Learning Objectives
By the end of this section, you will be able to:
- Define primary immunodeficiencies
- Differentiate between HIV and AIDS
The result of the malfunctioning of an underactive or weakened immune system is termed immunodeficiency. This is different from an autoimmune disease, in which the body’s immune system overreacts and mistakenly attacks the body. While autoimmune disease may attack normal, healthy tissue, immunodeficiency may lead to an inadequate response to pathogens. As such, immunodeficiency disorders impair the immune system’s ability to defend the body against foreign invaders. They may lead to infections that develop and recur often and that are typically more severe and longer lasting than usual. They may also result in the development of lymphomas or other types of cancers. There are two types of immunodeficiency disorders: primary and secondary.
Primary Immunodeficiencies
The primary immunodeficiencies generally are present at birth; they are genetic and usually hereditary. These disorders typically become evident during infancy or childhood, but some may not be recognized until adulthood. There are more than 100 primary immunodeficiency disorders, and all are quite rare.
Cultural Context
Race and Ethnicity in Primary Immunodeficiency
Because primary immunodeficiencies are linked to genes, the nurse should be aware of the cultural considerations when providing assessment and care. A patient’s racial/ethnic background may predispose them to certain primary immunodeficiencies. For example, while it is known that sickle cell disease is more prevalent in Black individuals, systemic racism and bias can often be barriers to diagnosis and treatment (Pokhrel et al., 2023). Navajo Nation and Apache populations have a higher incidence of Athabaskan severe combined immunodeficiency, a secondary combined immunodeficiency linked to the DCLRE1C gene (Kniffen, 2023).
Pathophysiology
Primary immunodeficiencies may be caused by mutations to a specific gene. For example, most immunodeficiencies are congenital and have an X-linked (autosomal recessive) inheritance pattern. Mutated genes on the X sex chromosome occur most often in males. Immune cells involved in immunodeficiencies include B and T lymphocytes. B cells transform into plasma cells that produce many antibodies (immunoglobulins) to fight off extracellular microorganisms. If there is a problem with B cells, a person has a high susceptibility to pneumonia, otitis, and other infections caused by extracellular bacteria.
T cells differentiate into helper, cytotoxic, or suppressor T cells. Helper T cells stimulate antibody production. In T-cell deficiencies, antibody production may be compromised to some degree. T cells also fight intracellular microorganisms, such as fungi and viruses, as well as tumors. Microorganisms can infect and tumors may flourish in individuals with HIV, severe combined immunodeficiency, hyper-immunoglobulin M syndrome, and other T-cell deficiencies (Valliant & Qurie, 2022).
Clinical Manifestations
Clinical manifestations of primary immunodeficiencies include infections with opportunistic or unusual organisms, persistent infections despite aggressive treatment, failure to thrive, and a positive family history. Signs of infection will vary depending on the disorder but may include fever, thrush, gingivitis, warts, pus-filled sores, loss of appetite, abdominal pain, liver or spleen enlargement, ear infections, and/or skin infections.
Assessment and Diagnostics
To diagnose an immunodeficiency, nurses should complete a full-body assessment to identify signs and symptoms that could be associated with a disorder. For example, the spleen may be enlarged. In addition, nurses must obtain a family history. The patient should be asked questions about recent infections and their signs, severity, and duration.
A true diagnosis may require genetic testing or blood tests. A complete blood cell count with manual differential can reveal abnormal levels of serum IgG, IgM, and IgA. Antibody responses to vaccines should also be evaluated to detect a humoral immunodeficiency. Genetic testing can assess for gene mutations and whether or not a genetic predisposition exists. Biopsies of samples of the bone marrow or lymph nodes can assess whether immune cells are present.
Prevention
Prevention of primary immunodeficiencies may include family planning to address future pregnancies. Genetic testing can be done in utero to determine the likelihood that the fetus will acquire the disorder. Live vaccines are contraindicated in patients with antibody deficiencies because their bodies may not be able to defend against the weakened form of the virus in the vaccine. Patients should always consult with their provider for the best course of action on an individual basis regarding vaccines. Furthermore, all patients with immunodeficiency should be educated on the measures that can be taken to reduce their risk of infection and illness, such as safe sex practices, antiretroviral drugs, cancer treatment when needed, and blood sugar control (Fernandez, 2023). Infection prevention measures like avoiding public places and masking when in public; frequent, proper hand hygiene; and social distancing are also important for immunocompromised patients.
Collaborative Management
Immunodeficiencies require an interdisciplinary approach. A primary care physician may begin initial testing but generally refers the patient to an immunologist, because affected individuals are at risk for developing severe infections and warrant early detection and medical management. Some individuals are psychologically affected by an immunodeficiency and may need group therapy or individual counseling with a licensed professional. Social work may be needed as well to help affected individuals find resources; treatment and care can become extensive and expensive. Depending on the course of treatment, the patient may need a stem cell transplant, typically performed by a hematologist or oncologist.
To prevent infections in those with immunodeficiencies, the nurse should educate the patient to perform good oral hygiene, avoid eating undercooked food or drinking contaminated water, receive immune globulin treatments if ordered by the physician, and avoid individuals with infections (Fernandez, 2023). Nurses should also educate the patient and family member how to administer therapy at home, if prescribed.
Interdisciplinary Plan of Care
Plan of Care for Patient with HIV
For a patient with HIV, a comprehensive interdisciplinary plan of care would be crucial to address both medical management and holistic support needs. Infectious disease specialists would lead the medical management, focusing on antiretroviral therapy (ART) initiation or adjustment based on viral load and CD4 count monitoring. They would collaborate with the patient’s primary care physician to coordinate overall health maintenance and manage potential drug interactions with other medications.
Other team members may include psychiatrists or psychologists, social workers, nutritionists, pharmacists, and infectious disease nurses. Psychiatrists or psychologists would provide crucial support in addressing mental health aspects, such as depression, anxiety, or adjustment issues related to the HIV diagnosis. They would conduct regular assessments, offer counseling, and monitor the patient’s emotional well-being throughout treatment.
Social workers would assess the patient’s social support network, financial resources, and housing stability. They would assist in navigating insurance coverage for medications, accessing community resources for HIV support groups, and addressing any legal or employment concerns.
Nutritionists would assess the patient’s dietary intake and nutritional status, providing tailored dietary recommendations to support immune function and overall health. They would monitor weight changes and collaborate with the health care team to manage any nutritional deficiencies or gastrointestinal symptoms related to HIV or its treatment.
Pharmacists specializing in HIV care would ensure proper medication adherence, educate the patient on potential side effects, and monitor for drug interactions. They would also assist in managing medication schedules and addressing any concerns the patient may have about their treatment regimen.
Infectious disease nurses provide ongoing education on HIV transmission prevention, medication adherence strategies, and symptom management. They monitor the patient for any signs of opportunistic infections and collaborate closely with the infectious disease specialist to optimize treatment outcomes.
This interdisciplinary approach ensures that the patient with HIV receives comprehensive care addressing medical, psychological, social, nutritional, and pharmaceutical aspects of their health. It emphasizes the collaborative effort needed to manage HIV effectively and improve the patient’s quality of life.
Human Immunodeficiency Virus and Acquired Immunodeficiency Syndrome
More common than a primary immunodeficiency is a secondary immunodeficiency. Secondary immunodeficiencies generally develop later in life and are caused by another disorder or the use of certain medications, such as chemotherapy or corticosteroids. A well-known example is acquired immunodeficiency syndrome (AIDS), which is caused by the human immunodeficiency virus (HIV) (see 22.2 Viral and Fungal Infections). This section briefly describes HIV to provide insight into how it relates to immunity. Human immunodeficiency virus is a blood-borne virus that may be spread by sexual contact, sharing needles, or accidentally sticking oneself with a needle previously used by an infected individual. Although the risk is low, blood transfusions are also associated with the spread of HIV, though most blood products today are screened for the virus. Maternal-child transmissions may occur through the placenta, during childbirth, or through breast milk.
Pathophysiology
Human immunodeficiency virus binds to and enters host T cells via CD4+ molecules and chemokine receptors. Then, RNA and several HIV-encoded enzymes are released into the host cell. Viral replication requires reverse transcriptase copy HIV RNA to produce viral DNA. This mechanism is altered with this infection and results in frequent mutations and new HIV genotypes. These mutations enable the generation of HIV that refuses control by the immune system. This causes progressive destruction of the cell-mediated immune system and eliminates CD4+ T-helper lymphocytes. Eventually, HIV progresses into AIDS, especially if untreated. Characterized by extremely low levels of helper T cells, AIDS leaves the affected individual at risk for a range of life-threatening infections. The time of progression varies depending on when treatment is started and how aggressive treatment is.
Prevention
Prevention includes safe sex practices, such as using condoms and limiting the number of sexual partners, as well as abstinence from sex or exchange of sexual fluids. The use of pre-exposure prophylaxis, such as tenofovir disoproxil fumarate and emtricitabine, may be necessary for high-risk patients, for example, to reduce their chances of acquiring HIV if they are exposed.
The drug treatment regimen of choice will differ depending on a person’s risk factors. For example, if they are at risk from sexual exposure or needles used to inject drugs, the treatment differs. The person-at-risk should undergo frequent testing every three months to ensure they have not become infected. Other preventive measures include avoiding sharing razors, toothbrushes, needles, sex toys, or any article that may be contaminated with blood. Affected individuals should be educated to ask if they will accept blood, plasma, sperm, or organ donation. In addition, they should be encouraged to inform any past, present, or future sexual partners of their diagnosis and to adhere to any treatment regimen that is prescribed.
Link to Learning
Visit this website from the U.S. Department of Health and Human Services for information about HIV prevention that includes where to get tested, what to expect when starting and continuing treatment, and how to live well with HIV. The initial diagnosis can be overwhelming, so the nurse should consider giving patients time to review the information at home.
Risk Reduction of Transmission to Health-Care Providers
To reduce the risk of transmission of blood-borne infection, take standard precautions with any patient, regardless of their HIV status. This includes hand hygiene, use of personal protective equipment, proper handling of soiled equipment and patient laundry, environmental control, respiratory hygiene, use of resuscitation equipment to avoid contact with oral secretions, and sharps safety. In the event of a needlestick injury, the provider should first wash their hands, then immediately report the incident to their infectious disease representative at their facility or per their policy. When possible, blood samples should be taken from the patient to confirm whether they have a blood-borne infection. Postexposure prophylaxis should also be taken as soon as possible, as long as it is with seventy-two hours of possible HIV exposure (CDC, 2024). This includes taking an antiretroviral medication.
Stages of HIV Infection
There are three stages of HIV infection. These stages vary and are dependent on history, physical examination, laboratory testing, signs and symptoms, and other infections or malignancies. Stage one is an acute HIV infection, stage two is a chronic HIV infection, and stage three is AIDS. A person’s CD4 count is a tool used to determine their stage of infection (Cachay, 2023), as shown in Table 29.5. If an affected person’s CD4 count increases with therapy, however, the patient does not return to a previous stage.
| Stage | Name | CD4 Count |
|---|---|---|
| 1 | Acute HIV | ≥500 cells/µL |
| 2 | Chronic HIV | 200 to 499 cells/µL |
| 3 | AIDS | <200 cells/µL |
Assessment, Diagnostics, and Clinical Manifestations
In the initial stages of HIV infection, a patient may be asymptomatic or have nonspecific flu-like symptoms, such as fatigue, headache, or rash. In the later stages of HIV, a patient may have more immune system–specific symptoms and sequelae, including the following:
- fever
- fatigue
- malaise
- dermatitis
- sore throat
- lymphadenopathy
- herpes
- vaginal candidiasis
- pneumococcal infections
- decreased appetite
- weight loss
- wasting syndrome
- cancer
- skin changes, such as crusted scabies, disseminated bartonellosis, anal carcinoma, and/or Kaposi sarcomas (Figure 29.6)
Life-Stage Context
Life Span Considerations and HIV
The number of adults 50 years of age and older who have HIV or AIDS is rising (HIVinfo, 2024). While some were diagnosed in younger years and are benefiting from treatment, there are others who are undiagnosed. Older adults are less likely to get tested, as the signs and symptoms of HIV can be mistaken for age-related changes, such as pain. Older adults with HIV are more at risk for other comorbidities, including heart disease and diabetes (Roomaney et al., 2022). It is therefore essential to assess patients and obtain a history to determine whether they may need to be tested.
Treatment of HIV Infections
Members of an interdisciplinary team for treating HIV infections may vary but could include primary care providers, pharmacists, psychiatrists or other licensed mental health professionals, social workers, and nurses. Antiretroviral therapy (ART) is a treatment designed to suppress HIV replication to reduce HIV-associated morbidity, restore and preserve immunological function, suppress plasma HIV viral load, and prevent HIV transmission. The nurse should encourage the patient to adhere to treatment regimens as well as educate the patient on their disease status, treatment, and prevention. Social workers may be needed to help patients locate resources, such as finances, housing, or food.
Complementary and alternative medicine (CAM) therapies may include mind-body practices, such as massage or yoga, which may be helpful at lowering blood pressure, pain, and anxiety. Other CAM therapies include exercise and adequate nutrition. Patients may be advised to eliminate food allergens, coffee, trans-fatty foods, and refined foods; eat quality protein sources and foods high in vitamins, antioxidants, and minerals; and drink at least 64 oz of filtered water each day (St. Luke’s Hospital, 2016).
Emotional and Ethical Concerns
A diagnosis of HIV or AIDS can be devastating to an individual. They may be unsure of when or how they contracted the disease or from whom. While it is important to contact previous sexual partners so they can be tested and treated, patients may find this task prohibitively embarrassing or even frightening. All health care providers on the team must remember to be sensitive to the patient’s feelings, preserve their dignity, and encourage therapeutic communication.
While the emphasis is on the patient, providers may also be confronted with complex emotions and reactions to treating patients with HIV. Nurses may fear exposure or infection and have concerns about the patient’s confidentiality. To assuage these fears, precautions should be meticulously followed to avoid the risk of contracting the infection. The nurse has a duty to inform other members of the interdisciplinary team to ensure they are able to continue providing quality care safely. The nurse should also safeguard and protect the patient’s privacy from others.
Despite the stigma that may still surround HIV and AIDS, there are legitimate reasons for infected patients to remain optimistic about their prognosis. The mortality rate of those infected was much higher in the 1980s, during the early years of AIDS. In recent decades, researchers have made significant progress, and patients are living longer today because more treatments are available to control the disease’s progression. Some people with HIV can go into remission, meaning that they have such low levels of the virus in their body, they no longer need to take ART to prevent progression (Deeks et al., 2021).