5 Hemophilia Nursing Care Plans

5 Hemophilia Nursing Care Plans

Hemophilia, an X-linked disorder, is a congenital inherited bleeding disorder of the clotting mechanism cause by an abnormal gene that produces a defective clotting factor protein with little or no clotting ability. The two most common forms of this disorder are Classic hemophilia (type A) which is caused by the lack of factor VIII and Christmas disease (type B) is caused by the deficiency in factor IX. Since both of these disorders are X-linked, the female is the carrier and the disorder is manifested only in males.

Hemophilia is classified into the following three groups, based on the severity of factor deficiency, mild (5-50%), moderate (1-5%) and severe (1%). Hemophiliacs are at risk for prolonged bleeding or hemorrhage as a result of minor trauma. Individuals with severe hemophilia, or less than 1% clotting factor, are also at risk to suffer from spontaneous bleeding without trauma or more severe prolonged bleeding after trauma. Bleeding can occur at any part of the body. Hemarthrosis, or bleeding into the joint spaces, is the most common complication of severe hemophilia. The knee joint is the most frequent joint involved.

Nursing Care Plans

The nursing care plan goals for a client with hemophilia may include absence of complications and pain, prevention of injury and bleeding, improved physical mobility, and understanding of the disease condition and its management.

Here are five (5) nursing care plans (NCP) for hemophilia:

1. Acute Pain

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Acute Pain: Unpleasant sensory and emotional experience arising from actual or potential tissue damage or described in terms of such damage; sudden or slow onset of any intensity from mild to severe with anticipated or predictable end and a duration of <6 months.

May be related to

• - Hemarthrosis
• - Traumatic injury to muscles

Possibly evidenced by

• - Verbal complaint of pain/discomfort
• - A feeling of stiffness
• - Tingling or aching in the affected joint, followed by a decrease in the ability to move a joint
• - Guarding behavior
• - Irritability, restlessness
• - Crying

Desired Outcomes

• - Client will experience decreased pain.

Nursing Interventions	Rationale
Assess the location, characteristics, and rate of pain (use pain scale).	Hemarthrosis (bleeding into joint) is the main manifestation of the disease. Common sites include the elbows, shoulders, hips, knees, and ankle joints.
Assess for joint swelling and ability to move affected limb.	Bleeding episodes should be managed at the onset of discomfort, which entails a deficient factor replacement.
Immobilize joints and apply elastic bandages to the affected joint if indicated; elevate affected and apply a cold compress to active bleeding sites, but must be used cautiously in young children to prevent skin breakdown.	Immobilization promotes comfort and decreases joint damage; elastic bandage most often avoids muscle bleeding; elevation of affected extremity/ joint will minimize swelling; cold application will promote vasoconstriction.
Provide bed cradle over painful joints and other sites of bleeding.	Prevents pressure of 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 to decreased bleeding and provide some relief.
Perform range of motion 48 hours after the acute bleeding episode and pain has subsided.	Maintains optimal joint movement.
Administer medications as indicated.	Acetaminophen (Tylenol), propoxyphene, corticosteroids, and codeine are given for pain management; NSAIDs such aspirin are contraindicated since they impair clotting and can cause gastric bleeding.
Administer factor VIII or other prescribed factor component immediately.	Controls the bleeding that is causing the pain.
Educate child about cause of pain and interventions to relieve it; how medications must be administered via per orem, while injections are not advised; to avoid taking aspirin or aspirin product for pain.	Promotes understanding of pain responses and methods to reduce it.
Instruct child to support and protect painful areas and in the importance of immobilization.	Promotes comfort and prevents further bleeding into joints.

2. Impaired Physical Mobility

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Impaired Physical Mobility: Limitation in independent, purposeful physical movement of the body or of one or more extremities.

May be related to

• - Pain and discomfort with the onset of bleeding episodes
• - Hemarthrosis.

Possibly evidenced by

• - Pain in affected joint
• - Decreased ability to move the joint
• - Immobilized joints (first 24 to 48 hours after a bleeding episode)
• - Potential contractures in affected joints

Desired Outcomes

• - Child will maintain optimal physical mobility as evidenced by normal range of motion (ROM) and activities of daily living within ability.

Nursing Interventions	Rationale
Assess for limited ROM, contractures, and bony changes in the joints when bleeding has stopped.	Recurrent bleeding of the joints can lead to bone destruction, permanent deformities, and crippling. This data gives the baseline for evaluating further constraints from bleeding.
Provide gentle, passive ROM exercise when the child’s condition is stable.	Clients who are experiencing active bleeding have restricted mobility or splinting. However, later ROM exercise can help maintain optimal muscle and joint movement.
Assist with the progression to active exercise as tolerated.	Non-weight bearing exercise facilitates optimal ROM without stimulating rebleeding. Extra weight should be avoided until swelling has subsided.
Educate on preventive measures, such as the application of protective gear and the administration of factor products.	Avoiding injury and hemarthrosis is essential for maintaining joint and limb mobility and use.
Provide assistive devices when needed.	The chronic joint deformity is a common complaint.
Refer for physical therapy, occupational therapy, and orthopedic consultations, as required.	Electrical stimulation of the muscles around the joints prevents muscle atrophy. Active physiotherapy helps in regaining joint movement and preventing fibrous build up.

3. Compromised Family Coping

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Compromised Family Coping: A usually supportive primary person (family member, significant other, or close friend) insufficient, ineffective, or compromised support, comfort, assistance or encouragement that may be needed by the individual to manage or master adaptive tasks related to his or her health challenge.

May be related to

• - Inadequate or incorrect information or understanding
• - Prolonged disease or disability progression that exhausts the physical and emotional supportive capacity of caretakers

Possibly evidenced by

• - Expression and/or confirmation of concern and inadequate knowledge about long-term care needs, problems, and complications,
• - Anxiety and guilt
• - Overprotection of child

Desired Outcomes

• - Family will cope effectively with child’s illness.

Nursing Interventions	Rationale
Assess family’s coping methods and their effectiveness; family interactions and expectations related to long-term care, developmental level of family; response of siblings; knowledge and use of support systems and resources; presence of guilt and anxiety; overprotection and/or overindulgent behaviors.	Identifies coping methods that work and the need to utilize new coping skills and behaviors, family attitudes; child with special long-term needs either strengthen or distort family relationships and an undue degree of overprotection may be detrimental to child’s growth and development such as disallowing school attendance or peer activities, avoiding discipline of child, and disallowing child to assume responsibility for ADL.
Encourage family members to verbalize problem areas and develop solutions on their own.	Decreases anxiety and enhances understanding; provides family an opportunity to recognize problems and create problem-solving strategies.
Encourage family members to express feelings, such as how they deal with the chronic needs of a family member and coping patterns that help or hinder adjustment to the problems.	Allows for venting of feelings, which relieves guilt and anxiety and helps determine need for information and support.
Assist family in setting short- and long-term goals for the child and integrate child into family activities, include participation of all family.	Promotes involvement and control over situations and maintains the role of family members and parents.
Provide information about long-term care and management.	Enhances family understanding of medical regimen and responsibilities of family members.
Inform family that overprotective behavior may hinder growth and development and that child should be treated as normally as possible.	Promotes understanding of the importance of making child one of the family and the adverse effects of overprotection of the child.
Provide assistance of social worker, counselor, or other as needed.	Gives support to the family faced with long-term care of a child with a serious illness.

4. Risk for Bleeding

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Risk for Bleeding: At risk for a decrease in blood volume that may compromise health.

May be related to

• - Decreased concentration of clotting factors circulating in the blood (factor VIII and factor IX)

Possibly evidenced by

• [not applicable]

Desired Outcomes

• - Child’s risk for injury from possible bleeding is decreased through the use of appropriate prophylactic measures.

Nursing Interventions	Rationale
Monitor the coagulation assays for factor VIII AND IX.	Decreased value indicate that factor replacement therapy is subtherapeutic.
Monitor the partial thromboplastin time (PTT).	PTT is prolonged because of a deficiency in the clotting system factors. The prothrombin time will be normal.
Assess for any signs of bruising and bleeding (note the extent of bleeding). Assess for prolonged bleeding after minor injuries.	Usual sites of external bleeding may include the bleeding in the mouth from a cut, bite, or from cutting or losing a tooth; nosebleeds for no obvious reasons; heavy bleeding from a minor cut, or bleeding from a cut that resumes after stopping for a short time. Hemophiliacs do not bleed faster or more frequently. Instead, they bleed longer due to a deficiency of clotting factor. Clients are often aware of bleeding before clinical manifestation. Bleeding can be life-threatening to these clients.
Assess for any pain and swelling over the entire body.	A headache, in the presence of a trauma history, may suggest intracranial hemorrhage. Abdominal pain may indicate an internal bleeding. Bleeding into a joint is usually reported as a peculiar tingling sensation felt well before pain or swelling is detected.
If spontaneous or traumatic bleeding is evident, monitor vital signs.	Hypovolemic shock can happen due to decreased circulatory volume with blood loss. Signs include hypotension and tachycardia.
- Monitor hemoglobin and hematocrit levels.	Hgb and Hct are monitored as indicators of blood loss.
- Assess for inhibitor antibody to factor VIII.	Clients who require frequent transfusions may develop inhibitor antibody and require a subsequent change in coagulation therapy to factor VIIa.
Anticipate or instruct in the need for prophylactic treatment before high-risk situations, such as invasive diagnostic or surgical procedures, or dental work.	Treatment may include cryoprecipitate, which contains factor VIII and fibrinogen, and factor VIII concentrate, or it may include desmopressin (DDAVP) for treating mild to moderate hemophilia A.
Perform the following measures to control bleeding: - Apply manual or mechanical pressure if active bleeding is noted. - Apply sterile dressings to wounds, and apply pressure. - Apply topical coagulants, such as fibrin foam and thrombin.	Controlling bleeding is a nursing priority. Nasal packing should be avoided, because the subsequent removal of the packing may precipitate further bleeding.
If bleeding is in a joint (hemarthrosis), elevate and immobilize the affected limb. Apply ice packs to control bleeding.	Repeated hemarthrosis can result in severe and crippling deformity.
Provide replacement therapy of deficient clotting factors.	Replacement of factors is the primary treatment for bleeding. Treatment includes factor VIII, which is an essential clotting factor needed to convert prothrombin to thrombin. This treatment can also be provided in the home. DDAVP is the treatment of choice for mild hemophilia. It is an analogue of vasopressin and is available intravenously and intranasally. Recombinant DNA factors are available. Because they are not produced from humans, they should reduce the risk for infectious transmission.
Administer plasma-derived factor VIIa for clients with antibodies against factor VIII.	Antibodies (inhibitors) to the clotting factors can destroy it before it is able to work and defeat the effect of replacement therapy. New therapies are available to neutralize the antibodies.
Administer tranexamic acid or epsilon aminocaproic acid as indicated.	These are second-line antifibrinolytic drugs that do not actually form clots but help hold clots in place by stopping plasmin activity. They do not replace factor therapy. They are commonly used before dental work, during bleeding episodes, and, in the rare case of a woman with hemophilia, during heavy menstrual bleeding.
Anticipate the need for blood replacements.	Volume expanders and O-negative blood should be immediately available in the event of life-threatening hemorrhage.

5. Risk for Injury

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Risk for Injury: Vulnerable for injury as a result of environmental conditions interacting with the individual’s adaptive and defensive resources, which may compromise health.

May be related to

• - Decreased clotting factor (VIII or IX)

Possibly evidenced by

• [not applicable]

Desired Outcomes

• - Client will not experience injury.

Nursing Interventions	Rationale
Assess signs and symptoms of bleeding; hemarthrosis (stiffness, tingling, or pain); subcutaneous and intramuscular hemorrhage; oral bleeding; epistaxis (is not a frequent sign); petechiae (are uncommon).	Early detection of bleeding episodes will delay initiation of factor replacement therapy and will minimize complications; oral bleeding is often caused by trauma to the gums; petechiae is caused by low platelet function versus a deficient clotting factor.
Advise adolescents to use an electric shaver versus manual razor devices (with blades).	High risk of bleeding is related to use of razor blades; minimal risk of bleeding is associated with the use of electric shaver.
Utilize appropriate toys (soft, not pointed or small sharp objects); for infants, may need to use padded bed rail sides on crib; avoid rectal temperatures.	All of these recommendations will minimize and/or prevent bleeding episodes due to trauma.
Provide appropriate oral hygiene (use of a water irrigating device; use of a soft toothbrush or softening the toothbrush with warm water before brushing; use of sponge-tipped toothbrush).	Implementation of appropriate oral hygiene will minimize trauma to the gums.
Substitute the subcutaneous route for intramuscular injections; utilize venipuncture blood drawing technique for all required blood testing samples versus the use of a finger or heel puncture.	Both of these measures are associated with less bleeding after implementing subcutaneous injection or venipuncture blood sample.
Recommend non-contact sports activities such as swimming, hiking, or bicycling.	These activities are considered a safe activity by the Hemophilia Foundation.
Avoid contact sports such as football, soccer, ice hockey, karate.	Contact sports will predispose the child to injury and bleeding episodes.
Limit use of helmets and padding of cause joints during participation in contact sports activities.	Daily use of these measures may the child to feel ostracized or may create emotional discomfort.
Maintain close supervision during play time to minimize injuries.	Prevent bleeding related to trauma in the child’s environment.
Institute the following measures to control and halt all bleeding episodes:
- Apply manual or mechanical pressure (10 to 15 mins) if active bleeding is observed.	Allows clot formation.
- Immobilize and elevate affected extremity above the heart	Decrease blood flow to control bleeding.
- Application of cold pack as indicated.	Promote vasoconstriction, but use caution with small children to avoid tissue damage.
- Institute factor replacement therapy (based on hospital protocol) and DDAVP (it can be given IV or intranasally)	Control and stop bleeding episode; Prevent crippling effects from joint bleeding.
- Complete bed rest for intramuscular hemorrhage of the lower spine area and non-weight bearing support.	Minimize hemorrhage in muscles of the lower spine.
- Assess laboratory values for blood clotting factors (VIII or IX) and vital signs;	These values determine current hemodynamic status and factor replacement therapy guidelines or protocols
- Terminate passive range-of-motion exercises after an acute episode of bleeding.	Avoid injury to the affected extremity or joint and to avoid recurrence of bleeding to these.
Educate the family and affected child about signs and symptoms of bleeding, and appropriate interventions to control bleeding at home.	Empowers others with accurate information to identify and manage bleeding episodes.
Instruct parents to include iron-rich foods in diet.	Maintains iron level to prevent anemia.
Advice to wear a medical alert bracelet.	Facilitates accurate diagnosis and treatment in case of an emergency.
Teach parents related to home health maintenance:
- The affected child should receive all routine immunizations (use subcutaneous route, recommend pressure and elastic bandage after injections)	Protect the child from childhood communicable diseases.
- Reinforce the importance of appropriate dental hygiene program;	Minimize oral trauma.
- Reinforce the provision of a safe but normal home environment, such as safety measures that are employed for all children of different ages are recommended; example: for the toddler, gates over stairs but avoid restraining the toddler’s attempt to master motor skills; for the older child, participating in sports activities (use helmets and padding);	Minimize emotional distress during the child’s progression through the different developmental stages.
- Provide a home environment free of hazards, including clear pathways, and supervise child during ambulation and play without being overprotective.	Minimize risk of trauma in the home by falls; infants and toddlers frequently fall or sustain injuries.
Instruct parents and child, if age appropriate, to administer factor VIII via IV if signs and symptoms appear, or before dental visits or other possible invasive procedures; instruct in mixing the precipitate, drawing into syringe, venipuncture, and application of pressure following IV, and allow for return demonstration.	Prevents or manages bleeding by factor replacement.
Teach parents and child of possible reactions to IV concentrate administration and that blood is tested for AIDS.	Decreases anxiety caused by risk of infections such as hepatitis and AIDS from replacement products.

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References and Sources : nurseslabs.com