Chapter 36: Management of Patients with Immunodeficiency Disorders
D (Feedback: The nurse should obtain height and weight before treatment to verify accurate dosing. IVIG can be administered through a peripheral line. Diuretics are not normally given prior to administration, and IVIG is not mixed with normal saline.)
IVIG has been ordered for the treatment of a patient with an immunodeficiency. Which of the following actions should the nurse perform before administering this blood product? A) Ensure that the patient has a patent central line. B) Ensure that the IVIG is appropriately mixed with normal saline. C) Administer furosemide before IVIG to prevent hypervolemia. D) Weigh the patient before administration to verify the correct dose.
B (Feedback: Hereditary angioneurotic edema results from the deficiency of C1esterase inhibitor, which opposes the release of inflammatory mediators. The clinical picture of this autosomal dominant disorder includes recurrent attacks of edema. A patient with this diagnosis does not lack interferons, IgG, or IgA.)
A 20-year-old patient with an immunodeficiency is admitted to the unit with an acute episode of upper airway edema. This is the fifth time in the past 3 months that the patient has had such as episode. As the nurse caring for this patient, you know that the patient may have a deficiency of what? A) Interferons B) C1esterase inhibitor C) IgG D) IgA
B (Feedback: Infants with X-linked agammaglobulinemia usually become symptomatic after the natural loss of maternally transmitted immunoglobulins (passive acquired immunity), which occurs at about 5 to 6 months of age. Opsonization is the coating of antigen-antibody molecules with a sticky substance to facilitate phagocytosis. Cell-mediated immunity and phagocytosis do not directly affect the timeline of the infant's symptoms.)
A 6-month-old infant has been diagnosed with X-linked agammaglobulinemia and the parents do not understand why their baby did not develop an infection during the first months of life. The nurse should describe what phenomenon? A) Cell-mediated immunity in infants B) Passive acquired immunity C) Phagocytosis D) Opsonization
A (Feedback: Encouraging the patient and family to be active partners in the management of the immunodeficiency is the key to successful outcomes and a favorable prognosis. This transcends the patient's activity and functional status. Medications should not be adjusted without consultation from the primary care provider. Fluid balance is not normally a central concern.)
A home health nurse is caring for a patient who has an immunodeficiency. What is the nurse's priority action to help ensure successful outcomes and a favorable prognosis? A) Encourage the patient and family to be active partners in the management of the immunodeficiency. B) Encourage the patient and family to manage the patient's activity level and activities of daily living effectively. C) Make sure that the patient and family understand the importance of monitoring fluid balance. D) Make sure that the patient and family know how to adjust dosages of the medications used in treatment.
D (Feedback: The patient must be made aware that all health-related instructions are lifelong. Immunizations may be contraindicated and infection usually requires inpatient treatment. Platelet transfusions are not indicated for most patients who have immunodeficiencies.)
A home health nurse is reinforcing health education with a patient who is immunosuppressed and his family. What statement best suggests that the patient has understood the nurse's teaching? A) "My family needs to understand when I can go get the seasonal flu shot." B) "I need to know how to treat my infections in a home setting." C) "I need to understand how to give my platelet transfusions." D) "My family needs to understand that I'll probably need lifelong treatment."
C (Feedback: The patient who is to receive IVIG at home will need information about the expected benefits and outcomes of the treatment as well as expected adverse reactions and their management. The home environment cannot be sterile and complementary alternatives to IVIG have not been identified. Fluid management is not a central concern.)
A home health nurse will soon begin administering IVIG to a new patient on a regular basis. What teaching should the nurse provide to the patient? A) The need for a sterile home environment B) Complementary alternatives to IVIG C) Expected benefits and outcomes of the treatment D) Technique for managing and monitoring daily fluid intake
C (Feedback: Continual monitoring of the patient's condition is critical, so that early signs of impending infection may be detected and treated before they seriously compromise the patient's status. Continual monitoring is not primarily motivated by the patient's functional needs or medication schedule. The nurse's documentation is important, but less than infection control.)
A nurse caring for a patient who has an immunosuppressive disorder knows that continual monitoring of the patient is critical. What is the primary rationale behind the need for continual monitoring? A) So that the patient's functional needs can be met immediately B) So that medications can be given as ordered and signs of adverse reactions noted C) So that early signs of impending infection can be detected and treated D) So that the nurse's documentation can be thorough and accurate
D (Feedback: Advances in medical treatment have meant that patients with primary immunodeficiencies live longer, thus increasing their overall risk of developing cancer. It does not mean that they are at increased risk of COPD, dementia, or pulmonary fibrosis.)
A nurse educator is explaining that patients with primary immunodeficiencies are living longer than in past decades because of advances in medical treatment. This increased longevity is associated with an increased risk of what? A) Chronic obstructive pulmonary disease B) Dementia C) Pulmonary fibrosis D) Cancer
B (Feedback: The optimal dosage of IVIG is determined by the patient's response. In most instances, an IV dose of 200 to 800 mg/kg of body weight is administered.)
A nurse has admitted a patient diagnosed with severe combined immunodeficiency disease (SCID) to the unit. The patient's orders include IVIG. How will the patient's dose of IVIG be determined? A) The patient will receive 25 to 50 mg/kg of body weight. B) The dose will be determined by the patient's response. C) The dose will be determined by body surface area. D) The patient will receive a one-time bolus followed by 100- to 150-mg doses.
B (Feedback: Pulse rate and respiratory rate should be counted for a full minute, because subtle changes can signal deterioration in the patient's clinical status. The rationale for this action is not because of the relationship between heart rate and respirations. These patients do not have a greatly increased risk of hemodynamic instability or dysrhythmias.)
A nurse has created a plan of care for an immunodeficient patient, specifying that care providers take the patient's pulse and respiratory rate for a full minute. What is the rationale for this aspect of care? A) Respirations affect heart rate in immunodeficient patients. B) These patients' blunted inflammatory responses can cause subtle changes in status. C) Hemodynamic instability is one of the main complications of immunodeficiency. D) Immunodeficient patients are prone to ventricular tachycardia and atrial fibrillation.
A (Feedback: The cardinal symptoms of immunodeficiency include chronic or recurrent severe infections, infections caused by unusual organisms or organisms that are normal body flora, poor response to treatment of infections, and chronic diarrhea. Hyperglycemia, rhinorrhea, and contact dermatitis are not symptoms the patient is likely to exhibit.)
A nurse is admitting a patient with an immunodeficiency to the medical unit. In planning the care of this patient, the nurse should assess for what common sign of immunodeficiency? A) Chronic diarrhea B) Hyperglycemia C) Rhinorrhea D) Contact dermatitis
C (Feedback: Ataxia-telangiectasia is an autosomal recessive neurodegenerative disorder characterized by cerebellar ataxia (loss of muscle coordination), telangiectasia (vascular lesions caused by dilated blood vessels), and immune deficiency. Decreased coordination is likely to constitute a risk for falls. The patient does not characteristically lose tactile sensation or experience pernicious anemia or constipation.)
A nurse is admitting an adolescent patient with a diagnosis of ataxia-telangiectasis. Which of the following nursing diagnoses should the nurse include in the patient's plan of care? A) Fatigue Related to Pernicious Anemia B) Risk for Constipation Related to Decreased Gastric Motility C) Risk for Falls Due to Loss of Muscle Coordination D) Disturbed Kinesthetic Sensory Perception Related to Vascular Changes
B (Feedback: Persistent diarrhea is among the varied signs and symptoms that may suggest infection in an immunocompromised patient. Aggression, pruritis, and constipation are less suggestive of an infectious etiology.)
A nurse is caring for a patient who has an immunodeficiency. What assessment finding should prompt the nurse to consider the possibility that the patient is developing an infection? A) Uncharacteristic aggression B) Persistent diarrhea C) Pruritis (itching) D) Constipation
B (Feedback: Hematopoietic stem cell transplantation (HSCT), another form of cell therapy, has proven to be a successful curative modality. Treatment with GM-CSF or G-CSF is not curative. Brachytherapy is not a treatment for immunodeficiency.)
A nurse is caring for a patient with a phagocytic cell disorder. The patient states, "My specialist says that I will likely be cured after I get my treatment tomorrow." To what treatment is the patient most likely referring? A) Treatment with granulocyte-macrophage colony-stimulating factor (GM-CSF) B) Hematopoietic stem cell transplantation C) Treatment with granulocyte colony-stimulating factor (G-CSF) D) Brachytherapy
D (Feedback: Hand hygiene is usually considered the most important aspect of infection control. IVIG and antibiotics are not considered infection control measures, though they enhance resistance to infection and treat infection. Gloves and goggles are sometimes indicated but are less effective than hand hygiene.)
A nurse is planning the care of a patient who requires immunosuppression to ensure engraftment of depleted bone marrow during a transplantation procedure. What is the most important component of infection control in the care of this patient? A) Administration of IVIG B) Antibiotic administration C) Appropriate use of gloves and goggles D) Thorough and consistent hand hygiene
A (Feedback: Diphenhydramine and acetaminophen are administered 30 minutes prior to an IVIG infusion.)
A nurse is preparing to administer a scheduled dose of IVIG to a patient who has a diagnosis of severe combined immunodeficiency disease (SCID). What medication should the nurse administer prior to initiating the infusion? A) Diphenhydramine B) Ibuprofen C) Hydromorphone D) Fentanyl
B (Feedback: The patient who is to receive IVIG at home will need information about adverse reactions and their management. A patient would not start a new IV site independently and the patient does not calculate changes in dose independently. Medications are not added to IVIG.)
A nurse is preparing to discharge a patient with an immunodeficiency. When preparing the patient for self-infusion of IVIG in the home setting, what education should the nurse prioritize? A) Sterile technique for establishing a new IV site B) Signs and symptoms of adverse reactions C) Formulas for calculating daily doses D) Technique for adding medications to the IVIG
D (Feedback: Many patients develop oral manifestations and need education about promoting good dental hygiene to diminish the oral discomfort and complications that frequently result in inadequate nutritional intake. Alcohol cleansers do not necessarily need to be avoided and nail care is not a central concern. Acne care is not a main focus of education, since it is not relevant to many patients.)
A nurse is providing health education regarding self-care to a patient with an immunodeficiency. What teaching point should the nurse emphasize? A) The importance of aggressive treatment of acne B) The importance of avoiding alcohol-based cleansers C) The need to keep fingernails and toenails closely trimmed D) The need for thorough oral hygiene
B (Feedback: A patient diagnosed with CVID often develops pernicious anemia; the patient's hemoglobin and vitamin B12 levels would be used to assess for this common complication of CVID. None of the other listed blood values directly relates to the signs and complications of CVID.)
A patient diagnosed with common variable immune deficiency (CVID) has been admitted to the acute medicine unit. When reviewing this patient's laboratory findings, the nurse should prioritize what values? A) Creatinine and blood urea nitrogen (BUN) B) Hemoglobin and vitamin B12 C) Sodium, potassium and magnesium D) D-dimer and c-reactive protein
B, C, D (Feedback: Patients with phagocytic cell disorders experience recurrent cutaneous abscesses, chronic eczema, bronchitis, pneumonia, chronic otitis media, and sinusitis. Irritable bowel syndrome and cognitive deficits are atypical.)
A patient has been admitted with a phagocytic cell disorder and the nurse is reviewing the most common health problems that accompany these disorders. The nurse should identify which of the following? Select all that apply. A) Inflammatory bowel disease B) Chronic otitis media C) Cutaneous abscesses D) Pneumonia E) Cognitive deficits
A (Feedback: Potential adverse effects of an IVIG infusion include hypotension, flank pain, chills, and tightness in chest, terminating with a slightly elevated body temperature and anaphylactic reaction. Hypertension, hypothermia, and joint pain are not usual adverse effects of IVIG.)
A patient is admitted for the treatment of a primary immunodeficiency and intravenous immunoglobulin (IVIG) is ordered. What should the nurse monitor for as a potential adverse effect of IVIG administration? A) Anaphylaxis B) Hypertension C) Hypothermia D) Joint pain
D (Feedback: Hand hygiene is imperative in infection control. A well-balanced diet is important, but for most patients this is secondary to hygiene as an infection-control measure. Prophylactic antibiotics are not normally used. Limiting physical activity will not protect the patient from infection.)
A patient who has received a heart transplant is taking cyclosporine, an immunosuppressant. What should the nurse emphasize during health education about infection prevention? A) Eat a high-calorie, high-protein diet. B) Limit physical activity in order to conserve energy. C) Take prophylactic antibiotics as ordered. D) Perform frequent handwashing.
A (Feedback: Patients with Wiskott-Aldrich syndrome (WAS) are at a grave risk for infection; infection prevention is a priority aspect of nursing care. Nutritional supplementation may be necessary, but infection prevention is paramount. Chest physiotherapy and FFP administration are not indicated.)
A patient with Wiskott-Aldrich syndrome is admitted to the medical unit. The nurse caring for the patient should prioritize which of the following? A) Protective isolation B) Fresh-frozen plasma administration C) Chest physiotherapy D) Nutritional supplementation
C (Feedback: Frequent respiratory tract infections in patients with CVID typically lead to chronic progressive bronchiectasis and pulmonary failure. Pulmonary edema is often a result of vascular insufficiency. A patient suffering from CVID is likely to develop gastric cancer, not lung cancer. The patient is not at risk for emphysema.)
A patient with a diagnosis of common variable immunodeficiency begins to develop thick, sticky, tenacious sputum. The patient has a history of episodes of pneumonia at least one time per year for the last 10 years. What does the nurse suspect the patient is developing? A) Pulmonary edema B) A pulmonary neoplasm C) Bronchiectasis D) Emphysema
C (Feedback: Dyspnea and cough are among the many signs and symptoms that may suggest infection in an immunocompromised patient. There is no indication for suctioning or the use of nebulizers. Deep breathing and coughing exercises do not address the patient's complaints or the likely etiology.)
A patient with a diagnosis of primary immunodeficiency informs the nurse that he has been experiencing a new onset of a dry cough and occasional shortness of breath. After determining that the patient's vital signs are within reference ranges, what action should the nurse take? A) Administer a nebulized bronchodilator. B) Perform oral suctioning. C) Assess the patient for signs and symptoms of infection. D) Teach the patient deep breathing and coughing exercises.
C (Feedback: In one rare type of phagocytic disorder, hyperimmunoglobulinemia E syndrome (formerly known as Job syndrome), white blood cells cannot initiate an inflammatory response to infectious organisms. The other listed health problems do not have this pathology.)
A patient's primary immunodeficiency disease is characterized by the inability of white blood cells to initiate an inflammatory response to infectious organisms. What is this patient's most likely diagnosis? A) Chronic granulomatous disease B) Wiskott-Aldrich syndrome C) Hyperimmunoglobulinemia E syndrome D) Common variable immunodeficiency
C (Feedback: Patients with phagocytic cell disorders may develop severe neutropenia. None of the other listed health problems is a common complication of phagocytic disorders.)
A teenager is diagnosed with cellulitis of the right knee and fails to respond to oral antibiotics. He then develops osteomyelitis of the right knee, prompting a detailed diagnostic workup that reveals a phagocytic disorder. This patient faces an increased risk of what complication? A) Thrombocytopenia B) HIV/AIDS C) Neutropenia D) Hemophilia
C (Feedback: Swollen lymph nodes are suggestive of infection and warrant prompt medical assessment and treatment. Acetaminophen is an ineffective response. The nurse should monitor the patient's vital signs closely, but the physician should also be informed. Standard precautions should be in place regardless of the patient's status.)
An immunocompromised patient is being treated in the hospital. The nurse's assessment reveals that the patient's submandibular lymph nodes are swollen, a finding that represents a change from the previous day. What is the nurse's most appropriate action? A) Administer a PRN dose of acetaminophen as ordered. B) Monitor the patient's vital signs q2h for the next 24 hours. C) Inform the patient's primary care provider of this finding. D) Implement standard precautions in the patient's care.
B (Feedback: There is no evidence of drug-induced thrombocytopenia or aplastic anemia. The child would have only suffered from severe neutropenia if there was evidence of bacterial or fungal infections. The fact the mother of this individual had him tested for gamma-globulin as a child would indicate that his sibling had X-linked agammaglobulinemia. More than 10% of patients with X-linked agammaglobulinemia are hospitalized for infection at less than 6 months of age. Since the condition is X-linked it is important for the couple to undergo genetic testing.)
A young couple visits the nurse practitioner stating that they want to start a family. The husband states that his brother died of a severe infection at age 6 months. He says he never knew what was wrong but his mother had him undergo "blood testing" as a child. Based on these statements, what health problem should the nurse practitioner suspect? A) Severe neutropenia B) X-linked agammaglobulinemia C) Drug-induced thrombocytopenia D) Aplastic anemia
A (Feedback: Although prophylactic drug treatment effectively prevents some bacterial and fungal infections, it must be used with caution because it has been implicated in the emergence of resistant organisms. Use of antibiotics does not directly increase the risk of viral infections.)
Family members of an immunocompromised patient have asked the nurse why antibiotics are not being given to the patient in order to prevent infection. How should the nurse best respond? A) "Using antibiotics to prevent infections can cause the growth of drug-resistant bacteria." B) "If an antibiotic is given to prevent a bacterial infection, the patient is at risk of a viral infection." C) "Antibiotics can never prevent an infection; they can only cure an infection that is fully developed." D) "Antibiotics cannot resolve infections in people who are immunocompromised."
A (Feedback: All foods must be cooked to avoid food-borne illness. The patient should avoid contact with individuals who have recently been ill or vaccinated. The nurse should apply creams and emollients to any dry, chaffed, or cracked skin. Vitamin supplements may or may not be indicated.)
Patient teaching regarding infection prevention for the patient with an immunodeficiency includes which of the following guidelines? A) Cook all food thoroughly. B) Refrain from using creams or emollients on skin. C) Maintain contact only with individuals who have recently been vaccinated. D) Take OTC vitamin supplements consistently.
D (Feedback: They must be informed of the need for continuous monitoring for subtle changes in the patient's physical health status and of the importance of seeking immediate health care if changes are detected. Nutrition is important, but infection control is the priority. Patients and families do not choose antibiotics independently. Vaccinations are often contraindicated in immunocompromised patients.)
The home health nurse is assessing a patient who is immunosuppressed following a liver transplant. What is the most essential teaching for this patient and the family? A) How to promote immune function through nutrition B) The importance of maintaining the patient's vaccination status C) How to choose antibiotics based on the patient's symptoms D) The need to report any slight changes in the patient's health status
C (Feedback: Primary immunodeficiency diseases are genetic in origin and result from intrinsic defects in the cells of the immune system. Primary immunodeficiency diseases do not always need IVIG as treatment, and they are not communicable. Primary immunodeficiencies do not result from intrauterine infection.)
The nurse educator is differentiating primary immunodeficiency diseases from secondary immunodeficiencies. What is the defining characteristic of primary immunodeficiency diseases? A) They require IVIG as treatment. B) They are the result of intrauterine infection. C) They have a genetic origin. D) They are communicable.
B (Feedback: Frequent causes of death in patients with ataxia-telangiectasiaare chronic pulmonary disease and malignancy. Peripheral edema, anaphylaxis, and GI bleeding are not noted to be common among patients with ataxia-telangiectasia.)
The nurse is admitting a patient to the unit with a diagnosis of ataxia-telangiectasia. The nurse's assessment should reflect the patient's increased risk for what complication? A) Peripheral edema B) Cancer C) Anaphylaxis D) Gastrointestinal bleeds
A, D, E (Feedback: Some of the key elements of standard precautions include performing hand hygiene; using appropriate personal protective equipment, depending on the expected type of exposure; and using safe injection practices. Isolation is an infection control strategy but is not a component of standard precautions.)
The nurse is applying standard precautions in the care of a patient who has an immunodeficiency. What are key elements of standard precautions? Select all that apply. A) Using appropriate personal protective equipment B) Placing patients in negative-pressure isolation rooms C) Placing patients in positive-pressure isolation rooms D) Using safe injection practices E) Performing hand hygiene
A (Feedback: Patients with paroxysmal nocturnal hemoglobinuriahave a high incidence of life-threatening venous thrombosis, which occurs most commonly in the abdominal and cerebral veins. This health problem is not linked to ARDS, MI, or hypertensive urgency.)
The nurse is caring for a patient who has a diagnosis of paroxysmal nocturnal hemoglobinuria. When planning this patient's care, the nurse should recognize the patient's heightened risk of what complication? A) Venous thromboembolism B) Acute respiratory distress syndrome (ARDS) C) Myocardial infarction D) Hypertensive urgency
A (Feedback: Immunodeficient patients often lack the typical objective and subjective signs and symptoms of infection. However, this does not mean that they wholly lack symptoms. Infections do not normally have a slower onset. Blood work may not be a reliable diagnostic tool, but that does not mean that the results are inaccurate.)
The nurse is caring for a patient with an immunodeficiency who has experienced sudden malaise. The nurse's colleague states, "I'm pretty sure that it's not an infection, because the most recent blood work looks fine." What principle should guide the nurse's response to the colleague? A) Immunodeficient patients will usually exhibit subtle and atypical signs of infection. B) Infections in immunodeficient patients have a slower onset but a more severe course. C) Laboratory blood work is often inaccurate in immunodeficient patients. D) Immunodeficient patients do not develop symptoms of infection.
D (Feedback: The nurse should administer pretreatment acetaminophen and diphenhydramine as prescribed 30 minutes before the start of the infusion. The patient should be weighed prior to the treatment and the IV infusion rate should not exceed 200 mL/hour. The nurse should stop the transfusion in the event of any signs of a reaction.)
The nurse is preparing to administer IVIG to a patient who has an immunodeficiency. What nursing guideline should the nurse apply? A) Do not exceed an infusion rate of 300 mL/hr. B) Slow the infusion rate if the patient exhibits signs of a transfusion reaction. C) Weigh the patient immediately after the infusion is complete. D) Administer pretreatment medications as ordered 30 minutes prior to infusion.
C (Feedback: Treatment options for SCID include stem cell and bone marrow transplantation, but HSCT is the definitive therapy for the disease and supersedes the importance of antibiotics. CSFs, radiation therapy, and chemotherapy are not indicated.)
The nurse is working with the interdisciplinary team to care for a patient who has recently been diagnosed with severe combined immunodeficiency disease (SCID). What treatment is likely of most benefit to this patient? A) Combined radiotherapy and chemotherapy B) Antibiotic therapy C) Hematopoietic stem cell transplantation (HSCT) D) Treatment with colony-stimulating factors (CSFs)
D (Feedback: Transplantation of fetal thymus, postnatal thymus, or human leukocyte antigen (HLA)-matched bone marrow has been used for permanent reconstitution of T-cell immunity in infants with DiGeorge syndrome. Antibiotics and chemotherapy do not address the etiology of the infant's disease. Stem cell transplantation is not a common treatment modality.)
The parents of a 1-month-old infant bring their child to the pediatrician with symptoms of congestive heart failure. The infant is ultimately diagnosed with DiGeorge syndrome. What will prolong this infant's survival? A) Stem cell transplantation B) Long-term antibiotics C) Chemotherapy D) Thymus gland transplantation
