Immunodeficiency PDF

Introduction to Immunodeficiency A healthy, functioning immune system is integral to defending the body against threats. When the immune system response is deficient, the host cannot adequately defend the body, leading to dysfunction. Human Immunodeficiency Virus...

Introduction to Immunodeficiency A healthy, functioning immune system is integral to defending the body against threats. When the immune system response is deficient, the host cannot adequately defend the body, leading to dysfunction. Human Immunodeficiency Virus Terms Term Description Rationale Opportunistic infection Infections that occur due to a Opportunistic infections are illnesses that weakened immune system occur when the immune system is compromised, a common complication as HIV advances. Seroconversion The development of Seroconversion is the period during which detectable antibodies to HIV HIV antibodies develop and become in the blood detectable, marking the transition from HIV-negative to HIV-positive status. Wasting syndrome A condition characterized by Wasting syndrome is characterized by significant weight loss, often unintended and significant weight loss accompanied by diarrhea, and may be seen as HIV advances. weakness, and fever Pneumocystis A type of opportunistic PCP is a severe lung infection that can pneumonia (PCP) infection in the lungs affect individuals with HIV, particularly when CD4+ T cell counts are very low.  CD4+ helper T cells The immune cells most Examining the CD4+ helper T cell count impacted by HIV provides a key indicator of immune function in individuals with HIV. This lab value is used to monitor disease progression and guide treatment decisions. The chronological order of the stages of human immunodeficiency virus (HIV) infection is: 1. Acute HIV infection: After initial infection, the acute HIV infection stage occurs first, typically 2–3 weeks after infection, characterized by high viral replication and dissemination throughout the body with the client potentially experiencing nonspecific flu-like symptoms as the immune system begins to respond to the virus. 2. Clinical latency: Clinical latency is the second stage of HIV infection, which can last for a year or more. This stage is characterized by low levels of HIV replication and persistent immune activation, with CD4+ helper T cell counts gradually declining. The client may not experience symptoms during this stage. 3. AIDS: AIDS is the final and most severe stage of infection, where the immune system is badly damaged. AIDS is characterized by a CD4+ T cell count below 200 cells/mm3 with the body becoming vulnerable to opportunistic infections, opportunistic malignancies, and neurological complications. Without treatment, survival time averages 3 years. HIV is classified as a secondary immunodeficiency disorder as genetic or congenital defects are not the cause. Rather, HIV is acquired during an individual’s life and caused by a factor external to the immune system. In contrast, primary immunodeficiency disorders are typically present at birth and are caused by intrinsic defects in the immune system. There are two types of immunodeficiency disorders: primary and secondary. The Healthy Immune System A healthy, functioning immune system defends the body against threats. In the human immune system, three lines of defense work together and take on different tasks for host defense. Click each section below to learn more about the first, second, and third lines of immune defense in healthy, functioning immune systems. First Line of Defense Intact physical, mechanical, and biochemical barriers prevent foreign threats from invading the body. Physical Barriers Mechanical Barriers Biochemical Barriers skin cilia mucous mucous membranes coughing perspiration intestinal epithelium sneezing saliva urinating tears vomiting earwax defecation Second Line of Defense If foreign substances penetrate the first-line barriers, the non-specific inflammatory response rapidly senses non-self invaders and sends immune resources to contain and neutralize the invading substance. Immune resources include the following: neutrophils o first responders that marginate, diapedeses, and phagocytize monocytes and macrophages o phagocytize and release cytokines plasma protein systems activated o complement system o clotting system o kinin system platelet-activating factor o releasing more mediators to assist in increasing vascular permeability, helping immune resources travel to the invader mast cells o releasing more inflammatory mediators Third Line of Defense Foreign antigens are phagocytized by antigen-presenting cells (macrophages and dendritic cells) and signal and bind to CD4+ helper T-cells to activate them. CD4+ helper T-cells control and coordinate host defense response through this mechanism. Then, CD4+ helper T-cells activate CD8 cytotoxic T-cells, macrophages, B-cells, and NK cells through cytokine release. Pathophysiology of Human Immunodeficiency Virus The human immunodeficiency virus (HIV) is an RNA retrovirus that primarily infects and destroys CD4+ helper T cells (although it can also infect macrophages and dendritic cells). Retroviruses like HIV use reverse transcriptase, allowing the virus to copy RNA into DNA and replicate inside the host’s cells. The attack on CD4+ helper T cells decreases CD4+ levels, leading to a deficient immune response. Viral Membrane Human immunodeficiency virus (HIV) is a virus that attacks specific immune cells called T cells. The anatomy of HIV plays an essential role in the replication and survival of the virus. The viral membrane is the outermost portion of HIV. This membrane is double-layered and comprised of fats called phospholipids. HIV Glycoproteins Specialized proteins called glycoproteins cover the viral membrane. These proteins enable HIV to attach to healthy cells, initiating viral replication. Glycoprotein 41 (gp41) extends through the viral membrane, and glycoprotein 120 (gp120) binds to gp41. Matrix Proteins Beneath the viral membrane is a layer of organized matrix proteins. Matrix proteins help the virus maintain its shape and protect the viral capsid underneath. Viral Capsid The HIV viral capsid acts as the HIV nucleus. The capsid houses HIV genetic information and the enzymes that help the virus replicate. Viral Enzymes and HIV RNA Within the viral capsid is the HIV genetic material (RNA) and the associated HIV enzymes. These enzymes include reverse transcriptase, integrase, and protease. Reverse transcriptase converts HIV RNA into DNA, integrase incorporates HIV DNA into healthy cell DNA, and protease separates newly formed HIV proteins. Clinical Application Jeremy Wittens (pronouns: he/him/his) tested positive for HIV 2.5 years ago. He receives routine follow- up care at the HIV clinic every 2–3 months, most recently six weeks ago. He has been on antiretroviral treatment since his initial diagnosis. Today, he presents to the primary care office with complaints of moderate dyspnea, a persistent and non-productive cough, and fatigue. Based on the information provided, what is the risk factor that is the most likely contributor to Jeremy’s HIV status? Unprotected anal sex Explaination: The most likely contributor to Jeremy's HIV status is unprotected anal sex. Unprotected sex with an HIV-positive partner is a significant risk factor for HIV transmission. The virus can be transmitted through sexual fluids during unprotected vaginal, anal, or oral sex, with the risk of transmission during anal sex especially high. In the United States, 70% of new HIV infections occur in populations comprising homosexual or bisexual men (McCance & Huether, 2019). Jeremy is a homosexual male, according to his electronic health record. The provider should not make assumptions about the definitive cause of HIV infection from the chart. However, noticing this risk factor in the chart should prompt the provider to ask additional questions about sexual practices to determine the most likely means of HIV transmission. HIV cannot be transmitted by sitting on toilet seats or through contact with surfaces used by an HIV- positive individual. While individuals can contract HIV through untested blood transfusions, there is no indication on the chart that Jeremy has received blood transfusions. While individuals can contract HIV in utero, according to his chart, Jeremy contracted HIV 2.5 years ago. As a 31-year-old male, that rules out contracting HIV in utero. The data within the electronic health record that indicates clinical manifestations potentially related to Jeremy's HIV status include the following: moderate dyspnea, cough, and fatigue decreased appetite and weight loss cervical and axillary lymphadenopathy bibasilar crackles auscultated tachypnea and fever These clinical manifestations may be related to HIV, including the presence of opportunistic infections (e.g., pneumocystis pneumonia [PCP], tuberculosis [TB]). The client is compliant with medications, is in normal sinus rhythm with a heart rate of 90, and has bowel sounds auscultated in all four quadrants, all of which are normal findings. Human Immunodeficiency Virus Risk Factors The primary routes for acquiring human immunodeficiency virus (HIV) include sex, blood, and perinatal transmission. Standardized testing has decreased the incidence of this virus in certain settings, including routine screening during maternity care, routine screening of donated blood products, and occupational safety standards to reduce and respond to accidental needlestick injuries. When universal precautions and post-exposure prophylaxis are followed in needlestick injuries, the incidence of HIV transmission is rare. Sex Unprotected vaginal or anal sex ○ Increased risk associated with anal sex Multiple sexual partners Blood Sharing needles (during drug use or in medical settings) Receiving infected blood products during transfusion Accidental needle sticks Perinatal Babies born to HIV-positive mothers can get HIV ○ before or during birth, or ○ during breastfeeding. Human Immunodeficiency Virus Clinical Manifestations The clinical manifestations of human immunodeficiency virus (HIV) vary depending on the progression of the disease, individual variations in lifestyle, and adherence to standardized care processes. Subjective and objective data are collected and analyzed to monitor disease progression, and individualized assessment and treatment are paramount to quality outcomes. Clients with HIV may be asymptomatic for up to one year after initial infection. If the disease process is untreated, the client’s condition will deteriorate and progress toward acquired immunodeficiency syndrome (AIDS). Labs: Decreased white blood cell (WBC) counts ○ Especially lymphocytes Decreased CD4+ counts Increased viral load Decreased platelets Increased erythrocyte sedimentation rate (ESR) Neurologic: Headache Fatigue  Memory loss Vision loss HIV encephalopathy Integumentary: Persistent skin rashes Opportunistic infections ○ Severe viral/fungal skin and/or mucous membrane infections (Candidiasis) ○ Oral and genital herpes simplex Opportunistic malignancies: Oral hairy leukoplakia Kaposi sarcoma Squamous cell carcinoma Basal cell carcinoma Cutaneous lymphomas Immune: Infections Fever Lymphadenopathy Absence of normal immune response to antigens ○ Absence of reaction to skin tests Respiratory: Flu-like symptoms Coughing, shortness of breath (SOB), dyspnea Opportunistic infections ○ Opportunistic pneumonias ○ Tuberculosis (TB) Parenchymal lung disease Gastrointestinal: Difficult or painful swallowing Nausea, vomiting Abdominal cramping Wasting syndrome  ○ Weight loss and diarrhea Musculoskeletal: Joint pain Muscle pain Pediatric: Growth delays Frequent illnesses Human Immunodeficiency Virus Diagnosis Organizations around the world advocate for guidelines for the prevention, screening, and detection of human immunodeficiency virus (HIV) infection. The Centers for Disease Control and Prevention (CDC, 2023) recommends routine screenings for all individuals aged 13-64 and annual screenings for men who have sex with men (MSM). Diagnosing a client with HIV requires a comprehensive individualized assessment. Diagnostic testing includes tests to detect HIV antibodies and antigens, with confirmation by HIV DNA tests. Viral load and CD4+ lab results establish infection severity and progression risk. Providers must also complete a thorough history and physical, reviewing signs and symptoms. HIV diagnosis can be challenging. Examine the image at the right to learn more about the challenges in diagnosing HIV. 1. Clients may be asymptomatic for up to a year after initial infection, resulting in a delay in seeking treatment. 2. The first signs and symptoms of HIV may be non-specific (e.g., flu-like symptoms) that can easily be mistaken for other viral illnesses. 3. If testing is done soon after exposure, the virus may be undetectable with standardized testing, leading to a false-negative. 4. HIV symptoms vary greatly from person to person, with some presenting with very mild symptoms and others with severe symptoms. 5. The presence of comorbidities may have symptoms that mask HIV symptoms, leading to missed or delayed diagnosis. 6. Access to testing may be limited in some areas, which can delay diagnosis.  Human Immunodeficiency Virus Treatment The World Health Organization (WHO, 2021) recommends a continuum of care for HIV, from prevention to palliative care, including the management of comorbidities and complications. Prevention Strategies to prevent HIV infection include education about risk behavior avoidance. Once infection and diagnosis occur, education shifts to medication adherence, medication side effects, lifestyle modifications, and monitoring for and reporting complications. Resistance Testing The prevalence of HIV resistance to medication therapy is increasing and can be present when individuals are first diagnosed with HIV. After diagnosis, resistance testing is performed prior to starting medication therapy to ensure optimal drug selection. Medication The National Institutes of Health (NIH, 2021) recommends immediate initiation of medication therapy once HIV is diagnosed. This includes targeted medication regimens that may include multiple antiretroviral therapy (ART) medications with a variety of mechanisms of action targeting different phases in viral infection and replication. These drugs are also referred to as highly active antiretroviral therapy (HAART) drugs. New drug therapies involving monoclonal antibodies are also being developed and may be incorporated into client medication regimens. Lifelong Treatment Due to the integration of the HIV virus into the host genome and organs throughout the body, clients must continue antiretroviral therapy (ART) for life. Long-term use of these drugs can cause many complications, such as dyslipidemia, insulin resistance, or a decrease in bone density, potentially requiring additional medical care or medications. Pathophysiology of Cancer-Induced Immunodeficiency Cancer is another disease process associated with secondary immunodeficiency. Cancer-induced immunodeficiency is a complex process where the disease process or treatment leads to a weakened immune system. This can occur through various mechanisms. Examine the image below to learn more about the pathophysiological mechanisms associated with cancer-induced immunodeficiency. Immunosuppression From Inflammation Malignancies often lead to chronic inflammation, which can suppress the adaptive immune system. Chronic inflammation also promotes angiogenesis (supporting tumor growth) and activates immunosuppressive cells, further reducing the immune response. Immunosuppression From Advanced Cancer Cancer cells can create an acidic environment, releasing metabolites that suppress immune cell function. Advanced cancers can lead to widespread immune response deficiency, increasing susceptibility to infection. Immunosuppression From Cancer Treatment Various drugs and therapies involved in cancer treatment inhibit the immune response. This includes corticosteroids, antirejection drugs, chemotherapy, and radiation. Clinical Application Eldine Bertil (pronouns she/her/hers) is a 64-year-old female with a history of small cell lung cancer (SCLC), an aggressive form of lung cancer. She was diagnosed 4 months ago by her oncologist and has been on chemotherapy and radiation treatment for 3.5 months. Today, she presents to her primary care provider’s office complaining of a more frequent cough, hemoptysis, fatigue, and unintentional weight loss of 8 pounds over the past week. Based on the information provided, which risk factors are the most likely contributors to Eldine’s cancer- induced immunodeficiency? Select all that Age-related immune decline Chemotherapy and radiation Diabetes history Small cell lung cancer (SCLC) The most likely contributors to Eldine's cancer-induced immunodeficiency include chemotherapy and radiation, small cell lung cancer, a history of diabetes, and age-related immune decline. Therapeutic interventions like chemotherapy and radiation suppress the immune response, contributing to immunodeficiency. Individuals with cancer are at an increased risk of immunosuppression due to the body’s immune response to the disease process. Medical comorbidities such as diabetes or HIV can weaken the immune response of clients with cancer, placing them at greater risk for immunodeficiency. Factors contributing to the poor underlying health of the client, including age-related immune decline, can weaken the immune response. While neutropenia is a potential result of cancer-induced immunodeficiency, it is not a risk factor. The data within the electronic health record that indicates clinical manifestations potentially related to Eldine's cancer-induced immunodeficiency status include the following: fatigue decreased appetite and unintentional weight loss abnormal labs including pancytopenia (leukopenia, anemia, thrombocytopenia) and neutropenia  fever These clinical manifestations could all be potentially related to cancer-induced immunodeficiency, influenced by the disease process itself or the treatment the client is receiving. The client receiving chemotherapy and radiation and her history of diabetes and smoking are risk factors, not clinical manifestations. The blood pressure of 130/88 is not a clinical manifestation of cancer-induced immunodeficiency. Action Diagnostic Treatment Rationale Obtaining a CBC with X A CBC with differential can provide differential diagnostic information about red blood cells, white blood cells, and platelets, as well as information about neutrophils. Immunodeficiency can cause decreases in these blood cells, making a CBC important for identification and ongoing monitoring of immune status. Administering X Immunotherapy is a treatment that can immunotherapy provide passive immunity to clients who are immunocompromised, helping to prevent infections by supplying antibodies their bodies may not be producing in sufficient quantities. Performing a bone X A bone marrow biopsy can help diagnose marrow biopsy bone marrow suppression, which can be caused by cancer treatments such as chemotherapy and radiation. Bone marrow suppression can lead to immunodeficiency by reducing the production of blood cells. Implementing X Neutropenic precautions include neutropenic treatment strategies to protect precautions immunocompromised clients from potential infections and include measures such as educating the client about using personal protective equipment (PPE), hand hygiene, and avoiding fresh flowers and fruits. Obtaining blood X Obtaining blood cultures can provide cultures diagnostic information about the types of pathogens causing infection, allowing treatment decisions to be targeted and specific. Administering a blood X Clients with anemia or thrombocytopenia transfusion may need to be treated by receiving transfusions of blood products for supportive care. Cancer-Induced Immunodeficiency Risk Factors Cancer types are numerous and are grouped according to the type of cell of origin. Individuals with cancer are at an increased risk of immunosuppression due to the disease process, lifestyle factors, and standard cancer treatment protocols (Garcia et al., 2020). Examine the image below to learn more about the intrinsic, extrinsic, and treatment-related factors placing clients with cancer at risk for developing cancer-induced immunodeficiency (Garcia et al., 2020; Norris, 2020). Intrinsic Risk Factors The ability of cancer cells to modify their antigenic expression and secrete substances that dampen immune responses directly contributes to immune suppression. Production of cytokines and chemokines by tumor cells can hinder the proliferation and activation of immune cells, suppressing the immune response. Extrinsic Risk Factors Factors contributing to the poor underlying health of the client, including malnutrition, age-related immune decline, and comorbidities such as diabetes or HIV can weaken the immune response. Lifestyle and environmental factors, such as smoking or chemical exposure, can further weaken the immune response. Treatment-Related Risk Factors Therapeutic interventions like chemotherapy, radiation, and other immunosuppressive drugs can cause bone marrow suppression, leading to neutropenia. Neutropenia significantly increases infection risk. Clients with cancer requiring surgery and in the post-operative recovery phases may experience reduced immune surveillance, leading to increased susceptibility to opportunistic infections. Cancer-Induced Immunodeficiency Clinical Manifestations Cancer-induced immunodeficiency may cause a variety of clinical manifestations, primarily resulting from the body’s diminished capacity to defend against infections and other diseases. Healthcare providers must monitor clients with cancer closely for signs and symptoms of immunodeficiency, as they necessitate urgent or emergent management and potential adjustment to the cancer treatment regimen (Garcia et al., 2020; Norris, 2020). Infection Clients with cancer-induced immunodeficiency may experience more frequent and severe infections than people with healthy immune systems. These infections may be opportunistic infections, may present in unusual sites, may present atypically, be recurrent, and are harder to resolve. Neutropenia can predispose the client to infections and is potentially life-threatening, especially in the presence of fever. Fever and Night Sweats Persistent, unexplained fever can be a direct result of the immune system’s response to unrecognized pathogens. Night sweats can be associated with infection or a direct symptom of cancer. Fatigue Constant activation or suppression of the immune system can cause overwhelming fatigue and general malaise, greatly affecting the client’s quality of life. Poor Wound Healing Cuts, wounds, or postoperative sites may take longer to heal due to delays in the body’s ability to repair and regenerate tissue. Hematologic Abnormalities Bone marrow suppression from cancer or cancer treatment can result in anemia, thrombocytopenia, or leukopenia, manifesting as fatigue, increased bruising, bleeding, and infections. Neutropenia can predispose the client to infections and is potentially life-threatening, especially in the presence of fever. Weight Loss and Cachexia Unintended weight loss and muscle wasting (cachexia) can be a consequence of chronic immune activation and inflammation. Lymphadenopathy Enlarged lymph nodes can result from cancer-induced immunodeficiency, although this can also be a direct effect of cancer metastasis. Cancer-Induced Immunodeficiency Diagnosis and Treatment Cancer-induced immunodeficiency is diagnosed through a combination of clinical assessment and laboratory testing. Treatment focuses on managing the underlying cancer diagnosis and mitigating the effects of immunosuppression. Diagnosis A clinical assessment, including a thorough history and physical examination, is required to assess for signs and symptoms of immunodeficiency. Lab testing, including a complete blood count (CBC) with differential, immunoglobulin levels, and cultures of anywhere infection is suspected can help identify immunodeficiency. Additional tests or imaging may also be required to assess for infection of specific organ systems. A bone marrow biopsy may be required if hematologic abnormalities are present. Treatment  Determine if chemotherapy, radiation, or drug treatment can be continued without adjustment or if adjustments are necessary to minimize immunosuppression. Prevent infections using isolation precautions to protect from exposure to infectious agents (such as neutropenic precautions). Treat infections prophylactically or when infections are identified with appropriate medications (usually beginning with broad-spectrum antibiotics pending culture results). Provide supportive treatments such as immunotherapy or blood transfusions. Consider holistic interventions to support the client, including lifestyle coaching (nutrition, smoking cessation, etc.). CASE STUDY: Immunodeficiency Li Xi (pronouns: she/her/hers) is a 70-year-old female with non-Hodgkin’s lymphoma who is currently undergoing chemotherapy. Li also has a history of type 2 diabetes and hypertension, both controlled with medication. She presents to the office of her primary care provider with a low-grade fever, fatigue, and sore throat.  Sign of Bone Clinical Manifestations and Sign of Marrow Lab Results Infection Suppression Rationale Low-grade fever X Low-grade fever can indicate that the client has an infection. Night sweats X Night sweats can be indicative of infection in immunodeficient clients. Bruising X Bruising can occur from thrombocytopenia due to bone marrow suppression.  Leukopenia X Leukopenia can result from bone marrow suppression. Neutropenia X Neutropenia can result from bone marrow suppression. Anemia X Anemia can result from bone marrow suppression. Thrombocytopenia X Thrombocytopenia can result from bone marrow suppression. Which of the following cues are key to understanding Li’s current condition? Neutropenia Low-grade fever Recent chemotherapy Chemotherapy is a common cause of bone marrow suppression leading to immunodeficiency. A low-grade fever is a manifestation of infection in clients with immunodeficiency. Neutropenia can be caused by bone marrow suppression and is an indicator of immunosuppression. It predisposes the client to infection as their immune system is less capable of defending against pathogens when suppressed. Controlled hypertension, while a comorbidity, is likely not related to Li’s current experience of immunodeficiency. The client’s normal oxygen saturation is not the cause for concern as it is within normal range. Considering Li’s presentation and lab results, select the most likely hypothesis from the drop-down list for each statement. The client’s low-grade fever suggests a potential infection, and neutropenia primarily indicates bone marrow suppression An allergic reaction Infection is a common complication in clients with cancer-induced immunodeficiency. There are not any indications from the client’s chart that they are having an allergic reaction. A potential infection [Correct answer] Infection is a common complication in clients with cancer-induced immunodeficiency. Fever is one common manifestation of infection. Bone marrow suppression [Correct answer] Neutropenia is a common manifestation of bone marrow suppression in clients receiving chemotherapy as blood cell production is decreased. This can lead to infection. Stable disease Neutropenia is not usually a sign of stable disease; rather, it indicates worsening bone marrow suppression from treatment or the disease process. Immediate care needs include reinforcing precautions and monitoring vital signs. Neutropenia predisposes the client to infection. Reinforcing neutropenic precautions can help reduce the risk of further infection. Monitoring vital signs is important to determine if the client’s condition is improving or declining. Diagnostic testing includes obtaining blood cultures, a chest x-ray, and urinalysis and culture. Diagnostic testing, including obtaining blood cultures, is critical to determining the presence of an infection as well as the type of infection. This is key to target future treatment decisions. Obtaining a chest x-ray is key to determining the location of the infection to help target future treatment decisions. Obtaining a urinalysis and culture is critical to determining the location of the infection to help target future treatment decisions. Broad-spectrum antibiotics should be administered after obtaining blood cultures to treat potential infections. Antibiotic therapy can then become more targeted after obtaining culture results. Blood cultures can provide information on the presence and type of microbe causing infection in the blood. This informs future treatment decisions, including antibiotic selection. Nutritional support is important to address potential malnutrition, which can further compromise immune function. IV fluids may not be immediately necessary as nothing in the chart indicates severe dehydration or hemodynamic instability. Immediate surgery is not indicated based on the information provided.  Changes in Li’s The Condition Better Same Worse Rationale WBC count of 4.1 X An increase in the WBC count from 1.8 initially to 4.1 two days later indicates that the client’s condition is getting better. Sore throat pain 0 X A decrease in the client’s throat pain from 5 to 0 two days later indicates that the client’s condition is getting better. Platelet count of X An increase in the client’s platelet count 140 from 100 initially to 140 two days later indicates that the client’s condition is getting better. Temperature of 98.5 X A decrease in the client’s temperature from F 100.5 °F initially to 98.5 °F two days later most likely indicates the client’s condition is getting better, especially since it is supported by other promising data. Application: Identifying Contributing Factors A nurse practitioner (NP) is evaluating a 45-year-old male named Clarence Goff (pronouns he/him/his). Clarence has a history of human immunodeficiency virus (HIV) and is presenting to the office with a recent onset of oral candidiasis and a CD4 lab result of 180 cells/mm3 from yesterday. Which of the following pathophysiological processes should the NP consider as contributing factors to the client’s current immunodeficient state? Increased destruction of CD4+ T helper lymphocytes by the HIV virus is a correct answer. The hallmark of HIV pathophysiology is the destruction of CD4+ T helper lymphocytes, which are critical for orchestrating the immune response. This client has a low CD4 lab result demonstrating a decline in immune competence. HIV-associated disruption of mucosal barriers is a correct answer. The disruption of mucosal barriers is a consequence of direct HIV effects and the loss of local immune protection, making mucosal surfaces like the oral cavity more susceptible to opportunistic infections such as candidiasis. HIV-associated immune suppression, leading to the presence of opportunistic infection is a correct answer. The client is presenting with candidiasis, an opportunistic infection. Opportunistic infections become more common as the client’s immune system function declines. Direct HIV infection of neutrophils leading to neutropenia is incorrect. While HIV can affect the bone marrow’s ability to produce various blood cells, it does not directly infect neutrophils. Increased destruction of CD8+ T helper lymphocytes by the HIV virus is incorrect. The hallmark of HIV pathophysiology is the destruction of CD4+ T helper lymphocytes, which are critical for orchestrating the immune response, not CD8+ cytotoxic T lymphocytes. Application: Identifying Next Steps A nurse practitioner (NP) is evaluating a 60-year-old female client named Yenesi Gonzales (pronouns she/her hers). Yenesi has a history of breast cancer and is currently undergoing chemotherapy. Yenesi presents to the office complaining of a fever of 101.5 °F, chills, and a productive cough with green sputum. The NP notes that the client has a neutrophil count of 500 cells/mm3 on her routine lab work from this morning. Which of the following is the most appropriate step in initial management for the client’s suspected condition? Place the client on neutropenic precautions and instruct them to go to the nearest Emergency Department for evaluation and treatment of potential neutropenic fever. Instruct the client to go to the nearest Emergency Department for evaluation and treatment of potential neutropenic fever is the correct answer. A client with cancer who is receiving chemotherapy and presenting with neutropenia, a fever, and signs of infection should be considered a medical emergency due to the high risk of severe complications. The client requires prompt evaluation and treatment at the Emergency Department, including administration of broad-spectrum intravenous antibiotics after obtaining the necessary cultures. Advise the client to take over-the-counter antipyretics and increase fluid intake, then reassess in 24 hours is incorrect. A client with cancer who is receiving chemotherapy and presenting with neutropenia, a fever, and signs of infection should be considered a medical emergency due to the high risk of severe complications. The client should not rely on self-management at home and needs inpatient treatment to avoid severe complications. Prescribe a course of oral antibiotics and arrange daily follow-up calls to monitor the client’s status is incorrect. A client with cancer who is receiving chemotherapy and presenting with neutropenia, a fever, and signs of infection should be considered a medical emergency due to the high risk of severe complications. The client requires prompt evaluation and treatment at the Emergency Department, including administration of broad-spectrum intravenous, not oral, antibiotics after obtaining the necessary cultures. Order a sputum culture before initiating antibiotics is incorrect. A client with cancer who is receiving chemotherapy and presenting with neutropenia, a fever, and signs of infection should be considered a medical emergency due to the high risk of severe complications. The client requires prompt evaluation and treatment at the Emergency Department, including administration of broad-spectrum intravenous antibiotics after obtaining the necessary cultures. Obtaining cultures should be done inpatient, not outpatient. Introduction to Immunodeficiency Every day our bodies are exposed to elements that present potential or real threats to us. Although our immune systems protect us from harm, there are times when the immune system overreacts (hypersensitivity) or underreacts (immune deficiency). Immunity is the physiological process that provides an individual with protection or defense against disease. The foundation of primary prevention of immune system diseases is based on vaccinations across the life span. The immune system is a group of lymphatic tissues, organs, and white blood cells that protect the body from foreign invaders (pathogens). Pathogens may include viruses, bacteria, cellular debris, or any other substance that may make the body sick. Our immune system provides our bodies with the ability to fight foreign substances. Immunodeficiency causes the body to have a compromised or lack of an immune response. This activity focuses on the immunodeficiency of the immune system. By completing this module, you will gain the knowledge and skills needed to: Differentiate between primary deficiencies and secondary acquired immunodeficiencies. Describe the pathophysiology of acquired immunodeficiency syndrome (AIDS). Understand the transmission, diagnostic testing, clinical manifestations, and nursing interventions for HIV and AIDS. Immunity is the body’s capacity to fight foreign substances. Immunodeficiency is a compromised or lack of an immune response. Primary Immunodeficiency Disorders Individuals with primary immunodeficiency disorders are most at risk for which alteration in health? Infection One of the primary roles of the immune system is to protect against pathogens that cause infections. When this system is weakened or there is a failure in the development of the immune system (primary disorders), as with immunodeficiency, there is an increased risk of infection. Transmission of Human Immunodeficiency Virus How is human immunodeficiency virus (HIV) transmitted? Blood Semen Vaginal secretions Causes of Immunodeficiencies There are a variety of invaders that activate the immune system. These include bacteria, viruses, fungi, and parasites. Some of these invaders release additional toxins normally or when they are destroyed. One example involves bacteria that may release endotoxins or exotoxins, depending upon the strain. All these things can stimulate the immune response and start the inflammatory process. Clinical manifestations may include headache, rhinitis, fever, cough, infection, pharyngitis, malaise, and pain. In an acute inflammatory response, cytokines increase plasma proteins that induce fever, histamines, prostaglandins, and increase the blood flow to the site. In an immunity or immune response, antigens (which stimulate an immune response), antibodies (specific protein molecules that are produced by the humoral response to bind with the antigen), and lymphatic tissues (remove foreign matter and aid in the immune response) are activated. When the immune system is not working well, we call it immune deficiency. A person may experience frequent infections or illnesses that other people do not typically get. This may alert the person to see their healthcare provider for further testing. Two types of immune deficiencies are primary (congenital) and secondary (acquired) Primary Immune Deficiencies Primary immune deficiencies are caused by a failure in the immune system, such as the production of stem cells from the bone marrow, the thymus, or the synthesis of antibodies. Some congenital deficiencies (hypogammaglobulinemia) are recognized at birth, whereas others do not show up until later in life. Secondary Immune Deficiencies Secondary immune deficiencies can occur with pregnancy, physical or emotional stress, medical treatments (splenectomy), or viral infections (i.e., HIV). These deficiencies can result in severe infections. Treatment modalities include replacing the missing immune components or treating the infection once it is in the body. Immune support using antiviral medications, vitamins, and increased healthy behaviors (exercise and diet) is the treatment for secondary immune deficiencies. Acute inflammatory components: cytokine (increase plasma proteins; induce fever) histamine (increase blood flow to site) prostaglandin (increase blood flow to site) Immunity or immune response cellular components: antigen (components of cells stimulating immune response) antibody (specific protein molecule produced by humoral response to bind with antigen) lymphatic tissue (remove foreign matter and aids immune responses) Exploring Human Immunodeficiency Virus The human immunodeficiency virus (HIV) is a virus that attacks specific immune cells called T cells. The anatomy of HIV plays an essential role in the replication and survival of the virus. The viral membrane is the outermost portion of HIV. This membrane is double-layered and comprised of fats called phospholipids. Effects of Immunodeficiency Immunodeficiency predisposes individuals to opportunistic infections. The microorganisms typically do not cause harm but, in an immune-compromised individual, they may cause damage. Often, this arises from resident body flora. Due to this vulnerability, anyone undergoing an invasive procedure must have a preventive antibiotic or prophylactic antimicrobial drug administered. Under certain circumstances, the immune system may mistake healthy body tissues for harmful pathogens. These cases are called "autoimmune attacks." During an autoimmune attack, specialized white blood cells called macrophages release inflammatory particles. When targeted at healthy tissues, inflammatory particles can cause cellular degeneration and damage, leading to conditions such as multiple sclerosis (MS) or rheumatoid arthritis (RA). Cancer is also a greater risk to an immune-compromised individual due to defective elimination of altered cancer cells or impaired immunity to eliminate cancer cells. Clinical Management of Immunodeficiencies The best defense against all invaders is avoidance. Good handwashing, thoughtful hygiene practices, and safe sexual practices help protect the spread of any of these invaders. Sometimes, by introducing our bodies to them (in the form of a vaccine), we are one step ahead and can minimize the damage they can cause. Vaccines are not only recommended by the Centers for Disease Control and Prevention (CDC) as a primary prevention strategy, but they are also recommended to protect individuals and communities. It is important to recall that not all microorganisms cause harm. Some are necessary for health. For example, individuals taking antibiotics may destroy bacteria used in the process of digestion. The clinical management of individuals with decreased immune systems (i.e., immunocompromised) or hyperimmune systems varies. These variations depend upon the type of condition, severity, age, health status, and any underlying medical conditions. Therefore, a thorough health history and physical examination are necessary to make clinical management decisions. Primary Prevention Strategies The foundation of primary prevention for immune system disorders is based on vaccinations across the life span. Other direct prevention interventions include: avoiding high-risk behaviors minimizing exposure to environmental hazards eating a proper diet regular exercise Secondary Prevention Strategies Secondary prevention strategies include screening for the presence or emergence of immune system disorders, for example, HIV testing for high-risk groups. These strategies are crucial to health promotion and disease prevention. Vaccinations The parents of a toddler question the need for their child to receive vaccinations. Which are true about vaccinations? mmunizations are considered part of primary prevention, help protect individuals from contracting specific diseases and spreading them to the community at large, and are recommended by the CDC. Immunizations are recommended for people of various ages across the life span. Vaccination does not guarantee that you will not get the disease, but it decreases the potential to contract the illness. No medication is risk-free. Risk Factors for Altered Immunity Populations At Risk As a future healthcare provider, it is important to recognize the populations at greatest risk for altered immune function. Suppressed Immune Response: Neonates, older persons, persons with leukemia or HIV, persons receiving chemotherapy or radiation, and those with a suppressed response are said to be immunocompromised. Immunocompromised persons are unable to defend against the invasion of microorganisms or foreign proteins adequately, placing them at risk for infection and, over time, for cancer. Exaggerated Immune Response: Persons with a family history of autoimmune disease and those who have an exaggerated or hyperimmune response are at risk. Examples of hyperimmune reactions are allergies that range from rhinitis (e.g., runny nose from exposure to pollen) to anaphylaxis (e.g., emergent response to food/medication or bee sting which may cause dizziness, hives, throat swelling, and/or respiratory distress) and autoimmune diseases, such as type 1 diabetes mellitus, where the immune system has attacked and destroyed the insulin-producing cells. Headache: This is caused by increased dilation of the blood vessels to the brain, alerting us to a problem, issue, or abnormality. Rhinitis: This symptom affects the mucous membranes of the nose, producing extra mucous to trap and remove inhaled particles that may harm us. Fever: This symptom is caused by an adjustment the hypothalamus makes to increase blood flow and vessel sizes for faster transport of immune and inflammatory mediators to the troublesome area. Infection: This is one major potential cause of immune system and inflammatory response activation. Pharyngitis: This symptom is another name for a sore throat and inflammation of the tonsils and other lymphoid tissue as it collects the byproducts of immune response. Cough: This inflammatory response causes an explosive, forceful outward movement of anything in the respiratory tract that may need removal. It works best when mucous has first trapped it. Human Immunodeficiency Virus and Acquired Immunodeficiency Syndrome Human immunodeficiency virus (HIV) is a virus that attacks specific immune cells called T cells. The anatomy of HIV plays an essential role in the replication and survival of the virus. The viral membrane is the outermost portion of HIV. This membrane is double-layered and comprised of fats called phospholipids. HIV has no symptoms. Acquired immunodeficiency syndrome (AIDS) occurs when a pathogen overwhelms the immune system, causing symptoms. Clinical manifestations vary among individuals and may initially present as flu-like systems, such as low fever, arthralgia, and sore throat. In the latent phase, individuals may present with no clinical signs or may have enlarged lymph nodes. In the final acute stage, individuals may experience opportunistic infections (herpes virus), gastrointestinal effects (wasting or anorexia), neurologic effects (dementia), secondary infections (pneumocystis carinii), and malignancies. HIV/AIDS HIV is transmitted  ○ unprotected sex ○ use of non-sterile syringes and tools ○ pregnancy, breastfeeding ○ blood transfusions ○ organ transplants HIV is not transmitted ○ food, drink, utensils ○ insect bites ○ kiss, touch ○ clothes, towels ○ toilet, shower Symptoms ○ muscle and joint pain ○ heat ○ weight loss ○ candidiasis of the oral cavity ○ rash ○ nausea ○ diarrhea ○ frequent viral infections Currently, only 75% of people with HIV know their status, and there is no vaccine. Do’s and Do Not’s ○ Do not use drugs ○ Use sterile tools ○ Use a condom ○ HIV blood test ○ Dementia is expected in the late stage of AIDS when immune deficiency is extremely low and complications are occurring in various body systems. Low-grade fever, sore throat, and arthralgia are expected in the initial first stage of AIDS in someone infected with HIV. Human Immunodeficiency Virus Treatment The treatment for clients with human immunodeficiency virus (HIV) or acquired immunodeficiency syndrome (AIDS) is multifaceted and includes specific medication classes, minimizing the effects of complications such as tuberculosis (TB). It is critical that treatment starts when the following occur: severe symptoms CD4 count under 500 pregnancy HIV-related kidney disease when an individual is treated for hepatitis B Treatment includes: antiviral drugs highly active antiretroviral therapy (HAART) antituberculosis drugs antidiarrheal drugs other drugs to control symptoms HAART is one of the most effective therapies for controlling the virus, reducing the viral load in the blood, and returning the CD4 cell counts to near-normal levels. A CD4 count below 500 warrants a prescribed treatment regimen by the healthcare provider. A count above 500 is considered a normal range for a healthy adult. Before any invasive procedure, what should be administered to immune-compromised individuals? Prophylactic antimicrobial drugs Prophylactic antimicrobial drugs help to prevent microorganisms that enter the body during an invasive procedure from causing an infection. Blood transfusion and vaccines are not correct. Vitamin C would be excellent complementary support, but prophylactic antimicrobial drugs will be most effective in preventing infection. Immunodeficiency puts individuals most at risk for which two conditions? Cancer and opportunistic infections Immunodeficient individuals are at higher risk of developing cancer and opportunistic infections. Malnutrition and kidney disease are common causes of primary immunodeficiency. DiGeorge syndrome is a primary cause of secondary immunodeficiency. HIV is transmitted by: use of non-sterile syringes and tools blood transfusion pregnancy, breastfeeding unprotected sex organ transplant HIV is not transmitted by: food, drink, utensils insect bites kiss, touch clothes, towels toilet, shower An individual living with HIV infection recently had their spleen removed. Which action should they use to prevent infection? Basic infection control techniques The spleen is one of the major organs of the immune system. Without the spleen, a person is at higher risk for infection. Basic infection control techniques, such as frequent handwashing and avoiding people who are sick, are most important. Limiting contact with the public and wearing a face mask when out may be required when airborne or droplet spread infections are high within the community, but not every time they are in public. Taking antibiotics as a precaution is not advised due to the risk of antibiotic resistance and side effects of long- term use. Case Study: Immune Disorder Manifestations A person with a history of misusing injectable drugs and type 2 diabetes has been experiencing flu-like symptoms of low-grade fever, weakness, arthralgia, and a headache for over a month. In the past few days, they have developed mouth sores and have no energy to get out of bed. Which cues strongly indicate this person may have an autoimmune disorder? Flu-like symptoms greater than a month No energy to get out of bed Mouth sores Headache The clinical manifestations that strongly indicate an autoimmune disease (acquired immune deficiency syndrome [AIDS]) are no energy to get out of bed, headache, flu-like symptoms lasting more than a month, and mouth sores. Persistent, clear sinus drainage is more closely associated with allergies and upper respiratory infections Based on the information provided, the person is most likely experiencing which alteration in health? Human immunodeficiency virus (HIV) This person is most likely experiencing human immunodeficiency virus (HIV) infection. People who participate in lifestyle behaviors, such as misusing injectable drugs or unprotected sex, have an increased risk of HIV. Additionally, flu-like symptoms are associated with early HIV infection. Cancer does not present with flu-like symptoms. Multiple sclerosis and rheumatoid arthritis are characterized by consistent autoimmune attacks that cause lasting pain or damage. Which type of test is used to confirm an infection with the human immunodeficiency virus (HIV)? Blood test The confirmation of HIV infection is done using blood tests for HIV antibodies. Sputum tests are prescribed for tuberculosis. CT scan and MRI are used to diagnose alterations inside the body. Which treatment options can the person expect to treat their human immunodeficiency virus (HIV) infection? Highly active antiretroviral therapy (HAART) Highly active antiretroviral therapy (HAART) is commonly used to treat HIV infection. Antibiotics are used to treat bacterial, not viral, infections. Immunosuppressing medications are not used to treat HIV since the illness already suppresses the person’s immune system. Aspirin may be used to treat the symptoms this person is experiencing, but it will not help the HIV infection.