Bambang Kegawatdaruratan Hemophilia

04/17/2023 TIN Ke-7 PHTDI Yogjakarta

EMERGENCY CARE IN HEMOPHILIA

Bambang Sudarmanto Department of Child HealthMedical Faculty Diponegoro University Kariadi Hospital Semarang


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

HEMOPHILIAS

• Hemophilia – Inherited– X-linked (recessive)

• A & B

– Autosomal• C

– Single factor deficiencies

– Anatomic soft tissue bleeding


HEMOPHILIAHemophilia A

Factor VIII Deficiency Antihemophilic FactorX-linked recessive disorderMost 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 disorderSymptoms range from mild to severe


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

Classification of hemophilia based on biological F VIII and FIX

Committee of the International Society of Thrombosis and Haemostasis


Type of bleeding Severe (<0.1)

Moderate(0.1 – 0.4)

Mild (0.5 – 4.0)

Age of onset < 1 year < 2 years > 2 yearsHemarthroses Spontanoeous Following minor trauma

++++++++

+++++

--

Muscle hematoma ++++ ++ -Central nervous system ++ + -Hematuria ++++ ++ -Surgery ++++ +++ ++Dental extraction ++++ +++ ++Trauma to soft tissue Mild Significant

++++++++

+++++

++

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

Bleeding manifestation with different severities of hemophilia A and B

04/17/2023

TIN Ke-7 PHTDI Yogjakarta

Serious•Joint•Muscle•Mouth/gums/nose•Hematuria

Life -threatening

•Central Nervous System (CNS)•Gastrointestinal•Neck/throat•Severe trauma •Pseudotumor ruptur

Bleeding Manifestation in Hemophilia

Incidence of different site of bleeding• hemarthros : 70%- 80%•Muscle/soft tissue : 10% - 20%•Other major bleeds : 5% - 10%•Central nervous System : < 5%



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


KEGAWAT DARURATAN PADA PASIEN HEMOPHILIA SEBAGAI SISTEM


NILAI HAKIKI PASIEN DENGAN HEMOPHILIA

“ SEHAT”“KEADILAN”

TUMBUH KEMBANG OPTIMAL

SAFE HEMOPHILIA EMERGENCYCARESERVICE

Pre hospitalIntra hospitalInterhospital

• Quick Response(Life Saving & Limb Saving)

•Peran HMHI• Peran Pemerintah

04/17/2023

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”

SISTEM PENANGGULANGAN GAWAT DARURAT HEMOPHILIA

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.



Prehospital Care– Rapid transport to definitive care is the mainstay of

prehospital care.– Prehospital care providers should do the following:

Apply aggressive hemostatic techniquesAssist patients capable of self-administered factor therapyGather focused historical data if the patient is

unable to communicate



Prehospital Care:- Call Center :

- Sebagai Call handling dari masyarakat - Sebagai media komunikasi jejaring RS- Data data penderita hemophilia



Prehospital Care:- Transportation: Ambulans

- Medic and Paramedic staf - Emergency kit ( aggressive hemostatic

tehnique)


INTRA HOSPITAL

EMERGENCY CARE

1. Medical support

2. Management Support


INTRA HOSPITAL CARE

Intra hospital Care– Emergency Department / IGD :• Competency of medical staff • Medical support:–Pharmacy –Laboratory – Imaging

• CFC Replacement ---AHF


EMERGENCY DEPARTMENT CARE

The type and severity of factor deficiencyThe nature of the hemorrhage or the planned

procedureThe patient's previous treatments with blood

productsWhether inhibitors are present and if so, their

probable titer


INTERDICIPLINARY MANAGEMENT

Patient


PERAN HMHI……• Genetic , Psychological and

emotional counseling• Education :

• Individual• familial

• Home infusion training• Support group• Advocacy with insurance companies• Research studies and clinical trials

GUIDELINES

STANDARD FASCILITY

PROCEDUR OPERATIONAL

R E G I S T R A S I

PEMERINTAH


PERAN PEMERINTAH

• REGULASI/KEBIJAKAN PELAYANAN HEMOPHILIA INDONESIA (Misal: PP/PERMENKES, etc) – KETERSEDIAAN Clotting factor concentrate

replacement profilaksi – PEMBIAYAAN biaya tinggi

SJSNBPJS-Kesehatan

Wilayah JEJARING HEMOPHILIA Jawa Tengah

RS MUWARDI SOLOUntuk Jawa Tengah Selatan dan sekitarnya

RSUD MARGONO Untuk Karisidenan Kedu

RSUP Dr. Kariadi Untuk Pantura dan Sebagian selatan



THANK YOU FOR YOUR ATTENTION


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Bambang Kegawatdaruratan Hemophilia