Hemophilia (Term 2)
A mother wants to donate her blood to her child who is having surgery, but discovers that she has a different blood type than her child. Which type of blood donation is the child most likely going to receive? Directed blood donation Xenogeneic blood donation Autologous blood donation Allogeneic blood donation
"Allogeneic blood donation" is correct Rationale: Allogeneic blood donation refers to a normal whole blood donation to anyone other than the donor himself. 'Allogeneic' means taken from individuals of the same species, which is how all anonymous blood donation is done in the United States. "Directed blood donation" is incorrect because a directed blood donation involves a person donating blood specifically for the purpose of giving it to a certain person. The donor must meet the same requirements as in a regular blood donation, and all the standard protocols are still in place. The blood must match the recipient's blood and be free of infection. "Autologous blood donation" is incorrect because this involves a person donating blood to be stored for themselves to use later. "Xenogeneic blood donation" is incorrect because xenogeneic refers to an outside species.
A child is brought into the emergency department with severe injuries. The physician orders a transfusion of one unit of whole blood to be administered immediately. The nurse has completed a rapid assessment of the child but there are no laboratory results available to know the child's blood type. Which of the following actions of the nurse is most appropriate? Ask the physician if he ordered a type and crossmatch for the child Administer oxygen and fluids until the laboratory results have returned Contact the blood bank and request one unit of 'O' Rh-negative blood for the child Contact the laboratory and request a cross-link between the lab outcomes and the blood type
"Contact the blood bank and request one unit of 'O' Rh-negative blood for the child" is correct. Rationale: When a patient requires a blood transfusion, the providers must send a sample of his blood for a type and crossmatch to ensure that he receives a matched blood product. In an emergency situation, however, the providers may transfuse 'O' Rh-negative blood without first checking the patient's type or performing a crossmatch, because a delay in this situation would do more harm than an immediate transfusion. "Ask the physician if he ordered a type and crossmatch for the child" is incorrect because this emergent situation requires the nurse to act immediately to get the blood product in the patient as safely and as soon as possible. "Administer oxygen and fluids until the laboratory results have returned" is incorrect because this indicates a delay in a life-saving treatment. The nurse should contact the blood bank for the 'O' negative blood as soon as possible. "Contact the laboratory and request a cross-link between the lab outcomes and the blood type" is incorrect. A type and crossmatch was not done in this situation, so any information obtained by the lab is not useful to match the blood product. The universal donor blood, type 'O' negative, should be given.
A child presents to the ER after falling down the stairs and getting a cut on the knee that won't stop bleeding. The child states, "I always bleed a lot because I am missing a clotting factor." Which of the following conditions does this client most likely have? Wiskott-Aldrich syndrome Idiopathic thrombocytopenic purpura Hemophilia Disseminated intravascular coagulation
"Hemophilia" is correct. Hemophilia is a disorder where the blood is missing certain clotting factors and clotting is abnormal. "Wiskott-Aldrich Syndrome" is incorrect. This is a rare disorder in which there is microthrombocytopenia, meaning that the platelets are small and limited in number causing prolonged bleeding. "Disseminated intravascular coagulation" is incorrect. This occurs because the activation of the clotting cascade causes mini clots to form all throughout the vasculature of the body. "Idiopathic thrombocytopenic purpura" is incorrect. This is an autoimmune disorder where platelets are destroyed and the client is unable to clot. The client will bruise easily.
An 8-year-old child must have a transfusion of whole blood following an acute hemorrhage. As the nurse is preparing the child for the transfusion, which of the following actions is most appropriate to relieve the child's fears about the blood? When the bag arrives, let the child handle it and look at it Talk to the child about what the blood will look like and how it will feel going in before starting the transfusion Cover the bag so that the child cannot see it during the transfusion Ask the parents to hold the child while the blood is being administered
"Talk to the child about what the blood will look like and how it will feel going in before starting the transfusion" is correct. When working with a child who must have a blood transfusion, the nurse can explain to the child what will happen and how the transfusion will feel, if the child is old enough to understand. An 8-year-old child should be able to understand some of the concepts of blood and the body and the nurse can ease some of his fears by talking to him about the blood before starting the transfusion. "When the bag arrives, let the child handle it and look at it" is incorrect because when giving a blood transfusion, allowing a child to handle the bag can result in greater risk of contamination. The nurse should not offer to have the child handle the blood. "Cover the bag so that the child cannot see it during the transfusion" is incorrect. It is important for the nurse to be able to visualize the blood bag during administration. Additionally, a school-age child typically has the ability to understand the reason for their treatment, and since the nurse has explained the procedure in terms the child can understand, there is no need to hide the blood. "Ask the parents to hold the child while the blood is being administered" is incorrect. For a school-age child, it is important to allow the child some control over their body and the situation. Asking a parent to hold the child could increase anxiety in the child.
The nurse is providing home care instructions to the parents of a 10-year-old child with hemophilia. Which sport activity should the nurse suggest for this child? 1. Soccer 2. Basketball 3. Swimming 4. Field hockey
3 Swimming Rationale: Hemophilia refers to a group of bleeding disorders resulting from a deficiency of specific coagulation proteins. Children with hemophilia need to avoid contact sports and to take precautions such as wearing elbow and knee pads and helmets with other sports. The safe activity for them is swimming. Test-Taking Strategy: Focus on the subject, a safe activity. Recalling that bleeding is a major concern in this condition, eliminate options 1, 2, and 4 because these activities are comparable or alike in that they present the potential for injury. Review: Home care and safety instructions for hemophilia
A 10-year-old child with hemophilia A has slipped on the ice and bumped his knee. The nurse should prepare to administer which prescription? 1. Injection of factor X 2. Intravenous infusion of iron 3. Intravenous infusion of factor VIII 4. Intramuscular injection of iron using the Z-track method
3. Intravenous infusion of factor VIII Rationale: Hemophilia refers to a group of bleeding disorders resulting from a deficiency of specific coagulation proteins. The primary treatment is replacement of the missing clotting factor; additional medications, such as agents to relieve pain, may be prescribed depending on the source of bleeding from the disorder. A child with hemophilia A is at risk for joint bleeding after a fall. Factor VIII would be prescribed intravenously to replace the missing clotting factor and minimize the bleeding. Factor X and iron are not used to treat children with hemophilia A. Test-Taking Strategy: Focus on the child's diagnosis. Eliminate options 2 and 4 because they are comparable or alike. Recalling that a child with hemophilia A is missing clotting factor VIII will direct you to the correct option from those remaining.
The nurse analyzes the laboratory results of a child with hemophilia. The nurse understands that which result will most likely be abnormal in this child? 1. Platelet count 2. Hematocrit level 3. Hemoglobin level 4. Partial thromboplastin time
4. Partial thromboplastin time Rationale: Hemophilia refers to a group of bleeding disorders resulting from a deficiency of specific coagulation proteins. Results of tests that measure platelet function are normal; results of tests that measure clotting factor function may be abnormal. Abnormal laboratory results in hemophilia indicate a prolonged partial thromboplastin time. The platelet count, hemoglobin level, and hematocrit level are normal in hemophilia. Test-Taking Strategy: Focus on the subject, laboratory tests used to monitor hemophilia, and note the strategic words, most likely. Recalling the pathophysiology associated with this disorder and recalling that it results from a deficiency of specific coagulation proteins will direct you to the correct option. Review: Laboratory tests used to monitor hemophilia
A nurse teaches a coworker that the treatment for hemophilia will likely include periodic self-administration of: 1. platelets. 2. whole blood. 3. factor concentrates. 4. fresh frozen plasma.
ANSWER: Factor Concentrates A person with hemophilia A is deficient in factor VIII; hemophilia B, factor IX; and von Willebrand's hemophilia, the von Willebrand's factor and factor VIII. Recombinant forms of the factors are available for the client to self-administer intravenously at home. Although whole blood and fresh frozen plasma contain the deficient factors, periodic administration of factor concentrates are safer. Platelets do not contain the deficient clotting factors.
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? A. Bleeding time B. Tourniquet test C. Clot retraction test D. Partial thromboplastin time (PTT)
Correct Answer: D. Partial thromboplastin time (PTT) Rationale: PTT measures the activity of thromboplastin, which is dependent on intrinsic clotting factors. In hemophilia, the intrinsic clotting factor VIII (antihemophilic factor) is deficient, resulting in a prolonged PTT. Bleeding time reflects platelet function; the tourniquet test measures vasoconstriction and platelet function; and the clot retraction test measures capillary fragility. All of these are unaffected in people with hemophilia.
A nurse is caring for a child with hemophilia who has developed hemarthrosis. Based on the nurse's understanding of hemarthrosis, the nurse knows to assess for which of the following? Decreased grip strength Purpura over the face and neck Joint pain and swelling Frequent epistaxis
Correct Answer: "Joint pain and swelling" Rationale: Hemarthrosis is a condition that develops as bleeding into a joint space. A client with hemophilia may suffer from hemarthrosis as one manifestation of easy bleeding and bruising. Signs and symptoms of this condition include joint pain and swelling, warmth and redness over a joint, and difficulties with movement. "Frequent epistaxis" is incorrect. Hemarthrosis involves internal bleeding into the joint space, not epistaxis. "Decreased grip strength" is incorrect. Hemarthrosis does affect range of motion of the affected joints, but does not affect muscle strength. "Purpura over the face and neck" is incorrect. Hemarthrosis does not cause purpura.
Which of the following assessments in a child with hemophilia would lead the nurse to suspect early hemarthrosis? A. Child's reluctance to move a body part B. Cool, pale, clammy extremity C. Ecchymosis formation around a joint D. Instability of a long bone in passive movement
Correct Answer: A. Child's reluctance to move a body part Rationale: Bleeding into the joints in the child with hemophilia leads to pain and tenderness, resulting in restricted movement. Therefore, an early sign of hemarthrosis would be the child's reluctance to move a body part. If the bleeding into the joint continues, the area becomes hot, swollen, and immobile—not cool, pale, and clammy. Ecchymosis formation around a joint would be difficult to assess. Instability of a long bone on passive movement is not associated with joint hemarthrosis.
Because of the risks associated with the administration of factor VIII concentrate, the nurse would teach the client's family to recognize and report which of the following? A. Yellowing of the skin B. Constipation C. Abdominal distention D. Puffiness around the eyes
Correct Answer: A. Yellowing of the skin Rationale: Because factor VIII concentrate is derived from large pools of human plasma, the risk of hepatitis is always present. Clinical manifestations of hepatitis include yellowing of the skin, mucous membranes, and sclera. Use of factor VIII concentrate is not associated with constipation, abdominal distention, or puffiness around the eyes.
A pediatric nurse health educator provides a teaching session to the nursing staff regarding hemophilia. Which of the following information regarding this disorder would the nurse plan to include in the discussion? A. Hemophilia is a Y linked hereditary disorder B. Males inherit hemophilia from their fathers C. Females inherit hemophilia from their mothers D. Hemophilia A results from a deficiency of factor VIII
Correct Answer: D. Hemophilia A results from a deficiency of factor VIII Rationale: Males inherit hemophilia from their mothers, and females inherit the carrier status from their fathers. Hemophilia is inherited in a recessive manner via a genetic defect on the X-chromosome. Hemophilia A results from a deficiency of factor VIII. Hemophilia B (Christmas disease) is a deficiency of factor IX.
A 27-year-old patient with hemophilia is seen at the care clinic with acute hemarthrosis. Which of the following best describes this condition? Development of fluid under the fascia that compresses the underlying nerves and joints The deposit of uric acid crystals into the joint capsule The growth of bacteria and infection that develops in a joint The process of bleeding in a joint
Correct Answer: The process of bleeding in a joint Rationale: Hemarthrosis occurs when there is bleeding into the joint space. It may develop among people who have clotting disorders and are more prone to bleeding, such as those with hemophilia. Hemarthrosis can cause joint pain and swelling, eventually leading to disability if the condition is not managed. The patient may need pain medication and may need to have the fluid aspirated out of the joint space.
A nurse has just completed client education prior to discharging a two-year-old who was just diagnosed with Hemophilia A. Which of the following statements from the parents indicate that the teaching was effective? Select all that apply. "If my child gets a fever I should give him ibuprofen instead of acetaminophen" "Hemophilia means that my child will bleed longer than people who don't have hemophilia" "Having hemophilia means that my child will bleed more intensely than people who don't have hemophilia" "Hemophilia is treatable with the right medications" "Hemophilia is a clotting disorder that occurs due to an excess of vitamin K"
Correct: "Hemophilia means that my child will bleed longer than people who don't have hemophilia" Rationale: Hemophilia affects the amount of time it takes for the body to clot which means they would bleed for longer, not bleed more intensely. "Hemophilia is treatable with the right medications" Rationale: By replacing the missing clotting factors, bleeds can be prevented. Incorrect: "If my child gets a fever I should give him ibuprofen instead of acetaminophen" Rationale: Ibuprofen increases the risk factors for bleeding. Acetaminophen does not have the side effect of causing bleeding and is, therefore, safer for a client with hemophilia. "Having hemophilia means that my child will bleed more intensely than people who don't have hemophilia" Rationale: Clients with hemophilia do not bleed more intensely. Rather, they bleed for a longer amount of time due to missing clotting factors. "Hemophilia is a clotting disorder that occurs due to an excess of vitamin K" Rationale: Some forms of acquired hemophilia may occur with a deficiency in Vitamin K.
The mother of a two-year-old boy who has been diagnosed with hemophilia is concerned because she is pregnant again. She says her husband is not a carrier of the gene and wants to know what the chances are that this baby will have hemophilia. The nurse accurately tells her which of the following? "If your baby is a girl there is a 50% chance that she will be a carrier of the gene and a 25% chance that she will have the disease." "If your baby is a boy there is a 50% chance he will have hemophilia and a 25% chance that he will be a carrier of the disease." "If your baby is a boy there is a 50% chance he will have hemophilia." "There is a 25% chance that this baby will have the disease."
Correct: "If your baby is a boy there is a 50% chance he will have hemophilia." Rationale: Hemophilia is an x-linked recessive disorder and the father in this scenario does not have the disorder. This means there is a 50% chance the mother will pass the disorder on to her son.
The nurse caring for a seven-year-old with hemophilia knows that the most common recurring issue for this child will be which of the following? Hemarthrosis Intracranial bleeds GI bleeds Hypovolemia
Correct: Hemarthrosis Rationale: The joints are the most common location for children to experience bleeding. Incorrect: While GI bleeds can happen, they are not the most common type of bleeding to occur in clients with hemophilia. While intracranial bleeds can occur, they are not the most common type of bleeding to occur in clients with hemophilia. While hypovolemia may occur with extreme blood loss and would be very concerning, this is not the most common recurring issue for children with hemophilia.
A nurse is caring for an eight-month-old who has recently been diagnosed with hemophilia A. The nurse instructs the parents that their child is missing which of the following clotting factors? IX XI X VIII
Correct: Hemophilia A is missing clotting factor VIII. Incorrect: Clotting factor X is Vitamin K dependent and not associated with hemophilia A. Hemophilia C is missing clotting factor XI, not hemophilia A. Hemophilia B is missing clotting factor IX, not hemophilia A.