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23 Cards in this Set
- Front
- Back
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What is hemophilia and what are the 2 most common types?
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X-linked disorders of blood coagulation
Factor VIII (hemophilia A) - 80 to 90% Factor IX (hemophilia B) - 9% |
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Most diabling consequence of hemophilia?
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Joint arthropathy from repeated hemarthroses
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What determines severity of hemophilia?
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Amount of factor in the blood
Mild = 5-25% of factor present Mod = 1-5% of factor present Severe = <1% factor present |
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What percentage of A and B have the "severe" form of hemophilia?
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60% of A
50% of B |
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Most common symptom of severe form of hemophilia?
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Recurrent hemorrhages
Spontaneous, from minor trauma |
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Most common places for hemorrhage with hemophilliacs?
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1. Joint cavity
2. mm (iliopsoas, gastroc, flexor forearm compartment) 3. Hematuria, mucous membrane, CNS |
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What joints does hemarthrosis effect? S/s?
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Hinge joints: knee, elbow,ankle
Joint bleeding: warm, swollen, painful, ROM restrction Recurrent episodes can lead to chronic synovitis and degenerative arthropathy |
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5 stages arthropathy from hemophilia
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I: no bony abnormalities, swelling present
II: osteoporosis and overgrowth of epiphyses, but integrity maintained III: visible subchnodral cysts, patellar squaring, intercondylar notch widened, trochlear notch widened when elbow involved. Articular cartilage still intact IV: joint space narraowed nad articular cartilage damage V: fibrous joint contracture, loss of jt space, total aticular cartilage destruction |
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Medical mngt of hemophilia
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Factor replacement to stop bleeding episodes
IV, extracted from normal plasma or manufactured fom recombinant DNA |
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Prodromal sensations
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Feel stiffness in joints... you know you need your factor
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What to do if hemophilia is actively bleeding in joint?
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Rest
Immobilization Avoid WB |
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When does joint destruction usually occur in hemophiliacs?
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Late adolescence/early adulthood
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Problems associated with hemophillia?
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Contractures
mm weakness Dec'd proprioception LLD Pain |
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Things to include in eval of hemophiliac
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Start with functional AROM
Freq of bleeding and locations Joint deformities Gait Balance/coordination Pain |
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PT intervention for hemophiliacs?
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Pain relief (biofeedback, TENS)
ROM Positioning Joint protection strategies Individualized fitness programs AD to relieve joint stress |
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Contraindications for hemophilliacs
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Heat, soft tissue, high level activity
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3 categories of activity for hemophiliac?
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Rec'd: aquatic, cycling
Benefits>risks = walking Risks>benefits = boxing, hockey, football |
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What is rickets? who is at high risk for development?
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VIT D deficiency that interferes with bone calcification
Mothers who breast feed a lot, kids who don't get enough sun or have some processing dysfxt 6-24 mo. is high risk period |
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Characteristics of rickets?
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bow legged (varus knees)
Possible hypotonia Possible spinal curvatures |
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Issues with rickets?
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Cosmesis (<4 can brace kids knee)
Can inerfere with play and ADLs Skeletal deformities, dental issues, seizures, short stature, mo. delay all possible |
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What is scurvy?
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Vit C (ascorbic acid) deficiency seen more in teenagers
Vit C imp for collagen formation (dec bone thickness wit out it) Can happen if weened to quickly from breast milk to cows milk |
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s/s scurvy
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Bleeding gums, loose teeth, stiff LE joints, slow wound healing, anemia
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Interventions for scurvy
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WB activities once approp Vit C supplementation
Possible splinting |
