Leukemia and SCD

Course Information

  • Course Title: NURS 2100

  • Institution: Central Community College, Nursing Department

Course Learning Objectives (Competencies & Outcomes)

  • Competencies:

    • Coordinate with patients and families to implement a plan of care that supports patient values and beliefs.

    • Promote the core values of the nursing profession.

    • Manage care of complex patients within the legal and ethical parameters of the nursing profession.

    • Correlate the impact evidence-based approaches have on optimal patient outcomes.

  • Criteria:

    • 2.1: Utilizes therapeutic communication with clients and their families.

    • 2.3: Provides education that supports client values and beliefs.

    • 7.3: Shares educational findings, experiences, and ideas with peers.

    • 8.1: Follows legal and ethical dilemmas for patients with complex health issues.

    • 11.1: Demonstrates best practices in client care.

Exemplar Topics

1. Leukemia: Cellular Regulation

Learning Outcomes

  • Analyze leukemia as it relates to cellular regulation.

  • Describe the pathophysiology of leukemia.

  • Describe the etiology of leukemia.

  • Compare the risk factors for and prevention of leukemia.

  • Identify clinical manifestations of leukemia.

  • Summarize diagnostic tests and therapies used in collaborative care for patients with leukemia.

  • Differentiate considerations for the care of patients with leukemia across their lifespan.

  • Apply the nursing process in providing culturally competent care to individuals with leukemia.

Leukemia: Pathophysiology and Overview

Definition & Characteristics

  • Leukemia: A chronic malignant disorder of WBCs and their precursors, characterized by the replacement of bone marrow by malignant immature WBCs and abnormal circulating WBCs, along with infiltration into other organs such as the liver, spleen, and lymph nodes.

Classification of Leukemia

  • By Acuity:

    • Acute Leukemias

    • Chronic Leukemias

  • By Predominant Cell Type:

    • Lymphocytic Leukemias

    • Myeloid Leukemias

Most Common Types

  • Children: Acute Lymphocytic (Lymphoblastic) Leukemia.

  • Adults: Acute Myeloid Leukemia, Chronic Lymphocytic Leukemia.

Specific Types of Leukemia

Acute Myeloid Leukemia (AML)

  • Uncontrolled proliferation of myeloblasts leading to bone marrow hyperplasia and splenomegaly.

  • Average Age at Diagnosis: 68 years old.

  • Clinical Manifestations:

    • Neutropenia and thrombocytopenia leading to:

    • Recurrent severe infections

    • Petechiae, purpura, ecchymosis

    • Epistaxis, hematomas

    • Hematuria, GI bleeding

    • Anemia: A late manifestation.

    • Causes of Death: Infection or hemorrhage.

    • CBC Findings: Normocytic, normochromic anemia.

Chronic Myeloid Leukemia (CML)

  • Abnormal proliferation of all bone marrow elements, more prevalent in men.

  • Average Age: Error in original content, specify.

  • Chromosomal Association: Philadelphia chromosome, can result from exposure to large doses of ionizing radiation.

  • Phases:

    • Chronic (early): Asymptomatic or vague symptoms.

    • Accelerated: Decreased appetite, weight loss, fever.

    • Acute (blast): Proliferation of blast cells, infiltration into tissues causing splenomegaly, bone damage, and atypical platelet counts.

Acute Lymphocytic Leukemia (ALL)

  • Most common in children and adolescents due to rapid onset.

  • Pathophysiology: Lymphoblasts proliferate in the bone marrow, suppressing normal hematopoiesis.

  • Clinical Manifestations:

    • Infections, bleeding episodes, anemia.

    • Lymphadenopathy, liver enlargement, bone pain, headaches, visual disturbances, vomiting, seizures.

  • Diagnostic Findings: Elevated WBC count with increased lymphocytes.

  • Treatment Success Rate: Combination therapies produce remission in 80-90% of adults, with a commonality of relapses; survival rate in children exceeds 90%.

Chronic Lymphocytic Leukemia (CLL)

  • Characterized by the proliferation and accumulation of small abnormal, mature lymphocytes.

  • The most common leukemia in adults, presenting with a slow onset and often vague symptoms.

  • Clinical Manifestations:

    • Anemia, infections, enlarged lymph nodes, spleen, liver.

    • 5-year Survival Rate: 85%.

Risk Factors for Leukemia

  • Gender Disparity: More frequently affects men than women.

  • Children with immunodeficiency states present increased risk.

  • Previous cancer treatment can contribute to development.

  • Certain genetic disorders can also be risk factors.

  • Environmental Factors:

    • Cigarette smoking.

    • Chemical exposures such as benzene.

    • High-dose ionizing radiation exposure.

  • Noteworthy: Few known causes of childhood leukemias.

Assessment and Clinical Manifestations of Leukemia

  • Clinical Manifestations may include:

    • Fatigue, pallor, significant risk of infection due to decreased WBC functionality.

    • Fever, night sweats, easy bruising, and bleeding manifestations (thrombocytopenia).

    • Bone pain and splenomegaly (enlargement of the spleen).

Nursing Problems Related to Leukemia

  • Potential for infection due to compromised immune function.

  • Risk for bleeding associated with thrombocytopenia.

  • Weight loss due to increased metabolic demands and poor intake.

  • Compromised oral mucous membranes due to treatments.

  • Side effects from various medications used in treatment.

  • Psychological impact leading to grieving processes.

Goals and Outcomes for Leukemia Patients

  • Patient Goals include:

    • Strategy discussions for reducing infection risk.

    • Demonstrating no signs or symptoms of infection.

    • Meeting developmental milestones as appropriate.

    • Expressing emotions related to their diagnosis.

    • Achieving adequate dietary intake for maintaining weight.

    • Reporting symptoms of complications early.

Collaborative Interventions for Leukemia

Surgical Interventions

  • Bone Marrow Transplant (BMT): Treatment of choice for certain leukemias, often in conjunction with chemotherapy.

  • Stem Cell Transplant (SCT): Serves as an alternative to BMT.

    • Involves complete sustained replacement of blood cell lines.

    • Requires closely matched donor tissue.

    • Hematopoietic growth factors are administered to donors before harvesting.

  • Graft-versus-host disease (GVHD): Occurs when immune cells from the donor attack the recipient's tissues, with acute and chronic forms.

Pharmacologic Therapy

  • Chemotherapy: Can be a single agent or combination; risk of tumor lysis syndrome (TLS) is significant with initial treatments.

  • Biological Therapy: Involves cytokines like interferons or interleukins to modify the body's response to cancer.

  • Radiation Therapy: Damages cellular DNA, primarily affecting rapidly dividing cells like cancer cells and bone marrow.

Nursing Interventions and Non-Pharmacologic Collaboration

  • Prevent and manage adverse medication effects.

  • Monitor daily weights and intake/output (I&O).

  • Regularly evaluate infusion sites for complications.

  • Monitor renal function for potential damage.

  • Prevent Infection: Particularly crucial for mucous membranes.

    • Monitor laboratory values: CBC, lactic acid, inflammatory markers.

  • Protect patients from injury related to bleeding; implement bleeding risk interventions and educate patients.

  • Conduct regular assessments of vital signs and body systems for signs of bleeding.

Lifespan Considerations for Leukemia

  • Children: Predominantly develop ALL; symptoms related to decreased RBCs and WBCs. Chemotherapy remains the principal treatment, with targeted therapy for specific high-risk patients.

  • Survival Rates:

    • ALL: >91% five-year survival rate.

    • AML: 66%.

  • Long-term Risk: Patients successfully treated for leukemia face increased health risks in adulthood.

  • Pregnant Women: Acute leukemia is rare but presents significant treatment challenges.

  • Older Adults: Treatment for CLL is often symptomatic due to comorbidities, while treatment for AML is highly individualized.

Evaluation of Outcomes for Leukemia

  • Expected outcomes for leukemia patients include:

    • Maintaining freedom from infections, adequate hydration, and normal electrolyte values.

    • Managing pain at tolerable levels.

    • Demonstrating knowledge of disease processes and treatment plans.

    • Achieving satisfactory quality of life.

Sickle Cell Disease (SCD): Cellular Regulation

Learning Outcomes

  • Analyze SCD as it relates to cellular regulation.

  • Describe the pathophysiology and etiology of SCD.

  • Compare risk factors for and prevention of SCD.

  • Identify clinical manifestations of SCD.

  • Summarize diagnostic tests and therapies used in collaborative care.

  • Differentiate considerations for the care of patients with SCD across their lifespan.

  • Apply the nursing process in providing culturally competent care to individuals with SCD.

Pathophysiology and Overview of SCD

  • Sickle Cell Disease: Defined as a hereditary hemoglobinopathy where normal hemoglobin is replaced by abnormal hemoglobin S (HbS).

  • Sickle Cell Anemia: The most common form of sickle cell disease, characterized by chronic hemolytic anemia.

Etiology and Risk Factors for SCD

  • Transmission: Autosomal recessive genetic defect leading to a 25% risk of having a child with SCD if both parents have the trait.

  • Prevalence is highest among individuals of African descent.

  • Carriers of the sickle cell trait have one HbS gene, often asymptomatic but at risk of developing SCD under severe hypoxic conditions.

Assessment of Clinical Manifestations in SCD

  • Acute and Chronic Manifestations include:

    • Pallor, fatigue, jaundice, irritability.

    • Significant risk of stroke; complications such as splenic enlargement and renal insufficiency.

    • Risk of gallstones, priapism, abdominal pain, skin ulcers, and aplastic crisis.

Diagnostic Collaboration for SCD

  • Diagnostics:

    • Prenatal diagnosis through amniocentesis.

    • Gene testing and counseling are important.

    • Mandatory newborn screening and routine ultrasound starting at age 2 for stroke prevention.

Nursing Problems Related to SCD

  • Inadequate gas exchange due to sickling.

  • Ineffective breathing patterns.

  • Potential for decreased cardiac tissue perfusion.

  • Potential for fluid volume deficiency.

  • Acute pain; caregiver burden, and impaired family functioning.

  • Risk for undernourishment and infection.

Goals and Outcomes for SCD Patients

  • Goals for patients may include:

    • Maintaining oxygen saturation above 90%.

    • Respiratory rates between 12 to 20 breaths per minute during rest and activity.

    • Absence of symptoms related to acute cardiac syndrome or stroke.

    • Consistent reporting of pain below a 3 on a 1-10 scale (adults) or tolerable/absent in children.

    • Meeting criteria for normal growth and development.

Collaborative and Pharmacologic Interventions for SCD

Collaborative Interventions

  • Ensuring optimal oxygenation and circulation through oxygen administration and blood transfusions; balancing activity and rest periods.

  • Maintaining fluid volume balance through I&O documentation and hydration as necessary.

  • Pain management strategies and infection prevention protocols, including prophylactic antibiotics.

Pharmacologic Interventions

  • Hydroxyurea: Used to decrease sickling and is an antineoplastic agent.

  • Analgesics for managing pain; essential for comfort during crises.

Non-Pharmacologic Interventions

  • Surgery Options: Bone marrow or hematopoietic stem cell transplant; note the potential recurrence in about 10% of cases. Splenectomy may be indicated for life-threatening splenic RBC sequestration.

  • Gene Editing: Emerging as a second curative option, enabling genetic modification of cells removed from the bone marrow.

  • Nutritional Management: Patients may have vitamin and macronutrient deficiencies; high-calorie and high-protein diet is recommended.

Lifespan Considerations for SCD

  • Children: Typically asymptomatic until 4-6 months; high risk for acute chest syndrome and stroke.

  • Pregnant Women: Increased risk for pain crises and complications.

  • Older Adults: Rare for individuals with SCD to reach past age 58, but some have managed to live into older adulthood with chronic SCD.

Evaluation of Outcomes for SCD

  • Expected outcomes include:

    • Demonstration of no signs or symptoms of hypoxia or dyspnea.

    • Absence of complications such as stroke or acute chest syndrome.

    • Indicators of adequate hydration and no signs of infection.

    • Pediatric patients should meet growth and developmental milestones.

    • Family education on the disease and treatment regimens is essential, including alternative pain relief measures when necessary.

Nursing Process: Care Plan (DAPI E)

  • Patient Interview: Critical questions to assess the patient.

  • Physical Examination: Key assessment measures to consider.

  • Problem Analysis: Identify what is going wrong in the patient’s body.

  • Prioritization: Define priority issues for care.

  • Goal Setting: Establish achievable patient goals to improve issues.

  • Outcomes Assessment: Determine if goals were met, unmet, ongoing, or partially met. Adjust care plans as necessary.