Hem
Hem
R = Replacement of clotting factor. Treat with factor as directed by your treatment centre.
R = Rest. Do not use this joint while it is painful or swollen. Use crutches or a wheelchair
when there is a joint bleed in the leg. Use a sling to rest arm bleeds
I = Ice. Ice may help to decrease pain. An ice pack, gel pack or crushed ice can be used.
Always wrap the ice in a damp towel first; do not apply ice directly to the skin. For best
relief, wrap the ice completely around the joint. For small joints, leave the ice on for 5-10
minutes; for larger joints, up to 20 minutes may be needed. Reapply the ice every two hours
for the first two to three days, or until the pain has decreased. Do not use ice if it is making
the pain worse.
I = Immobilization. If the joint is very swollen or if movement is lost, a splint or half cast
may be used to protect the joint. Splints can also be useful for young children who may not
understand that they need to rest. Your HTC/physiotherapist will advise you what type of
immobilization should be used and for how long.
C = Compression. A tensor bandage or an elastic sleeve will support the joint and help to
control the swelling; however compression may not be tolerated if the joint is very tender.
Circulation must be watched carefully. If there is any numbness or tingling, remove the
compression immediately.
E = Elevation. Keeping the injured part six inches above the level of the heart will decrease
the pressure on the damaged blood vessels and help slow the swelling.
For people with hemophilia, the WFH recommends the use of antifibrinolytic drugs (e.g., tranexamic
acid, epsilon aminocaproic acid [EACA]) alone or as adjuvant treatment, particularly in controlling
mucosal bleeds and for invasive dental procedures.
LIFE THREATENING BLEEDS
What is Hemophilia?
Hemophilia is a genetic disorder characterized by a deficiency or absence of one of the
clotting proteins in plasma. The result is delayed clotting. Deficiencies of factor VIII (8)
Hemophilia prevents the formation of a firm, fibrin clot and results in a soft, unstable clot.
Persons with hemophilia do not bleed faster than others; rather the bleeding is continuous.
Clinical diagnosis
• Hemophilia should be suspected in individuals presenting with a history of any of these symptoms: – easy
bruising; – “spontaneous” bleeding (i.e., bleeding for no apparent/known reason), particularly into the joints,
• Early symptoms of joint bleeds in children at a very young age are a key indicator of severe hemophilia.5 (See
• If hemophilia is suspected, the clinician should obtain the patient’s bleeding history and family history of
abnormal or unexplained bleeding experienced by any siblings or maternal male relatives (i.e., maternal cousin,
uncle, or grandfather) to assess patterns of inheritance and assist with diagnosis. • Accurate diagnosis of
Severity of deficiency:
The amount of bleeding expected in an individual with hemophilia depends upon the severity of the deficiency.
Normal plasma levels of factor VIII (8) and IX (9) range from 50-150%.
Those with < 1% factor VIII (8) and IX (9) are considered to have severe hemophilia.
Persons with factor levels of 1-5% are considered to have moderate hemophilia.
Persons with > 5% factor activity are considered to have mild hemophilia .
Some carrier girls and women (called symptomatic carriers) can have lower than normal plasma levels of factor
VIII (8) or IX (9) and thus may exhibit symptoms of mild hemophilia.
Oral Cavity:
Bleeding in the mouth can be hard to control. The patient will probably need factor. A single infusion of a
routine dose of factor* may temporarily stop the bleeding, but clots break down normally on days 3-5 and
bleeding may start again at that time. An anti-fibrinolytic may be indicated to maintain hemostasis. A modified
diet should be started at the same time as factor therapy. Bleeding may occur with erupting or exfoliating teeth.
It is more common with exfoliating teeth, especially a tooth that is very loose.
1.Diet should be restricted to soft, cool, or lukewarm foods until the area is fully healed.
2. Avoid milk products and foods made with milk. Milk products may contribute to clot breakdown and may
also cause nausea and vomiting if the patient has swallowed blood.
3. Avoid using a straw. Negative pressure from the sucking action can dislodge the clot and aggravate the
bleeding site.
4. Avoid hard foods like chips, popcorn, tacos, etc.
Inhibitors:
An inhibitor is an antibody that some individuals with hemophilia develop against factor VIII (8) or IX (9).
These antibodies neutralize the factor procoagulant activity, thus counteracting the desired effect of an infusion
of factor concentrate.
How inhibitors are measured?
Labs express the presence of an inhibitor in terms of Bethesda units (BU). One Bethesda unit is the amount of
antibody that destroys half of the factor VIII in an equal mixture of normal and patient plasma in two hours.
Indications for inhibitor testing
• After initial factor exposure
• After intensive factor exposure, e.g., daily exposure for more than 5 days
• For recurrent bleeds or target joint bleeds, despite adequate CFC replacement therapy
• For failure to respond to adequate CFC replacement therapy
• For lower than expected factor recovery or half-life after CFC replacement therapy
• For suboptimal clinical or laboratory response to CFC replacement therapy
• Before surgery
• For suboptimal post-operative response to CFC replacement therapy
– bleed recognition;
– record-keeping of bleeds and treatment;
– self-administration of CFCs or other hemostasis products;
– self-care (i.e., nutrition and physical fitness)
-medicines management (i.e., record-keeping, treatment routines, maintenance of adequate treatment supply,
proper storage, reconstitution, and administration of treatment products);
– pain management;
– risk management;
– participation in outcome reporting and documentation
People with hemophilia may have an increased risk of low bone mineral density compared to the general
population due to risk factors including hemophilia severity and hemophilic arthropathy and resulting
immobility.Ways to promote bone health include preventing hemarthrosis, regular exercise, and adequate
vitamin D and calcium intake.
• For those with significant musculoskeletal dysfunction, weight-bearing activities that promote development
and maintenance of good bone density should be encouraged to the extent their joint health permits.
• Non-contact sports such as swimming, walking, jogging, golf, badminton, archery, cycling, rowing, sailing,
and table tennis should be encouraged.
• High-contact and collision sports such as soccer, hockey, rugby, boxing, and wrestling, and high-velocity
activities such as motocross racing and skiing are not advised due to the potential for life-threatening injuries,
unless the individual is on adequate prophylaxis to cover such activities and is well educated on the potential
risks.
• Ideally, individuals with hemophilia (or their family caregivers) should consult a physical therapist before
engaging in new sports and physical activities to discuss their appropriateness, required protective gear,
prophylaxis (factor coverage and other measures), and required physical skills prior to beginning the activity.
This is particularly important if the individual has any joint with recurrent bleeding (i.e., target joint).
• Target joints can be protected with braces or splints during physical activity, especially in the absence of factor
coverage.
CIRCUMCISION:
• In patients with hemophilia, the circumcision procedure should be performed electively by an experienced
surgeon and hematology team in a resourced hematology treatment centre with access to clotting factor
concentrates.
• In patients with hemophilia, the plasma factor level should be raised to 80-100 IU/dL just prior to the
procedure.
• In patients with hemophilia undergoing circumcision, intraoperative care should be taken to cauterize all
bleeding vessels.
• For patients with hemophilia undergoing circumcision, the WFH suggests use of topical fibrin sealant as an
adjunctive therapy, using a product manufactured with robust viral reduction/inactivation processes if available,
to minimize the risk of bloodborne pathogen transmission.
• For patients with hemophilia undergoing circumcision, the WFH recommends adjusting clotting factor
replacement to the clinical course of the procedure. If continued clotting factor replacement is required, the goal
would be to maintain factor levels above 50 IU/dL for the first 3 days, and above 30 IU/dL for the subsequent 4-
8 days
• In patients with hemophilia post-circumcision, inhibitor measurement should be repeated if there is intractable
bleeding that is poorly responsive to replacement therapy and local hemostatic measures. • In patients with
hemophilia post-circumcision, nondissolvable stitches (if used) should be removed 10-14 days postsurgery; the
inevitable bleeding should be managed with clotting factor replacement. • In hemophilia patients with
intractable bleeding postcircumcision, all angles should be considered, including blood vessel bleeding, clotting
factor deficiency, and platelet abnormalities.
Vaccinations:
Should we include: