NCLEX-PN ExamPrep: Care of the Client with Hematological Disorders

Date: Jun 21, 2011

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This chapter discusses the most common types of hematological disorders to assist in preparing for the NCLEX-PN exam.

The hematologic system consists of blood, blood cells, and blood forming organs. Because the circulation of blood provides oxygen and nutrients to all body systems, a functioning hematological system is essential to health and well-being. A disorder in the system might result from a lack of function, a reduction in production, or an increase in the destruction of blood cells. This chapter reviews common disorders affecting the blood.

Anemia

When anemia occurs, people have a decrease in the number of red blood cells or a decrease in the capability of these red blood cells to carry oxygen. Anemia is not a disease but is a symptom of other disorders, such as thalassemia and iron deficiency anemia. The causes of anemia are as follows:

The symptoms of anemia are as follows:

CAUTION

Children with persistent anemia might experience frequent bouts of otitis media and upper respiratory infections.

Pernicious Anemia

The intrinsic factor is missing in pernicious anemia, resulting in an inability to absorb vitamin B12. Pernicious anemia is common in the elderly and clients who have had a gastric resection. It can also occur from poor dietary intake of foods containing B12, especially in vegetarian diets or those lacking dairy products. Symptoms of pernicious anemia include

The treatment for pernicious anemia is the administration of injections of cyanocobalamin (vitamin B12). The injections are given weekly until adequate levels are reached, and then monthly for maintenance. The nurse should be aware of the following points when giving this drug:

Aplastic Anemia

Aplastic anemia is a rare disorder that occurs when there is depression of the blood-forming elements of the bone marrow. The cells are replaced with fat. Symptoms associated with aplastic anemia include

Aplastic anemia can be either primary (congenital) or secondary (acquired). Approximately half of all acquired cases are from unknown causes. Several factors can contribute to the development of aplastic anemia and can include

Treatment of acquired aplastic anemia is accomplished by first identifying that something is preventing the bone marrow from carrying out its basic function. The focus of treatment is on the identification and removal of the offending agent. The client might also receive immunosuppressive therapies, including

Because of the possible fatal course that can develop from this disease, a bone marrow transplantation is also a treatment for consideration early in the disease.

Sickle Cell Anemia

Sickle cell anemia is the most common genetic disease found in the United States. It is more common in the African-American population. This disorder is inherited as autosomal recessive. A disease inherited in this manner is characterized by each parent having the sickle cell trait, resulting in a 25% chance of producing offspring with sickle cell anemia with each pregnancy.

A client with sickle cell anemia has red blood cells that have an abnormal crescent shape, as illustrated in Figure 10.1. Because they cannot flow easily through tiny capillary beds, they can become clumped, cause obstruction, and become an impairment in tissue perfusion. Red blood cells containing Hgb S are prone to sickling when exposed to decreased oxygen in the blood. After they become sickled, they are more fragile, rigid, and rapidly destroyed.

Figure 10.1 Normal and sickled red blood cells.

The most common forms of this disease in the United States are

Some clients might have sickle cell trait. When this occurs a child inherits normal hemoglobin from one parent and hemoglobin S (the abnormal hemoglobin) from the other. The same defect exists as in sickle cell anemia, but only portions of the hemoglobin are Hgb S. The majority of the blood is Hgb A (normal hemoglobin). These clients might not exhibit symptoms. If these clients are exposed to low oxygen levels, symptomology and severe anemia can occur. Non-painful gross hematuria is the major complication that might occur with sickle cell trait.

The defect in sickle cell anemia can result in an obstruction in blood flow due to sickling or increased destruction of red blood cells (RBCs). Clinical manifestations of the disease are related to these defects and include

Several crises or exacerbations happen with sickle cell anemia. Table 10.1 outlines the types of crises associated with sickle cell anemia. The basic pathophysiology for most of the crises involves circulatory compromise in the micro circulation caused by the sickled cells. You should study this table, differentiating the types and placing primary focus on the most common type of crisis: vaso-occlusive.

Table 10.1. Sickle Cell Anemia Crises*

Type of Crisis

Description

Associated Symptoms Include

Vaso-occlusive

Usually appears after the age of five. Sickling of cells results in obstruction of blood vessels, leading to a lack of oxygen to the area, which causes localized hypoxia and necrosis. It's usually not life-threatening. Hand-foot syndrome can be a result of this crisis. It is a skeletal problem that occurs in children six months to two years of age. Swelling is found in the hands and feet but usually resolves itself in two weeks.

Severe pain: Bone, abdominal, muscular, or thoracic. Jaundice, dark urine, priapism, fever, leukocytosis, lethargy, fatigue, sleepiness.

Sequestration

Occurs between the age of two months and five years. There is massive pooling of RBCs in the liver and spleen.

Lethargy, pale skin, hypovolemia (tachycardia, decrease in urinary output, and so on).

Aplastic

Results from bone marrow depression, is associated with viral infections (especially human parvovirus), and leads to RBC lysis and severe anemia.

Lethargy, pale skin, shortness of breath, altered mental status.

Hyperhemolytic

Rare; the result of certain drugs or infections. RBC destruction occurs.

Abdominal distention, jaundice, dark urine, reticulocytosis. If reticulocytosis occurs, it suggests some other associated problem (for example, a deficiency in glucose-6 phosphate dehydrogenase [G6PD]).

Stroke

Sickled cells block major blood vessels in the brain leading to an infarction.

Neurological impairments (see Chapter 11, "Care of the Client with Disorders or the Neurological System," for clinical manifestations of a stroke).

Chest Syndrome

Similar to pneumonia; due to sickling in lung blood vessels.

Chest pain, fever, cough, anemia.

Overwhelming Infection

Especially streptococcus pneumonia, Haemophilus influenzae. It is due to a defect in spleen function. Alert: This is the number one cause of death in children under the age of five!

Symptoms of infection and septicemia (high fever, tachycardia, tachypnea, and so on).

NOTE

The vaso-occlusive crisis is the primary crisis type that causes the client to have pain.

The treatment for sickle cell anemia is twofold. The goals are to prevent sickling and treat sickle cell crises. This is accomplished by several measures, including

TIP

An easy way to remember general nursing care for clients with sickle cell anemia is to remember the following:

Iron Deficiency Anemia

There is a simple lack of iron in this disorder. It is the most prevalent nutritional disorder in the United States. The cause could be the result of poor dietary intake of iron sources. Age and financial status have an influence in this disorder with 25% of children 6–24 months of age in the low socioeconomic population having a diagnosis of anemia. Adolescents are at risk due to rapid growth needs and inadequate nutritional eating practices. Premature and multiple birth babies have reduced fetal iron supplies, making them at risk of iron deficiency anemia. Other causes are associated factors include

The symptoms of iron deficiency anemia are the same as general anemia. There are a few symptoms for severe, prolonged anemia that are different (included here):

The management of iron deficiency anemia is conservative. The cause of the anemia is explored. The healthcare provider will focus on determining the source of the blood loss. A stool specimen for occult blood and endoscopic exam might be ordered to rule out these common GI sources of bleeding. A thorough menstrual history is obtained from female patients with anemia to determine whether blood loss might be the source of the anemia.

The treatment for iron deficiency anemia includes treating the underlying cause of the anemia, medications for iron replacement, client education, and administration of blood transfusions in severe cases of depletion. The drugs used during therapy include ferrous sulfate (Feosol) and iron dextran injection (Imferon).

TIP

IM iron is called IMferon.

The examinee should remember the following teaching and administration points when giving iron:

Thalassemia

Thalassemia is an autosomal-recessive group of hereditary blood disorders that is found mostly in the African-Americans, Asians, and the Middle East populations. The most common forms are

The signs and symptoms associated with thalassemia are frequently associated with anemia and include

The treatment of thalassemia revolves around maintenance of adequate hemoglobin that will allow oxygenation of tissues and prevention and treatment of complications. The following objectives will be accomplished by the nurse:

Polycythemia Vera

The polycythemia vera disorder is characterized by thicker than normal blood. With polycythemia vera, there is an increase in the client's hemoglobin to levels of 18 g/dL, RBC of 6 million/mm, or hematocrit at 55% or greater and increased platelets.

The clinical manifestations that are assessed in clients with polycythemia vera relate to the increased viscosity of the blood. The following list describes some of the symptoms that are associated with polycythemia vera:

If polycythemia vera is not treated, few patients will live longer than two years. Treatments revolve around reducing the thickness of the client's blood and providing an easier flow of blood through the blood vessel. This is accomplished by

CAUTION

With polycythemia, the client is at risk for cerebrovascular accident (CVA), myocardial infarction (MI), and bleeding due to dysfunctional platelets.

Hemophilia

In hemophilia, there is a deficiency of one of the factors necessary for blood coagulation. An abnormal clotting pattern occurs, resulting in an ineffective clot. Hemophilia is inherited as a sex-linked disorder. It is an x-linked recessive disorder. The mother passes this disorder to her male children. When a female inherits the gene from her father, she has a 50% chance of transmitting it to her son. Approximately 40% of patients with hemophilia have no hereditary genetic links. Clients lacking factor VIII have hemophilia A (Classic hemophilia); clients lacking factor IX have hemophilia B (Christmas disease). The discussion that follows focuses on Factor VIII deficiencies because they are the most common (75% of cases).

There are varying degrees of severity associated with hemophilia that relate to the amount of Factor VIII activity. Severe hemophilia occurs when there is 1% of Factor VIII activity. This client can have spontaneous bleeding without trauma. Moderate hemophilia happens with 1%–5% of Factor VIII activity and bleedings occurs with trauma. The last degree of severity is the mild form that occurs with 5%–50% of Factor VIII active. Clients with mild hemophilia bleed with severe trauma or when surgery is performed. Signs or symptoms of hemophilia include

CAUTION

Intracranial bleeding is the major cause of death in clients with hemophilia.

The primary goals of treatment for clients with hemophilia are to promote adequate blood clotting and to prevent and treat complications or problems associated with the disease. Nursing measures for goal accomplishment include the following:

CAUTION

Cryoprecipitates are no longer used for treatment of hemophilia because HIV and hepatitis cannot be removed.

Transfusion Reactions

When clients lack blood or blood components, it might be necessary for these components to be replaced. Possible causes of the need for a transfusion include trauma, red blood cell destruction disorders, and bone marrow depression. Table 10.2 outlines types of reactions associated with blood transfusions. Study the symptoms extensively and expect to see items related to reactions on your nursing exams.

Table 10.2. Blood Transfusion Reactions

Type of Reaction

Symptoms

Treatment

Hemolytic (due to blood type or Rh incompatibility)

Headache, chest pain, anxiety, lower back pain, hypotension, tachycardia, tachypnea, hemoglobinemia, bronchospasm, vascular collapse

Stop blood transfusion. Send tubing and blood to laboratory. Maintain blood volume and renal perfusion.

Febrile reaction

Chills, tachycardia, fever, hypotension

Antipyretic administration; pretreat with future transfusions or give washed RBCs.

Allergic reaction (patient usually has a history of allergies)

Urticaria, itching, respiratory distress, anaphylaxis

Pretreat with antihistamine.

Bacterial reaction due to contaminated blood (not a common occurrence)

Tachycardia, hypotension, fever, chills, shock

Same treatment as septic shock.

Circulatory overload (more likely in elderly, children, and clients with multiple transfusions of whole blood)

Symptoms of congestive heart failure: hypertension, bounding pulse, distended neck veins

Monitor intake and output. Infuse blood slowly. Administer ordered diuretics.

Case Study

Joey is a 5-year-old boy admitted to the pediatric unit with a diagnosis of sickle cell anemia. He is in pain, and his parents reported that he had been running a temperature for three days due to bronchitis. The doctor diagnoses vaso-occlusive crisis. Joey's temperature is 101.8° F and you assess edema of the right knee.

  1. What events could have triggered this crisis? Give a description of vaso-occlusive crisis, as well as signs and symptoms associated with the disorder.

  2. Recommend three priority nursing diagnosis on admission of the child.

  3. Which plans do you expect to be included in the physician's prescription?

  4. This is Joey's first admission with this diagnosis. Name five teaching plans you will recommend to educate Joey's parents prior to discharge.

Answers to Case Study

  1. Triggers of a crisis include low oxygen levels, stress, infection, and dehydration. A probable cause of Joey's crisis is the infection and fever noted in the client history.

    Vaso-occlusive crisis occurs when sickling cells cause occlusion of vessels, decreasing the amount of oxygen to the localized site causing pain and necrosis. Signs and symptoms include fever, pain, and tissue engorgement.

  2. Priority nursing diagnoses include

    • Risk for injury
    • Infection
    • Pain
    • Impaired tissue perfusion

  3. The nurse would expect plans to include

    • Hydration implementation PO or IV
    • Oxygen orders and/or blood products to improve tissue perfusion and prevent further sickling
    • Pain medication around the clock
    • A heating pad to involved areas to improve comfort

  4. Education needs are related to the disease process and include

    • Preventing crisis (adequate hydration, avoiding altitudes and low oxygen levels, avoiding stress)
    • Signs to observe that indicate a crisis episode
    • Signs and symptoms of infection
    • Resumption of age-appropriate activities that are no contact; for example, swimming
    • Explanation of the disease process and genetic factors
    • Explanation of the healthcare regimen prescribed by the physician

Key Concepts

This chapter discussed the most common types of hematological disorders. The key concepts will help the nursing graduate on the NCLEX exam by focusing on the most commonly used key terms, diagnostic exams, and pharmacological agents used to treat these disorders. This section is covered on the NCLEX exam in the area of physiological integrity.

Key Terms

Diagnostic Tests for Review

The diagnostic tests for a client with hematological disorders are the same as any other routine hospitalization of a client (CBC, urinalysis, and chest x-ray). Specific tests, such as the Schilling test for B12 deficiency, and sickledex and Hgb electrophoresis, are used to diagnose sickle cell anemia and thalassemia. Review these tests prior to taking an exam for a better understanding of the disease process:

Pharmacological Agents Used in Hematological Disorders

A client with a hematological disorder will receive a number of medications to stimulate red blood cell production and replace needed vitamins or nutrients. Analgesics are also a requirement for the pain associated with some diseases. You'll need to review the drugs listed in Table 10.3 prior to an exam for knowledge of their effects, side effects, adverse reactions, and nursing implications. These medications are not inclusive of all the agents used to treat gastrointestinal disorders; therefore, you will want to keep a current pharmacology text handy for reference.

Table 10.3. Hematological Pharmacological Agents

Name

Action

Side Effects Include

Pertinent Nursing Implications

Vitamin B12 (cyanocobalamin)

Necessary for metabolic processes and required for RBC formation. Prevents B12 deficiency and treats pernicious anemia.

Diarrhea, itching, rash, hypokalemia, anaphylaxis, pain at IM site.

Available IM, subcutaneously, intra-nasal (useful by this route only if need is due to a nutritional deficiency). Teach foods high in vitamin B12: meats, seafood, egg yolk, and fermented cheese. PO can be administered with meals or mixed with fruit juice. Intranasal (use within an hour of hot food or liquid). Instruct of lifelong need after gastrectomy.

Ferrous sulfate (Femiron, Feostat) Imferon Tip: IM iron is Imferon

An essential mineral found in hemoglobin. Prevents and treats iron deficiency.

IM and IV: dizziness, seizures, tachycardia, hypotension. IM: skin staining, arthralgia, myalgia, anaphylaxis. PO: constipation, dark stools, diarrhea, pain in epigastric region, staining of teeth with liquid preparations.

Assess for signs of anaphylaxis (rash, itching, laryngeal edema with wheezing). Monitor Hgb and Hct levels. Monitor for overdose: stomach pain, fever, nausea and vomiting, bluish lips and fingernails, seizures, and tachycardia. Avoid antacids, coffee, tea, dairy products, or whole grain breads when on PO iron. Dilute liquid iron in water or fruit juice. Administer with a straw or place drops at back of throat. Give iron with citrus fruit or juice to enhance absorption (can also be taken with vitamin C pill).

Hydroxyurea (Droxia)

Reduces painful crises in sickle cell anemia. An anticancer agent.

Anorexia, nausea, vomiting, consti-pation, hepatitis, alopecia, rashes, pruritus, leukopenia, anemia, thrombocytopenia.

Monitor laboratory values for signs of leukopenia, anemia, and thrombocytopenia in the client. Assess for side and adverse effects.

Apply Your Knowledge

This chapter provided information regarding the care of clients with hematological disorders. The following questions test your knowledge regarding the safe, effective care and management of these clients. The nurse should also refer to the chapter content, key terms, diagnostics, and pharmacological agents sections to assist in answering these questions.

Exam Questions

  1. The nurse can best promote hydration in a 4-year-old with sickle cell anemia by which of the following?

    1. Telling the child how important it is to drink fluids
    2. Forcing fluids of bottled water every two hours
    3. Providing soup on the lunch and dinner meals
    4. Offering flavored ice pops or iced Slurpees

  2. The nurse administering a blood transfusion suspects a reaction has occurred. Which signs and symptoms would the nurse expect with an allergic reaction to blood?

    1. Fever and chills
    2. Hypotension and tachycardia
    3. Rash and hives
    4. Decreased urinary output and hypertension

  3. The nurse is caring for an adult with hemophilia. Which clinical manifestation causes the nurse the most concern?

    1. Hemarthrosis of the elbow
    2. Bruise of the ankle
    3. Oozing of blood at the IV site
    4. Unilateral numbness and lack of movement of arm

  4. The nurse is contributing to a parent education program about sickle cell anemia. Which of the following information should the nurse recommend including?

    1. Apply cold to painful areas
    2. Limit fluid intake
    3. Increase activity during exacerbations
    4. Avoid high altitudes

  5. Which should the nurse observe for as a complication of Factor VIII administration?

    1. Fluid volume excess
    2. Sepsis
    3. Blood transfusion reaction
    4. Thrombus formation

  6. Which of the following is not a named sickle cell anemia crisis?

    1. Aplastic
    2. Vaso-occlusive
    3. Splenic sequestration
    4. Erythropoiesis

  7. A client receiving a blood transfusion exhibits lower back pain, fever, and dyspnea. What is the nurse's initial action?

    1. Stop the blood transfusion and keep the vein open with normal saline
    2. Administer epinephrine per unit protocol
    3. Notify the physician
    4. Obtain a set of vital signs

  8. A client has been admitted with sickle cell anemia in crisis. Which physician prescription would the nurse anticipate?

    1. Restrict fluids 200mL/shift
    2. Ice to painful joints
    3. Start O2 at 3 L/min
    4. Ambulate in hall every two hours

  9. A child is in the hospital with a diagnosis of thalassemia major. Multiple blood transfusions have been ordered along with a chelating agent. The father asks the nurse, "Why does my son get this drug every time he gets blood?" What does the nurse explain as the purpose of chelating drugs?

    1. Stabilize clots in the vascular system
    2. Decrease the chance of a blood reaction
    3. Eliminate iron excess
    4. Boost oxygen delivery to the cells

  10. Which does the nurse recognize as the most accurate in diagnosing sickle cell anemia?

    1. Sickledex
    2. Hemoglobin electrophoresis
    3. Partial thromboplastin time
    4. Complete blood count

Answers to Exam Questions

  1. Answer D is correct. A child will likely accept the fluids in this answer better than the others listed. The child is too young to understand the statement in answer A, so it is inappropriate. Answers B and C are good sources of fluids but would not be best or acceptable for a 3-year-old, so they are incorrect.
  2. Answer C is correct. Other symptoms include respiratory distress and anaphylaxis. Answer A describes febrile nonhemolytic reaction, so it is incorrect. Answer B occurs with hemolytic reaction and answer D is not associated with a blood transfusion reaction, so they are incorrect.
  3. Answer D is correct. The neurological symptoms could mean an intracranial bleed has occurred. The answers in A, B, and C are reasons for concern, but they are not the priority, so they are incorrect.
  4. Answer D is correct. High altitudes can increase oxygen consumption and trigger a crisis. The client should apply heat to painful areas, increase hydration, and rest during crisis making A, B, and C incorrect options.
  5. Answer C is correct. Factor VIII is a blood product, so the nurse would monitor for a transfusion reaction. Answer A is unlikely due to the small volume of fluid administered, so it is wrong. Answers B and D are not immediate concerns for this short-term infusion, so they are incorrect.
  6. Answer D is correct. Erythropoiesis is the formation of RBCs not related to sickle cell crisis. The answers in A, B, and C are types of sickle cell anemia crises, so they are incorrect.
  7. Answer A is correct. The nurse would first ensure that the patient doesn't get any more of the wrong blood due to displaying symptoms of a hemolytic blood reaction. Answers B, C, and D are proper actions with a blood transfusion reaction, but none is the initial action, so they are incorrect.
  8. Answer C is correct. It is not unusual for patients to receive oxygen to prevent additional sickling of cells. The answers in A and B are incorrect because these are contraindicated in sickle cell anemia. Answer D is not recommended for patients in sickle cell crisis, so it is incorrect.
  9. Answer C is correct. A chelating agent such as deferoxamine (Desferal) is given to eliminate excess iron. The answers in A, B, and D are not the action of chelating agents, so they are incorrect.
  10. Answer B is correct. Hemoglobin electrophoresis is the test that verifies the diagnosis of sickle cell and is the most accurate because it separates the different hemoglobins. Answer A is a screening tool that lacks the accuracy of electrophoresis, so it is incorrect. The answers in C and D are not directly related to sickle cell, so they are incorrect.

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