{
  "name": "Hemophilia",
  "slug": "hemophilia",
  "url": "https://nursingplex.com/care-plans/hemophilia",
  "category": "Hematologic & Lymphatic",
  "summary": "Inherited factor deficiency; injury prevention, factor replacement and joint bleed care.",
  "nursing_diagnoses": [
    "Risk for bleeding",
    "Acute pain",
    "Impaired physical mobility"
  ],
  "overview": [
    "Hemophilia is an inherited deficiency of a clotting factor: hemophilia A lacks factor VIII, hemophilia B (Christmas disease) lacks factor IX. Both are X-linked recessive, so they overwhelmingly affect males while females are usually carriers. The defect is in the intrinsic pathway, which is why the aPTT is prolonged while PT, platelet count and bleeding time stay normal.",
    "Patients do not bleed faster — they bleed longer, and they bleed into places that matter. Hemarthrosis into knees, ankles and elbows is the signature problem; repeated joint bleeds destroy cartilage and produce chronic arthropathy and deformity by young adulthood. Intracranial bleeding after even mild head injury is the leading cause of death.",
    "Modern care is prophylactic factor replacement, prompt treatment of any suspected bleed, and lifelong avoidance of trauma, intramuscular injections and platelet-inhibiting drugs. Nursing care focuses on immediate bleed management, joint preservation, safe activity choices and teaching families home infusion."
  ],
  "key_facts": [
    {
      "label": "Genetics",
      "text": "X-linked recessive: affects males, carried by females; hemophilia A is factor VIII, B is factor IX."
    },
    {
      "label": "Lab pattern",
      "text": "Prolonged aPTT with normal PT, platelet count and bleeding time."
    },
    {
      "label": "Signature bleed",
      "text": "Hemarthrosis — joint warmth, tingling, stiffness and pain before visible swelling."
    },
    {
      "label": "Acute joint care",
      "text": "RICE: rest, ice, compression, elevation — plus factor replacement first, not after."
    },
    {
      "label": "Absolute avoidances",
      "text": "No aspirin or NSAIDs, no IM injections, no rectal temperatures, no contact sports."
    }
  ],
  "nursing_priorities": [
    "Stop and prevent bleeding with prompt factor replacement.",
    "Preserve joint function and prevent chronic arthropathy.",
    "Recognize life-threatening bleeds: intracranial, airway, retroperitoneal.",
    "Control pain without platelet-inhibiting drugs.",
    "Teach the family home infusion and safe activity.",
    "Support normal development and psychosocial adjustment."
  ],
  "assessment": {
    "subjective": [
      "Tingling, warmth or 'bubbling' in a joint — the earliest sensation of a bleed",
      "Joint pain and stiffness limiting movement",
      "Headache, visual change, drowsiness or vomiting after head injury",
      "Family reports of prolonged bleeding after circumcision, tooth loss or minor cuts",
      "Frustration with activity restrictions, especially in school-age boys"
    ],
    "objective": [
      "Warm, swollen, painful joint held in flexion with limited range of motion",
      "Large deep bruises, hematomas and prolonged oozing from small wounds",
      "Epistaxis, hematuria, melena or bleeding gums",
      "Prolonged aPTT with reduced factor VIII or IX assay",
      "Falling hemoglobin, tachycardia and hypotension in significant blood loss",
      "Neurologic change after head trauma; abdominal or flank pain with retroperitoneal bleeding",
      "Chronic joint deformity, muscle atrophy and contracture in older patients"
    ],
    "related_factors": [
      "Deficiency of factor VIII or IX with impaired clot stabilization",
      "Trauma, invasive procedures, dental work and surgery",
      "Bleeding into joints, muscles and closed spaces",
      "Development of factor inhibitors reducing treatment effectiveness",
      "Age-appropriate risk-taking and normal childhood activity"
    ]
  },
  "goals": [
    "The client will have bleeding episodes recognized and treated within the first hour of symptoms.",
    "The client will maintain full range of motion in affected joints without contracture.",
    "The client will report pain controlled with approved analgesics.",
    "The client and family will demonstrate correct factor reconstitution and infusion.",
    "The client will participate in safe physical activity appropriate to age.",
    "The client will remain free of intracranial and airway bleeding."
  ],
  "interventions": [
    {
      "title": "1. Treating an acute bleed",
      "points": [
        "Administer factor replacement immediately at the first symptom — treat on suspicion, never wait for swelling or imaging.",
        "Apply RICE to a bleeding joint: rest and immobilize in a position of function, apply ice, gentle compression and elevation.",
        "Hold firm direct pressure over external bleeding for at least 10–15 minutes without lifting to peek.",
        "Use topical hemostatics and antifibrinolytics such as aminocaproic or tranexamic acid for mucosal and dental bleeding as ordered.",
        "Give desmopressin for mild hemophilia A when prescribed; it does not work for hemophilia B.",
        "Monitor hemoglobin, vital signs and the size of any hematoma with a marked outline."
      ]
    },
    {
      "title": "2. Preventing bleeding",
      "points": [
        "Avoid intramuscular injections; use the subcutaneous route with the smallest needle and apply pressure afterward.",
        "Avoid rectal temperatures, suppositories and unnecessary invasive procedures.",
        "Prohibit aspirin, NSAIDs and other antiplatelet agents; use acetaminophen for routine pain.",
        "Provide a soft toothbrush, supervised dental hygiene and regular preventive dental visits with factor cover before work.",
        "Pad the crib and play areas for toddlers and use knee and elbow pads during learning-to-walk years.",
        "Verify a medical alert bracelet is worn."
      ]
    },
    {
      "title": "3. Preserving joints and mobility",
      "points": [
        "Immobilize an acutely bleeding joint only briefly, then begin gentle range of motion once bleeding is controlled, as directed by physical therapy.",
        "Never perform passive range of motion on an actively bleeding joint — it extends the bleed.",
        "Encourage strengthening of surrounding muscles between episodes; strong muscles protect joints.",
        "Guide activity selection: swimming, cycling with a helmet, walking and golf rather than football, wrestling or hockey.",
        "Refer to physical therapy for a long-term joint-preservation program."
      ]
    },
    {
      "title": "4. Recognizing emergencies",
      "points": [
        "After any head injury, give factor first and seek imaging; treat every head bump as potentially intracranial bleeding.",
        "Monitor for headache, vomiting, irritability, drowsiness, unequal pupils or seizure.",
        "Watch for airway compromise from neck, throat or tongue bleeding — hoarseness, drooling or stridor.",
        "Suspect retroperitoneal or iliopsoas bleeding with abdominal, hip or groin pain and a hip held in flexion."
      ]
    },
    {
      "title": "5. Family teaching and psychosocial support",
      "points": [
        "Teach reconstitution, venipuncture and infusion step by step with return demonstration until the family is confident.",
        "Help the family keep a bleed log with date, site, dose and response, which guides prophylaxis adjustments.",
        "Coordinate with the school nurse on activity, emergency plans and factor storage.",
        "Discuss inheritance and offer genetic counseling to carriers and prospective parents.",
        "Address overprotection: children need age-appropriate independence and risk to develop normally.",
        "Connect with a hemophilia treatment center and peer camps."
      ]
    }
  ],
  "patient_teaching": [
    "Infuse factor at the first sign of a bleed — tingling or warmth in a joint counts.",
    "Go to the emergency department after any head injury, even a minor one, after giving factor.",
    "Never take aspirin or ibuprofen; use acetaminophen instead and check every new medication.",
    "Wear a medical alert bracelet at all times.",
    "Choose non-contact sports and keep muscles strong to protect joints.",
    "Tell every dentist and surgeon about hemophilia before any procedure so factor cover can be arranged.",
    "Keep the bleed log and bring it to every clinic visit."
  ]
}