Immunologic Deficiency Disorder
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What type of therapy has significantly decreased the transmission of HIV in infected pregnant women?
HAART (highly active antiretroviral therapy)
Etiology of HIV
retrovirus that is transmitted by lymphocytes and monocytes. Found in blood, semen, vaginal secretions and breast milk. Incubation period is months to years.
Horizontal transmission of HIV:
occurs through intimate sexual contact or parenteral exposure to blood or body fluids containing visible blood
Perinatal (Vertical) transmission of HIV:
occurs when and HIV-infected pregnant woman passes the infection to her infant
Pathology of HIV
primarily infects a specific subset of T lymphocytes, the CD4+ T cells. Virus takes over the machinery of the CD4+ lymphocytes, using it to replace itself, rendering the cell dysfunctional. the count gradually decreases over time leading to progressive immunodeficiency.
Clinical manifestations of HIV infection
- *oral candidiasis
- *failure to thrive
- *developmental delay
Common defining conditions of AIDS:
pneumonia, recurrent bacterial infections, wasting syndrome, HIV encephalopathy, herpes, short stature, malnutrition...etc.
Diagnostic evaluation for HIV and AIDS:
- After 18 months of age, the ELISA (HIV enzyme-linked immunosorbent assay) and Western blot immunoassay are used for determination.
- In babies <18 months, the maternal antibodies cover this up so the PCR (HIV polymerase chain reaction) is used for detection
Therapeutic management of HIV and AIDS
Goals: slowing growth of virus, preventing and treating opportunistic infections, and providing nutritional support and symptomatic treatment.
work at various stages of HIV life cycles to prevent reproduction of functional new virus particles. NOT CURABLE but these drugs can suppress viral replication and prevent further deterioration of immune system. THERAPY IS LIFE LONG!
What is the most common opportunistic infection of children infected with HIV? and at what age?
Pneumocystis carinii pneumonia (PCP). Most frequently b/t 3-6 months of age.
Immunization and HIV
Immunization against all common childhood illnesses is recommended to children exposed and infected with HIV. If severely immunocompromised....don't give chicken pox and MMR vaccine. Immediate prophylaxis after exposure to vaccines is warrented bc of poor antibody production to vaccines
Nursing care management of HIV/AIDS
- Education on transmission and control of infections is essential, safety issues, ongoing education about HIV to dispel myths, PREVENTION IS KEY to education....routes of transmission, hazards of IV drug use, sex, etc..
- Ongoing assessment of pain is crucial (emla cream, tylenol, nsaids, opiods)
Severe combined immunodeficiency disease (SCID)
absence of both humoral and cell-mediated immunity. (terms swiss-type lymphopenic agammaglobulinemia and X linked lymphopenic agammaglobulinemia) also describe this disorder
Susceptability to infection occurs early in life (first month). Failure to thrive is a consequence of this illness.
Diagnosis of SCID
Usually based on history of recurrent, severe infections from early infancy, familial history of the disorder and specific laboratory findings, which include lymphopenia, lack of lymphocyte response to antigens, and absence of plasma cells in bone marrow.
Therapeutic management of SCID
Definitive treatment is HSCT from histocompatible donor (usually parent) or matched un-related donor. IVIG infusions and PCP prophylaxis are used to augment the humoral immunity until the transplant is performed
Nursing care managment of SCID
Preventing infection and supporting child and family. Since prognosis is VERY poor, if there is no compatible donor, nursing care is directed at supporting family in caring for a child with a life-threatening illness.
Wiskott-aldrich syndrome (WAS)
WAS is an X-linked recessive disorder characterized by a triad: thrombocytopenia, eczema, and immunodeficiency of selective functions of B lymphocytes and T-lymphocytes.
Wiskott-aldrich syndrome presenting symptoms at birth:
bloody diarrhea from thrombocytopenia, as child grows older....recurrent infection and eczeme become more severe and bleeding becomes less frequent. Chronic infection with herpes is frequent and may lead to vision loss.
Medical treatment for Wiskott-aldrich syndrome
Counteracting the bleeding tendencies with platelet transfusions, using IV gamma globulin to provide passive immunity and administering prophylactic antibiotics to prevent and control infection
Only curative therapy is HSCT from matched donor
Nursing care management for Wiskott-aldrich syndrome
Poor prognosis, main consideration is supporting family in the care of a fatally ill child. Controlling physical problems, prevention or control of infections.
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