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Transcript
Management of Patients with
Immunodeficiency Disorders
and HIV/AIDS
By Linda Self
Immunodeficiency defined
Decreased or compromised abiity to
respond to antigenic stimuli by appropriate
cellular immunity reaction.
 May be secondary to loss of
immunoglobulins or an abnormality of B or
T cell lymphocytes

Immunodeficiency
Primary—inborn errors. Can affect
lymphocytes, phagocytes, complement
system
 Secondary—More common and may be
related to underlying diseases or the
treatment of these diseases.

Primary immunodeficiency
disorders
More than 100 identified
 Examples include: Immunoglobulin A
deficiency, thymic hypoplasia, severe
combined immunodeficiency disease,
granulomatoses

Causes of secondary
immunodeficiency
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Malnutrition
Burns
Uremia
Diabetes mellitus
Immunotoxic medications
Self-medication of recreational drugs and
alcohol
AIDS
Nursing Management for Patient
with Immunodeficiencies in general
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Assess for infection
Fever
White patches in oral cavity
Adenopathy
Persistent diarrhea
Frequency, urgency or pain upon urination
Redness, drainage or swelling of skin lesions
Persistent vaginal discharge
Cough with or w/o sputum
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Monitor lab values
Sputum
Urine
Blood cultures
CBC
Monitor vital signs, weight
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Assess nutritional status
?use of tobacco, alcohol or drugs
Personal hygiene practices
Dental hygiene
Teaching patient s/s of infection
Preventing Infection in the
hospitalized neutropenic patient
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Still w/o evidence-based consensus
Private room
Low microbial foods
Protective clothing
Skin asepsis
Ice chips to prevent mucositis
Avoid rectal introduction of meds, treatments
HIV/AIDS
Public awareness in 1981
 First antiretroviral drug in 1987
 PCP prevention started in 1988
 1995 protease inhibitors
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Epidemiology
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CDC surveillance/reportable illness
African-Americans account for 50% of cases in
200
Highest incidence in CA, NY, and FL
Kills more than 8000 cases per day worldwide
Now considered a disease of chronicity
Older adults at risk for HIV infection
Women are fastest growing group w/HIV
 Women have poorer outcomes
 Fatality rate is 60%
 Hits hardest between 25-44 years of age

Sexual Transmission
Virus is most concentrated in semen and
blood
 Most easily transmitted when infected
body fluids come into contact with mucous
membranes or non-intact skin
 Genital, anal or oral sexual contact with
exposure of mucous membranes to
infected semen or vaginal secretions

Viral Load
Higher the blood level of HIV, the greater
the risk for sexual and perinatal
transmission
 Effects of highly active antiretroviral
therapy have been instrumental in causing
the viral load to drastically drop in some
individuals

HIV Transmission
Not by casual contact
 By body fluids such as breast milk, semen,
vaginal secretions, amniotic fluid, and
blood

Pathophysiology
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1.
2.
3.
4.
5.
6.
7.
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Caused by an RNA virus or retrovirus
Replication goes as follows:
HIV GP 120 and GP41 to CD4 cell receptor
Viral core empties into cell
Reverse transcriptase copies RNA into double stranded DNA
Integration of viral DNA into host DNA w/aid of integrase
Using integrated DNA as blueprint, makes new viral proteins
Translation of viral messenger RNA to create polyprotein
Protease cleaves the strand
Assembly and release of new virus
Pathophysiology
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HIV attacks T cells
T Cells are lymphocytes
Have a special receptor on cell surface
Different subsets of T cells
CD4+ helper T Cells (release cytokines)
instrumental in initiating the immune response
both with B lymphocytes, macrophages and NK
cells
Pathophysiology cont.
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Cytotoxic T cells or CD8+ cells destroy virally infected
cells, tumor cells and induce transplant rejection
Memory T cells-persist long-term. Can be CD4 or CD8
cells
Regulatory T cells-suppressor T cells. Shut down T cell
mediated immunity at end of immune reaction
Natural killer cells bridge adaptive immune system with
innate immune system. Target damaged, infected or
dysfunctional cells.
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AIDS renders the immune function ineffective
Targets cells with the CD4 and CCR5
glycoprotein receptors which is primarily on T
lymphocytes, monocytes, dendritic cell
Mutation of CCR5 occurs in some Caucasians
allowing for greater posssible immunity
Stages of HIV Disease
Clinical Category A
 Includes one or more of the following in an
adult or adolescent and w/o conditions in
clinical categories B and C
 Asymptomatic HIV infection, persistent
generalized lymphadenopathy, acute HIV
infection with accompanying illness

Stages cont.
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Clinical Category B
Candidiasis, oropharyngeal or vulvovaginal (persistent
and poorly responsive)
Cervical dysplasia/cervical CA in situ
Fever or diarrhea exceeding one month
Hairy leukoplakia
Herpes zoster affecting more than one dermatome
ITP
PID
Peripheral neuropathy
Stages cont.
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Clinical Category C
Candida of esophagus, trachea +/or bronchi
Invasive cervical cancer
Cryptosporidiosis exceeding one month
CMV disease (other than liver, spleen or lymph)
HIV encephalopathy
CMV retinitis
Histoplasmosis
Kaposi’s sarcoma
MAC, MTB
Toxoplasmosis of brain
Wasting syndrome
Pneumcystis jiroveci
CD4+ T-cell Categories
A, B, C
1. greater than or equal to 500/uL
2. 200-499/uL
3. <200 u/L
People with AIDS indicator conditions (clinical
category C) and those in categories A3 or B3
are considered to have AIDS
Assessment and Diagnostic
Findings
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Health history
IV injection drug use
Receipt of blood transfusions
Exposure to body fluids
HIV antibody tests—EIA (enzyme
immunoassay), Western blot confirms EIA
OraSure saliva test and the OraQuick Rapid HIV
antibody test (approx. 20 minutes and is 99.6%
accurate)
Viral Load
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Measures plasma HIV RNA levels
Reverse transcriptase polymerase chain
reaction and nucleic acid sequence based
amplification
Both are target amplification methods to quantify
HIV RNA or DNA levels
Viral load is a better predictor of the risk for HIV
disease progression than is the CD4+count
Lower the load, longer the time to AIDS
Treatment
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CD4+ count is most important consideration in
starting HAART
Antiretroviral medications should be offered to
those w/T cell count less than 350 cells/mm3 or
plasma HIV RNA levels >100,000 copies/mL
Goals: sustain suppression of viral load,
preserve or restore immunologic function,
improve quality of life, reduce HIV morbidity and
mortality
Treatment
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Ongoing treatment response evaluation
Viral load levels before initiation of HAART and
again after 2-8 weeks
CD4+ count should increase by 100 to 150
cells/mm3 per year
Viral load should continue to decline over
several weeks and drop below detectable levels
<50 RNA copies/mL by 16-20 weeks
HAART
Mechanics of virus replication targeted
 Medications target reverse transcriptase,
integrase, protease and actual receptors
to slow disease progression
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Antiretroviral Agents
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Currently over twenty agents in more than four
classes
NRTIs—Abacavir (ABC), Videx (didanosine),
Epivir (lamivudine), Zerit (stavudine)
NNRTIs—Rescriptor (delavirdine), Viramune
(nevirapine)
Protease Inhibitors—Agenerase (amprenavir),
Norvir (ritonavir), Viracept (nelfinavir)
Fusion Inhibitors—Fuzeon (enfuvirtide)
Treatment
Always combination therapy
 Decrease pill burden
 Compliance is an issue
 All medications have adverse effects—
lipodystrophy, hyperlipidemia, heart
disease and diabetes, changes in body
image

Drug Resistance
Results from spontaneous genetic
mutation of the pathogens or in response
to exposure to the medication
 Contributing factors—monotherapy,
suboptimal treatment regimens,
nonadherence, late initiation of therapy
 Virologic failure will manifest first, then
immunologic failure

Drug Resistance
To better predict the likely susceptibility of
drug therapy, genotype testing
determines amino acid mutations
 Phenotype testing determines the drug
concentration needed to inhibit replication
of a recombinant virus by 50% of a
patient’s isolate
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Clinical manifestations
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May affect any organ
Can develop immune reconstitution
syndromes w/ MAC, MTB, PCP, toxoplasmosis,
hepatitis B &C, CMV, VZ, cryptococcal infection,
PML (progressive multifocal
leukoencephalopathy)
Is a paradoxical inflammatory reaction when
innate immunity improves after tx with HAART
Will have high fevers and worsening infection
symptoms temporarily
Respiratory Manifestations
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PCP—most common infection with AIDs.
Pneumocystis pneumonia
Now called Pneumocystis jiroveci
Without prevention, 80% of those with HIV will
develop PCP
Fever, chills, SOB, productive cough
Bronchoscopy
Treat with Bactrim which also confers cross
protection to toxoplasmosis
Mycobacterium Avium complex
Common opportunistic infection
 Group of acid-fast bacilli that includes M.
avium, M. intracellulare and M.
scrofulaceum
 Treat with Zithromax or Biaxin, possibly
Mycobutin.
 Duration of tx depends on CD4+counts
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Tuberculosis
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Occurs late in HIV infection
Absence of immune response to TB
test=Anergy
Infection may become disseminated
Multi-resistance occurring
Paradoxical reaction more commonly with this
infection. Seen as high fevers, worsening
pulmonary infiltrates, expanding CNS lesions
and worsening lymphadenopathy. Seen in those
with TB and HIV who are on anti-retrovirals.
Gastrointestinal Manifestations
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Loss of appetite
Nausea and vomiting
Oral and esophageal candidiasis
Chronic diarrhea
Pathogens include: Cryptosporidium muris, salmonella,
Giardia, Clostridium difficule and Isospora belli.
May have to treat with sandostatin if s/s not controlled
(decreases motility and secretion of water)
GI Manifestations
Oral candidiasis
 Wasting syndrome—weight loss of more
than 10% and diarrhea for more than one
month Anorexia, diarrhea, GI
malabsorption and lack of nutrition in
chronic disease are contributors
 Hypermetabolic state resulting in cachexia
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Oncologic Manifestations
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Results from altered immune response and possible
stimulation of developing cancer cells
Kaposi’s sarcoma—most common HIV related
malignancy
Is associated with Herpes virus 8
Skin signs manifest as CD4+ count declines
Appear brownish pink to deep purple, flat or raised and
surrounded by ecchymoses and edema
Involvement can occur of internal organs
Confirm by biopsy
Tx-surgical excision, radiation, alpha-interferon IV
Oncologic Manifestations
B cell lymphomas
 Second most common malignancy in AIDS
 Seen in younger individuals, develop most
commonly in brain, bone marrow, GI tract
 Aggressive, high grade and usually in multiple
sites
 Treatment less effective due to numerous other
co-existent problems
 Tx—radiation and chemotherapy
Neurologic Manifestations
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Include central, peripheral and autonomic
functions
HIV encephalopathy characterized by
progressive decline in cognitive, behavioral and
motor functions.
Direct result of HIV infection
Initiates release of toxins or lymphokines that
result in cellular dysfunction
HIV Encephalopathy cont.
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Signs and symptoms: fatigue, depression,
memory deficits, headache, confusion,
psychomotor slowing, apathy, and ataxia.
Later stages reveal vacant stare,spastic
paraparesis, hyperreflexia, hallucinations,
tremor, incontinence, seizures, mutism and
death.
Diagnosis—by CT, MRI, LP and brain biopsy
Neurologic Manifestations
Cryptococcal meningitis by C. neoformans.
 S/S– fever, headache, malaise, stiff neck,
nausea, vomiting, mental status changes, and
seizures.
 Dx—LP.
 Tx—IV amphotericin B w/or w/o Diflucan. May
need to give amphotericin intrathecally if not
responsive IV. Oral Diflucan for lifelong
suppressive therapy.
Neurologic Manifestations cont.
Progressive multifocal leukoencephalopathy—
a demyelinating CNS disorder that affects the
oligodendoglia. Caused by JC virus, a papoma
virus.
 S/S—blindness, aphasia, muscle weakness,
paresis and death.
 Tx—antiretroviral therapy, research being done
on interferon
Gynecologic manifestations
Ulcerative STDs—chancroid, syphilis,
herpes
 Genital warts
 Cervical intraepithelial neoplasia
 PID
 Menstrual irregularities
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Integumentary Manifestations
KS
 Herpes zoster
 Herpes simplex
 Molluscum contagiosum
 Generalized folliculitis
 Skin rash from Bactrim
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Endocrine Manifestations
Any endocrine organ can be affected
 S/S will present according to organ
involved
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Cytomegalovirus Retinitis
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Leading cause of blindness in patients with AIDS
Prophylaxis with oral ganciclovir may be
indicated in those with CD4+ counts less than 50
cells/mm3
Also can use Foscavir (foscarnet)
Must be taken for life
Adverse effect to ganciclovir is neutropenia
Must be cautious with co-administration of these
meds with antiretroviral drugs
CMV Retinitis
Nutrition Therapy
High protein, high calorie diet
 Enteral feedings
 Appetite stimulants such as Megace
(inhibits cytokine IL-1),Marinol
 Advera-special nutritional supplement
developed for those with HIV

Antidepressant therapy
Ritalin as a psychostimulant (but consider
effect on appetite and sleep)
 ECT when severe
 Prozac, Tofranil and Norpramin per text
(consider drug interactions)
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Nursing Diagnoses
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Diarrhea related to enteric pathogens or HIV infection
Risk for infection related to immunodeficiency
Ineffective airway clearance related to PCP, increased
secretions, decreased ability to cough
Imbalanced nutrition, less than body requirements
PC: opportunistic infections; impaired breathing; wasting
syndrome; fluid and electrolyte imbalances; adverse
reactions to medications
Social isolation related to stigma of disease, fear of
infecting others
Nursing Assessments
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Identification of risky sexual practices, drug use
(IV)
Physical assessment
Respiratory status
Nutritional status
Skin integrity
Neurologic status
Fluid and electrolyte balance
Knowledge level
Nursing Interventions
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Improving airway clearance
Preventing infections
Maintaining thought processes
Improving activity tolerance
Promoting skin integrity
Promoting usual bowel patterns
Relieving pain and discomfort
Improving nutritional status
Nursing Interventions
Decreasing sense of isolation
 Coping with grief
 Monitoring and managing potential
complications-respiratory failure, cachexia,
side effects of medications
 Teach self-care
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Evaluation
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Maintains effective airway
Maintains usual level of thought processes
Resumes usual bowel habits
Maintains skin integrity
Experiences no infections
Maintains adequate level of activity tolerance
Maintains adequate nutritional status
Progresses through grief process
Remains free of complications
Case Study

JO is a 42 year old male who resides with
his partner. JO was diagnosed several
months ago when he presented to the
doctor’s office with dysphagia, chronic
cough, diarrhea, weight loss, night sweats,
lymphadenopathy and fever, all lasting
well over a month.

What pathogen or pathogens might
account for his cough?
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What medication(s) might we use to treat
him?
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Why does he have diarrhea? What
causative pathogens could be causing
this?
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Why do patients with AIDS lose weight?
What medications might we give to
increase appetite?

Why might JO have a sore throat?

JO subsequently developed visual
blurring. What pathogen might be
causative? How would we treat this?

What lab tests may have been used to
arrive at his diagnosis of AIDS? What
category would he be, given his
conditions?

What treatments might we institute to slow
the progression of his illness?

JO was started on didanosine, nevira and norvir.
Thereafter, JO’s symptoms markedly improve.
His CD4 count improved from 200 to 500.
However, after being on this combination
therapy for several months, JO developed
lipoatrophy of his face and legs and his
triglycerides were 6000. Which drug warrants
discontinuation?

JO is now on Trizir and is doing
satisfactorily.