Primary immune deficiency disorders (PAtho)
*Genetic
*Majority diagnosed in infancy; some may be diagnosed during adolescence
*Occasionally, adults may present with persistent, recurrent, or resistant infections
*Prevent body from developing normal immune responses
*May affect phagocytic function, B cells or T cells, or the complement system
clincal manifestations of PIDD
*Multiple infection despite treatment
*Infection with unusual/opportunistic organisms
*Failure to thrive, poor growth, +family hx
Assessment of PIDD
labs identify antibody deficiencies, T cell defects, neutrophil disorders and complement deficiencies; CBC with differential, lymphocytopenia may indicate an immunologic abnormality; serum Ig levels and antibody responses to vaccines should be assessed
prevention of PIDD
*live vaccines are contraindicated in pts with antibody deficiency
medical mgmt PIDD
refer to immunologist, neutropenia=increased risk of infection, infection control practices evaluated esp with multidrug resistant organisms; HSCT is curative, IVIG and subcutaneous immunoglobin to provide functional antibodies
what kind of live vaccines do you not want to give?
MMR and chicken box
nursing mgmt for PIDD
*Nursing care is meticulous because often PIDD pts have autoimmune disorders
what are the strategies to reduce risk of infection
oHand hygiene
oInfection prevention precautions per institution policy
oContinual monitoring for early signs of infection
oTeach patients and caregivers to administer medications and therapy at home see Chart 36-1 for home administration of Ig replacement therapy
HIV
the virus that causes AIDS
what are some important aspects of care for a pt with HIV?
Prevention, early detection, and ongoing treatment
who is most profoundly affected by HIV?
Gay, bisexual, and other men who have sex with men of all races remain the population most
modes of trasmission (HIV)
*Inflammation and breaks in skin or mucosa increase probability of HIV exposure will lead to infection
HIV-1 transmitted in body fluids that contain infected cells
oBlood and blood products
oSeminal fluid
oVaginal secretions
oMother-to-child: Amniotic fluid, breast milk, during delivery
oNot through casual contact (sharing infected injection drug use equipment, sexual relations with infected individuals, infants born to mothers with HIV or who are breastfed by, those who received transplants or blood products esp between 1978-1985)
HIV prevention
*use of condoms; some pts don't have this freedom= may require pre-exposure prophylaxis where one pill with two HIV medications (tenofovir disoproxil fumarate and emtricitabine) are taken daily to avoid risk of acquiring HIV; abstinence and effective use of latex condoms (non-latex will not prevent) are only way to prevent sexual transmission; male circumcision reduces risk of heterosexually acquired HIV by 60%; females condoms provide barrier to genital secretions containing HIV
oHIV testing; needle exchange programs
oLinkage to treatment and care
patient education HIV (ABstain)
reduce # of partners to 1; use latex condoms- nonlatex will not prevent HIV, don't reuse condoms, avoid cervical caps/diaphragms without a condom as well
if anal intercourse is unavoidable what can you teach the patient ?
if not avoidable use lubricant; avoid "fisting", inform sexual partners; put on a new condom before any kind of sex, hold condom at the tip to squeeze out the air, unroll all the way over the erect penis, have sex, hold condom so it cannot come off the penis, pull out, use new condom if you want to have sex again or in a different place (keep condoms cool and dry, never use skin lotions, baby oil, petroleum jelly, cold cream= can cause latex condom to break= products made with water- KY jelly are safer to use)
LBGTQ
are at higher risk of contracting HIV, experience significant challenges due to family rejection, lack of support, stigma, isolation, abuse, and harassment- nurses should be nonjudgmental in order to educate this population about prevention
Harm reduction framework for people who inject drugs
oNeedle exchange
oMay use bleach to clean used needles and syringes
oAvoid sharing needles and syringes
oAvoid sharing needles, razors, toothbrushes, sex toys, blood contaminated articles
when does the standard precautions need?
hand hygiene when? Gloves, gown, mask when? Needles and sharps? Resuscitation? Patient placement? Respiratory hygiene/cough etiquette?
WHat is the most effective measure to prevent transmission of organisms?
Hand Hygiene
Post exposure prophylaxis
oAntiretroviral medications within 72 hours of exposure
o2 to 3 drugs prescribed for 28 days
o0.23% risk of becoming HIV infected when exposed to needle stick involving HIV blood
oOccupational exposure- urgent- the sooner the better for treatment- every hour counts
Post HIV Exposure Prophylaxis for HCP
*Alert supervisor and initiate exposure reporting system
*Determine pts HIV status to guide post exposure prophylaxis; use rapid testing if HIV status is unknown
*Use precautions: barrier contraception, avoid blood/tissue donations, pregnancy, breast feeding to prevent secondary transmission esp first 6-12 weeks after
*Reevaluation of exposed HCP 72 hr after
*HIV testing at baseline, 6 wk, 12 wk, and 6 mo after exposure
Currently no vaccine for HIV
CD4+ normal range is
500-1000
HIV patho
is in the subfamily of lentiviruses and is a retrovirus because it carries its genetic material in the form of ribonucleic acid (RNA) rather than deoxyribonucleic acid (DNA)
HIV patho #2
targets cells with CD4+ receptors, which are expressed on the surface of T lymphocytes, monocytes, dendritic cells, and brain microglia
Stage 0
oEarly HIV infection; initially pt may test - initially but in 2-3 weeks antibodies can be detected in the sera of people infected with HIV but most of the antibodies at this time lack the ability to control the virus
stage 1 primary/acute
oPeriod from infection with HIV to the development of HIV-specific antibodies; once antibodies can be detected HIV is firmly est in host
oDramatic drops in CD4+ T-cell counts normally 500 to 1500 cells/mm3 of blood
Stage 2
*Occurs when CD4+ T-lymphocyte cells are between 200 and 499
Stage 3
oCD4+count drops below 200 cells/mm3 of blood
oConsidered to have AIDS for surveillance purposes
oOnce classified as surveillance severity stage is cannot be classified into a less severe stage even if CD4 increases= severe immune dysfunction allows them to qualify for disability, housing, food stamps
unknown
No information on CD4+ T-lymphocyte count or percentage
during the first stage of HIV pts may exhibit what symptoms?
fatigue or skin rash
Respiratory Manifestations (HiV)
oShortness of breath, dyspnea, cough, chest pain
oPneumocystis pneumonia (on exertion= tachypnea, tachycardia, diffuse dry rales, oral thrush is common, hypoxemia, elevated lactate), Mycobacterium avium complex (typically occurs when CD4 count is less than 50 cells/mm, caused by infection with different types of mycobacterium- fever, night sweats, wt loss, fatigue, diarrhea, abd pain) , TB (at diagnosis of TB should test for latent TB- once pt is stage 3 should be retested; latent TB treated with isoniazid and pyridoxine)
GI manifestations of HIV
Loss of appetite, N and V, oral candidiasis (painless, creamy white, plaque like lesions), diarrhea
what do you want to give a pt with HIV with stomach issues?
OCTREOTIDE (slows GI motility and intestinal secretion of water and electrolytes)
wasting syndrome
weight loss, decrease in muscular strength, appetite, and mental activity; associated with AIDS
if pt has wasting syndrome what medicine can you give them as appetite stimulant?
Megestrol acetate
HIV pt has nause and vomiting , what can be prescribed?
dronabinol
Kaposi's sarcoma
may spread through sexual contact, localized cutaneous lesions that are brownish-pink- deep purple (Figure 36-3) can disrupt skin integrity= infection
AIDS
HL (reed steenurg) and NHL
neurological manifestations HIV
Effects on cognition, motor function, visual memory, demyelination, degeneration, HIV encephalopathy (HIV in brain and CSF, memory deficits, HA, difficulty concentrating, progressive confusion, ataxia, hallucinations, tremor, seizures), peripheral neuropathy
what are some SE of ART drugs
pain in feet and hands
neurological (HIV) cont...
oFungal infection, Cryptococcus meningitis- fever, HA, malaise, stiff neck, N/V, MS change, seizures; diagnosis= CSF analysis
oDepression and apathy (fluoxetine)
herpes zoster
painful vesicles that disrupt skin integrity
Gynecologic Manifestations
oGenital ulcers: increases risk of transmission of HIV
oPersistent, recurrent vaginal candidiasis
oMenstrual abnormalities: amenorrhea or bleeding between periods
art
oOverarching goal to suppress HIV replication; CD4 count serves as the major laboratory indicator of immune function
oReduce HIV-associated morbidity and prolong duration and quality of life
oRestore and preserve immunologic function
oMaximally and durably suppress plasma HIV viral load below the level of detection (HIV RNA less than 20-75 copies/mL)
oPrevent HIV transmission
gOaLS FOR ART
Achieving viral suppression requires at least 2 and preferably 3 active drugs from 2 or more classes (if viral loads are decreasing usually because pt isn't adhering to treatment plan
Goal for ART
*Adequate CD4 for pts on ART is an increase of 50-150 mm per year; viral load should be measured at baseline and regularly after because it's the best indicator of response to ART therapy
Adverse reactions for ART
hepatotoxicity, nephrotoxicity, osteopenia, increased risk of cardiovascular disease and MI, cause fat redistribution and metabolic alterations (dyslipidemia, insulin resistance), facial wasting, lipodystrophy= disturbed body image may be the reason they stop treatment
ART resistance
1- transmission of drug resistant HIV at time of initial infection 2- selective drug resistance in pts who are receiving non-suppressive regimens
EIA (enzyme immunoassay)
determines presence of antibodies to HIV
RT-PCR
measures viral load, used along with CD4 count, indicates level of immune dysfunction, assess stage and severity of HIV infection
Before ART what do you assess?
extent of damage to immune system
you want the viral load to ? CD4 count?
viral load decrease
cd4 count increase
Promoting Adherence to ART therapy
*Nonjudgmental providers
*Consider pts knowledge base, provide info about HIV, viral load, and CD4 count, the consequences of nonadherence to therapy
*Assess behavioral and psychosocial challenges
*Assess structural issues (housing, lack of income, lack of prescription drug coverage, lack of continuous access to meds)
*Mental health/ substance abuse
Identify reason for nonadherance
physical assessment pt 1
*Identification of potential risk factors: risky sexual practices or IV drug use
*Physical status
*Psychological status
*Immune system functioning
*Nutritional status: dietary hx, ability to purchase, prepare, and store food, wt, serum protein/albumin
physical assessment pt 2
*Skin Integrity: inspect skin/mucous membranes daily for breakdown, ulceration, infection; monitor oral cavity for redness, ulcerations, creamy-white patches= candidiasis
*Respiratory status: cough, sputum (amount/color), SOB
*Neurologic status: LOC, orientation, sensory/ motor deficits
*Fluid and electrolyte balance: skin turgor, dryness, increased thirst, decreased UOP, weak rapid pulse, urine specific gravity of 1.025 or more
preventing infection
monitor for S&S=fever, chills, night sweats, cough, SOB, dyspnea, oral pain/dysphagia, wt loss, swollen nodes, N/V/D, increased frequency urgency or pain with urination; culture would drainage, skin lesions, urine, stool, sputum, mouth, blood (monitor WBC, cultures, cook meat/eggs thoroughly)
NI for preventing infections
oClean kitchen/bathroom surfaces regularly with disinfectant; pets- allow someone else to clean areas soiled by animals such as birdcages and litter boxes if not possible wear gloves and wash hands after
oAvoid exposure to others who are sick or had recent vaccines esp live
oAvoid smoking, alcohol, street drugs
oIf HIV positive or injected drug user don't donate blood
oAvoid sharing drug equipment
Improving activity tolerance
monitor ability to perform ADLs, energy conservation techniques- sitting while washing/preparing meals, frequently used items remain within reach
Promoting skin integrity
*position changes Q2hrs, alternating pressure mattresses, avoid adhesive tape, linens free of wrinkles, avoid tight/restrictive clothing, foot lesions present encourage cotton socks/shoes that don't cause perspiration
Promoting usual bowel patterns
avoid bowel irritants such as fatty fried foods, nuts, raw fruits/ veggies, popcorn, carbonated beverages, spicy foods, food of extreme temp; suggest BRAT diet: banannas, rice, applesauce, tea, and toast; small frequent meals
Maintaining Coherent Though Process
is AMS speak in simple, clear language and give pt sufficient time to respond, reorient as needed
Improving airway clearance
pulmonary therapy- cough, deep breathing, postural drainage, percussion, and vibration every 2 hrs to prevent stasis of secretions; maintain semi-high fowlers position to facilitate breathing
relieving pain
NSAIDS, opioids w/ relaxation techniques
Improving nutritional status
wt, dietary intakes, albumin, BUN, Protein, antiemetic's, if oral lesions/sore throat can give viscous lidocaine (adm?) encourage food that easy to swallow, avoid spicy or sticky foods, enhance nutritional value of meals by adding eggs, butter, or fortified milk (powdered skim milk has been added to increase calories), gravies, soups, milkshakes- high calories foods such as pudding; electrolytes monitored (oranges and bananas to increase potassium; cheese and soups to increase sodium), 3L or more per day unless contraindicated
Decreasing sense of isolation
oMay have guilt because of lifestyle or they may have infected others/ May feel anger toward person who transmitted the virus to them
oProvide atmosphere of acceptance and understanding
oAssess baseline for social interaction so you can detect changes (decreased interaction, nonadherence)
oEncourage to express feelings, provide info for how to protect themselves/others, help them avoid isolation
oInform family/friends HIV isn't spread by casual contact
oCoping with Grief: verbalize feelings, identify resources for support-AIDS support groups, encourage to continue usual activities whenever possible