EMERGENCY CARE IN HEMOPHILIA

Bambang Sudarmanto
Department of Child Health Medical Faculty Diponegoro University Kariadi Hospital Semarang

5/27/2013

TIN Ke-7 PHTDI Yogjakarta

INTRODUCTION
Patient with hemophilia are at risk for severe bleeding that may quickly evolve into life or limb threatening circumstances Bleed that require immediate assessment and intervention are characteristic by:
Bleeding into an enclosed space Compression of vital tissue Potential loss of life, limb or function
5/27/2013 TIN Ke-7 PHTDI Yogjakarta

HEMOPHILIAS
Hemophilia
Inherited X-linked (recessive)
A&B

Autosomal
C

Single factor deficiencies Anatomic soft tissue bleeding

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HEMOPHILIA
Hemophilia A

Factor VIII Deficiency

Antihemophilic Factor X-linked recessive disorder Most common type of hemophilia

Hemophilia B

Factor IX Deficiency
Christmas Factor (from family of first patients diagnosed with the disorder) X-linked recessive disorder

Hemophilia C

Factor XI Deficiency
Autosomal recessive disorder Symptoms range from mild to severe

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TIN Ke-7 PHTDI Yogjakarta

Classification of hemophilia based on biological F VIII and FIX

F VIII/FIX Concentration

Severe hemophilia

< 0.01 IU/ml

< 1% of normal

Moderate hemophilia

0.01 0.05 IU/ml

2 5% of normal

Mild hemophilia

0.05 0.4 IU/ml

5 40 % of normal

Committee of the International Society of Thrombosis and Haemostasis

5/27/2013 TIN Ke-7 PHTDI Yogjakarta

Bleeding manifestation with different severities of hemophilia A and B

Type of bleeding
Age of onset
Hemarthroses Spontanoeous Following minor trauma

Severe (<0.1)
< 1 year
++++ ++++

Moderate (0.1 0.4)

< 2 years
++ +++

Mild (0.5 4.0)

> 2 years
-

Muscle hematoma
Central nervous system Hematuria Surgery

++++
++ ++++ ++++

++
+ ++ +++

++

Dental extraction
Trauma to soft tissue Mild Significant

++++
++++ ++++

+++
++ +++

++
+ +
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+++, Usual; +++ common; ++ not usual + can occur;; - rare

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Bleeding Manifestation in Hemophilia

Serious Joint Muscle Mouth/gums/nose Hematuria Life -threatening Central Nervous System (CNS) Gastrointestinal Neck/throat Severe trauma Pseudotumor ruptur

Incidence of different site of bleeding

hemarthros

: 70%- 80% Muscle/soft tissue : 10% - 20% Other major bleeds : 5% - 10% Central nervous System : < 5%
5/27/2013 TIN Ke-7 PHTDI Yogjakarta

MANAGEMENT OF HEMOPHILIA
Prevention of bleeding should be the goal Acute bleed should be treated early Home therapy All severe bleeds should be manage in the clinic or hospital setting Clotting factor concentrate replacement or DDAVP Avoid use of drug : ASA, NSAIDs Sport
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KEGAWAT DARURATAN PADA PASIEN HEMOPHILIA SEBAGAI SISTEM

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TIN Ke-7 PHTDI Yogjakarta

NILAI HAKIKI PASIEN DENGAN HEMOPHILIA

SEHAT KEADILAN
SERVICE
Pre hospital Intra hospital Interhospital

SAFE HEMOPHILIA
TUMBUH KEMBANG OPTIMAL
Peran HMHI Peran Pemerintah

EMERGENCY CARE
Quick Response
(Life Saving & Limb Saving)

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TIN Ke-7 PHTDI Yogjakarta

SISTEM PENANGGULANGAN GAWAT DARURAT HEMOPHILIA

SISTEM YANG TERDIRI DARI KOMPONEN :

PRE HOSPITAL CARE INTRA HOSPITAL INTER HOSPITAL CARE RS KOMUNIKASI DAN TRANSPORTASI SDM KESEHATAN DI RUMAH SAKIT KETERSEDIAAN CFC Replacement

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TIN Ke-7 PHTDI Yogjakarta

PRE HOSPITAL CARE (1)

Prehospital Care: (patient with hemophilia) - Planning Ahead For Emergencies :
recognize the signs of the type of injury or bleed that might require a trip to the ER. Prepare by packing an ER bag with everything you might need. Having your ER bag ready to go at all times.

- Preparing For The ER In Advance

- find the one most familiar with the treatment of people with hemophilia.
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PRE HOSPITAL CARE (2)

Prehospital Care
Rapid transport to definitive care is the mainstay of prehospital care. Prehospital care providers should do the following: Apply aggressive hemostatic techniques Assist patients capable of self-administered factor therapy Gather focused historical data if the patient is unable to communicate

5/27/2013

TIN Ke-7 PHTDI Yogjakarta

PRE HOSPITAL CARE (2)

Prehospital Care:
- Call Center :
- Sebagai Call handling dari masyarakat - Sebagai media komunikasi jejaring RS - Data data penderita hemophilia

5/27/2013

TIN Ke-7 PHTDI Yogjakarta

PRE HOSPITAL CARE (3)

Prehospital Care: - Transportation: Ambulans
- Medic and Paramedic staf - Emergency kit ( aggressive hemostatic tehnique)

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TIN Ke-7 PHTDI Yogjakarta

INTRA HOSPITAL

IGD
Antar RS

ICU
EMERGENCY CARE
1. Medical support 2. Management Support

Kamar Jenazah

HCU
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5/27/2013

INTRA HOSPITAL CARE

Intra hospital Care
Emergency Department / IGD : Competency of medical staff Medical support: Pharmacy Laboratory Imaging CFC Replacement ---AHF
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EMERGENCY DEPARTMENT CARE

The type and severity of factor deficiency The nature of the hemorrhage or the planned procedure The patient's previous treatments with blood products Whether inhibitors are present and if so, their probable titer

5/27/2013

TIN Ke-7 PHTDI Yogjakarta

INTERDICIPLINARY MANAGEMENT

Hematologist Physical Therapists

Orthopedists

Patient
Laboratory Technicians Nurses

Social Workers

Dentists

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TIN Ke-7 PHTDI Yogjakarta

PERAN HMHI
Genetic , Psychological and emotional counseling Education : REGISTRASI Individual GUIDELINES familial STANDARD FASCILITY Home infusion training Support group PROCEDUR OPERATIONAL Advocacy with insurance companies Research studies and clinical trials

PEMERINTAH
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PERAN PEMERINTAH
REGULASI/KEBIJAKAN PELAYANAN HEMOPHILIA INDONESIA (Misal: PP/PERMENKES, etc)
KETERSEDIAAN Clotting factor concentrate replacement profilaksi PEMBIAYAAN biaya tinggi

SJSN BPJS-Kesehatan
5/27/2013 TIN Ke-7 PHTDI Yogjakarta

Wilayah JEJARING HEMOPHILIA Jawa Tengah

RSUP Dr. Kariadi Untuk Pantura dan Sebagian selatan

RSUD MARGONO Untuk Karisidenan Kedu

5/27/2013

RS MUWARDI SOLO Untuk Jawa Tengah Selatan dan sekitarnya

TIN Ke-7 PHTDI Yogjakarta

THANK YOU
FOR YOUR ATTENTION

5/27/2013

TIN Ke-7 PHTDI Yogjakarta

5/27/2013

TIN Ke-7 PHTDI Yogjakarta