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MC II Exam 2 > Hemophelia > Flashcards

Hemophelia Flashcards

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
Q

Discuss the use of Fusion/Arthrodesis in treating patients with hemophelia

A

Late in the course of joint destruction
Usually ankle or knee
Usually does not require physical therapy

26
Q

Discuss the use of Total Joint Replacement in treating patients with hemophelia

A 
In end stage joint disease
Usually knee or hip
Can do shoulder, elbow and ankle
5-10 day hospital stay
Aggressive physical therapy
27
Q

Discuss the use of elbow prostheses in patients with hemophelia

A

Not typically done in hemophilia patients

Lifting restrictions

28
Q

Post-op Care

A

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
Q

What positive changes are occuring with respect to the management of hemophelia?

A

Shorter hospital stays for total joint surgery
Longer acting factor products coming into the market place
Prevention of orthopaedic complications

30
Q

What is the primary manifestation of hemophelia?

A

Bleeding into the joints

31
Q

What are the deleterious effects of bleeding in the joints in hemophelia?

A

Degeneration of articular cartilage and severe joint athropathy over time
Loss of joint range of motion, strength and function and severe pain

32
Q

How long does it take for bleeding into a joint to produce adverse effects (in vitro studies)?

A

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
Q

How long will it take for the effects of bleeding into a joint to be irreversible (in vitro studies)?

A

After 2 days of exposure to blood load of 10% v/v, adverse effects on cartilage were NOT reversible

34
Q

What are the major findings from an in vivo study examining the effects of bleeding into a joint?

A

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
Q

Which joints are typically involved in patients with hemophelia?

A 
Knees
Elbows
Ankles
Shoulders 
Hips
36
Q

What is evaluated in an acute bleed in patients with hemophelia?

A 
Swelling
Temperature
Pain
Active motion
Muscle function
Circumference
Gait
Nerve involvement
37
Q

Rehab immediately following an acute bleed in hemophelia?

A

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
Q

What 4 criteria can contraindicate weight bearing in a patient with hemophelia?

A

When blood is in the joint
Flexion position > 15º (knee or hip)
Weaker than Grade 4 quadriceps
Pain is minimal or absent

39
Q

What happens when a joint that has blood in it is loaded?

A

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
Q

What is the general exercise progression for patients with hemophelia?

A

Isometric
Gentle active motion
Progress to resistive

Avoid passive motion

41
Q

What sorts of foot/ankle orthotic devices are used for patients with hemophelia?

A

Rigid, Lace-Up, Neoprene, and Compressive ankle supports
Custom functional foot orthoses - Fit to position of comfort
Floor-reaction carbon fiber AFO

42
Q

What differential diagnosis must be performed for a patient with hemophelia with a hip issue?

A

Hip joint bleed vs. iliopsoas muscle bleed vs non-bleeding-related pathology (e.g. appendicitis)

43
Q

What are unique aspects of a hip joint evaluation in patients with hemophelia (as opposed to other joints)?

A 
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
Q

What is involved in a muscle bleeding evaluation?

A

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
Q

How long does it take for the body to complete repair of a muscle?

A

20-40 days

46
Q

What are the major treatments for muscle rehab following a muscle bleed?

A

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
Q

What is important to avoid when rehabbing from a muscle bleed?

A

Recurrence - occurs easily in patients with hemophelia

48
Q

What is a possible complication of a forearm bleed?

A

Volkmann’s Ischaemic Contracture

49
Q

Is compartment syndrome a medical emergency?

A

Yes

50
Q

Where do compartment syndromes commonly occur?

A

Forearms and shins

51
Q

True or false, small bleeds can cause ischemia and affect nerve function

A

True

52
Q

What rehab considerations must be made for patients with hemophelia who also have inhibitor antibodies?

A

More likely to immobilize or immobilize longer
Rest period may be longer
Exercise started later, less aggressively and progressed more slowly

53
Q

Why might ice not be helpful in treating patients with hemophelia and an acute bleed?

A

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
Q

What is the current recommendation on using ice in treating patients with hemophelia and an acute bleed?

A

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
Q

What modalities should not be used to treat an acute bleed?

A

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
Q

What principles should be used when weight training a patient with hemophelia?

A

Begin slowly – set realistic goals
Low load – high repetitions
Work in pain-free arcs

57
Q

What are the benefits of splinting following an acute bleed in a patient with hemophelia?

A

Helps control pain
Helps “quiet” the area
May improve healing time

58
Q

True or false, splinting following an acute bleed in a patient with hemophelia is a long-term treatment

A

False, Short-term intervention - 24-48 hours

59
Q

When should you use splinting following an acute bleed in a patient with hemophelia?

A

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
Q

Bleeds involving which body parts require more aggressive treatment?

A

Any joint bleed - esp. shoulder and hip

Muscle bleeds that may cause compartment syndromes - psoas, gastroc / soleus, forearm mm, tibialis anterior

61
Q

When should a PT involve an orthopedist in managing a patient with hemophelia?

A 
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
Q

True of false, patients with hemophelia may minimize their pain on a pain scale

A

True, often report lower number than “typical” patient

MC II Exam 2 (5 decks)

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