Greek Assosiation of HAE Stavroula Labiri HAE Representative for - - PowerPoint PPT Presentation

Stavroula Labiri

HAE Representative for Greece

Greece Greek Assosiation of HAE

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). Cont ntact act Detai ails ls: Telephone: + 30 6977 412779 E-mail: haegroup.greece@gmail.com Facebook group: http://www.facebook.com/groups/HAE.GREECE

Let’s talk in numbers !!!

• 10

10,815 815,19 197 7 is the total al popul ulatio ation n in Gr Greece ce

• 120

120 HAE AE p patie ients nts - 43 non related ted famili ilies es

• 300 patie

ients nts estim timate ated d based ed on globa bal l preva vale lenc nce e (1/90,0 0,000 00)

Age range 4 – 88 Years

Greece

= 63 = 57

...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 t 5 years rs

• Overall diagnosis of 16.5 years
• 7 confirmed deaths from Laryngeal edema

Greece

...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

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

Population 838,897 # of patients 2 # of HAE Physicians 2 Greece

Let’s talk geography !!! Cyprus

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).

• f general physicians about HAE in Greece is still

very poor. There is NO NO specialist center for HAE in Greece.

Greece

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

• r 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

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

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

Management of HAE: Medication and treating attacks

In case e of Em Emergenc ncy y : – HAE is NOT NOT recognized by doctors in the Emergency Rooms – Medication is NOT OT available, patients must bring their own medication – Medical personnel is NOT NOT trained to administer Berinert (patients or care givers must give instructions)

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

Special Acknowledgment: Dr Fotis Psarros Allergist in Athens Naval Hospital