Seven Hemophilia Nursing Care Plans

Hemophilia patients do not bleed faster, they bleed longer. A missing clotting factor means a minor cut keeps oozing and a bumped joint keeps filling. The bleeds you cannot see, into joints, muscle, or the skull, are the ones that cripple and kill. On the floor your job is simple to state and hard to do: catch bleeding early, control it fast, replace the deficient factor, and keep these patients from injuring themselves in the first place.

What is Hemophilia?

Hemophilia is an X-linked congenital bleeding disorder. An abnormal gene produces a defective clotting factor protein with little or no clotting ability. The two common forms are type A (classic hemophilia), caused by a lack of factor VIII, and type B (Christmas disease), caused by a deficiency of factor IX. Because the trait is X-linked, females carry it and males show it.

Severity tracks the level of circulating factor: mild (5-50%), moderate (1-5%), and severe (under 1%). Anyone with hemophilia can hemorrhage after minor trauma. Patients with severe disease, under 1% factor, also bleed spontaneously with no trauma at all. Bleeding can occur anywhere, but hemarthrosis (bleeding into the joint spaces) is the most common complication of severe hemophilia, and the knee is the joint involved most often.

Nursing Care Plans and Management

Care goals center on no complications, controlled pain, prevention of injury and bleeding, preserved physical mobility, and a family that understands the disease and its management.

Nursing Problem Priorities

1. Bleeding prevention and management
2. Monitoring for bleeding complications
3. Clotting factor replacement therapy
4. Injury prevention and selfcare
5. Pain control during bleeding episodes
6. Physical mobility and joint protection
7. Adherence to treatment and medication regimens

Nursing Assessment

Assess for the following subjective and objective data:

• Joint tenderness, swelling, or limited range of motion from hemarthrosis
• Ecchymosis (bruising) or hematoma
• Gum bleeding
• Epistaxis
• Blood in urine or stool
• Heavy menstrual bleeding (in females with hemophilia)
• Prolonged bleeding after dental work or minor trauma
• Unexplained or prolonged bleeding after vaccination

Factors related to the cause:

• Hemarthrosis
• Traumatic injury to muscles
• Decreased circulating clotting factors (factor VIII and factor IX)

Nursing Diagnosis

A focused assessment lets you formulate an accurate nursing diagnosis that drives a care plan built around this patient's bleeding pattern, joints, and home situation.

Nursing Goals

• The client reports decreased pain.
• The child understands and takes part in selfcare during bleeds, including applying pressure and seeking help when needed.
• Bleeding episodes get timely treatment, with reduced blood loss, pain relief, and resolution.
• The child stays free of injury.
• The child engages in physical therapy and joint protection to maintain mobility and prevent joint damage.
• The child and family demonstrate safe technique for daily activities.
• The child maintains mobility with normal range of motion and ADLs within ability.
• The family copes effectively with the child's illness.

Nursing Interventions and Actions

1. Preventing Bleeding and Injury

Low circulating clotting factor means impaired clotting and prolonged bleeding after any injury. That puts these children at high risk for bruises, cuts, and joint bleeds, with the pain, lost mobility, and joint destruction that follow.

Assess for bruising and bleeding, and note the extent. Watch for bleeding that continues after a minor injury. Common external sites are the mouth (a cut, bite, or lost tooth), the nose (nosebleeds for no clear reason), and any minor cut that bleeds heavily or restarts after it appeared to stop. Patients often sense a bleed before you can see it, so listen to them. These bleeds can be life threatening.

Assess for pain and swelling anywhere on the body. Headache after any head trauma may signal intracranial hemorrhage. Abdominal pain may mean internal bleeding. A joint bleed often starts as a peculiar tingling, felt well before pain or swelling shows.

With spontaneous or traumatic bleeding, monitor vital signs. Blood loss drops circulating volume and can cause hypovolemic shock. Watch for hypotension and tachycardia.

Monitor coagulation assays for factor VIII and factor IX. Decreased values mean factor replacement is subtherapeutic.

Monitor the activated partial thromboplastin time (aPTT), and hemoglobin and hematocrit. A prolonged aPTT points to factor deficiency. Hgb and Hct track blood loss.

Assess for inhibitor antibody to factor VIII. Patients who need frequent transfusions can develop inhibitor antibodies and require a switch to factor VIIa.

Anticipate prophylactic treatment before high-risk events: invasive diagnostics, surgery, or dental work. Options include cryoprecipitate (which contains factor VIII and fibrinogen), factor VIII concentrate, or desmopressin (DDAVP) for mild to moderate hemophilia A.

Provide replacement therapy for the deficient clotting factor. Factor replacement is the primary treatment for bleeding. Factor VIII converts prothrombin to thrombin, and replacement can be given at home as well as in hospital.

Give plasma-derived factor VIIa for patients with antibodies against factor VIII. Inhibitor antibodies destroy replaced factor before it works. Factor VIIa bypasses that block.

Give tranexamic acid or epsilon aminocaproic acid as ordered. These antifibrinolytics hold clots in place rather than form them.

Keep blood replacement available. Volume expanders and O-negative blood should be immediately on hand in case of life-threatening hemorrhage.

To control and stop a bleeding episode:

• Apply manual or mechanical pressure for 10 to 15 minutes at the active bleeding site to allow clot formation. Avoid nasal packing, since removing it can restart the bleed.
• Immobilize and elevate the affected limb above the heart to slow blood flow.
• Apply a cold pack to promote vasoconstriction. Use caution in small children to avoid tissue damage.
• For hemarthrosis, elevate and immobilize the joint and apply ice. Repeated joint bleeds cause severe, crippling deformity.
• Start factor replacement per protocol, plus DDAVP (IV or intranasal) to stop the bleed and prevent crippling joint damage.
• Apply sterile dressings and topical coagulants such as fibrin foam and thrombin to wounds.
• For intramuscular hemorrhage of the lower spine, keep the patient on bed rest with non-weight-bearing support.
• Stop passive range-of-motion exercise after an acute bleed to avoid restarting it.

Assess for early bleeding signs: hemarthrosis (stiffness, tingling, pain), subcutaneous and intramuscular hemorrhage, oral bleeding, epistaxis (uncommon), and petechiae (uncommon). Catching a bleed early gets factor replacement started sooner and limits complications. Oral bleeding usually comes from gum trauma. Petechiae reflect low platelet function rather than a clotting factor deficiency.

Injury-prevention teaching for the child and family:

• Use an electric shaver, never a blade razor. Blades carry a high bleeding risk.
• Use soft, non-pointed toys. Pad crib rails for infants. Avoid rectal temperatures.
• Provide gentle oral care with a water irrigating device, a soft or warm-water-softened toothbrush, or a sponge-tipped brush to spare the gums.
• Use the subcutaneous route instead of intramuscular injection, and draw labs by venipuncture rather than finger or heel stick. Both bleed less.
• Encourage noncontact sports such as swimming, hiking, and bicycling, which the Hemophilia Foundation considers safe.
• Avoid contact sports such as football, soccer, ice hockey, and karate, which invite injury and bleeds.
• Reserve helmets and joint padding for activities that need them rather than constant daily wear, which can make the child feel singled out.
• Supervise play closely to prevent trauma without smothering the child.
• Have the child wear a medical alert bracelet so an emergency team knows the diagnosis.
• Include iron-rich foods in the diet to prevent anemia.

Home health teaching for parents:

• Give all routine immunizations by the subcutaneous route, then apply pressure and an elastic bandage. Vaccines protect against communicable disease.
• Keep up dental hygiene to limit oral trauma.
• Keep the home safe but normal for the child's age: stair gates for toddlers without blocking their drive to master motor skills, helmets and padding for the older child in sports. Overprotection harms development.
• Clear pathways and supervise ambulation and play. Infants and toddlers fall often.
• Teach the family, and the child if old enough, to give factor VIII by IV at the first sign of a bleed or before dental and other invasive procedures. Cover mixing the concentrate, drawing it up, venipuncture, and applying pressure afterward, and have them return-demonstrate.
• Explain possible reactions to IV concentrate and that blood products are screened for HIV and hepatitis. This eases fear of transfusion-transmitted infection.

2. Managing Acute Pain

Pain in hemophilia comes from hemarthrosis or muscle bleeds, spontaneous or after activity, with bleeding, inflammation, and swelling driving it.

Assess pain location, character, and severity with a pain scale. Hemarthrosis is the main pain source. Common sites are the elbows, shoulders, hips, knees, and ankles.

Assess joint swelling and ability to move the limb. Treat at the first sign of discomfort with factor replacement.

Immobilize the joint, elevate the limb, and apply a cold compress to active bleeding sites. Use cold cautiously in young children to protect the skin. Immobilization and elevation ease pain and limit joint damage and swelling, and cold promotes vasoconstriction.

Place a bed cradle over painful joints and bleeding sites to keep linen weight off them.

Keep the limb immobilized during the acute phase (24 to 48 hours) with a splint or sling as needed to reduce bleeding and pain.

Resume range of motion 48 hours after the bleed stops and pain subsides to maintain joint movement.

Give analgesics as ordered. Acetaminophen (Tylenol), propoxyphene, corticosteroids, and codeine are used. NSAIDs, including aspirin, are contraindicated because they impair clotting and can cause gastric bleeding.

Give factor VIII or the prescribed factor immediately to control the bleeding that is causing the pain.

Teach the child that pain meds are taken by mouth, not by injection, and to avoid aspirin and aspirin-containing products.

Teach the child to support and protect painful areas and why immobilization matters.

3. Improving Physical Mobility

Hemarthrosis, with its swelling, inflammation, pain, and restricted range of motion, limits movement and the ability to stay active.

Assess for limited ROM, contractures, and bony joint changes once bleeding stops. Recurrent joint bleeds destroy bone and cause permanent deformity. This is your baseline for tracking further loss.

Provide gentle passive ROM once the child is stable. During active bleeding, mobility is restricted. Later ROM maintains muscle and joint movement.

Progress to active exercise as tolerated. Non-weight-bearing exercise restores ROM without restarting the bleed. Avoid added weight until swelling subsides.

Teach preventive measures: protective gear and factor products to avoid injury and hemarthrosis and keep the joint usable.

Provide assistive devices for chronic joint deformity.

Refer to physical therapy, occupational therapy, and orthopedics as needed. Muscle stimulation around the joint prevents atrophy, and active physiotherapy restores movement and limits fibrous buildup.

4. Promoting Effective Family Coping

A chronic, unpredictable disease with financial and emotional strain, frequent medical care, possible complications, and activity limits taxes a family's ability to cope.

Assess the family's coping methods and how well they work: family interactions and expectations around long-term care, developmental level, the siblings' response, use of support systems, and any guilt, anxiety, overprotection, or overindulgence. A child with long-term needs can strengthen or strain a family. Excessive overprotection, blocking school or peer activities, avoiding discipline, or doing the child's ADLs, harms growth and development.

Have family members name problem areas and work out their own solutions to lower anxiety and build problem-solving skills.

Encourage them to express feelings about the chronic demands and the coping patterns that help or hurt, which relieves guilt and anxiety and shows where they need information and support.

Help the family set short- and long-term goals and fold the child into family activities, keeping every member involved.

Provide information on long-term care and management so the family understands the regimen and their roles.

Tell the family that overprotection hinders development and the child should be treated as normally as possible.

Refer to a social worker or counselor as needed for support through long-term care.

5. Assessing and Monitoring for Complications

Children with hemophilia clot poorly, so watch closely for bleeding, infection, and other complications and act early.

Assess for bleeding: excessive bruising, hematomas, joint pain or swelling, GI bleeding (blood in stool), or intracranial bleeding (headache, altered mental status). Reduced clotting ability makes prolonged and spontaneous bleeds more likely.

Monitor for infection and treat promptly with antibiotics when suspected. Frequent IV access and joint injections raise the risk of introducing pathogens into the bloodstream.

Assess for acute compartment syndrome: severe pain, swelling, or loss of sensation, and notify the provider immediately. Internal bleeding can raise pressure within a muscle compartment, choking off blood flow and damaging tissue.

Teach joint protection and avoidance of high-impact activity and excess joint stress to cut the risk of joint bleeds and preserve mobility.

6. Medications and Pharmacologic Support

Drug therapy replaces deficient clotting factors, stabilizes clots, manages pain, prevents bleeding, and protects against infection.

Clotting Factor Concentrates. Recombinant or plasma-derived factor VIII (hemophilia A) or factor IX (hemophilia B) is given IV to replace the deficient factor, either to treat a bleed or as prophylaxis.

Desmopressin (DDAVP). The treatment of choice for mild hemophilia. This vasopressin analogue stimulates release of von Willebrand factor and raises factor VIII in some patients with mild or moderate hemophilia A or von Willebrand disease. It can be given IV, intranasally, or subcutaneously.

Antifibrinolytics. Second-line agents that do not form clots but hold them in place by blocking plasmin. They do not replace factor therapy. They are used before dental work, during bleeds, and in the rare woman with hemophilia during heavy menses. Given orally or IV.

Pain medications. NSAIDs or opioids may be prescribed for bleed- or procedure-related pain, with the NSAID caution noted above.

Immunizations. Vaccines protect against infections that can trigger bleeds. Give routine childhood vaccines on schedule.

7. Laboratory and Diagnostic Procedures

These tests gauge factor levels, clotting times, and platelet function, and separate hemophilia from other bleeding disorders.

Complete Blood Count (CBC). Reports red cells, white cells, and platelets, and helps evaluate anemia and platelet counts.

Activated Partial Thromboplastin Time (aPTT). Measures the intrinsic coagulation pathway. A prolonged aPTT suggests the factor deficiency seen in hemophilia.

Bleeding Time. Times how long a small skin incision bleeds. Used less often now, it adds information on platelet function and overall bleeding tendency.

Factor VIII Assay. Measures factor VIII, deficient in hemophilia A, to grade severity and track treatment.

Factor IX Assay. Measures factor IX, deficient in hemophilia B, for the same purpose.

Von Willebrand Factor Assay. Because von Willebrand disease can mimic mild hemophilia A, testing von Willebrand factor level and activity helps tell them apart.