Chronic Care Exam 1
1. Discuss the etiology, clinical manifestations, complications, diagnostic studies, nursing management and collaborative
care (including diet and patient education) of chronic hematologic, cardiovascular, gastrointestinal, and renal conditions.
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Sickle Cell Disease: Group of inherited, autosomal recessive disorders characterized by an abnormal form of
hemoglobin in erythrocytes
o Major types: Sickle cell anemia, Sickle-cell thalassemia, sickle cell Hgb c Disease, and sickle cell trait
o Etiology:
 Sickle cell anemia
 Most severe of SCD syndromes.
 When person is homozygous for hemoglobin S (Hgb SS)
 Sickle Cell thalassemia & sickle cell Hgb C
 When person inherits Hgb S from one parent & another type of abnormal (ex. Thalassemia or
Hgb C) from the other parent
 Sickle Cell Trait
 When person is heterozygous for Hemoglobin S (Hbg AS)
o Inherits Hgb S from one parent and Hgb A from other
o Typically mild to asymptomatic condition
o Pathophysiology:
 Major physiologic event of SCD is sickling of erythrocytes
 Sickling episodes most commonly trigger by a low oxygen tension in the blood
 Sickled RBCs become more rigid and elongated crescent- shaped
 Sickled cells can’t easily pass through capillaries/small vessels and can cause vascular
occlusion, leading to acute or chronic tissue injury
 Resulting homeostasis promotes a self-perpetuating cycle of hypoxia, deoxygenation of more
erythrocytes, and more sickling
o Circulating sickle cells are hemolyzed by the spleen , leading to anemia
o Condition is eventually irreversible due to cell membrane damage
o Diagnostic studies:
 Blood smear abnormal reticulocytes/sickled cells
 Hgb S exposed by sickling test (exposing erythrocytes to deoxygenating agent)
 Spleen size may decrease in size & become nonfunctioning
 Bone & joint deformities, stroke, DVT on x-ray, MRI, doppler respectively
o Clinical manifestations: #1 symptom is pain as a result of tissue ischemia
 Back, chest, extremities, and abdomen are most common areas affected
 Symptoms: fever, swelling, tenderness, tachypnea, HTN, N/V
 Organ issues: heart enlarges which may lead to CHF, lungs acute chest syndrome, spleen become
small and nonfunctioning, brain stroke, kidneys AKI
Sickle Cells Disease & Sickle Cell Crisis
o Primary symptom is pain b/b of damaged sickle cells clump together and adhere to blood vessels
impaired blood flow
 Lack of blood flow to vital organs extreme pain and should be addressed 1st
o Medications: optimal pain control large doses of opioid analgesics + breakthrough analgesia often in PCAs
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Hydroxyurea is the only anti-sickling agent that has helped
 It increases production of Hgb F (fetal hemoglobin) which decreases hemolysis and increases
Hgb concentration
 Fluids and electrolytes are administered to treat hypoxemia & control sickling
 Transfusion therapy is indicated when aplastic crisis occurs
o These pts may also need iron chelation therapy to remove excess iron from the blood
 Iron overload headaches, GI probs, bloody stools
Patient Education for Sickle Cell Disease
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Teach patient ways to avoid sickle cell crises like avoiding high altitudes, avoiding hypoxemia and
dehydration, and seeking medical attention immediately to counteract crises possible causes
Teach pain control and medication compliance
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Thalassemia
o Groups of diseases involving inadequate production of normal hemoglobin, and therefore decreased
erythrocyte production
 Hemolysis also occurs in thalassemia
o Commonly found in ethnic groups w/ origins near the Mediterranean Sea and equatorial/near equatorial
regions of Asia, Middle East, and Africa.
o Etiology:
 Autosomal recessive genetic mode of inheritance
 1. Thalassemia Minor (Thalassemia trait): person is heterozygous w/ one normal gene and one
thalassemia gene
 2. Thalassemia Minor: Person is homozygous w/ two thalassemia genes
 This is the severe form of the disease
o Clinical Manifestations & Diagnostics:
 Symptoms usually develop by the age of 2
 Blood tests will reveal alterations in the body’s erythrocytes and is used to confirm Dx
 Prenatal testing can be done to dx in utero
 Thalassemia Minor Symptoms:
 Frequently asymptomatic
 Mild to moderate anemia w/ microcytosis (small cells) and hypochromia (pale cells)
 Thalassemia Major Symptoms:
 Delayed physical and mental development
 Pallor
 Splenomegaly, hepatomegaly, and jaundice from hemolysis of RBCs
 Chronic bone marrow hyperplasia causing expansion of the marrow space
 Thickening of the cranium and maxillary cavity
Thrombocytopenia
o Condition in which blood has decreased number of platelets (<150,000)
o 3 Types: Inherited, acquired immune, acquired nonimmune
 Inherited thrombocytopenia causes: Fanconi anemia (pancytopenia), hereditary thrombocytopenia
 Acquired-immune thrombocytopenia causes: immune or idiopathic thrombocytopenia purpura (ITP),
Neonatal alloimmune thrombocytopenia
 Acquired non-immune thrombocytopenia causes:
 shortened circulation (increased consumption)
o Thrombotic thrombocytopenic purpura (TTP), disseminated intravascular coagulation
(DIC), heparin-induced thrombocytopenia, splenomegaly or splenic sequestration
 Turbulent blood flow (hemangiomas, abnormal cardiac valves, intra-aortic balloon pump)
 Decreased production
o Drug induced marrow suppression, chemotherapy, bacterial infections (sepsis),
alcoholism, bone marrow suppression, viral infections (HIV, hepatitis,
cytomegalovirus), myelofibrosis, aplastic anemia, radiation bone, solid tumor
infiltrating bone marrow, hematologic malignancy (leukemias, lymphomas,
myelomas)
o Idiopathic thrombocytopenia purpura (ITP) AUTOIMMUNE DISEASE
 most common acquired thrombocytopenia
 syndrome of abnormal circulating platelet destruction
 in ITP, platelets are coated with antibodies. They function normally but die faster than normal RBCs (810 days is normal), b/c when they reach the spleen they are recognized as foreign and are destroyed by
macrophages
 Chronic ITP is most common in WOMEN ages 15-40
 Is has a gradual onset and transient remissions occur
o Thrombotic thrombocytopenia purpura (TTP)
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Uncommon syndrome characterized by hemolytic anemia, thrombocytopenia, neurological
abnormalities, fever (in the absence of infection), and renal abnormalities
 usually caused by deficiency of plasma enzyme (ADAMSTS13) that breaks the Vonwillebrand
(vWF) clotting factor down into normal size
o If ADAMSTS13 is not in the body, vWF clotting factors become too large and attach to
activated platelets increased platelet aggregation
 It is idiopathic or caused by drug toxicity, pregnancy or preeclampsia, infection, or known
autoimmune disease
 Is a MEDICAL EMERGENCY b/c bleeding and clotting occur simultaneously
o Heparin Induced Thrombocytopenia (HIT)
 LIFE-THREATENING condition, should be suspected if platelet count falls more than 50% below
baseline or below 100,000
 Symptoms of bleeding are rare b/c platelet count rarely drops below 20,000
 Platelet destruction and vascular endothelial injury are major responses to an immune-mediated
response to heparin
 Platelet factor 4 binds to heparin
 This complex then binds to the platelet surface and further platelet activation + increased
release of PF4= positive feedback loop
 Antibodies are created against this complex and removed prematurely from circulation
thrombocytopenia and platelet-fibrin thrombi
 ** pts are at greater risk for clots than bleeding
 Major complication of HIT is venous thrombosis (Ex. DVT, PE, thrombotic stroke, MI)
 Collaborative Care of HIT:
 Discontinue heparin when HIT is 1st recognized to stop platelet destruction and decrease
endothelial injury
 Start pt. on direct thrombin inhibitor, like Argatroban or Fondaparinux, an Xa inhibitor
o Only start warfarin once platelet count is 150,000 or greater
 Pts with HIT do not ever get heparin or LMW heparins
o Goals for thrombocytopenia pts:
 Have no gross/occult bleeding
 Maintain vascular integrity
 Manage homecare to prevent any complications r/t increased risk of bleeding
Disseminated Intravascular Coagulation (DIC)
o Serious bleeding and thrombotic disorder from abnormally initiated and accelerated clotting
 Subsequent decrease in clotting factors and platelets= possible uncontrollable hemorrhage
o Characterized by profuse bleeding resulting from decreased platelets and clotting factor
o Etiology:
 DIC is abnormal response to the normal clotting cascade stimulated by the disease process or disorder
 Disorders that predispose to DIC: shock, sepsis, placental abruption, acute liver failure,
malignancies, severe head injuries, heat stroke, massive tissue trauma, crush injuries,
pulmonary emboli
 Can occur acutely, catastrophic condition, or may exist at subacute or chronic level
 Chronic/subacute is often seen in pts. w/ long-standing illness like malignant disorders or
autoimmune diseases
Clinical manifestations for
 Hypertension
o Etiology
o Clinical manifestations
o Complications:
o Diagnostic studies:
o Nursing management & collaborative care (+ diet & pt. education)
2. Identify treatment options and pharmacologic interventions for chronic hematologic,
cardiovascular, gastrointestinal, and renal conditions.
3. Review blood products and indications for use (should be review from Foundations).
4. Describe possible acute transfusion reactions, clinical manifestations, and treatment.
5. Differentiate between enteral vs parenteral nutrition and the indications of each.
6. Identify problems related to tube feedings and corrective measures.
7. Identify complications of parenteral nutrition.
8. Determine appropriate delegation and prioritization for these disorders.