ThalassemiaThalassemia

Demographics & StatisticsDemographics & Statistics

Year 2008 Year 2008

Genetic Multi-factorial Environment

Blood group,

Thalassemia,

Down syndrome

Diabetes,

Hypertension,

Cancer

Tuberculosis,

Malaria,

Typhoid fever

DISEASE

Genetic blood diseases in Arab World

1. Thalassemia

2. Sickle cell

3. G6PD deficiency

Carrier Disease

Children born with Thalassemia Disease:

World: 100,000/year 12 children in one-hour

India: 10,000/year 1 child every hour

UAE: 50/year 1 child per week

New cases with Thalassemia disease in UAE

59 54 5364

56

0

20

40

60

80

1996

1997

1998

1999

2000

Thalassemia patients (n=702) Distribution on Nationality (Dec 2001)

UAE47%

GCC14%

Arab5% Iranian

10%

Asian22%

Others2%

75

204 225266

325382 388

452497

550613

670 702

0100

200300400

500600

700800

1989

1990

1991

1992

1993

1994

1995

1996

1997

1998

1999

2000

2001

No. of Patients

Cost of Treatment (every year):

One patient: AED 40,000

700 patients: AED 16 million

New cases over next 40 yrs: AED 32 billion

Ideal outcome following introduction of screening/education programme for

Thalassaemia Major

CANADA(Am J Hum Genet 1996 Oct;59(4):793-8)

1972-1992

Screened 25,274 High school Students

693 were carriers

Virtually all the carriers identified remembered their status,

had their partner tested

took up the options to prevent

EARLIER STUDY

When to Intervene ?

College/ University Vs Pre-Marital

Screening Strategies for the UAE The AIM of a screening program must be to

offer carrier testing to every member of the population (or to one member of each couple) ideally before they have children.

The PURPOSE is to identify carrier couples

and inform them of their risk and options for avoiding it.

The PRIME TARGETS of screening are those

with -thal, -thal and Hb S, C, D, E in a well-selected target population.

Cause & EffectCause & Effect 

The relatively high prevalence of carriers in the UAE may be attributed to:

• Consanguineous marriagesConsanguineous marriages• Founder effectFounder effect• Lack of information and awareness Lack of information and awareness programs programs • Absence of thalassemia screening Absence of thalassemia screening programsprograms• Unavailability of dedicated Unavailability of dedicated laboratorieslaboratories• Missed diagnosis of carrier state Missed diagnosis of carrier state

LIFE EXPECTANCYLIFE EXPECTANCY

Male:Male:

Female:Female:72.73 y72.73 y

77.87 y77.87 y

ETHNIC GROUPSETHNIC GROUPS

Emirati 19%Emirati 19% South Asian South Asian 50%50%

Other Arab & Other Arab & Iranian 23%Iranian 23%

Westerners Westerners and East and East Asians 8%Asians 8%

July 2005 est.July 2005 est.

Population:Population: 4.04 million4.04 million

Age 0-14 years: Age 0-14 years: 25.3% 25.3%

15-64 years: 15-64 years: 71.1% 71.1%

65 years and 65 years and over:over:

3.6% 3.6%

Fertility rate:Fertility rate: 2.94 children 2.94 children born/womanborn/woman

0

20

40

60

80

100

120

1972

1973

1974

1975

1976

1977

1978

1979

1980

1981

1982

1983

1984

1985

1986

1987

1988

1989

1990

1991

1992

1993

1994

Cyprus

Greece

Italy

UK

BT

M b

irth

s %

Pre Natal Diagnosis PROGRAMS

The graph demonstrates how thalassemic births were prevented through the implementation of PND programs in various countries