Hemophilia

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Hemophilia

• Suzan Tibbitts, RN BSN MSN
• Granite School District Nurse

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HEMOPHILIA

• A rare genetic disorder characterized by the
  deficiency, or the absence of, one of the
  clotting proteins found in blood plasma
• The result is delayed clotting in an affected
  individual
• There is no cure for hemophilia

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INCIDENCE

• Affects one out of every 7,500 live male births
• In the US, approximately 18,000 are affected
• Occurs in all racial, ethnic, and socioeconomic
  groups

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INHERITANCE

• Genetic disorder
• Abnormal gene carried on the X chromosome

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INHERITANCE

• In one third cases, no known prior family history
  exists
• They are thought to be a spontaneous genetic
  mutation

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DIAGNOSIS

• Family history
• Excess bruising
• Prolonged circumcision bleeding
• Confirmed by blood test

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NORMAL COAGULATION

• Injured blood vessels constrict to reduce the
  flow of blood
• Platelets adhere to the capillary surface and
  form a temporary plug
• Plasma clotting proteins (factors) are activated
  in sequence to form a firm fibrin clot

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THE DEFECT

• With hemophilia, the first two clotting stages
  function normally
• Mild bleeding will stop
• Due to decreased or absent factors, the fibrin
  clot fails to form and the end result is a mushy
  clot
• Platelet plug breaks up and bleeding resumes

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LEVELS OF SEVERITY

• Moderate
• 1 to 5 Factor
• Minor trauma
• Mild
• gt 5 Factor
• Surgery or severe trauma

• Severe
• lt 1 Factor
• Spontaneous bleeds

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SITES OF BLEEDS

• Wrist
• Knee
• Ankle
• Toe

• Head
• Mouth
• Shoulder
• Elbow
• Hip

• Usually the bleeds you cant see are the most
  worrisome

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Site of Bleeds

• Hemophilia causes blood to accumulate in the
  joints - an excruciatingly painful condition.

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MAJOR BLEEDS

• Can be life threatening or have serious
  consequences
• Head
• Gastro-intestinal
• Neck/Throat
• Trauma
• Forearms
• Advanced joint bleeds

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MINOR BLEEDS

• Early joint bleeds
• Muscle/soft tissue bleeds
• Mouth/Gums
• Nose bleeds
• Blood in urine

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RECOGNITION OF BLEEDS

• Early signs
• Injury
• Tingling
• Bubbling
• Pain

• Early signs
• Guarding
• Fatigue
• Irritability

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RECOGNITION OF BLEEDS

• Late signs
• Warm to touch
• Stiffness
• Swelling
• Increased pain

• Late signs
• Refusal to straighten joint or bear weight
• Limited movement

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TREATMENT

• IV infusions of deficient Factor
• Releasing stored Factor in mild severity with
  nasal spray
• Episodic On demand

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TREATMENT

• Prophylactic Scheduled
• Port-A-Cath Venous Access Devices common
• Treatment reduces severity

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HIV AND AIDS

• Before 1985, 50 of persons with hemophilia were
  infected with HIV
• No new cases of HIV transmission through factor
  since heat treatments began in 1985

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INHIBITORS

• Body produces Antibodies to Factor
• Replacement Factor seen as foreign
• Inhibitors destroy factor before it is utilized

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BLEEDS AT SCHOOL

• Basic First Aid
• ICE muscles/ joints as necessary
• School Health Plan
• Granite Health Services 685-4246
• Develop care plan with family and Treatment
  Center
• Emergency contact numbers

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RESOURCES

• National Hemophilia Foundation
• 1-800-42HANDI www.hemophilia.org
• Utah Hemophilia Foundation
• 801-484-3205 www.hemophiliautah.org
• Hemophilia Resources of America
• 800-549-2654 www.hrahemo.com
• Treatment Center _at_ PCMC
• 801-588-2680

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Questions?
Call your School Nurse Granite District Nursing
Services 685-4246