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Stavroula Labiri HAE Representative for Greece
Greece
Greek Assosiation of HAE
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Who we are?
Greek Association of HAE
Stavroula Labiri, HAE Representative for Greece
• We are members of the HAEi and the Greek Alliance for Rare
Diseases.
• Our Facebook Group has 38 members representing 54 people.
• Cyprus is also a member of our team with currently 2 members
(1
patient & 1 doctor).
Contact Details:
Telephone: + 30 6977 412779
E-mail: [email protected]
Facebook group: http://www.facebook.com/groups/HAE.GREECE
mailto:[email protected]://www.facebook.com/groups/HAE.GREECE
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Let’s talk in numbers !!! • 10,815,197 is the total population
in Greece
• 120 HAE patients - 43 non related families
• 300 patients estimated based on global
prevalence (1/90,000)
Age range 4 – 88 Years Greece
= 63 = 57
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...Let’s talk in numbers !!!
• 80% of the patients in Greece have Type I HAE
• 19% of patients in Greece have Type II HAE
– 1 patient with acquired angioedema
• The average age of disease onset is 12 years
• In 40% of the patients, the diagnosis of HAE was
established only during the last 5 years
• Overall diagnosis of 16.5 years
• 7 confirmed deaths from Laryngeal edema
Greece
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...Let’s talk more in numbers !!!
Greece
Affected Sites Greece Bibliography
Larynx 60 % ~50%
Abdomen 88 % 70-90%
Skin 98,5 % ~95%
• 22% unnecessary surgeries for
acute abdomen vs 30% in literature
• Intubation or cricothyreotomy (14%)
94,20%
78,60%
74%
42,30%
33,70%
28,00%
19,20%
without obvious reason
emotional stress
trauma
viral infection
ACE inh
Dental procedures
Surgery
Triggers of HAE attacks in Greece
% of patients per trigger
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Let’s talk geography !!!
Greece
Geographic District Patients %
Attika 25 21%
Macedonia 15 13%
Peloponnese 15 13%
Islands in Aegean Sea
25 21%
Epirus 11 10%
Rest of Central Greece
9 8%
Crete 8 5%
Thessaly 6 5%
Thrace 5 4%
Islands in Ionian Sea 1 1%
Greece
Lesvos island has 25 patients Total polulation
86,000
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Population 838,897
# of patients 2
# of HAE Physicians 2
Greece
Let’s talk geography !!! Cyprus
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Management of HAE:
Diagnosis and doctors
The doctors that usually treat HAE in Greece are Allergists,
Immunologists or ENTs.
In 2009 the Hellenic Society of Allergiology and Clinical
Immunology created GHAER (Greek Hereditary Angioedema Registry), in
order to locate and register all HAE Cases in Greece.
An Awareness campaign started in 2010 in order for all
allergists and all allergy hospital departments in the country to
be informed regarding HAE and gradually was extended addressing
specialists in all fields involved in the diagnosis and treatment
of the disease (e.g. pediatricians and ENT specialists).
of general physicians about HAE in Greece is still very
poor.
There is NO specialist center for HAE in Greece.
Greece
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Management of HAE:
Diagnosis and doctors
Greece
Personal & Family Medical History Phobia concerning the
availability of appropriate drugs at the next episode
C4 blood test Phobia concerning insurance coverage
C1 inh blood test Refusal of emergency room physicians to
administer(particularly i.v.) the drugs that the patients had
brought with them during the episodes (due to unfamiliarity with
the disease or the drugs)
Genetic Defect Testing
• Unfortunately Rare diseases and HAE are not a priority in
Greece. Diseases such as cancer, HIV, HCV, diseases affecting the
heart and diabetes are more important
• HAE is underdiagnosed in Greece with obvious implications for
the treatment , the mortality and the deterioration of quality of
life of the patients
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Management of HAE:
Diagnosis and doctors
Symptoms Greece
34 pregnancies Literature
Improvement 85% 56%
Unchanged 5% 8%
Worsening 10% 36%
51% 49%
Prodromal symptoms
With prodromal Without prodromal
Most common Symptoms: • Erythema
marginatum • Fatigue • Nausea
Most common Triggers: • Hormones • Viral Infections
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Management of HAE:
Medication and treating attacks Long Term Treatment
Danatrol caps. 100,200mg
Not reimbursed No restrictions on prescriptions
Transamin caps. 250mg
Not reimbursed
No restrictions on prescriptions
Treating Attacks
Berinert-P inj. 500IU/vial Only administered in hospitals
Reimbursed There is a limit of 10 vials per month that the
doctor can prescribe
Firazyr inj. Sol 30mg Self administered
Reimbursed There is a limit of 10 vials per month that the
doctor can prescribe
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Management of HAE:
Medication and treating attacks
In case of Emergency :
– HAE is NOT recognized by doctors in the
Emergency Rooms
– Medication is NOT available, patients must
bring their own medication
– Medical personnel is NOT trained to
administer Berinert (patients or care givers
must give instructions)
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Future of HAE in Greece:
Actions - Plans
• Raise HAE awareness in Greece & Cyprus – Create a national
website, hosted by HAEi
– Recruit more members in our Facebook group
– Collaborate with local HAE specialists to raise awareness
among their co-workers (ER specialists, ENTs, Allergists etc)
– Host a Patient meeting
– Participate in global meetings to be kept up to speed of all
HAE innovations and news
• Inform & Encourage people to get tested.
• Appoint one of the main Hospitals in Athens as a HAE
center
• Train patients to self-administer their medication
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Special Acknowledgment:
Dr Fotis Psarros Allergist in Athens Naval Hospital
