Hemophilia, Thrombocytopenia, Sickle Cell Flashcards
hemostasis
- initiating clot formation to stop bleeding
- involves an interaction among the blood vessel wall, platelets and plasma coagulation proteins and a number signaling molecules
Von Willebrand’s Disease
- caused by a lack of or dysfunction of vWF
- can causes excessive bleeding because they do not have the factor to enhance adhesiveness of platelets
- most common inherited bleeding disorder
vWF
von willebrand factor
- released from platelets following vascular injury
hematoma
collection of blood outside of blood vessels
hemarthrosis
blood moving into the joints
hemophilia
bleeding disorder inherited as a sex-linked autosomal recessive trait (X chromosome)
what is hemophilia caused by
lack/deficiency of/in a clotting factor
how is hemophilia classified
according to the percentage of clotting factor present in plasma
when do you see hemophilia with newborns
occur with heel sticks, immunizations, blood draws, circumcision
when do you see hemophilia with adults
- bruising
- bleeding from the mouth or frenulum
- intracranial bleeding
- hematomas of the head
- hemarthrosis with ambulation
when do you see hemophilia in women
- excessive bleeding during menstrual cycle
- abnormal bleeding from bruising
- dental extractions
- abortion/miscarriage
- complication with pregnancy
- nosebleeds
- minor trauma
how would hemophilia affect joints?
- hemarthrosis, most common clinical manifestations
- causes the joint to become distended, causing swelling, pain, warmth and stiffness
how can weight bearing affect the joints with hemophilia?
may further damage the synovium –> hypertrophic synovium which leads to further bleeding, swelling, narrowing of the joint space and joint pain
what would you see with muscle clinical manifestations and hemophilia
- muscle hemorrhages/space occupying
- often flexor muscles
- pain and limited ROM
- can compress neurovascular bundle
hemophilia A
- lack of the clotting factor VIII
- 80% of all cases
hemophilia B
- deficiency of factor IX
- 15% of people
things to remember about hemophilia
- no cure
- avoid injury and meds that promote bleeding
- good nutrition
- good dental hygiene
- IV administration of deficient clotting factor
signs/symptoms of hemophilia
- intracranial hemorrhage
- prolonged nosebleeds
- bruises easily
- warm, painful swollen joints with movement
- GI hemorrhage
- coffee ground emesis
- cola colored urine
- tarry stool
what exercise program is recommended for pt with hemophilia
- regular program
- strength training –> increase strength, stabilize joint, improves circulation, reduce risk of injury & spontaneous bleeding
- pain free ROM
what is not recommended within exercise program for pt with hemophilia
- greater BMI increases risk of joint injury
- avoid contact sports, rock climbing, wrestling
- no body building, powerlifting, competitive weightlifting
- 1RM testing
what are early signs of bleeding with hemophilia
- stiffening into the position of comfort
- decreased ROM –> swelling
- pain/tenderness
- joint swelling with increased warmth
- gradually intense pain
- loss of sensation
thrombocytopenia
platelet count below 150,000
causes of thrombocytopenia
- metastatic neoplasms
- leukemia
- lymphoma
- myeloma
- bacterial infections
- viral infections
- myelofibrosis
- myelodysplatic syndrome
- drugs
- ending a chemo regime
S&S of thrombocytopenia
- mucosal bleeding
- nose
- brushing ones teeth
- GI tract (melena)
- urinary tract (hematuria)
what is important for PTs when working with pt’s who have thrombocytopenia
- can cause bleeding into the muscles or joints
- take BP cautiously
- exercise: type, intensity
- AE of aspirin and NSAIDs on platelet aggregation
hemoglobinopathies
sickle cell disease or sickle cell anemia
sickle cell disease
- autosomal recessive disorder
- abnormal form of Hb namely hemoglobin S
- causes RBC to “sickle”
sickle cell crisis
- strenuous or excessive exercise
- occur in low O2 situation
- reduced pH
- hypovolemia
- sudden change in temp
- too many cells sickle and stiffen
- vaso-occlusion –> down stream ischemia –> painful
primary pathophysiologic features of sickle cell disorders
- chronic hemolytic anemia
- vaso-occlusion
- restricts blood flow to an organ –> ischemia, pain, necrosis, organ damage
- stroke
- pulmonary defects
sickle cell disease and PT involvement
- teaching joint protection/prescribing assistive devices
- patient education
- movement to combat serious pulmonary and other systemic complications
- post troke management
- aquatic therapy
