Hemophilia

Question 1

Hemophilia

Description & Epi & Etiology

Answer 1

A bleeding disorder in which a person’s blood does not clot normally leading to delays in coagulation after injury
- Not bleeding profusely rather just bleeding for longer
- Majority of people with hemophilia have severe hemophilia & may bleed spontaneously or w/ slight trauma (especially iinto the joints & deep mm)

M>F
- Gene for clotting is on the “X” chromosome - inherited

Typically hereditary, rarely aquired (result of an autoimmune disease attacking the clotting factors

Question 2

Clinical Manifestations

(6)

Answer 2

1. Hematoma formation (may result from injections or firm holding) - can be from relatively minor contact
2. Excessive bruising from minor trauma
3. Delayed hemorrhage after a moinor injury
4. Persistent bleeding after cuts
5. Hemarthrosis (bleeding in the joints)
   Very common manifestation of hemophilia
6. Episodes of spontaneous bleeding into joints, muscles, & internal organs (does not commonly occur in mild-moderate hemophilia - more severe cases)

Question 3

Hemarthrosis

Def + (6)

Answer 3

Bleeding into the joint spaces

1. Most common clinical manifestation of hemophilia
2. Significantly affects synovial joints
3. Joints that have recurrent bleedings are known as “Target Joints”
4. Most commonly affected joint is the knee, followed by: ankle, elbow, hip, shoulder & wrist
5. Joints with hemarthrosis become distended causing pain, swelling, warmth, stiffness (Not only d/t swelling that occurs in there BUT also the blood in the joint space makes it more difficult to move)
6. Blood is an irritant to the synovium, causing inflammation of the synovium (synovitis)
   Chronic synovitis may lead to erosive damage of the cartilage = OA (very severe)

Question 4

Intramuscular Hemorrhage

Def & S/S

Answer 4

Bleeding into the muscles

May lead to:
- Pain
- Swelling
- Limitations of motion
- Protective spasm
- Warmth
- Palpable hematoma
- Neurological signs (numbness & tingling) < swelling & bleeding begins to irritate a nerve (compresses a nerve in the area)

Bruising is not always present & may be ONLY indicative of a superficial soft tissue bleed

Question 5

Medical Management

(3)

Answer 5

1. Factor replacement therapy
2. Pain medications - AVOID ibreprofen & Advil because they are blood thinners
3. Corticosteriod to treat chroinc synovitis

Question 6

Rehabilitation Management of Active Bleed:
Joint (Hemarthosis): Acute Stage

(6)

Answer 6

1. Factor replacemet
2. Pain medication
   1-2 = outside scope of practice
3. RICE - help to reduce swelling
4. Pain-free movement - help maintain ROM
5. Non (or minimal) weight-bearing - want to find strategies to reduce wt bearing - ex prescribe an assistive aid
6. Splinting & support as appropriate

Question 7

Rehabilitation Management of Active Bleed:
Joint (Hemarthosis): Subacute Stage

(3)

Answer 7

1. Factor replacement (if indicated)
2. Progressive weight-bearing, movement, and other exercsies
3. Wean from splints and slings

Question 8

Rehabilitation Management of Active Bleed:
Muscle (Intramuscular Hemorrhage): Acute Stage

(4)

Answer 8

1. Factor replacement
2. RICE
3. Progressive movement
4. Appropraite weight-bearing status
   ** Recommend or advise to help DEC irritation in that area to allow for healing

Question 9

Rehabilitation Management: Post-Bleed

(5)

Answer 9

1. Education
   Benefits of exercise - what they can do safely
   Injury risk - activites that have a higher risk of injury
   Protection strategies - joint protection (similar to RA) - equipment to help prevent bleeding episodes
2. Isometric mm exercises - try to prevent atrophy of mm
3. Slowly progressing strengthening exercises when joint is pain free though full ROM
4. Slow, progressive Return to weight bearing activities
5. Proprioception exercises
   ACTIVE bleed = swelling & bleeding in the joint = affects propripception deep receptors

Question 10

Rehabilitation Considerations

(5)

Answer 10

1. The use of heat is contraindicated during a joint or mm bleed
2. Joint manipulation is contraindicated
3. Contact sports such as hockey, football, boxing, & wrestling are potentialy dangerous and are NOT recommended
4. Activities with a high risk of falls or serious traumatic injury such as riding a motorcycle are not recommended
5. Heavy weight lifting & powerlifting is not recommended d/t micro-tears in the msucles and risk of intramuscular bleeds
   - Heavy = excessive micro-tearing - risks of the training outweight the benefits. Advised against.
   - Eccentrics = should also be cautioned b/c of the extra micro-tearing = more difficult for hemophilia pts to recover from