Dr. Leila Jeddane & Prof. Aziz Bousfiha - Ipopi

IPOPI Global Leader Meeting,
4-5 November 2011
Jeddane Leïla, Ailal Fatima, Bousfiha Aziz,
& ASID BOARD :
Bejaoui M, Barbouche MR
Esser M & Eley B, Boukari R & Kechout N,
ElMarsafi A & Boutros J, Wann AT,
Ould Khalifa I & Ould Mohamed Awdih A,

• Access to treatment
or access to diagnosis
• Methodology
• Results
• Perspectives

Access to treatment or
access to diagnosis
Region
Population in
2010
Number of
PID in 2010*
Number of
new cases in
2010**
Registry 2010
Registry
coverage
Africa 1,022,234,400 882,188 105,290 1,016 0.12%
Europe 738,198,601 637,065 76,034 13,017 2.04%
World 6,895,889,018 5,951,152 710,276 46,450 0.78%
Only 1,2‰ of PID cases notified in ASID registries !!
First objective: improve diagnosis in Africa
*Based on prevalence in USA (Boyle & Buckley, 2007)
**Based on incidence in USA (Joshi et al., 2009)

Population : 1 045 922 624
Number of countries : 58
Life expectancy
: 57.4 years
Birth rate : 34.0 ‰
Death rate : 10.8 ‰
Infant mortality rate : 71.4 ‰
Number of languages : 1302
GDP / capita 2007: $ 1 380 USD

Methodology
• ASID data
• Mails and phone calls to ASID members
• Searching contacts outside ASID members

What is ASID
• African Society for Primary Immunodeficiencies
• Founded in Casablanca; October 2008
• Objectives:
– Education
– Improvement of Diagnosis
– Access to treatment

ASID has identified referent persons and
encouraged the creation of reference centers.
2008
NUMBER OF
COUNTRIES
2011
NUMBER OF
COUNTRIES
At least one
person
referent
4
At least one
person
referent
14
Basic
Exploration :
Ig GAME
+ SPL
IV-Ig
15
10
Reference
Center 4
Basic
Exploration :
Ig GAME
+ SPL
IV-Ig
25
15
Reference
Center 6
BMT
2
BMT
3

Access to treatment
Availability
Region/ Country IV Ig SC Ig HSCT Reference
center
Referent
person
Bousfiha A, Ailal F x x (2011) x 1 4
Bejaoui M, Mellouli F X x x 1 6
Boukari R x o o 3
El Marsafi A x o x 5 8
Esser M x o x 2
Ould Mohamed Awdih
o
(imported)
o o 2
x o o 0 (laboratory) 0

Ig substitution therapy
Region/ Country Coverage Brand
Bousfiha A 80%
TEGELINE®, KIOVIG®
(SC)
Mellouli F 100%
INTRATECT ® , TEGELINE®, KIOVIG®
SANDOGLOBULINE ® , OCTAGAM®
(stopped)
Boukari R
SANDOGLOBULINE ®
KIOVIG® , INTRAGLOBINE® (stopped)
GREEN CROSS CORPORATION, OCTAGAMMA®
El Marsafi A
SANDOGLOBULINE ® (unaffordable)
GAMMAGUARD ® (stopped)
Esser M, Eley B 100%
POLYGAM ® , INTRAGAM IMI ® (SC)
Intraglobin F ® Pentaglobin ® Megalotect ®

Ig substitution therapy
• IVIg generally available BUT
• Expensive
• Geographic contraints
• Families not covered by social services
• Subcutaneous Ig generally NOT available,
not even discussed in some countries

HSCT
Region/country Local center African
center
Extracontinental
center
Coverage
Morocco x x x
Tunisia x o o
Algeria x x
Egypt
x
Long awaiting
list
South Africa x o o

HSCT
• Need for an intracontinental
cooperation
• Need for african donors

Reference centers
• 6 reference centers in Africa
• Need for at least one reference center by region:
• Middle Africa
• Eastern Africa
• Western Africa
• Education to train at least one referral person by
country

Gene therapy
• Is it the best option in Africa
• Need collaboration with specialized
centers to provide gene therapy for
african patients
• Compensate lack of donors for BMT

Our objective
Laboratory
sponsors
Improved
diagnosis
• Reference center
• Increased needs in
IVIg
• Association
Training