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MC II Exam 2 > Hemophelia > Flashcards

Hemophelia Flashcards

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1
Q

Define hemophelia

A

A group of hereditary bleeding disorders in which there is a deficiency of one of the factors necessary for coagulation of blood

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2
Q

Define hemophelia A

A

Absence or deficiency of FVIII

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3
Q

Define hemophelia B

A

Absence or deficiency of FIX

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4
Q

Define Von Willebrands

A

vWHF is missing or faulty

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5
Q

What is the inheritance pattern of hemophelia?

A

X-linked, carried on the X chromosome
Male are affected
Women are carriers
20% of carrier women have decreased levels as well

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6
Q

Explain the role of inheritance vs. mutation in hemophelia

A

The disease is mainly hereditary, but up to 30% of cases are caused by spontaneous mutation

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7
Q

How is the severity of hemophelia determined?

A

Severe is < 1% factor level
Moderate is > 1 - 5% factor level
Mild is > 5% factor level

Side note - 50-150% factor level is considered normal

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8
Q

What are the common hemorrhage sites in hemophelia?

A

soft tissue, muscle, joint

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9
Q

What types of bleeds are considered life threatening in hemophelia?

A

head, neck, abdomen

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10
Q

How are bleeding episodes treated in hemophelia?

A

Replace the missing clotting factor
RICE
Splinting

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11
Q

What are the types of factor replacement in hemophelia

A

On demand and prophylaxis

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12
Q

What are considerations for factor replacement in hemophelia?

A

Has to be given IV
Venous access is often an issue
May require port for prophylaxis or a PICC line following a surgery
Disruption of family life

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13
Q

What is the purpose of “on demand” therapy in hemophelia?

A

Prompt infusion of the missing clotting factor reduces chronic complications

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14
Q

What is the purpose of prophylaxis therapy in hemophelia?

A

Standard of care for severe hemophilia
Regular infusions to reduce bleeding
Primarily recommended in children
Adults tend to use prophylaxis according to their life style

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15
Q

What are common complications related to hemophelia?

A

Joint destruction
Exposure to plasma viruses
Inhibitor development

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16
Q

Is viral exposure a concern for patients with hemophelia?

A

Yes, but risk has been reduced due to improved viral inactivation procedures and the use of recombinant products

Viruses of concern include HIV, Hepatitis, CJD,
Parvovirus

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17
Q

What are “inhibitors” with respect to patients with hemophelia?

A

Circulating antibody to factor VIII or IX

Affects 20-30% of severe FVIII hemophiliacs

Affects 1 - 3% of severe FIX patients

Treatment - increase factor, bypassing products,
immune tolerance

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18
Q

What are the steps in the “bleeding cycle” in hemophelia?

A

Hemarthrosis
Hypertrophy and inflammation of the synovium( after 2-3 hemarthrosis)
Release of proteolytic enzymes
Damage to articular cartilage

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19
Q

What are common non-surgical medical interventions for hemopehlia?

A 
NSAIDs
Prednisone
Alternative medicine
Radionuclide Synovectomy
Intra-Articular Injections
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20
Q

How is PT involved with patients with hemophelia?

A

Baseline assessments
Evaluation of bleeding/treatment
Pre-operative assessments
Post surgical therapy

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21
Q

Why might a patient with hemophelia undergo orthopedic surgery?

A

Decrease the number of bleeding episodes
Decrease pain
Increase ROM
Promote independence

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22
Q

What are necessary elements of preoperative PT assessment in patients with hemophelia?

A 
Education
Pain management
Laboratory studies
Measurements
Gait and transfers
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23
Q

Discuss the use of Arthroscopic Synovectomy in treating patients with hemophelia

A

Early in the course of target joint development
Usually knee and ankle
+ / - overnight hospital stay
Additional physical therapy in most cases

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24
Q

Discuss the use of Open Synovectomy in treating patients with hemophelia

A

Early in the course of target joint development
Ankle, knee, elbow or shoulder
Hospital admission
Additional physical therapy

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25
Discuss the use of Fusion/Arthrodesis in treating patients with hemophelia
Late in the course of joint destruction Usually ankle or knee Usually does not require physical therapy
26
Discuss the use of Total Joint Replacement in treating patients with hemophelia
``` In end stage joint disease Usually knee or hip Can do shoulder, elbow and ankle 5-10 day hospital stay Aggressive physical therapy ```
27
Discuss the use of elbow prostheses in patients with hemophelia
Not typically done in hemophilia patients | Lifting restrictions
28
Post-op Care
Prevent hazards of bedrest and post-op complications Promote comfort, early function, and safe movement Maximum protection Moderate protection/controlled motion Minimum protection/return to function
29
What positive changes are occuring with respect to the management of hemophelia?
Shorter hospital stays for total joint surgery Longer acting factor products coming into the market place Prevention of orthopaedic complications
30
What is the primary manifestation of hemophelia?
Bleeding into the joints
31
What are the deleterious effects of bleeding in the joints in hemophelia?
Degeneration of articular cartilage and severe joint athropathy over time Loss of joint range of motion, strength and function and severe pain
32
How long does it take for bleeding into a joint to produce adverse effects (in vitro studies)?
Adverse effects persisted after initial exposure of ≥ 2 days Exposure for 4 days led to concentration-dependent adverse changes Effects persisted when concentration ≥ 10% v/v
33
How long will it take for the effects of bleeding into a joint to be irreversible (in vitro studies)?
After 2 days of exposure to blood load of 10% v/v, adverse effects on cartilage were NOT reversible
34
What are the major findings from an in vivo study examining the effects of bleeding into a joint?
Day 4 - Proteoglycan (PG) synthesis was inhibited and proteoglycan content of cartilage was  in both groups Degree of inhibition of PG synthesis significantly > in the younger dogs Day 16 – PG synthesis was  in both groups, but more elevated in older cartilage Concluded: Cartilage in younger dogs more susceptible than that in older dogs
35
Which joints are typically involved in patients with hemophelia?
``` Knees Elbows Ankles Shoulders Hips ```
36
What is evaluated in an acute bleed in patients with hemophelia?
``` Swelling Temperature Pain Active motion Muscle function Circumference Gait Nerve involvement ```
37
Rehab immediately following an acute bleed in hemophelia?
Avoid active movement of involved joint in first 24 hours RICE Active exercises for adjacent joints Splint for support and comfort initially, possibly immobilization After 24 hours if factor is helping and some movement is comfortable patient can begin to move within his comfort range
38
What 4 criteria can contraindicate weight bearing in a patient with hemophelia?
When blood is in the joint Flexion position > 15º (knee or hip) Weaker than Grade 4 quadriceps Pain is minimal or absent
39
What happens when a joint that has blood in it is loaded?
Rate of proteoglycan synthesis ↑ Newly formed proteoglycan release ↑ Total loss of proteoglycans from cartilage matrix ↑ Cartilage matrix integrity adversely altered Signs of synovial inflammation None of this was present in non-weightbearing controls
40
What is the general exercise progression for patients with hemophelia?
Isometric Gentle active motion Progress to resistive Avoid passive motion
41
What sorts of foot/ankle orthotic devices are used for patients with hemophelia?
Rigid, Lace-Up, Neoprene, and Compressive ankle supports Custom functional foot orthoses - Fit to position of comfort Floor-reaction carbon fiber AFO
42
What differential diagnosis must be performed for a patient with hemophelia with a hip issue?
Hip joint bleed vs. iliopsoas muscle bleed vs non-bleeding-related pathology (e.g. appendicitis)
43
What are unique aspects of a hip joint evaluation in patients with hemophelia (as opposed to other joints)?
``` Swelling difficult to discern Position of comfort in hip flexion All motions may be painful Rotation may be most pain Possible sequelae: avascular necrosis of the femoral head ```
44
What is involved in a muscle bleeding evaluation?
Description of area of bleeding - size, temperature, degree of swelling - firmness ROM assessment at 2 adjacent joints Assessment of nerve and vascular integrity numbness, weakness, pulse Pain assessment
45
How long does it take for the body to complete repair of a muscle?
20-40 days
46
What are the major treatments for muscle rehab following a muscle bleed?
RICE + Infusion therapy Compression may be contra-indicated if risk of compartment syndrome Support in position of comfort NWB gait Begin movement at one joint at a time Exercise must concentrate on muscle stretching and strengthening
47
What is important to avoid when rehabbing from a muscle bleed?
Recurrence - occurs easily in patients with hemophelia
48
What is a possible complication of a forearm bleed?
Volkmann’s Ischaemic Contracture
49
Is compartment syndrome a medical emergency?
Yes
50
Where do compartment syndromes commonly occur?
Forearms and shins
51
True or false, small bleeds can cause ischemia and affect nerve function
True
52
What rehab considerations must be made for patients with hemophelia who also have inhibitor antibodies?
More likely to immobilize or immobilize longer Rest period may be longer Exercise started later, less aggressively and progressed more slowly
53
Why might ice not be helpful in treating patients with hemophelia and an acute bleed?
Cooling of blood can inhibit platelet aggregation and activate fibrinolysis, impeding the clotting process Systematic review AJSM in 2004 showed conflicting evidence and poorly controlled studies.
54
What is the current recommendation on using ice in treating patients with hemophelia and an acute bleed?
Initial consensus that addition of ice to compression may be no more effective than compression alone Ice used alone to slow bleeding may challenge an already weakened system Bottom line: Recommendations are to give clotting factor, combined with compression and ice
55
What modalities should not be used to treat an acute bleed?
NEVER use heat on an acute bleed, though warm whirlpools very beneficial in treating large muscle bleeds Ultrasound -potential danger - not recommended for use in resorption of blood
56
What principles should be used when weight training a patient with hemophelia?
Begin slowly – set realistic goals Low load – high repetitions Work in pain-free arcs
57
What are the benefits of splinting following an acute bleed in a patient with hemophelia?
Helps control pain Helps “quiet” the area May improve healing time
58
True or false, splinting following an acute bleed in a patient with hemophelia is a long-term treatment
False, Short-term intervention - 24-48 hours
59
When should you use splinting following an acute bleed in a patient with hemophelia?
Any bleed lasting longer than 1-2 days Any joint or muscle that is constantly in a low level state of bleeding Bleeds that don’t resolve after 1-2 treatments with factor
60
Bleeds involving which body parts require more aggressive treatment?
Any joint bleed - esp. shoulder and hip | Muscle bleeds that may cause compartment syndromes - psoas, gastroc / soleus, forearm mm, tibialis anterior
61
When should a PT involve an orthopedist in managing a patient with hemophelia?
``` Concern of compartment syndrome Management of a severe hemarthrosis Concern over true hip bleed Concern over an orthopedic injury Non-responsive joint bleeding Concern of infection Complaint of chronic pain When surgical intervention is indicated ```
62
True of false, patients with hemophelia may minimize their pain on a pain scale
True, often report lower number than "typical" patient

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