Chapter 36 Management of Patients with Immune Deficiency Disorder
Vocabulary:
Candidiasis: fungal infection of the skin and mucus membranes that causes Candida species
Enzyme immunoassay (EIA): blood test that can determine presence of antibodies to HIV in blood or saliva, a variant of
this test is called enzyme-linked immunosorbent assay (ELISA)
HIV-1: retrovirus isolated and recognized as the etiologic agent of HIV disease
HIV encephalopathy: clinical syndrome characterized by a progressive decline in cognitive, behavioral, and motor function
Immune reconstitution inflammatory syndrome (IRIS): syndrome that results from rapid restoration of pathogen-
specific immune response to opportunistic infections
Kaposi sarcoma: malignancy that involved the epithelial layer of blood and lymphatic vessels
Latent reservoir: the integrated HIV provirus within the CD4+ T cell during the resting memory state, does not express viral
proteins and is invisible to the immune system and antiviral medications
Mycobacterium avium complex (MAC): infection caused by mycobacterial organisms that commonly causes respiratory
illness but can also infect other body systems
Opportunistic infection: illness caused by various organisms some of which usually do not cause diseases in normal
immune system patients
Peripheral neuropathy: disorder characterized by sensory loss, pain, muscle weakness and wasting of muscles in the hands
or legs and feet
Pneumocystis pneumonia (PCP): common opportunistic lung infection; pathogen implication is most commonly fungus
Polymerase chain reaction: sensitive lab technique that can detect and quantify HIV in person’s blood or lymph nodes
Post-exposure prophylaxis (PEP): taking antiretroviral medications ASAP, but not more than 72 hours after possible
exposure to HIV; 2-3 drugs are usually prescribed which must be taken for 28 days
Pre-exposure prophylaxis (PrEP): prevention method for HIV-negative people who are @ risk for contraction, taking
specific combination of HIV medicines daily; use with condoms and other preventive tools
Progressive multifocal leukoencephalopathy: opportunistic infection that infects brain tissue and causes damage to the
brain and spinal cord
Retrovirus: virus that carries genetic material in RNA instead on DNA and contains reverse transcriptase
Reverse transcriptase: enzyme that transforms single-stranded RNA into double stranded DNA
Viral load test: measures the quantity of HIV RNA or DNA in the blood
Viral set point: amount of virus present in the blood after the initial burst of viremia and the immune response that follows
Wasting syndrome: involuntary weight loss consisting of both lean and fat body mass
Reading Notes:
Introduction
Immune system protects against foreign substances, proliferation of neoplastic cells, and key role in inflammation and
healing
Primary Immune Deficiencies
Majority of PIDDs are diagnosed in infancy with male to female ratio of 5 to 1
Early adulthood diagnosis are frequently confounded by frequent use of antibiotics that mask symptoms
Pathophysiology
More than 270 different genes are associated with PIDDs
Prevent the body from developing normal immune responses resulting in numerous disorders with differing clinical
symptoms
Clinical Manifestations
Multiple infections despite aggressive treatment, infections with unusual or opportunistic organisms, failure to thrive and
poor growth, positive family history
Look at chart 36-1 on pg. 1023
Assessment and Diagnostic Findings
Onset in delay between onset of symptoms and time of Dx of PIDDs
, Chapter 36 Management of Patients with Immune Deficiency Disorder
Family Hx should be carefully checked because of genetic origins of PIDDs
Lab tests are used to identify antibody deficiencies, cellular T cell defects, neutrophil disorders and complement deficiencies
CBC with diff should be analyzed first
Lymphopenia may indicate an immunologic abnormality; serum Ig levels and antibody responses to vaccines should be
assessed to detect a humoral immune defect
o Use age-matched normal ranges -> levels change as person ages
Prevention
Live vaccines are contradicted in patients with antibody deficiency disorders -> unable to generate antibodies and live
substance in vaccine can cause death
Prenatal testing should be done
Medical Management
Indication for PIDD include unusually frequent and severe infections -> referral to immunologist
Neutroepnic patients are at increased risk for development of severe infections
Infection control -> emergence of multi-drug-resistant organisms
HSCT is curative -> stem cells may be from embryo or adults -> toxicity and reduced efficacy are limitations
Another therapy uses cells as vehicles for delivery of genes or gene products -> gene therapy -> many AE such as toxicity
Pharmacologic Therapy
Depends on type and severity of presenting infections in the PIDD Dx
PPx drug treatment can prevent some bacterial and fungal infections -> emergence of resistant organisms
Patients with antibody deficiencies receive regular Ig replacement therapy including immunoglobulin IV (IVIG) and subQ
immunoglobulin (SCIG) to provide functional antibodies
Nursing Management
Many patients have comorbid autoimmune disorders such as thyroid disease, RA, and IBD
Many require immunosuppressant therapy to ensure engraftment is successful -> nursing care must be meticulous
Hand hygiene and infection prevention look at chart 71-2 for methods of prevention
Identify S/S of infection early
Education for how to administer therapy @ home chart 36-1 pg. 1023
Acquired Immune Deficiency
Can be acquired from chemotherapy treatment or from infection such as with HIV
Prevention, early detection, and ongoing treatment are important aspects of care for patient living with HIV/AIDS
o PLWHA
HIV Infection and AIDS
1st decade since detection focused on recognition and treatment of opportunistic disease and introduction of PPx against
opportunistic infections
2nd decade had progress in development of highly active antiretroviral drug therapy (HAART)
3rd decade focused on issues of preventing new infections, adherence to antiretroviral therapy (ART), development of 2 nd
generation combination medications that affect different stages of viral lifestyle, and continued need for effective vaccine
HIV antibody test: enzyme immunoassay (EIA) or variant ELISA -> 1984 allowing for early Dx before onset of symptoms
HIV is managed more as a chronic disease in outpatient setting while AIDS may involve acute conditions that require
hospitalization
Epidemiology
Lab evidence is preferred overall clinical S/S
HIV can be classified between 0-unknown stages
o 0 indicates early infection inferred from lab tests
o Stages 1, 2, 3 are based on CD4+ T-lymphocyte count
o Cases with no info on CD4+ T-lymphocyte are classified as unknown
Vocabulary:
Candidiasis: fungal infection of the skin and mucus membranes that causes Candida species
Enzyme immunoassay (EIA): blood test that can determine presence of antibodies to HIV in blood or saliva, a variant of
this test is called enzyme-linked immunosorbent assay (ELISA)
HIV-1: retrovirus isolated and recognized as the etiologic agent of HIV disease
HIV encephalopathy: clinical syndrome characterized by a progressive decline in cognitive, behavioral, and motor function
Immune reconstitution inflammatory syndrome (IRIS): syndrome that results from rapid restoration of pathogen-
specific immune response to opportunistic infections
Kaposi sarcoma: malignancy that involved the epithelial layer of blood and lymphatic vessels
Latent reservoir: the integrated HIV provirus within the CD4+ T cell during the resting memory state, does not express viral
proteins and is invisible to the immune system and antiviral medications
Mycobacterium avium complex (MAC): infection caused by mycobacterial organisms that commonly causes respiratory
illness but can also infect other body systems
Opportunistic infection: illness caused by various organisms some of which usually do not cause diseases in normal
immune system patients
Peripheral neuropathy: disorder characterized by sensory loss, pain, muscle weakness and wasting of muscles in the hands
or legs and feet
Pneumocystis pneumonia (PCP): common opportunistic lung infection; pathogen implication is most commonly fungus
Polymerase chain reaction: sensitive lab technique that can detect and quantify HIV in person’s blood or lymph nodes
Post-exposure prophylaxis (PEP): taking antiretroviral medications ASAP, but not more than 72 hours after possible
exposure to HIV; 2-3 drugs are usually prescribed which must be taken for 28 days
Pre-exposure prophylaxis (PrEP): prevention method for HIV-negative people who are @ risk for contraction, taking
specific combination of HIV medicines daily; use with condoms and other preventive tools
Progressive multifocal leukoencephalopathy: opportunistic infection that infects brain tissue and causes damage to the
brain and spinal cord
Retrovirus: virus that carries genetic material in RNA instead on DNA and contains reverse transcriptase
Reverse transcriptase: enzyme that transforms single-stranded RNA into double stranded DNA
Viral load test: measures the quantity of HIV RNA or DNA in the blood
Viral set point: amount of virus present in the blood after the initial burst of viremia and the immune response that follows
Wasting syndrome: involuntary weight loss consisting of both lean and fat body mass
Reading Notes:
Introduction
Immune system protects against foreign substances, proliferation of neoplastic cells, and key role in inflammation and
healing
Primary Immune Deficiencies
Majority of PIDDs are diagnosed in infancy with male to female ratio of 5 to 1
Early adulthood diagnosis are frequently confounded by frequent use of antibiotics that mask symptoms
Pathophysiology
More than 270 different genes are associated with PIDDs
Prevent the body from developing normal immune responses resulting in numerous disorders with differing clinical
symptoms
Clinical Manifestations
Multiple infections despite aggressive treatment, infections with unusual or opportunistic organisms, failure to thrive and
poor growth, positive family history
Look at chart 36-1 on pg. 1023
Assessment and Diagnostic Findings
Onset in delay between onset of symptoms and time of Dx of PIDDs
, Chapter 36 Management of Patients with Immune Deficiency Disorder
Family Hx should be carefully checked because of genetic origins of PIDDs
Lab tests are used to identify antibody deficiencies, cellular T cell defects, neutrophil disorders and complement deficiencies
CBC with diff should be analyzed first
Lymphopenia may indicate an immunologic abnormality; serum Ig levels and antibody responses to vaccines should be
assessed to detect a humoral immune defect
o Use age-matched normal ranges -> levels change as person ages
Prevention
Live vaccines are contradicted in patients with antibody deficiency disorders -> unable to generate antibodies and live
substance in vaccine can cause death
Prenatal testing should be done
Medical Management
Indication for PIDD include unusually frequent and severe infections -> referral to immunologist
Neutroepnic patients are at increased risk for development of severe infections
Infection control -> emergence of multi-drug-resistant organisms
HSCT is curative -> stem cells may be from embryo or adults -> toxicity and reduced efficacy are limitations
Another therapy uses cells as vehicles for delivery of genes or gene products -> gene therapy -> many AE such as toxicity
Pharmacologic Therapy
Depends on type and severity of presenting infections in the PIDD Dx
PPx drug treatment can prevent some bacterial and fungal infections -> emergence of resistant organisms
Patients with antibody deficiencies receive regular Ig replacement therapy including immunoglobulin IV (IVIG) and subQ
immunoglobulin (SCIG) to provide functional antibodies
Nursing Management
Many patients have comorbid autoimmune disorders such as thyroid disease, RA, and IBD
Many require immunosuppressant therapy to ensure engraftment is successful -> nursing care must be meticulous
Hand hygiene and infection prevention look at chart 71-2 for methods of prevention
Identify S/S of infection early
Education for how to administer therapy @ home chart 36-1 pg. 1023
Acquired Immune Deficiency
Can be acquired from chemotherapy treatment or from infection such as with HIV
Prevention, early detection, and ongoing treatment are important aspects of care for patient living with HIV/AIDS
o PLWHA
HIV Infection and AIDS
1st decade since detection focused on recognition and treatment of opportunistic disease and introduction of PPx against
opportunistic infections
2nd decade had progress in development of highly active antiretroviral drug therapy (HAART)
3rd decade focused on issues of preventing new infections, adherence to antiretroviral therapy (ART), development of 2 nd
generation combination medications that affect different stages of viral lifestyle, and continued need for effective vaccine
HIV antibody test: enzyme immunoassay (EIA) or variant ELISA -> 1984 allowing for early Dx before onset of symptoms
HIV is managed more as a chronic disease in outpatient setting while AIDS may involve acute conditions that require
hospitalization
Epidemiology
Lab evidence is preferred overall clinical S/S
HIV can be classified between 0-unknown stages
o 0 indicates early infection inferred from lab tests
o Stages 1, 2, 3 are based on CD4+ T-lymphocyte count
o Cases with no info on CD4+ T-lymphocyte are classified as unknown
