Hemophilia

Clinical manifestations

-Bleeding from anywhere in the body -Hemorrhage from trauma -Excessive bruising -SubQ and IM bleeding -Hemarthrosis (bleeding into joints) -Hematomas -Spontaneous hematuria -Internal bleeding -Intracranial bleeding -Episaxis -Tooth extractions

Hemophilia B (Christmas disease)

-Deficiency in factor IX -15% of all cases

Hemophilia A (classic hemophilia)

-Deficiency of Factor VIII aka AHF (antihemophilic factor) -Factor VIII is made by the liver and is necessary for the formation of thromboplastin in Phase I of the blood coag process. accounts for -80-90% of all cases -Occurs in 1/5000 males -X linked recessive trait - only males are affected - females are carriers of the disease. -Degree of bleeding depends on the amount of clotting factor they have (AHF) and severity of the given injury.

treating hemophilia

-Treated by giving clotting factors found in plasma - given prophylactically on a regular basis. -DDAVP - DESMOPRESSIN - for mild hemophilia A - increases Factor 8 by 2-4 times. If trauma happens at home patients and their family are taught how to administer the AHF -For superficial bleed apply pressure for at least 15b min. -RICE - rest, ice, compression, elevation -Elevate and immobilize joint, ROM after bleeding stops to prevent contracture -Obesity places pressure on joints -Close supervision of children -soft toothbrush, no floss only water pic -Only use electric razor -Wear medical ID bracelet

The client with hemophilia A is experiencing hemarthrosis. Which intervention should the nurse recommend to the client? 1. Alternate aspirin and acetaminophen to help with the pain. 2. Apply cold packs for 24 to 48 hours to the affected area. 3. Perform active range-of-motion exercise on the extremity. 4. Put the affected extremity in the dependent position.

1. Alternate aspirin and acetaminophen to help with the pain.

Which sign/symptom should the nurse expect to assess in the client diagnosed with Hemophilia A? 1. Epistaxis 2. Petechiae. 3. Subcutaneous emphysema. 4. Intermittent claudication.

1. Epistaxis

Which measures would the RN know to avoid when planning care for a child with hemophilia? (Select all that apply) 1. Frequent blood pressures 2. Rectal suppositories 3. Rectal temperatures 4.Administering aspirin for pain 5. Carbonated beverages

1. Frequent blood pressures 2. Rectal suppositories 3. Rectal temperatures 4.Administering aspirin for pain 5. Carbonated beverages

While administering a blood transfusion to a child with a hematologic disorder, the nurse notes the child develops urticaria and wheezing. Which collaborative interventions will the nurse begin? Select all that apply. 1. Obtain a blood culture. 2. Give an antihistamine. 3. Discontinue the transfusion. 4. Administer a diuretic. 5. Apply oxygen as needed.

1. Obtain a blood culture. 2. Give an antihistamine. 3. Discontinue the transfusion. 4. Administer a diuretic. 5. Apply oxygen as needed.

The nurse is reviewing the results of a clotting study for a healthy 6-year-old. Which of the following would the nurse document as a normal prothrombin finding? a) 16.0 to 18.0 seconds b) 6.0 to 9.0 seconds c) 11.0 to 13.0 seconds d) 21.0 to 35.0 seconds

11.0 to 13.0 seconds Explanation: The nurse would identify a prothrombin time of 11.0 to 13.0 seconds as normal for a healthy child. A result of 21.0 to 35.0 seconds would be the expected range for partial thromboplastin time and activated partial thromboplastin time. Findings of 6.0 to 9.0 seconds and 16.0 to 18.0 seconds are outside the normal range.

Which nursing interventions should the nurse implement when caring for a client diagnosed with hemophilia A? Select all that apply. 1. Instruct the client to use a razor blade to shave. 2. Avoid administering enemas to the client. 3. Encourage participation in noncontact sports. 4. Teach the client how to apply direct pressure if bleeding occurs. 5. Explain the importance of not flossing the gums

3. Encourage participation in noncontact sports. 4. Teach the client how to apply direct pressure if bleeding occurs. 5. Explain the importance of not flossing the gums

The (UAP) asks the primary nurse, "How does someone get hemophilia A?" Which statement would be the primary nurse's best response? 1. "It is an inherited X-linked recessive disorder." 2. "There is a deficiency of the clotting factor VIII." 3. "The person is born with hemophilia A." 4. "The mother carries the gene and gives it to the son."

4. "The mother carries the gene and gives it to the son."

The physician has ordered several laboratory tests to help diagnose an infant's bleeding disorder. Which of the following tests, if abnormal, would the nurse interpret as most likely to indicate hemophilia? 1. Bleeding time 2. Tourniquet test 3. Clot retraction test 4. Partial thromboplastin time

4. Partial thromboplastin time

A woman is aware that she is the carrier of a sex-linked recessive disease (hemophilia A); her husband is free of the disease. What frequency of this disease could she expect to see in her children? A) All male children will inherit it. B) All female children will be carriers like she is. C) There is a 50% chance her male children will inherit the disease. D) There is a 50% chance her female children will inherit the disease.

Ans: C Feedback: With X-linked inheritance, there is a 50% chance male children will be affected. There is a 50% chance female children will be carriers of the disease.

Hemophilia

Hereditary bleeding disorders that result from deficiencies of clotting factors. Diagnosing: -Evidence of x-linked inheritance -Diagnosis is made from a hx of bleeding episodes and lab testing such as PTT

A nurse is caring for a 7-year-old boy with hemophilia who requires an infusion of factor VIII. He is fearful about the process and is resisting treatment. How should the nurse respond? a) "Will you help me apply this band-aid?" b) "Please be brave; we need to stop the bleeding" c) "Would you help me dilute this and mix it up?" d) "Would you like to administer the infusion?"

c) "Would you help me dilute this and mix it up?" Explanation: The best response for a 7-year-old is to use distraction and involve him in the infusion process in a developmentally appropriate manner. A 7-year-old is old enough to assist with the dilution and mixing of the factor. Asking for help with the band-aid would be best for a younger child. Teens should be taught to administer their own factor infusions. Telling him to be brave is not helpful and does not teach.

An 11-year-old male is diagnosed with mild hemophilia. Upon assessment, the nurse documents the following factor level for this category of hemophilia: a) Factor level less than 1% b) Factor level of 1% to 5% c) Factor level of 5% to 50% d) Factor level greater than 50%

c) Factor level of 5% to 50% Explanation: Mild hemophilia is characterized by a factor level of 5% to 50%. People with mild hemophilia experience prolonged bleeding only when injured. Thus, their condition may not be diagnosed unless they have trauma or surgery.