Hemolytic Diseases

Anemia

The lowered concentration of hemoglobin within the blood. It is not a disease but rather a symptom of an underlying disease; it is the most common hematologic condition. There are three cardinal signs for anemia: weakness, fatigue, and pallor. Most other general symptoms only appear in severe or acute anemia.

• General Diagnostic Procedures: Hematologic Studies (CBC), Iron Studies, Serum Vitamin B9 and B12 Levels, and Bone Marrow Aspiration
• General Medical Management: correction of the underlying cause and blood transfusion in severe or acute anemia.
• Nursing Diagnoses
  • Fatigue related to decreased hemoglobin and diminished oxygen-carrying capacity of the blood.
  • Altered nutrition, less than body requirements, related to inadequate intake of essential nutrients.
  • Altered tissue perfusion related to inadequate hemoglobin and hematocrit.
  • Noncompliance with prescribed therapy.
• Potential Complications: Heart Failure, Angina, Paresthesia, Confusion.
• Planning and Intervention:
  • Decrease Fatigue: planning activities and maintenance of physical activity and exercise.
  • Improve Nutrition: diet education + supplementation.
  • Maintain Perfusion: monitor vital signs and pulse oximetry; oxygenation, IV therapy.
  • Compliance
  • Absence of Complications: assess for heart failure and neurologic assessment for megaloblastic anemia.
• Evaluation: decreased fatigue, adequate nutrition, adequate perfusion, no or minimal complications.

Aplastic Anemia

Anemia caused by damaged or ineffective production of blood cells from the bone marrow, resulting in thrombocytopenia, anemia (erythrocytopenia), and leukocytopenia, all together being encompassed by pancytopenia: low concentrations of all cellular blood components.

• Clinical Manifestations: infection (from leukopenia), anemic S/S triad (from anemia), bleeding/bruising (from thrombocytopenia), lymphadenopathy (from infection)
• Diagnostics: bone marrow aspiration
• Medical Management: bone marrow transplant (<60 y.o.), sometimes immunosuppressive therapy
• Nursing Interventions: monitor for drug side effects, problems related to pancytopenia, and compliance.

Iron-Deficiency Anemia (IDA)

The most common type of anemia. Iron may be lost from bleeding, from inability to be absorbed, or simply from not being present in the diet. In premenopausal women, the most common cause is menstruation. In men and postmenopausal women, the most common cause is GI bleeding.

• Clinical Manifestations: anemia S/S triad; in prolonged or severe cases, mouth ulceration (angular cheilosis) and smooth/sore tongue, and brittle/ridged nails.
• Diagnostics: bone marrow aspiration, Iron studies (Serum Iron and Ferritin, CBC, Hgb, Hct)
• Medical Management: iron supplementation, elimination of curable cause (GI bleeding, ulcers, etc.), assessment of occult blood in stool.
• Nursing Interventions: health education about use of iron supplements
  • PO: (if liquid) take with straw, as it may stain the teeth; take before meals for best absorption (may cause GI upset), take after meal to avoid GI upset, do not take with antacids or milk (diminishes absorption). Intake of Vit. C (orange juice) with the supplement helps with absorption.
  • IM: deep IM (Z-Track); use an aspirating needle; check for anaphylactic reaction with a small test dose.

Megaloblastic Anemia

Megaloblastic anemia is the proliferation of immature erythrocytes caused by inadequacies in B9 (Folate), B12 (Cobalamin), or Intrinsic Factor (IF; required for B12 absorption). Their manifestations are similar, except for B9 not having neurological manifestations.

• Clinical Manifestations: anemia S/S, neurological manifestations (B12 is required for neurological activity), altered LOC, jaundice (from low lifespan of megaloblasts)
• Diagnostics: Schilling’s Test (testing Intrinsic Factor effectivity), Serum tests for B9, B12, and a CBC (very high MCV result).
• Medical Management
  • B9 Deficiency: diet improvement and B9 supplementation, 1 mg OD, IM if malabsorption is present. Re-assess and adjust supplementation as necessary.
  • B12 Deficiency: diet improvement if vegetarian; supplementation with vitamins or fortified soy milk. If due to IF deficiency, monthly IM supplementation continued for life.
    • Even if IF is deficient, a small amount may still be absorbed if delivered per orem. A large dose should be used (2 mg/day) if IM supplementation is not preferred.
• Nursing Interventions:
  • Assess for jaundice, vitiligo, and premature graying of skin.
  • Assess gait (the need for assistive devices, safety, and therapy)
  • Explain the process and importance for compliance with the 24-hour urine test for Schilling’s test.
  • Explain the importance for compliance with the lifelong B12 supplementation therapy.

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Disseminated Intravascular Coagulation (DIC)

A coagulation disorder causing an over-activation of the clotting cascade. This results in simultaneous thrombosis and hemorrhage. May be acute or chronic; highly fatal. Because of the overuse of clotting factors, bleeding is a problem despite the abnormal rates of coagulation. Two contrasting problems cause high difficulty in treatment for severe cases.

• Signs and Symptoms:
  • Clotting: Chest pain, shortness of breath, myocardial infarction (from obstruction of coronary arteries), cerebral effects from decreased perfusion (LOC changes)
  • Internal Bleeding: hematuria, melena/occult blood in stool, hemoptysis, cerebral hemorrhaging (changes in LOC)
  • External Bleeding: hematoma (subcutaneous bleeding), mucosal bleeding, purpura/petechiae/bruising.
• Diagnostics: CBC (Platelet count and clotting factor counts are reduced, PT and PTT times are increased)
• Medical Management: treat the underlying cause (if known).
  • Secondarily, correct ischemia by improving oxygenation, replacing fluids, correcting electrolyte imbalances, and administering vasopressor medications.
  • Replace overused clotting factors and platelets via transfusion of cryoprecipitate and fresh-frozen plasma.
  • Controversial in use, but heparin may also be used
• Nursing Interventions:
  • Maintain hemodynamic status: observe procedures to minimize wounding and risk for bleeding.
  • Monitor for imbalanced fluid volume: faulty circulation may result in fluid retention. Assess for edema and monitor breath sounds. Administer diuretics and minimize IV volume as prescribed.
  • Assess for ineffective tissue perfusion related to microthrombi: assess neurologic, pulmonic, and skin systems.

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Idiopathic Thrombocytopenic Purpura

Lysis of platelets after binding to antiplatelet autoantibodies. Compensatory production of platelets in the bone marrow occurs following this. Its acute form is mostly self-limiting, but chronic forms may last.

• Clinical Manifestations: mostly asymptomatic; easy bruising, petechiae, purpura, heavy menses and mucosal bleeding, thrombocytopenia
• Diagnostics: Decreased platelet count, increased bleeding time, and increased megakaryocyte (immature thrombocyte) counts in a bone marrow aspiration procedure.
• Medical Management: maintenance of a safe platelet count. Platelet infusions are avoided, as they will simply be destroyed. It is only used for cases of catastrophic bleeding.
  • A splenectomy may be done (the spleen being the producer of the antiplatelet autoantibody and site of destruction). Unsure, but has been shown to work in 85% of patients.
• Pharmacologic Therapy: Immunosuppressants are the treatment of choice. Bone disease should be monitored for patients on long-term management. Supplementation of Calcium and Vitamin D may be beneficial.
• Nursing Interventions:
  • Bleeding: risk assessment based on lifestyle, medications being taken, and physical assessment for sites of bleeding. Assess neurologic signs for bleeding.
  • Teach the patient to avoid vigorous sexual activity when platelet count is below 10k/mm³.

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Hypertension

Normal blood pressure is defined as being <120/<80 mmHg. Hypertension is a persistent blood pressure of >140/>90 mmHg.

Classification	Systolic	Diastolic
Normal	<120	<80
Prehypertensive	>120	<80
Stage 1	>130	<90
Stage 2	>140	>90
Crisis	>180	>120

• Clinical Manifestations: A major risk factor for atherosclerosis, CAD, retinal manifestations (hemorrhage, exudates, spots), HF
• Diagnostics: Historical, physical, and retinal examination; urinalysis, blood chemistry (electrolytes, sugar, cholesterols), ECG, and ECHO to assess for left ventricular hypertrophy.
• Medical Management: Goal is to maintain blood pressure at or below 140/90 mmHg. Lifestyle changes include stopping vices, reducing weight (if applicable), and improving diet (using DASH, low fat, low sodium). First-line pharmacologic interventions involve diuretics and beta-blockers.
• Nursing Assessment: regular checking of blood pressure, secondary organ damage, pulse assessment.
• Nursing Diagnoses:
  • Deficient knowledge regarding the relationship between the treatment regimen and control of the disease process.
  • Noncompliance with therapeutic regimen related to side effects of prescribed therapy.

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Beurger’s Disease

A repeating inflammation of the intermediate and small arteries and veins of the lower and upper extremities. It results in thrombus formation and segmental occlusion of the vessels. Most commonly occurs in men from 20 to 35 years old. Smoking or tobacco is an aggravating or causative factor.

• Clinical Manifestation: pain, changes in color, (if in feet) instep claudication.
• Diagnostics: segmental blood pressure, contrast angiography, duplex ultrasonography.
• Medical Management: stop tobacco use, regional sympathetic block/ganglionectomy to prevent vasodilation, debridement of necrotic tissue, if a toe is gangrenous, BKA/AKA indicated for severe gangrene, severe resting pain or severe sepsis.
  • Vasodilators are not prescribed as they only cause vasodilation for healthy vessels.

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Raynaud’s Disease

Intermittent arteriolar vasoconstriction resulting in coldness, pain, and pallor or the fingertips or toes (Raynaud’s Phenomenon). Most commonly occurs in women from 16 to 40 years old.

• Clinical Manifestations: pallor followed by cyanosis, then followed by rubor when vasodilation occurs. (Pink → White → Blue → Red → Pink); paresthesia; bilateral involvement.
• Medical Management: avoiding causative stimuli; calcium-channel blockers; sympathectomy.
• Nursing Interventions: avoiding situations that cause stress, being cold, nicotine, sharp objects, and postural hypertension that some medications may cause.

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Aneurysm

An aneurysm is the enlargement of an artery caused by weakness of the arterial wall. It is often asymptomatic until a rupture occurs. Most commonly comes in tandem with atherosclerosis.

• Clinical Manifestations: usually clinically silent until rupture. Non-ruptured aneurysms may cause obstruction or promote clotting (thromboembolism) which may lead to a stroke or other complications. Thoracic aneurysms may cause dyspnea or dysphagia.
• Complications:
  • Severe chest or back pain may indicate aortic aneurysm.
  • Acute severe headache may indicate an SAH.
  • Angina (chest pain) may indicate obstruction of the coronary arteries by an embolus.
  • Any ruptures may cause pain, hypotension, tachycardia, and lightheadedness.
• Risk Factors include hypertension, poor diet, sedentary living, and obesity. The most common is smoking (it increases risk of both an aneurysm and a rupture).
• Nursing Assessment: anticipate a rupture. Assess for activity of all the organs.

Aortic Aneurysm

The largest artery. Most commonly affected site is the abdominal aorta: Abdominal Aortic Aneurysm (AAA; Triple A). It is normally 2 cm in diameter, and may dilate up to 5 cm. Past 5.5 cm, surgery is necessary. Uncommonly, a Thoracic Aortic Aneurysm (TAA) may occur. It has a higher survival rate but presents the same.

Cerebral Aneurysm

Also known as “berry” aneurysms, as they appear like small berries on branches. Ruptures of these berry aneurysms can cause death within 24 hours; 40% of ruptures being fatal and 66% resulting in permanent neurological damage. These are the most common type of stroke: subarachnoid hemorrhages (SAHs)

Peripheral Aneurysm

Less likely to rupture, but still occur.

• Popliteal Aneurysm: behind the knee. The most common peripheral aneurysm.
• Splenic Artery Aneurysm: near the spleen.
• Mesenteric Artery Aneurysm: the artery supplying the intestines.
• Femoral Artery Aneurysm: the femoral artery by the groin.
• Carotid Artery Aneurysm: the major neck artery.
• Visceral Aneurysm: arteries that supply the bowel or kidneys.

Intracranial Aneurysm Care

• Nursing Assessment: Complete neurologic assessment, pupillary reaction, motor and sensory function, speech, ocular, etc. Detect subtle changes, especially changes in LOC.
• Nursing Diagnoses:
  • Ineffective tissue perfusion (cerebral) related to bleeding or vasospasm.
  • Disturbed sensory perception due to the restriction of aneurysm precautions.
  • Anxiety due to illness or restrictions of aneurysm precautions.
• Complications: vasospasm, seizures, hydrocephalus, re-bleeding, hyponatremia.
• Nursing Interventions:
  • Tissue Perfusion: assess regularly, avoid clotting, avoid increases in intracranial pressure (elevate head, don’t strain, no caffeine, etc.)

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Vein Disorders

Venous Thrombosis, Thrombophlebitis, Phlebothrombosis, and Deep Vein Thrombosis

Caused by Virchow’s Triad: Venous Stasis, Hypercoagulability, and Endothelial Lesions. Thrombophlebitis involves a clot involved with inflammation. If a clot occurs without inflammation, it is a phlebothrombosis.

• Clinical Manifestations: Non-specific. Edema and swelling, tenderness, redness, and warmth.
• Diagnostics: Doppler ultrasonography, duplex ultrasonography, air plethysmography, contrast phlebography (venography)
• Medical Management: prevent the thrombus from growing and fragmenting, resolving current thrombi, and prevent recurrence
• Pharmacologic Therapy: Heparin, Coumadin, Thrombolytics, and for prophylaxis, Fondaparinux SC
• Nursing Interventions: monitor anticoagulant therapy (PT, PTT), monitor complications

Varicosities

Aneurysms but for veins. May be caused by venous insufficiency (valvular defects, pregnancy, thrombophlebitis)

• Clinical Manifestations: swelling, tenderness, burning, aching, itching, and in severe cases skin changes and open sores.
• Management: wear compression stockings, elevate the legs, avoid standing or sitting for long periods of time, ambulate and exercise regularly.