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Physiotherapy Management On Hemophilia

Introduction

Hemophilia is the blood clotting disorder caused by deficiency of clotting factor in the blood. Some study concluded that Hemophilia is one of hereditary genetic disorders. It can occur to anyone. Hemophilia is more likely to occur in males than females.

Clotting factor is used to stop the bleeding when the blood vessel is broken. The clotting factor will stimulate the blood vessel to constrict when the bleeding happens. The cell will aggregate on each other until they stop the bleeding (PICTURE 1).

The coagulation activity will be impaired when abnormal clotting process occurs (PICTURE 1.).

How Hemophilia Occur?

The coagulation factors needed for a normal clotting process. People with Hemophilia have lower clotting factor level of blood plasma. PICTURE 1 shows the comparison of the blood clotting process among normal and the person with Hemophilia.

Normal person have normal clotting factor level of blood plasma that needed for a normal clotting process.

 Example - Bleeding in normal person
Example: Bleeding in normal person (PICTURE 1)
Source: genetic-disorder-wiki.wikispaces.com
Normal Hemophilia
a)    Injured blood vessel will cause bleeding.

b)    Constriction of the blood vessel occurs.

c)     Injured vessel blocked by trombosit.

d)    Coagulation factors will stimulate the fibrin formation which is necessary to maintain the blood clot.

 

a)  Thus when a blood vessel is injured and forms the temporary scab

b)  Constriction of the blood vessel occurs.

c)   Injured vessel blocked by trombosit.

d)  Missing coagulation factors prevent fibrin formation, which is necessary to maintain the blood clot. A hemophiliac does not bleed more intensely than a person without it, but can bleed for a much longer time.

Factors

There are two types of Hemophilia:

  1. Hemophilia A

Hemophilia A (clotting factor VIII deficiency) is the most common forms of the disorder. From the statistic by International Hemophilia Congress of United State, 85% of Hemophilia are from this group.

2. Hemophilia B

Hemophilia B occurs with factor IX deficiency.

Aim of Physiotherapy Management

  1. To control the pain and inflammation
  2. To maintain available affected and unaffected joint range
  3. To increase the affected joint range
  4. To strengthen the affected muscle
  5. To assist in maximum daily function
  6. To prevent deformity

Signs & Symptoms

Hemophilia can cause spontaneous and prolong bleeding at any muscle, joint and internal organ. The complication is not only pain but can cause handicap and death.

Complications

  • Limitation in daily activity due to pain
  • Spontaneous bleeding over the joint
  • Inflammation over the joint cartilage that can cause deformity if not treated
  • Muscle atrophy (wasting) can cause weakness if not used
  • Muscle shortening can cause contracture if not stretched
  • Brain bleeding can cause permanently paralyze and mental retardation if not treated early
  • Bleeding inside the important muscles of pelvic

Prevention

  • Avoid participation in high loading activity eg jumping and contact sports eg soccer, rugby, boxing, jumping, basketball, skating, skiing etc
  • Must always be cautious and carefull to prevent any injury in sport
  • Low impact activity eg cycling and walking and non contact sports eg bowling, tennis, jogging, snooker, golf, ping pong, darts are advisable

Physiotherapy treatment

  1. Acute Phase (bleeding)
i Rest : Support and rest the affected limb on the pillow (PICTURE 2)
ii Ice : Wrap the affected joint with the ice & let it for 15-20 minutes. Do not apply any heat ointment before the treatment to prevent any complication
iii Support : Support the affected joint with the splint for enhance the healing process (PICTURE 3)
iv Exercise : Keep moving the unaffected joint to maintain optimum joint range and muscle length
Example - Support the limb  splinting
Example: Support the limb (PICTURE 2) Example: Splinting (PICTURE 3)
Source: piratstudenterna.se Source: www2.aofoundation.org

2. Subacute Phase (reduction of pain & inflammation)

i Rest : Support and rest the affected limb on the pillow
ii Ice : Wrap the affected joint with the ice & let it for 15-20 minutes. Do not apply any heat ointment before the treatment to prevent any complication
iii Support : Apply the bandaging on the affected joint to reduce the inflammation (PICTURE 4)
iv Exercise : a)     Move actively the affected joint to maintain the available range

b)     Keep moving the unaffected joint to maintain optimum joint range and muscle length

Example - Bandaging
Example: Bandaging (PICTURE 4)
Source: painbehindkneetreatment.com

Chronic Phase

i Doctor Management : Replacement factor should be prescribed before exercise to prevent any complication (as doctors’ advice)
ii Exercise : a)     Strengthening exercise to strengthen the affected and unaffected limb

b)     Coordination and balancing exercise to improve body support and trunk control

c)     Gait training to relearn the normal walking pattern

d)     Hydrotherapy to improve whole body function and enhance cardiovascular fitness (PICTURE 5)

Example - Hydrotherapy
Example: Hydrotherapy (PICTURE 5)
Source: www.gosh.nhs.uk

Exercise Prescription:

No Exercise Demostration
1 Mobilising Exercise

 

i Position : Long sitting
ii Instruction : Slide the heel downward & upward
iii Frequency : 5 repetitions (3 sets)
 Long sitting
2 Mobilising Exercise

 

i Position : Lying (on the stomach)
ii Instruction : Bend the knee & slowly lowering it down
iii Frequency : 5 repetitions (3 sets)
 Lying (on the stomach)
3 Strengthening Exercise (without resistance bag)

i Position : Sitting
ii Instruction : Cross the leg & resist the affected leg upward within 10 secs
iii Frequency : 5 repetitions (3 sets)
 Strengthening Exercise (without resistance bag)
4 Strengthening Exercise (with resistance bag)

i Position : Lying (on the back) & affected knee support with a roll towel
ii Instruction : Straighten the knee
iii Frequency : Hold 5 secs (5 reps) (3 sets)
 Strengthening Exercise (with resistance bag)
5 Isometric Exercise

i Position : Lying (on the back)
ii Instruction : Lift up the ankle & full straighten the knee
iii Frequency : Hold 5 secs (5 reps) (3 sets)

 

 

 Isometric Exercise
6 Balancing Exercise

i Position : Standing on the affected leg
ii Instruction : Open the eyes & balance the body and can progress to:

a)   Close the eyes

b)   Soft surface

iii Frequency : Hold 30 secs (3 sets)
 Standing on the affected leg

Example: Exercise Prescription

Source: Jabatan Fisioterapi, Hospital Kuala Lumpur

Reference

  1. Care protocol OA Knee oleh Jawatankuasa Teknikal Profesion Fisioterapi, KKM
  2. Guidelines in the management of Hemophilia and von willebrand disease, Draft copy Pusat Darah Negara, 2004

Source Image

  1. Retrieved Jun 3 2015. genetic-disorder-wiki.wikispaces.com
  2. Retrieved Jun 3 2015. se
  3. Retrieved Jun 3 2015. aofoundation.org
  4. Retrieved Jun 3 2015. com
  5. Retrieved Jun 3 2015. gosh.nhs.uk
  6. (2012) Jabatan Fisioterapi Hospital Kuala Lumpur. Retrieved July 4 2013.
Last Reviewed : 18 January 2016
Writer : Pauzillah binti Hj. Dollah
Translator : Naqiuddin bin Mohd Johar
Accreditor : Se To Phui Lin