A look at what might occur across the globe in relation to hameoglobinopathies as a result of conflict and the resulting migration

1. The Cyprus
Institute of
Neurology
and Genetics
Potential Impact of Conflict on
Haemoglobinopathies
Carsten W. Lederer
The Cyprus Institute of Neurology and Genetics
The Cyprus School of Molecular Medicine
Lederer@cing.ac.cy
https://en.wikipedia.org/wiki/Damascus
UNESCO Headquarters
Paris, France
30 – 31st May 2016
Human Variome Project
Consortium
6th Biennial Meeting
GG2020 Fringe Meeting

2. The Cyprus
Institute of
Neurology
and Genetics
Haemoglobinopathy
Epidemiology and Trends
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3. The Cyprus
Institute of
Neurology
and Genetics
Epidemiology 3
WHO: Eliminating malaria: learning from the past, looking ahead
Modell and Darlison 2008 PMID18568278
 High prevalence in traditional malaria regions
 Even after malaria eradication, the inherited disease stays behind
 Annual birth of 330,000 babies with major haemoglobinopathies
 275,000 SCD (Sub-Saharan Africa: 50 – 80% die before age 5)
 55,000 Thal (Western Pacific, Eastern Mediterranean, South-East Asia)
 Globally >20 Million SCD and >100,000 Thal patients

4. The Cyprus
Institute of
Neurology
and Genetics
Epidemiologic & Medical Trends 4
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 Increasing & global migration from source to sink countries
 New health challenges for high-income countries, e.g. USA & UK with SCD
 Increasing life expectancy of patients, e.g. Thal survival rates in Italy
Piel et al. 2014 PMID 24748392
Borgna-Pignatti et al. 2004 PMID15477202

5. The Cyprus
Institute of
Neurology
and Genetics
Cost in High-income Economies
 Concrete cost per patient
 Thal, Northern Israel (2014) $63,660/a $1,971,380/lifetime
 Thal, UK (1999) $20,750/a $1,245,030/lifetime
 Thal, UK (2016) $14,404/a $ 720,201/lifetime
 SCD, USA (2009) $16,000/a $ 460,151/lifetime
annually $1.1 billion for approx. 70,000 US SCD patients
 Increasing cost to health systems over time, e.g.
USA with SCD
in million $
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http://www.grandviewresearch.com/industry-analysis/hemoglobinopathies-market
Koren et al. 2014 PMID24678389
Karnon et al. 1999 PMID10671989
Weidlich et al. 2016 PMID27041389
Kauf et al. 2009 PMID19358302

6. The Cyprus
Institute of
Neurology
and Genetics
Impact of Conflicts on
Haemoglobinopathies – The
Example of Syria
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7. The Cyprus
Institute of
Neurology
and Genetics
Situation in Syria
 Thalassaemia baseline
 8000 Thal patients (2009), estimated increase 10%/a
 Estimated up to 5% Thal carrier rate
 Without prevention programme
 0.25% of pregnancies at risk
 0.0625% of newborns thal patients
 Impact of conflict
 654 medical personnel killed (9/2015)
 Exodus of healthcare workers
 Health facilities only partially operative or closed down
 57% of 113 public hospitals
 51% of 1783 health centres
 Aggravating factors
 Burden of IDPs on urban health centres
 60% drop in local drug production
 50% increase in drug prices
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http://www.emro.who.int/images/stories/WHO_SitRep_October2015_reduced.pdf?ua=1
Al-Kir2009PMID20001629
HamamyandAl-Allawi2013PMID23224852
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8. The Cyprus
Institute of
Neurology
and Genetics
Situation in Syria
 Whole of Syria – Health Sector/Cluster People in Need
 Urban areas as main foci
http://www.who.int/hac/crises/syr/sitreps/syria_health_sector_cluster_news_september2015.pdf?ua=1

9. The Cyprus
Institute of
Neurology
and Genetics
Situation in Syria
 Whole of Syria – Health Sector/Cluster Severity Ranking
 Impacted by local accessibility and available health professionals
http://www.who.int/hac/crises/syr/sitreps/syria_health_sector_cluster_news_september2015.pdf?ua=1

10. The Cyprus
Institute of
Neurology
and Genetics
Impact on the Region
http://data.unhcr.org/syrianrefugees/regional.php
[3.5% of 75 Mio]
[26% of 4.1 Mio]

11. The Cyprus
Institute of
Neurology
and Genetics
Financial Aid
for the Region
file:///C:/Users/Lederer/Downloads/2016-05-24-UNHCRFundingUpdate-SyriaSituation-3RPandHRP.pdf

12. The Cyprus
Institute of
Neurology
and Genetics
Emigration from the Region
 Aid shortfall amongst major push factors, e.g. Jordan
 Non-communicable chronic disease (NCD) patients without health care
2014: 23%
2015: 58%
 >50% of Syrian refugee household with at least 1 NCD family member
 Immigrant refuge population with elevated carrier rates
 Serious medical conditions as agreed criteria for UNHCR-aided
resettlement
 Thalassaemia success stories as advertising
material for open-door policies
 Hope of thalassaemia treatment as
key pull factor for any affected family
http://www.unhcr.org/560523f26.html
https://www.youtube.com/watch?v=HAkBmqqe16g
http://www.unhcr.org/548835e59.html
Doocy et al. 2015 PMID 26521231

13. The Cyprus
Institute of
Neurology
and Genetics
Impact on EU Sink Countries
 Impact on Germany (81 Mio; 484,000 refugees)
 In 2014 with 500–600 thalassaemics in total
 Based on simplifying (and inaccurate) assumptions:
5% carrier rate with 11/2015 estimates and 1:1 gender distribution (242,000 couples) @
1 child/couple: 150 new thalassaemia patients amongst first-borns in coming years
 Based on incidence rates from Swedish study: 99 new cases/year
 Expected: in 5 years doubling of current thalassaemia numbers from the Syrian refugee
population alone
 Impact on Sweden (9.6 Mio; 107,966 refugees)
 Historical incidence rates for Syrians in Sweden 20.5/100,000/year
 22 new cases/year
 Impact on Greece (11 Mio; 496,119 refugees)
 Based on incidence rates from Swedish study:102 new cases/year
 Indigenous national screening programme in place
 Requirement of national screening programmes for major sink
countries, at least for at-risk ethnic groups!
 “The refugee crisis challenges national healthcare systems”
Eber and Dickerhoff 2014 PMID24557998 Hemminki et al. 2015 PMID27092253
Hunger 2015 PMID26964894 https://en.wikipedia.org/wiki/Refugees_of_the_Syrian_Civil_War

14. The Cyprus
Institute of
Neurology
and Genetics
Required Action:
Local disease control
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http://http://www.thalassaemia.org.cy/
Annual Number of Thalassaemic Births
% of Expected
 Removal of obstacles to progress ($)
1. Establish working relationships with local clinicians and
educators, through or despite local authorities
2. Identify and address specific local
infrastructural and educational needs
 Establishment of autonomy
 Disease management ($$$)
(keeping patients healthy)
 Disease prevention ($)
(enabling good quality of care)
 Education of patients ($)
 Local prevention measures and treatment options
 Real-life service provision in potential target countries
 Education of clinicians & decision makers ($)
 Best-practice prevention and treatment programmes

15. The Cyprus
Institute of
Neurology
and Genetics
Shared Databases:
Means and End
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 Link between international clinicians and researchers
 Symbolisation of regional and global commonalities
 Establishment of cross-border collaboration
 Affordability (compared to other biomedical undertakings)
 Avoidance of bioethics hurdles inherent to biological samples
 Critical community resource
 Tangible benefit for clinicians and researchers
 Asset to attract future collaborations and funding
 Deepening or widening of collaborations by
future database expansions

16. The Cyprus
Institute of
Neurology
and Genetics
This project has received funding from the European
Union’s Sixth and Seventh Framework Programmes for
research, technological development and demonstration
under grant agreement nos. 26539 (ITHANET) and 306201
(ThalaMoSS) and from the Cypriot Government as core
funding and through the Research Promotion Foundation of
Cyprus.
Thank you for your attention! 16
https://en.wikipedia.org/wiki/Damascus