scaling up role of medical ihs in hemophilia management : more

SCALING UP ROLE OF MEDICAL IHS IN
HEMOPHILIA MANAGEMENT :
More Than Factor Replacement Prescription
IDG. Ugrasena
Hematology-oncology division
Child Health Departement of Dr. Soetomo Hospital - Faculty of Medicine Airlangga University
Introduction
Gen Defect
(hereditary, spontaneous)
 Activity/ and Level of VIII/IX
(mild, moderate, severe)
 Effectivity
coagulation of factor VIII
Introduction
• Hemophilia is congenital an inherited
genetic condition.
• This condition isn’t curable,
• But it can be treated to minimize
symptoms and prevent future health
complications.
• The treatment should be clotting factor
General management of hemophilia
Principle
of care
Diagnosis
inhibitor
General
management
of hemophilia
Home
therapy
carriers
surgery
Psychoso
cial issue
Dental
care
Vaccinati
on
Prophylaxis is a very important development in the
lives of people with haemophilia.
Regular administration of clotting
factor and therefore requires a
regular time commitment from the
person with haemophilia and, if
required, their carer(s).
Good quality of life
Need more than clotting factor prescription
• Hemophilia is a relatively rare but
complex disorder in terms of
diagnosis and management.
• Optimal management of these
patients, especially those with
severe forms of the disease,
• requires more than the treatment
and prevention of acute bleeding.
Need more than clotting factor prescription
• Haemophilia care does not consist only of
replacement therapy and haematologic follow up.
• The haematologist’s clinical and laboratory
expertise should be conjugated to other diagnostic
and therapeutic facilities for the management of
bleeding at various sites, surgery and chronic
complications.
• The need for a multidisciplinary integrated
approach at specialised centres for this rare
congenital disease requiring complex management
Psychologic problem in hemophilia
Unfortunately, all of the problems of the haemophiliacs have not been solved by
the availability of concentrated factor products.
Patients still are faced with the crippling effects of arthritis, problems with
employment, problems with ignorance even in a sophisticated, treatmentoriented community
Key to improve health and quality of life
Keys to improvement of health and quality of life include:
• Prevention of bleeding;
• Long-term management of joint and muscle damage and other
sequelae of bleeding;
• Management of complications from treatment including:
- Inhibitor development; and
- Viral infection(s) transmitted through blood products requiring long-term
management.
Best met by a team of healthcare professionals .
Current service provision In our country
• Currently there is no national standard or
holistic approach in the management of
patients with haemophilia
• The haematology service started in 1980s
and hence haemophilia care is provided
by each department.
• Few hemophilia treatment center
• Haemophilia patients receive their
treatment and referred to the
haematology or paediatric wards when
required.
Problems of caring hemophilia in our country
• The incidence should be 25.000, but registered only
1280
• Stressfull due to receive of clotting factor as long as
their life
• Currently there is no national standard to ensure high
quality multidisciplinary approach to improve patient
outcomes and optimise resource utilisation.
530
• Every hospital has its own policy
180 230
• Non uniform policy implementation hemophilia
services in hospitals tertiary
2000 2001
• National guidelines have not had medical care (PNPK)
for hemophilia
1210
1270
1280
1084
2004
2009 2010
Data : Indonesian Hemophilia Society 2010
MENINGKATKAN PERAN TIM MEDIS HMHI DALAM
TATALAKSANA HEMOFILI ?
Bukan hanya sekedar menyuntikkan faktor koagulasi
Comprehensive care and multidiciplinary team
• Hemophilia merupakan penyakit yang kronis
dengan rentang masalah yang sangat kompleks.
• Masalah tidak bisa diselesaikan hanya dengan
pemberian faktor VIII saja,
• dibutuhkan cara yang bijak yaitu penanganan
secara komprehensif dengan pendekatan tim
secara multidisiplin dari para professional
Hemophilia Treatment Center Team Members
Infectio
us
orthope
Genetic
disease
dist
s
Pharma
physiot
cy
herapist
Social
worker
hemato
logist
Primary
care
Patients
Dental
Hepatol
ogy
Patient
and
family
Nurse
Perlu “ Comprehensive hemophilia treatment centres ”
• Comprehensive care promotes :
- physical and
- psychosocial health and
- quality of life , while
Inhibitor
Arthropathy,
Pseudotumor,
Joint damage
Bleeding,
pain,
Decreasing morbidity and mortality
anemia,
infection
Comprehensive means
• Komprehensif yang dimaksud merupakan suatu sistim
pelayanan bagi penyandang hemophilia dengan
memanfaatkan pengalaman/keahlian dari tim.
• Tim yang terdiri dari dokter ahli ini tidak hanya
memberi/menyuntikkan faktor VIII saja
• Tapi harus berpartner dengan keluarga penyandang
untuk merencanakan penanganan masalah yang
dihadapi sepanjang hidupnya.
Role of Hemophilia Treatment Centers
State-of-the-art medical treatment for persons
with hemophilia through the life span
• Education
• Research
• Outreach
• Model of comprehensive care
for chronic disease
Comprehensive care
• Perawatan komprehensif juga memberi layanan yang dibutuhkan
tidak hanya secara fisik, tapi juga psikososial, edukasi, tanpa
memandang ras, agama, kultur, mampu bayar atau tidak.
• Jangkauan dari penanganan ini juga harus sampai/ melibatkan
masyarakat misalnya ke sekolah, perusahan asuransi swasta atau
pemerintah serta pekerja yang membantu penyandang hemophilia
• Menerangkan/ mengajari apa itu hemophilia, bagaimana mengenal
tanda kedaruratan pada hemophilia.
Comprehensive care
Model penanganan terpadu ini mampu
- meningkatkan kesehatan penyandang hemophilia dan
- menurunkan biaya yang dibutuhkan,
- menurunkan kunjungan ke unit gawat darurat rumahsakit,
- menurunkan hari rawat inap, dan bolos sekolah .
• Menurut laporan dari CDC tahun 2005 menunjukkan lama rawat
inapnya bisa menurun hingga 40 % lebih rendah dari penyakit lainnya.
More than factor replacement prescription
• Didalam pusat layanan hemophilia ini peran dokter tidak hanya
berkewajiban meresepkan faktor VIII saja
• tapi juga memberikan edukasi bagi penyandang, orang tua, pengasuh
prihal apa itu hemophilia, bagimana melakukan penyuntikan faktor
konsentrat ini saat dirumah.
• Sehingga hal ini mampu mengurangi rasa tidak enak dan meningkatkan
rasa kenyamanan saat terjadi perdarahan karena penderita tidak harus
pergi unit gawat darurat dan menunggu di rumahsakit.
Tidak
More
hanya
thansekedar
factor replacement
memberi faktor
prescription
pengganti
• Dokter harus melakukan asesmen prihal gaya hidup pasien (life style),
lingkungan rumahnya dllnya,
• sehingga dokter bisa menilai kemampuan orang tua/wali untuk
memberi perawatan bila pengetahuan tentang tatalaksana
perdarahan yang kurang baik.
Tidak
More
hanya
thansekedar
factor replacement
memberi faktor
prescription
pengganti
• Pasien dan keluarga harus diberi suatu jaminan bahwa mereka mendapat
akses secara penuh mulai dari kebutuhan layanan hingga penanganan
terhadap keluhan penyakitnya.
• Merupakan kunci dari cara untuk :
- meningkatkan kesehatan termasuk pencegahan terhadap timbulnya
perdarahan,
- penanganan jangka panjang kerusakan sendi dan otot,
- penanganan komplikasi mulai terbentuknya inhibitor dan infeksi karena
transfusi.