 4327

4055 4198

4648

1549

2001 2002 2003 2004 2005 2006 2007 2008 2009 2010 2011

No of blood transfusions given in NTC anually

TILL

AP

RIL

292

253

Data obtained on 3rd March 2013 from NTC

Management of thalassemia

Fig 7

Fig 8

http://www.slideshare.net/Thalassaemia_Intl_Fed/overview-of-the-national-thalassaemia-programme-in-the-maldives#btnNext

Iron chelation therapy is used to remove excess iron from the body, which tends to build up

when a person receivers regular blood transfusions.Too much iron in the body can lead to

Cirrhosis ,Liver cancer,Cardiac arrhythmias,Diabetes,Alzheimer's disease etc.Iron chelation

drugs currently used are Deferoxamine injections,Deferiprone capsule and capsule.

(http://www.nejm.org/doi/full/10.1056/nejm199409013310902)

http://www.nejm.org/doi/full/10.1056/nejm199409013310902

CONCLUSIONS

The early use of deferoxamine in an amount proportional to the

transfusional iron load reduces the body iron burden and helps protect against

diabetes mellitus, cardiac disease, and early death in patients with thalassemia

major.

CONCLUSIONS

Oral deferiprone induces sustained decreases in

body iron to concentrations compatible with the

avoidance of complications from iron overload. The risk of

agranulocytosis associated with deferiprone may restrict

its administration to patients who are unable or unwilling to use

deferoxamine.

Fig 9

Fig 10

Curative methods

Born marrow transplant is a procedure where the diseased bornmarrow of the patient is

eliminated and healthy born marrow from a donor is transplanted into the patient. After

transplantation, if procedure was successful the new bone marrow migrates to the cavities of

the large bones, engrafts and begins producing normal blood cells. Bone marrow transplantation can be performed using one's own bone marrow, called an autologous bone

marrow transplant, or by using another person's bone marrow, called an allogeneic bone

marrow transplant. Approximately 25% of thalassemic patients could have a sibling donor

whose bone marrow is a perfect match.For those patients older than 16 years at the time of

transplant, the probabilities of survival and of event-free survival are 78 and 74%, respectively.

In Maldives this procedure is not available , hence patients wanting to undergo this treatment

has to travel abroad. So far 32 maldivians has undergone bone marrow transplant. Majority of

the patients has done the procedure in India. Several patients underwent the surgery ,in Italy

under an agreement between Maldives government and Italy.

born marrow transplant center total no of BMT done

successful BMT

rejection death

Pesaro (ITALY) 15 12 1 2

CMC Vellore ( INDIA) (1997-2009) 19 11 0 8

Bangalore (INDIA) 1 1 0 0

Bangkok (THAILAND) ( 1992 & 1996)

2 2 0 0

IMPLICATIONS

*Economic

Thalassemia, is a condition in which the patient has to undergo regular blood transfusions, iron

chelation therapy and they are hospitalized frequently which creates a financial burden in the

long run.In Maldives, it is estimated that the government spends roughly $5000 on every

thalassemic patient above the age of 12.This is a great deal of potential financial support which

could be invested to improve the quality of other aspects of the healthcare system in Maldives

(eg- Regional hospitals). The only cure yet to have yielded positive results is the very risky and

costly Born marrow transplantation. This procedure is listed as one of the most expensive

medical procedures in the USA with an estimated value of $676,800.In India ,which is the

country most Maldivians underwent the born marrow transplant is estimated to have cost Rs 7-10

lakh for the surgery ($12879.3668- $18399.0954)

Data obtained on 3rd March 2013 from NTC

Fig 11

Due to the nature of the disorder, thalassemia patients has to be very health conscious and spend

a lot of time on their treatments. Such chronic diseases are known to cause psychosocial burden

on the patients and their family. A study done in India to study the psychosocial life aspects of

Indian adolescents suffering from thalassemia showed that ,70% patients expressed that

thalassemia had an adverse affect on their education, since they had to take leave from school for

transfusions and other related problems. Over 2/3 rd related physical weakness to thalassemia

which limited their indulgence in sports. No problems were noted regarding family interactions

and 80% reported the disease had no impact on their social life.

RISK AND BENEFITS

The currently available only cure for thalassemia which is born marrow transplantation , though

it is risky and expensive , it can cure thalassemia major patients. After the surgery during the

recovery period which is 6 to 12 months , there is risk of infections and other complications so

frequent checkups with the doctor are done. Due to this reason many Maldivians tend to migrate

to the country temporarily for ease. If the born marrow transplant is successful the individual

would be able to play an important role in the society. Major risks associated with born marrow

transplant include susceptibility to infection, anemia, graft failure, respiratory distress, and

excess fluid, which can lead to pneumonia and liver dysfunction. Also in cases of a mismatch

between donor and recipient an immune response is triggered which could cause graft-versus-

host disease (GVHD),a condition in which graft cells attack the host cells.

ALTERNATIVE SOLUTIONS

Gene therapy for beta thalassemia

Gene therapy holds the promise of fixing one's own bone marrow cells by transferring the

normal -globin or -globin gene into hematopoietic stem cells (HSCs) to permanently produce

normal red blood cells. Various types of vectors are been considered for gene transfer into stem

cells whilst retroviruses are leading candidates. In 2007 , the first successful gene therapy clinical

trial for thalassemia was done on a 18 year old and three years after, by 2010 he was found to be

only mildly anemic and didnt require blood transfusions, thus proving to be successful.

Researchers are working with hope that in a near future they would be able to make the

procedure safer ,along with less complications so it could be available for all.

*Social

For the first time, a patient with severe beta-thalassemia is living without the need for

transfusions over a sustained period of time. These results are not only world, but

also represents a significant step forward for the field of autologous stem cell therapy

as an emerging therapeutic modality.- Marina Cavazzana-Calvo, M.D.

EVALUATION

The statistical data of thalassemia in Maldives was collected from my visit to the National

Thalassemia Society, so the data ia as accurate as it is in the records kept in National

Thalassemia Society. Some of the statistics (fig5&7) , how ever was extracted from the internet,

one of them been a presentation done by Dr.Farzana Khatoon and the presentation was uploaded

into the account of Thalassemia International Federation. On my visit to NTS I did meet with

Dr.Farzana Khatoon and I came to know that , she has worked in NTS for 16 years. So with such

immense experience, her presenting an erroneous statistics and that been uploaded by an

international federation seems absurd. Also all the statistics I obtained from NTS were mutual to

hers, hence I decided it was valid.

I found the website http://www.nhlbi.nih.gov/ to be very helpful on several occasions. It gave

detailed and comprehensive information on thalassemia . Moreover, several external links and

clinical trials related to the topic were provided, which proved to be time saving and very

informative.

BIBILOGRAPHY

1)Maldivess Population

en.wikipedia.org/wiki/Maldives

2) thalassemia information

http://www.alohamaldives.com/Healthcare/Thalassemia.html

http://www.mhsc.com.mv/en/?p=307

http://thalassaemia.org.mv/

http://www.childrenshospital.org/az/Site1707/mainpageS1707P0.html

3)Article by Dr.Faisal Saeed, posted on Abdulla Waheedblog , Status of Thalassmia in Maldives

http://abdullahwaheedsblog.blogspot.com/2009/02/status-of-thalassemia-in-maldives.html

4)Article by Beautiful Maldives

http://beautifulmaldives.wordpress.com/2009/05/11/highest-number-of-thalassemia-patients-registered-in-2008-ntc

5) Article by Dr.Mercola, The selfish reason to donate your blood, September 01, 2012

http://articles.mercola.com/sites/articles/archive/2012/09/01/too-much-iron.aspx

6) BONE MARROW TRANSPLANTATION IN THALASSEMIA

(http://www.annualreviews.org/doi/abs/10.1146/annurev.med.46.1.319)

Claudio Giardini, M.D., Mariella Galimberti, M.D., and Guido Lucarelli, M.D.

Annual Review of Medicine

Vol. 46: 319-330 (Volume publication date February 1995)

DOI: 10.1146/annurev.med.46.1.319

7) Minivan news , Maldivian bone-marrow transplant patient recovering in Italy ,March 14th 2011

http://minivannews.com/news-in-brief/maldivian-bone-marrow-transplant-patient-recovering-in-italy-17101

8) by Dr. Pietro Sodani ,Thalassemia FAQ

http://www.curethalassemia.org/faq

9)Atlantic Health System, Family Planning Options for Beta-Thalassemia Carriers

https://atlantichealth.dnadirect.com/grc/patient-site/beta-thalassemia-carrier-screening/family-planning-options-for-beta-

thalassemia-carriers.html?x0yo9r8P5O4iTboEmiynk5_

10)Article on thalassemia by : Yi-Bin Chen, MD, 2/7/2012, reviewed by David Zieve, MD

http://www.nlm.nih.gov/medlineplus/ency/article/000587.htm

11)Investopedia,Top 10 most expensive medical procedures, March 18, 2010

http://www.investopedia.com/financial-edge/0310/top-10-most-expensive-medical-procedures.aspx#axzz2N3foU2se

12)The times of India, Sahara Hospital to have bone marrow transplant unit http://articles.timesofindia.indiatimes.com/2011-09-25/lucknow/30200513_1_bone-marrow-transplant-donor

13)What to expect after Bornmarrow

http://www.nhlbi.nih.gov/health/health-topics/topics/bmsct/after.html

14)Encylopedia Britannica ,Born marrow transplant

http://www.britannica.com/EBchecked/topic/72962/bone-marrow-transplant

15) A Phase I/II Clinical Trial of -Globin Gene Therapy for -Thalassemia

http://onlinelibrary.wiley.com/doi/10.1196/annals.1345.007/abstract

1. ARTHUR BANK ,RONALD DORAZIO , PHILIPPE LEBOULCH1

Article first published online: 6 JAN 2006

DOI: 10.1196/annals.1345.007 , Volume 1054, Cooley's Anemia: Eighth Symposium pages 308316, November 2005

16)Helen Briggs, Gene therapy for blood disorder a success, 15 September 2010

http://www.bbc.co.uk/news/health-11313273

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http://www.bulletins-electroniques.com/actualites/64617.htm

18)Jeffrey S.Dungan, MD , Sherman Elias, MD , Prenatal Diagnostic Testing , November 2008

http://www.merckmanuals.com/home/womens_health_issues/genetic_disorders_detection/prenatal_diagnostic_testing.html

19) UCSF Medical Center, Matched Unrelated Donor Transplantation

http://www.ucsfhealth.org/education/matched_unrelated_donor_transplantation/index.html

20) Dr Farzana Khatoon,Maldives-current situation in control strategies and health systems in Asia,upload by Thalassemia

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http://www.slideshare.net/Thalassaemia_Intl_Fed/overview-of-the-national-thalassaemia-programme-in-the-

maldives#btnNext

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http://medind.nic.in/icb/t06/i10/icbt06i10p877.pdf

Indian Journal of Pediatrics, Volume 73October, 2006

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