· 

       Can be give first treatment hemophilia therapy before

referring the patient to specialist

Transfusion platelet

Frozen plasma

creopresipitate

Permanent and irreversible joint damage (also known as hemophilic

arthropathy) is the most common complication of hemophilia that
leads to disability. It is caused by repeated bleeding into the same
joint, and over time results in the destruction of the cartilage and
synovium (the lining of the joint).

1. Prophylaxis = pencegahan pendarahan lanjut untuk anak dan

dewasa, terutama pada persendian karena sering banget terjadi
pendarahan di sana.

1. Primary Prevention - trying to prevent yourself from getting a disease.

2. Secondary Prevention - trying to detect a disease early and prevent it from getting worse.
3. Tertiary Prevention - trying to improve your quality of life and reduce the symptoms of a disease
you already have.

Prophylaxis treatment regimen, ada 3 hal yang menentukan:

(1) the given resources/concentrate availability to target a specific trough level and/or
intervals of substitutions, which both reflect the costs;

(2) the bleeding trigger, which comprises physical activity, presence and degree of
arthropathy, and presence of chronic synovitis; and
 (3) the number of bleeds, especially joint bleeds, that are regarded as acceptable. 

Kalau misalnya salah satu dari ini berubah, maka 2 yang lain akan menyesuaikan. Profilaksis
treatment akan berbeda terhadap setiap orang sesuai umur dan keadaan. International
Society on Thrombosis and Haemostasis (ISTH) definitions.

Primary profilaksis, should aim to prevent any joint damage. (Berdasarkan data
yang saya peroleh, profilaksis bermula saat masih anak” sejak ada kejadian
pertama sebelum umur 3 tahun.)

Secondary profilaksis, commences after 2 or more joint bleeds, tapi sebelum

ketahuan terdiagnosa oleh imaging studies. These patients may already have a
significant risk of developing joint arthropathy. 

Tertiary prophylaxis is defined as treatment initiation after the onset of joint

disease at any age of the patient. Objectives in those patients include slowing
down progression of joint disease, reducing pain and inflammation, and
maintaining mobility, especially in adult hemophiliacs with already advanced joint
disease.

The Canadian primary prophylaxis experience begins with a once-weekly

regimen (50 U/kg body weight [BW]), that, depending on number of bleeds, is
intensified in a first step to twice-weekly treatment (2 times 30 U/kg BW) and, in a
third step, to every-other-day therapy (25 IU/kg BW).11  Median ages at switching
to steps 2 and 3 were 4.1 and 9.7 years, respectively

Dalam penelitian profilaksis dewasa dibagi ke dalam 2 grup, grup pertama yang
udah mulai dari awal primary dan secondary profilaksis bisa mempertahankan
kondisi sendi yang sehat. In the German cohort, 90% of patients showed some,
mostly mild, arthropathy, mainly in their ankle joints after a 26-year follow-up.
The joint ABR(annual bleeding rate) should not be higher than during an
intensive prophylaxis regimen, which is about 1 to 2 joint bleeds within 2 years.

The second group yang udah kena athropathy dan melaksanakan tertiary
prophylactic regimen. Evaluate the Effect of Secondary Prophylaxis with rFVIII
Therapy in Severe Hemophilia A Adult and/or Adolescent Subjects Compared to
That of Episodic Treatment (SPINART study). about 20% of the prophylaxis
group, with 25 IU/kg BW 3 times per week, still had a significant number of
bleeds.

Several studies have shown that the level of the VWF has a major influence
on the FVIII half-life.

Before a patient with hemophilia is treated, the following information should be

obtained:
 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
  Any previous history of desmopressin acetate (DDAVP) use (mild
hemophilia A only), with the degree of response and clinical outcome.

As a rule, FVIII 1 U/kg increases FVIII plasma levels by 2%. The half life of infused
factor is usually 8-12 hours. Incremental recovery (i.e. the amount increase of factor
activity per unit per kg) and half life vary from patient to patient and ideally should be
individualized based on that patient's experience.
Target levels by hemorrhage severity are as follows:
 Mild hemorrhages (ie, early hemarthrosis, epistaxis, gingival bleeding):
Maintain an FVIII level of 30%
 Major hemorrhages (ie, hemarthrosis or muscle bleeds with pain and
swelling, prophylaxis after head trauma with negative findings on
examination): Maintain an FVIII level of 50%
 Life-threatening bleeding episodes (ie, major trauma or surgery,
advanced or recurrent hemarthrosis): Maintain an FVIII level of 80-90%
until stabilization; after stabilization, maintain levels above 40-50% for a
minimum of 7-10 days
2. management of bleeding episodes,

Desmopressin
Desmopressin vasopressin analog, or 1-deamino-8-D-arginine
vasopressin (DDAVP), is considered the treatment of choice for mild
and moderate hemophilia A. It is not effective in the treatment of
severe hemophilia.
 DDAVP may result in sufficient hemostasis to stop a bleeding
episode or to prepare patients for dental and minor surgical
procedures. A test dose should be performed before prophylactic
use. It can be intravenously administered at a dose of 0.3 mcg/kg of
body weight in the inpatient setting. Its peak effect is observed in 30-
60 minutes.

A concentrated DDAVP intranasal spray (1.5 mg/mL) is available for

outpatient use. Its effectiveness is similar to that of the intravenous
preparation, although its peak effect is observed later, at 60-90
minutes after administration.

ATAU BISA JUGA RICE

(rest, ice, compression, elevation) segera dilakukan. Sendi yang

mengalami perdarahan diistirahatkan dan diimobilisasi.
Kompres dengan es atau handuk basah yang dingin,
kemudian dilakukan penekanan dan meninggikan daerah
perdarahan.
Musculoskeletal bleeding = dilakukan RICE, infusion

Early infusion upon the recognition of initial symptoms of a joint bleed may
often eliminate the need for a second infusion by preventing the
inflammatory reaction in the joint. Prompt and adequate replacement
therapy is the key to preventing long-term complications. Cases in which
treatment begins late or causes no response may require repeated
infusions for 2-3 days.
Oral bleeding
Combine adequate replacement therapy with an antifibrinolytic agent
(epsilon-aminocaproic acid [EACA] or tranexamic acid [TA]) to neutralize
the fibrinolytic activity in the oral cavity. Topical agents such as fibrin
sealant, bovine thrombin, and human recombinant thrombin can also be
used. 20 mg/kg dose of EACA.
Gastrointestinal bleeding
IV infusion continuesly

Intracranial bleeding
Intracranial hemorrhage is often trauma induced; spontaneous intracranial
hemorrhages are rare. If CNS hemorrhage is suspected, immediately begin an
infusion prior to radiologic confirmation. Maintain the factor level in the normal range
for 7-10 days until a permanent clot is established.

3. treatment of factor VIII (FVIII) inhibitors,

Other approaches to treating patients with FVIII inhibitors include the following:
 Porcine FVIII, which has low cross-reactivity with human FVIII antibody
 Activated prothrombin complex concentrate (aPCC)
 Activated recombinant FVII (rFVIIa)
 Desensitization
 Immune tolerance induction (ITI)
 Monoclonal antibodies that bridge FIXa and FX (eg, emicizumab)

4. and rehabilitation of hemophilia synovitis

Routine immunizations that require injection (eg, diphtheria, tetanus toxoids, and
pertussis [DPT] or measles-mumps-rubella [MMR] vaccines) may be given by
means of a deep subcutaneous (rather than deep intramuscular) injection with a
fine-gauge needle. Administer the hepatitis B vaccine (now routinely administered
to all children) soon after birth to all infants with hemophilia. Administer the
hepatitis A vaccine to those individuals with hemophilia and no hepatitis A virus
antibody in their serum.

In severe hemophilia, consider prophylactic or scheduled factor VIII. Prophylactic

replacement of FVIII is used to maintain a measurable level at all times, with the
goal of avoiding hemarthrosis and the vicious cycle of repetitive bleeding and
inflammation that results in destructive arthritis.  [58] This goal is achieved by
administering factor 2-3 times a week. The National Hemophilia Foundation has
 recommended the administration of primary prophylaxis, beginning at the age of
1-2 years.
·        Understand prevention complication of Hemophilia.

Recognize bleeding symptoms

Many people who have hemophilia know when they are bleeding even before there are many symptoms.

Bleeding in a joint

Bleeding into a joint (hemarthrosis), often without an injury, is the most common bleeding problem in
people who have severe hemophilia. Bleeding usually occurs in one joint at a time. Bleeding may occur in
any joint, but knees, elbows, and ankles are most commonly affected. Sometimes one particular joint,
called a target joint, will tend to bleed most often.
Symptoms of bleeding into a joint include:

 Warmth or tingling in the joint during the early stages of hemarthrosis. This is called an aura. If
bleeding is not treated, mild discomfort can progress to severe pain.
 Swelling and inflammation in the joint, caused by repeated episodes of bleeding. If episodes
continue, the swelling may lead to chronic pain and destruction of the joint.
 An infant or child not wanting to move an arm or leg because of bleeding into an affected joint.
This is often first noticed when a child begins to walk.
Bleeding in a muscle

Another common symptom of hemophilia is bleeding into a muscle (hematoma), which can be mild or
severe. There are many possible symptoms of bleeding into muscle, including:

 Bruising.
 Swelling.
 Muscle hardening.
 Tenderness.
 Pain, especially when large muscle groups are affected.
It is important to begin infusion with clotting factors as soon as possible after a bleeding episode has
started, before any physical signs develop. Even with treatment, bleeding is sometimes hard to control.
Frequent bleeding episodes or a serious injury can lead to complications and excessive blood loss.
Work with your doctor to make a plan for what to do if you or your child has a bleed.

Harus tetap bugar tapi hati”

Sports and activities that are typically recommended for adults and children who have hemophilia
include:

 Swimming.
 Bicycling (be sure to wear a helmet).
 Walking.
 Tennis.
Sports that are possible but carry an increased risk of bleeding include:

 Baseball.
 Basketball.
Sports that have a high risk for bleeding include:

 Soccer.
 Football.
 Hockey.
 Weight lifting (with heavy weights).
 Wrestling.

JANGAN MINUM OBAT” BERIKUT

Medicines that people with hemophilia should not take include the following:
 Aspirin
 Ibuprofen, such as Advil or Motrin
 Medicines that contain salicylate. This ingredient is closely related to aspirin. Alka-Seltzer, Pepto-
Bismol, and many cough medicines contain salicylates.
 Other non-steroidal anti-inflammatory medicines, such as Aleve. These medicines are designed to
reduce swelling and reduce pain.

Prevent injuries and accidents

Because a bleeding episode often begins with an injury, it is important to help prevent falls in the home.

 Remove and repair household hazards that can cause falls, including:
o Slippery floors. Use non-skid floor wax, and wipe up spills immediately.
o Poor lighting.
o Cluttered walkways. Rearrange furniture to keep furniture out of walking paths.
o Throw rugs. Fasten area carpets to the floor with tape or tacks, and do not use rugs that
slip easily.
o Raised doorway thresholds.
o Electrical cords. Rearrange cords to keep them out of walking paths.
 Keep furniture or other items that have sharp edges away from normal pathways through
your house.
o Remove square or rectangular coffee tables.
o Pad the rocks around fireplaces.
 Take precautions when you go outdoors.
o If you live in an area that gets snow and ice in the winter, have a family member or friend
sprinkle salt or sand on slippery steps and sidewalks.
 Check the condition of your shoes on a regular basis.
o Check the heels and soles of your shoes for wear.
o Repair or replace worn heels or soles.
o Wear low-heeled shoes that fit well and give your feet good support.
o Avoid loose-fitting shoes, which can cause you to lose your balance and fall.
o Wear slippers or shoes that have non-skid soles.

·        Understand strategies of prevention for surgery in Bleeding

Disorders.
Penatalaksaan Pasien Hemofilia di Kedokteran Gigi
Beberapa perawatan gigi dapat dilakukan tanpa penambahan faktor
pembekuan darah, seperti pemeriksaan, fissure sealants, restorasi
oklusal yang kecil, dan
scaling supragingiva.7 Tapi pada perawatan yang memerlukan
penambahan faktor
pembekuan, terdapat tiga pilihan terapi yang dapat dilakukan
tergantung dari jenis
Hemofilianya, yaitu:
a) Terapi faktor pembekuan pengganti
Terapi faktor pembekuan pengganti merupakan terapi utama yang
dilakukan
pada pasien Hemofilia A atau B yang sedang maupun parah.
Pemberian
faktor pembekuan pengganti ini dilakukan secara intravena. Hal
yang paling
penting dalam pemberian faktor pembekuan pengganti ini adalah
waktu
pemberiannya karena konsentrasi faktor pembekuan pengganti ini
akan
semakin menurun. Jadi, waktu perawatan gigi yang akan dilakukan
harus
sedekat mungkin dengan waktu pemberian faktor pembekuan
penggantin,
biasanya 30 menit - 1 jam sebelum dilakukan perawatan gigi. Selain
itu,
faktor pembekuan pengganti ini juga dapat diberikan ketika terjadi
perdarahan selama perawatan. Kelemahan terapi ini adalah
harganya yang
mahal dan menyebabkan berkembangnya inhibitor atau antibodi
yang
meniadakan efek terapi ini.7
b) Desmopressin (Desamino-8-D-Arginine Vasopressin [DDAVP])
Desmopressin merupakan hormon sintetis yang menstimulasi
pelepasan FVIII dan faktor von Willebrand (vWF) dari dalam tubuh
pasien sehingga
efektif untuk pasien penderita Hemofilia A dan von Willebrand
disease (vWD). Desmopressin diberikan satu jam sebelum dilakukan
perawatan secara subkutan dengan dosis 0,3 µg/kgBB dengan
kadar 15 µg/ml atau secara intravena dengan dosis 0,3 µg/kgBB
dengan kadar 4 µg/ml yang dicampur ke dalam cairan infus dan
diberikan selama 20-30 menit. Secara intranasal, desmopressin
diberikan dengan dosis 150 µg ke satu lubang hidung untuk pasien
dengan berat <50 kg dan 150 µg ke kedua lubang hidung untuk
pasien dengan berat ≥50 kg. Pemberian desmopressin dihindari
pada pasien dengan penyakit jantung iskemik dan pasien
anakanak.7
c) Antifibrinolytic Agents
Asam traneksamat berfungsi untuk menghambat pengaktifan
plasminogen menjadi plasmin sehingga juga menghambat bekuan
fibrin lisis. Asam traneksamat tersedia dalam sediaan oral dan obat
kumur. Asam traneksamat sediaan oral diberikan dengan dosis 15-
25 mg/kg BB dan maksimal kira- kira 1 g untuk sebagian besar
orang dewasa setiap 6-8 jam. Pemberian dilakukan 2 jam sebelum
perawatan dan dilanjutkan hingga 7-10 hari setelah perawatan.
Asam traneksamat sediaan obat kumur diberikan tepat sebelum
perawatan dan berlanjut setiap 6 jam selama 7-10 hari. Pada orang
dewasa, obat dikumur selama 2-3 menit kemudian ditelan atau
dibuang, sedangkan pada anak-anak obat setelah dikumur harus
dibuang.7

·        Understand laboratory result  bleeding  disorders  , vascular

disorder and platelets disorder