NCLEX-PN ExamPrep: Care of the Client with Hematological Disorders
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.
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:
- Increased red blood cell destruction
- Blood loss
- Poor dietary iron intake
- Poor absorption
The symptoms of anemia are as follows:
- Pallor (skin might be waxy)
- Cardiac changes (heart murmur, cardiomegaly)
- Growth retardation
- Late sexual maturation
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
- Smooth, beefy red tongue (glossitis)
- Weight loss
- Problems with proprioception (sense of position in space)
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:
- Do not mix in a syringe with other medications.
- Administer IM or deep subcutaneously.
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
- Decreased erythrocytes
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
- Drug toxicity, especially chemotherapeutic agents and the antibiotic chloramphenicol
- Multiple blood transfusions
- Radiation exposure
- Autoimmune states
- Sickle cell syndrome
- Hepatitis B
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
- Antilymphocyte globulin (ALG) or antithymocyte globulin (ATG)
- Cyclosporine (Sandimmune)
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
- Sickle cell anemia: Homozygous form (Hgb S). This is the most severe form; there is no cure.
- Sickle cell-C disease: Heterozygous (Hgb S and Hgb C).
- S thalassemia disease: A combination of sickle cell trait and B+–thalassemia trait (patients can still produce normal hemoglobin).
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
- Dilute urine
- Skeletal deformities
- Exercise intolerance
- Leg ulcers
- Growth retardation
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
Associated Symptoms Include
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.
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).
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.
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]).
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).
Similar to pneumonia; due to sickling in lung blood vessels.
Chest pain, fever, cough, anemia.
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).
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
- Decrease energy needs by the use of bedrest during exacerbations or crises.
- Provide frequent rest periods during everyday activities.
- Provide hydration.
- Avoid contact sports due to splenomegaly.
- Replace missing electrolytes.
- Administer prescribed antibiotics.
- Provide vaccination with HIB, pneumococcal, and meningococcal immunizations.
- Administer oxygen as prescribed.
- Monitor the administration of ordered short-term blood transfusions:
- Packed RBC in aplastic crises, hyperhemolytic, splenic sequestration, chest syndrome, and stroke.
- Blood is also given before major surgery to prevent anoxia and to suppress sickle cell formation.
- A splenectomy might be performed for splenic sequestration.
- Manage pain; apply heat to painful areas, if appropriate.
- Assist with teaching the client to seek medical attention with signs of infection and instruct when to seek medical attention.
- Reinforce teaching that the client should avoid high altitudes.
- Assess vital signs.
- Monitor intake and output.
- Pharmacological interventions include
- Pain medication including morphine sulfate, patient-controlled analgesia, oxycodone, Tylenol with codeine, and NSAIDs
- Hydroxurea (Droxia)
- Participate in reinforcing genetic counseling, including the following information:
- Sickle cell anemia is an autosomal-recessive disorder.
- There is a 25% chance of passing the disease to a child.
- If a child acquires one gene, the child is also a carrier.
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
- People on vegetarian diets
- Maternal iron deficiency
- Malabsorptive disorders
- Parasite infestations
- B12 and folic acid deficiency
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):
- Brittle nails
- Cheilosis (ulcers in the corner of the mouth)
- Sore tongue
- Koilonchyia (concave or spoon-shaped fingernails)
- Pica (craving to eat unusual substances such as clay or starch)
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).
The examinee should remember the following teaching and administration points when giving iron:
- Administer iron preparations with orange juice to enhance absorption.
- If giving an iron elixir, it should be administered through a straw to prevent staining the teeth.
- When administering iron IM, it should be given through the IM Z track method.
- Instruct the patient that her stools might be dark green or black when taking iron preparations.
- When administering IM or IV, monitor closely for anaphylaxis.
- Client education should focus on drug administration and side effects. The client should be instructed to increase dietary iron intake (good sources of iron include egg yolk; green, leafy vegetables; iron-fortified cereals; peanut butter; raisins; molasses; beans; oatmeal; dried fruit; and organ meats).