Hemophilia is a hereditary bleeding disorder caused by a defective gene that results in insufficient clotting factor production. There are two main types: Classic hemophilia (type A), caused by factor VIII deficiency, and Christmas disease (type B), caused by factor IX deficiency. Since hemophilia is X-linked, females typically carry the gene while males manifest the disorder.

Severity of hemophilia is classified as mild (5-50% clotting factor), moderate (1-5%), or severe (less than 1%). Individuals with severe hemophilia are particularly susceptible to prolonged bleeding from minor injuries or spontaneous bleeding without trauma. Bleeding can occur in any part of the body, with hemarthrosis, or bleeding into joint spaces, being a common complication, especially in severe cases. The knee joint is most frequently affected by hemarthrosis.

The nursing priorities for patients with hemophilia include:

Preventing and managing bleeding episodes.
Monitoring for signs of bleeding complications.
Administering clotting factor replacement therapy.
Implementing injury prevention strategies and providing self-care education.
Managing pain during bleeding episodes.
Promoting physical mobility and protecting joints.
Ensuring compliance with treatment plans and medication regimens.

Assess for the following subjective and objective data:

Joint tenderness, swelling, or limited range of motion due to hemarthrosis (bleeding into joints).
Presence of ecchymosis (bruising) or hematoma.
Gum bleeding.
Epistaxis.
Blood in urine or stool.
Excessive bleeding during menstruation (in females with hemophilia).
Excessive bleeding following dental procedures or minor trauma.
Unexplained or prolonged bleeding after vaccinations.

Assess for factors related to the cause of hemophilia:

Hemarthrosis.
Traumatic injury to muscles.
Decreased concentration of clotting factors circulating in the blood (factor VIII and factor IX).

Goals and expected outcomes may include:

The client will experience decreased pain.
The child will understand and participate in self-care measures during bleeding episodes, such as applying pressure to bleeding sites and seeking medical assistance when needed.
The child will receive timely and appropriate treatment for bleeding episodes, resulting in reduced blood loss, pain relief, and resolution of bleeding.
The child will not experience injury.
The child will actively engage in physical therapy and joint protection techniques to maintain joint mobility and prevent joint damage.
The child and family members will demonstrate proper techniques for handling daily activities to minimize the risk of injury.
The child will maintain optimal physical mobility as evidenced by normal range of motion (ROM) and activities of daily living within the ability.
The family will cope effectively with the child’s illness.

Therapeutic interventions and nursing actions for patients with hemophilia may include:

Preventing Bleeding Risk and Injury:

Assess for any signs of bruising and bleeding (note the extent of bleeding) and prolonged bleeding after minor injuries.
Monitor for pain and swelling over the entire body, especially in joints.
Monitor vital signs if spontaneous or traumatic bleeding is evident.
Monitor coagulation assays for factors VIII and IX to assess clotting ability.
Monitor activated partial thromboplastin time (aPTT) and hemoglobin/hematocrit levels.
If bleeding occurs in a joint (hemarthrosis), elevate and immobilize the affected limb and apply ice packs to control bleeding. Repeated hemarthrosis can lead to severe and crippling deformity.
Provide replacement therapy for deficient clotting factors. Replacement of clotting factors is the primary treatment for bleeding. Treatment includes factor VIII, which is essential for converting prothrombin to thrombin. This treatment can also be administered at home.
Administer plasma-derived factor VIIa for clients with antibodies against factor VIII. Antibodies (inhibitors) to clotting factors can destroy them before they can work, rendering replacement therapy ineffective. New therapies are available to neutralize these antibodies.
Assess for inhibitor antibody to factor VIII, especially in clients requiring frequent transfusions.
Anticipate or instruct in the need for prophylactic treatment before high-risk situations, such as invasive procedures or dental work, using factor replacement therapy or desmopressin (DDAVP) for mild to moderate hemophilia.

Managing Acute Pain

Clients with hemophilia may experience acute pain due to hemarthrosis (bleeding into joints) or traumatic muscle injury, which can occur as a result of injury or trauma. Hemophilia causes a deficiency of blood clotting factors, leading to prolonged bleeding, inflammation, and pain, which can occur spontaneously or after physical activity or injury.

Assess the location, characteristics, and intensity of pain (use a pain scale). Hemarthrosis (bleeding into the joint) is the primary manifestation of the disease. Common sites include the elbows, shoulders, hips, knees, and ankles.
Assess for joint swelling and evaluate the ability to move the affected limb. Bleeding episodes should be managed at the onset of discomfort, which involves replacement of deficient factors.
Immobilize joints and apply elastic bandages to the affected joint if indicated; elevate the affected limb and apply a cold compress to active bleeding sites, but use caution in young children to prevent skin breakdown. Immobilization promotes comfort and decreases joint damage; elastic bandages often prevent muscle bleeding; elevation of the affected extremity/joint minimizes swelling; cold application promotes vasoconstriction.
Provide a bed cradle over painful joints and other sites of bleeding. This prevents pressure from bed linens on affected sites, especially joints.
Maintain immobilization of the affected extremity during the acute phase (24 to 48 hours); apply a splint or sling to the affected extremity if indicated. Immobilization of the affected joint helps decrease bleeding and provide reliefe.

Improving Physical Mobility

Clients with hemophilia may experience impaired physical mobility due to hemarthrosis (bleeding into joints), pain, and discomfort. Hemarthrosis can lead to swelling and inflammation, which can cause restricted movement and reduced range of motion. Pain and discomfort can also limit mobility and affect a person’s ability to engage in physical activities.

Assess for limited range of motion (ROM), contractures, and bony changes in the joints when bleeding has stopped. Recurrent bleeding into the joints can lead to bone destruction, permanent deformities, and crippling. This assessment provides a baseline for evaluating further constraints resulting from bleeding.
Provide gentle, passive ROM exercises when the client’s condition is stable. Clients experiencing active bleeding may have restricted mobility or require splinting. However, once bleeding has subsided, gentle passive ROM exercises can help maintain optimal muscle and joint movement.

Promoting Effective Family Coping

Children with hemophilia and their families may experience compromised family coping due to the chronic and unpredictable nature of the disease, as well as the financial and emotional stressors that may accompany it. The need for frequent medical care, potential complications, and limitations on physical activity can also impact the family’s ability to cope with the challenges of the condition.

- Provide information about long-term care and management to enhance family understanding of the medical regimen and the responsibilities of family members.
- Inform the family that overprotective behavior may hinder growth and development and that the child should be treated as normally as possible. This promotes understanding of the importance of integrating the child into family life and the potential adverse effects of overprotecting the child.
- Provide assistance from a social worker, counselor, or other support professionals as needed to offer additional support to the family faced with the long-term care of a child with a serious illness.
- Assess the family’s coping methods and their effectiveness, including family interactions and expectations related to long-term care, the developmental level of the family, the response of siblings, knowledge and use of support systems and resources, and the presence of guilt and anxiety, overprotection, and/or overindulgent behaviors. This assessment identifies coping methods that work and the need to utilize new coping skills and behaviors, as well as understanding family attitudes and dynamics.
- Encourage family members to verbalize problem areas and develop solutions on their own. By encouraging open communication, families can identify challenges and collaboratively develop strategies to address them, reducing anxiety and enhancing understanding.
- Encourage family members to express feelings, including how they deal with the chronic needs of a family member and coping patterns that help or hinder adjustment to the problems. Allowing for the expression of emotions can relieve guilt and anxiety while helping identify the need for information and support.
- Assist the family in setting short- and long-term goals for the child and integrate the child into family activities, including the participation of all family members. This promotes involvement and control over situations, maintaining the child’s role within the family unit.

Assessing and Monitoring for Potential Complications

Nurses play a critical role in assessing and monitoring potential complications for children with hemophilia to detect early signs of bleeding, infections, or other issues. This proactive approach allows for prompt intervention and prevention of further complications, ensuring the child’s safety and well-being through timely and appropriate care.

Assess for signs of bleeding, including excessive bruising, hematomas, joint pain or swelling, gastrointestinal bleeding (e.g., blood in stools), or symptoms indicative of intracranial bleeding (e.g., headache, altered mental status).
Children with hemophilia experience challenges in forming blood clots, heightening their vulnerability to prolonged or spontaneous bleeding episodes. Vigilant assessment for these signs enables early intervention to manage bleeding episodes effectively.
Monitor for signs of infection and promptly initiate treatment with appropriate antibiotics if an infection is suspected.
Children with hemophilia often require frequent medical procedures, such as intravenous access or joint injections, which can increase the risk of introducing pathogens into their bloodstream.
Early detection and treatment of infections are essential for preventing further complications.

Administering Medications and Pharmacologic Support

Medications used in hemophilia include clotting factor concentrates (Factor VIII for Hemophilia A and Factor IX for Hemophilia B), desmopressin (DDAVP), antifibrinolytics, pain medications, and prophylactic antibiotics.
These medications serve various purposes such as replacing deficient clotting factors, promoting clot stability, managing pain, preventing bleeding, and protecting against infections.
Clotting Factor Concentrates Recombinant or plasma-derived clotting factor concentrates, such as Factor VIII (for Hemophilia A) or Factor IX (for Hemophilia B), are administered intravenously.
They replace the deficient clotting factor, aiding in blood clotting during bleeding episodes or as prophylactic treatment to prevent bleeding.
Desmopressin (DDAVP) DDAVP is the preferred treatment for mild hemophilia. It acts as an analogue of vasopressin, stimulating the release of von Willebrand factor and increasing Factor VIII levels in some individuals with mild or moderate hemophilia A or von Willebrand disease. It can be administered intravenously, intranasally, or subcutaneously.

Hemophilia Nursing Management

Introduction

The nursing priorities for patients with hemophilia include:

Conclusion

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