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MODULE 18 PPT.pdf
1. PRE-FINALS MODULE NO. 18
NURSING CARE OF A FAMILY WHEN A CHILD HAS AN
IMMUNE SYSTEM DISORDER
JASON ADOYOGAN, RN
NCM 109 LECTURER
2. MODULE OBJECTIVES
1.Discuss briefly the normal anatomy and
physiology associated with the Immune system.
2.Explain how alterations in the normal anatomy
and physiology of a child’s immune system leads
to the development of certain disorders.
3.Enumerate the most common and significant
disorders of the immune system
3. MODULE OBJECTIVES CONT..
4.Discuss how to assess the health of children
with immune system disorders.
5.Formulate nursing diagnoses for a child with a
disorder of the immune system.
6.Establish Nursing care outcomes and plan of
care for children with specific disorders of the
immune system.
4. LECTURE
CONTENTS
A. Brief review of the Immune system.
B. Common immune system disorders – Brief overview, assessment,
diagnostics, treatment (medical, nursing), nursing considerations
1) Immunodeficiency disorders
i. Primary immunodeficiency disorders
ii. Secondary (Acquired) immunodeficiency disorders
2) Allergy/ Hypersensitivity Disorders
i. 4 types of hypersensitivity reactions
ii. Anaphylactic shock
iii.Urticaria and Angioedema
iv.Atopic disorders
v. Drug and food allergies
vi.Stinging insect Hypersensitivity
vii.Contact Dermatitis
6. BREIF REVIEW OFTHE
IMMUNE SYSTEM
Immune System –
A system of tissues,
organs, and cells that
protects the body against
pathogenic organisms and
other foreign bodies.
8.  The immune system protects the body initially by
creating local barriers and inflammation. The local
barriers provide chemical and mechanical defenses
through the skin, the mucous membranes, and the
conjunctiva. Inflammation draws polymorphonuclear
leukocytes and neutrophils to the site of injury, where
these phagocytes engulf the invading pathogens. If these
first-line defenses fail or are inadequate to protect the
body, the humoral immune response and the cell-
mediated immune response are activated.
9. DEFINITION OFTERMS
Antigen – foreign
substances. Antigens
include pathogens
(bacteria, fungi, or
viruses), food proteins,
and pollens.
10. DEFINITION OFTERMS
 Antibody – An antibody,
also known as an
immunoglobulin, is a large, Y-
shaped protein used by the
immune system to identify
and neutralize foreign objects
such as pathogenic bacteria
and viruses.
11. DEFINITION OFTERMS
 Immunity - the quality of being insusceptible to or
unaffected by a particular disease or condition.
(Mosby’s medical dictionary). It is the ability to
destroy antigens (Pilitteri, et al)
12. DEFINITION OFTERMS
 Immunogen – It refers to an antigen capable of
stimulating the body to illicit immunity/immune response.
 Allergen - If, during the immune response, mediating
substances are released that cause tissue injury and
allergic symptoms, the antigen is termed an allergen.
13. DEFINITION OFTERMS
B-Lymphocytes - B Cells originate in
the bone marrow where they develop
from plasma or memory cells. Primary
purpose is to produce immunoglobulins.
14. DEFINITION OFTERMS
 T lymphocytes (also called T cells) are major
components of the adaptive immune system. Their
roles include directly killing infected host cells,
activating other immune cells, producing cytokines
and regulating the immune response.
 Account for 70-80% of blood lymphocytes and are
responsible for cell mediated immunity.
15. T LYMPHOCYTE TYPES
1. Cytotoxic (killer) T-cells
- Have a specific ability to bind to the surface of antigens
and directly destroy the cell membrane and, therefore, the
cell.
2. Helper T (CD4) Cells
- Stimulates B lymphocytes to divide and mature into
plasma cells so the B cells can begin secreting
immunoglobulins.
3. Suppressor T-cells
- are specific cells that reduce the production of
immunoglobulins against a specific antigen and prevent
their overproduction.
19. DEFINITION OFTERMS
Autoimmunity - is the result of the immune
system being unable to distinguish self from
non-self, causing the immune system to trigger
immune responses against normal cells and
tissue rather than invading antigens.
21. HUMORAL IMMUNITY VS
CELL-MEDIATED IMMUNITY
 Humoral immunity refers
to immunity created by
antibody production or B
lymphocyte involvement
whereas Cell-mediated
immunity is the type of
immune response caused
by T-lymphocyte activity
22. NATURAL IMMUNITY VS ARTIFICIAL IMMUNITY
Natural immunity occurs through contact
with a disease-causing agent, when the
contact was not deliberate, whereas
artificial immunity develops only through
deliberate actions of exposure.
23. ACTIVE VS PASSIVE IMMUNITY
 Active immunity refers to a form of long-term, acquired
immunity. It is a result of antibodies that develop naturally
after an initial infection or artificially after a
vaccination whereas passive immunity is a form of
acquired immunity resulting from antibodies that are
transmitted naturally through the placenta to a fetus,
through the colostrum to an infant, or artificially by
injection of antiserum for treatment or prophylaxis.
Passive immunity is not permanent and does not last as
long as active immunity
25. IMMUNODEFICIENCY DISORDERS
 When any portion of the immune system is missing
or not functioning properly, an immunodeficiency
can result. An immunodeficiency can be primary
(congenital) or acquired (secondary to a viral
infection, exposure to a toxic substance or some
drugs). If a child is suspected to have a humoral
immunodeficiency, live viral vaccines are
contraindicated, and all blood products should be
irradiated
26. PRIMARY IMMUNODEFICIENCY DISORDERS
Comprise over 150 inherited disorders.
They can present and be diagnosed at various
ages and affect all parts of the immune
system, including humoral defects, cell-
mediated defects, complement deficiencies,
and phagocyte disorders
27. Humoral Deficiency
Children with humoral defects are generally
well until 4 to 6 months of age because of the
presence of maternal antibody which crosses
the placenta. As this antibody wanes and the
child fails to produce his or her own antibody,
infections can begin to present.
28. X-LINKED AGAMMAGLOBULINEMIA &
COMMON VARIABLE IMMUNODEFICIENCY
• In X-linked agammaglobulinemia, male children
lack the enzyme necessary for B cells to mature;
• the B cells are incapable of maturing into antibody,
and patients are susceptible to infection from a
wide variety of pathogens.
• In patients with CVID, the levels of
immunoglobulins are low but usually not absent,
and antibody development is impaired.
29. X-LINKED AGAMMAGLOBULINEMIA &
COMMON VARIABLE IMMUNODEFICIENCY
• The cellular or T-lymphocyte response
remains adequate in CVID, protecting the
child from some pathogens.
• There is also an association with a higher
prevalence of autoimmune diseases and
lymphoreticular cancers in children with CVID.
30. DIAGNOSTICS
• Low immunoglobulin levels and absent B cells
• Genetic testing
Diagnosis of X-linked agammaglobulinemia is by
detecting low (at least 2 standard deviations below
the mean) levels of immunoglobulins (IgG, IgA, IgM)
and absent B cells (< 1% of all lymphocytes are
CD19+ cells, detected by flow cytometry). Transient
neutropenia may also be present.
31. MANAGEMENT
• Regular infusion of pooled human immunoglobulin.
• Immunoglobulin can be delivered intravenously
every 3 to 4 weeks or subcutaneously every 1 to 2
weeks.
• Health education - importance of early recognition
of signs and symptoms of infection.
• May need longer courses of antibiotic treatment
than immunocompetent children with the same
infection.
32.
33. B. SELECTIVE IGA DEFICIENCY
• The most common of the humoral
immunodeficiencies.
• May result from an increase in IgA suppressor
cells or defect in helper T-cells necessary for
IgA production.
• Also associated with atopic diseases
(allergies)
34. DIAGNOSTIC
• The clinical definition for this disorder is an
undetectable level of IgA, not merely a low level.
• The disorder can occur in as frequently as 1:500
people.
• Most people who lack IgA are completely
asymptomatic, and the deficiency is noted when the
person is being evaluated for another illness, such
as celiac disease.
35. MANAGEMENT
• There is no treatment specific for IgA
deficiency because there is no way to provide
IgA to the body.
• Protect the child from opportunistic infections.
• Health teaching on recognizing early signs of
infections.
36. C. T-LYMPHOCYTE DEFICIENCIES
• Involve inadequate numbers or inadequate
functioning of one or more types of T lymphocytes.
• Affects cell-mediated immunity and, because of
helper T-lymphocyte function, possibly humoral
immunity as well.
• An example is DiGeorge syndrome - a
chromosomal deletion syndrome with tremendous
phenotypic variability.
38. D. COMBINED T- AND
B-LYMPHOCYTE DEFICIENCY
• Severe combined immunodeficiencies (SCID) are
a group of inherited rare disorders associated with
large defects in T- and B-cell immunity.
• Children cannot respond directly to antigen
invasion, and no antibodies are produced.
 SCID is 100% fatal if untreated, and early
recognition is key to early treatment and survival
40. TREATMENT
The definitive treatment for children with
SCID is correction of the immunologic
defect by hematopoietic stem cell
transplantation, possibly from cord
blood.
42. SECONDARY (ACQUIRED) IMMUNODEFICIENCY DISORDERS
 Secondary immunodeficiency, or loss of immune system
response, can occur from factors such as:
• severe systemic infection
• cancer
• radiation therapy
• severe stress
• Malnutrition
• monoclonal antibody
therapy targeted at B cells
• other immunosuppressive
therapy and
• aging.
43. Anything that causes the body to lose
protein such as renal disease or protein-
losing enteropathies may also cause a
secondary immunodeficiency. There can
be complete or partial loss of both B- and
T-lymphocyte response.
44. 1. HIV INFECTION AND AIDS
HIV is a slowly replicating retrovirus and has at least two
main divisions, HIV-1 and HIV-2, followed by a variety of
further subtypes.
It is found in the blood, semen, vaginal secretions, and
breast milk. It has an incubation or latency period of
months to years. There are different strains of HIV.
Whereas HIV-2 is prevalent in Africa, HIV-1 is the dominant
strain in the United States and elsewhere.
45. TRANSMISSION
 Horizontal transmission
• occurs through intimate sexual contact or parenteral exposure to
blood or body fluids containing visible blood. (M. Hockenberry, et
al., 2017)
 Perinatal (vertical) transmission
• occurs when an HIV-infected pregnant woman passes the
infection to her infant. There is no evidence that casual contact
between infected and uninfected individuals can spread the virus.
(M. Hockenberry, et al., 2017)
46. PATHOPHYSIOLOGY
• The HIV virus primarily infects a specific subset of T
lymphocytes, the CD4+ T cells, but it can also
invade cells of the monocyte-macrophage lineage.
The virus takes over the machinery of the CD4+
lymphocyte, using it to replicate itself, rendering the
CD4+ cell dysfunctional. The CD4+ lymphocyte
count gradually decreases over time, at some point,
physical symptoms appear. (M. Hockenberry, et al.,
2017)
47. PATHOPHYSIOLOGY CONT.
• There is no effective way to destroy the HIV,
so it remains in the body for life and can
activate if the immune system becomes
depressed. When monocytes and
macrophages become affected, the person
with HIV infection cannot resist usual
infections such as the common cold.
48. PATHOPHYSIOLOGY CONT.
• When the CD4 count falls below 500 cells/mm3 or
the viral load rises above 5,000 copies/ml, it is
difficult for infected individuals to resist
opportunistic infections such as fungal infections.
The final result is that both the immune response
and the ability to screen and remove malignant
cells from the body are lost (Smith, 2011). (Silbert-
Flagg & Pillitteri, 2018)
49. SIGNS AND SYMPTOMS
 Some people have flu-like symptoms within 2 to 4 weeks
after infection (called acute HIV infection). These symptoms
may last for a few days or several weeks. Possible
symptoms include
• Fever,
• Chills,
• Rash,
• Night sweats,
• Muscle aches,
• Sore throat,
• Fatigue,
• Swollen lymph nodes, and
• Mouth ulcers.
50.
51. CLINICAL MANIFESTATIONS
Common Clinical Manifestations of HIV Infection
in Children (M. Hockenberry, et al., 2017):
• Lymphadenopathy
• Hepatosplenomegaly
• Oral candidiasis
• Chronic or recurrent
diarrhea
• Failure to thrive
• Developmental delay
• Parotitis
52. Common Defining Conditions for Acquired Immune Deficiency
Syndrome in Children (M. Hockenberry, et al., 2017):
• Pneumocystis carinii pneumonia
(PCP)
• Lymphoid interstitial pneumonitis
(LIP)
• Recurrent bacterial infections
• Wasting syndrome
• Candidal esophagitis
• Human immunodeficiency virus
(HIV) encephalopathy
• Cytomegalovirus disease
• Mycobacterium avium-
intracellulare complex infection
• pulmonary candidiasis
• Herpes simplex disease
• Cryptosporidiosis
53. DIAGNOSTICS
Tests to detect the antigen are termed
polymerase chain reaction (PCR) tests.
Those for the antibody are termed
enzyme-linked immunosorbent assay
(ELISA) or Western blot confirmation.
57. MANAGEMENT (MAIN GOALS)
1.Prevention – For those people at risk for
developing/acquiring the disease.And also for infected
person from spreading it.
2.ReduceViral Load to an undetectable level.
3.Prevent opportunistic Infections.
4.Improvement of health/quality of life.
58. NURSING MANAGEMENT
• Health Education concerning transmission and
control of infectious diseases, including HIV
infection, is essential for children with HIV
infection and anyone involved in their care.
• Maintain strict personal hygiene practices.
• Cater to the child’s developmental needs.
59. NURSING MANAGEMENT CONT.
• Encourage adolescents at risk to undergo HIV
testing and counselling.
• The multiple complications associated with
HIV disease are potentially painful (Ezekowitz,
2009). Ongoing assessment of pain is crucial
and is most easily accomplished in older
children who are able to communicate.
61. PRECAUTIONS CONT.
• Disposable gloves must be worn when there is a
direct contact or possibility of contact with blood,
body fluids, mucous membrane and non-intact skin of
all patients. Gloves should preferably be changed after
patient contact and before administering care to
another patient. Gloves must be changed whenever
they are torn and when a needle-stick or other injury
occurs and when they are visibly dirty with blood
62. PRECAUTIONS CONT.
•Mask, eye protection or face shield,
and gown must be worn as
appropriate during procedures and
patient care activity that may result in
splashing of blood and body fluids.
63. PRECAUTIONS
•Proper handling of sharps.
Precautions should be taken to
prevent injuries caused by needles,
scalpels and other sharp instruments
64. MEDICAL MANAGEMENT
• Four classes of drugs are the mainstay of therapy:
1. nucleoside reverse transcriptase inhibitors
(NRTIs)
â–Şdesigned to block the production of viral DNA,
limiting the ability of the virus to infect cells.
â–ŞEx.: Zidovudine
65. MEDICAL MANAGEMENT
2.Non-nucleoside reverse transcriptase
inhibitors (NNRTIs)
â–Şinhibit the DNA synthesis of viruses but act
at different sites on the viral enzyme;
â–ŞEx.: nevirapine and efavirenz.
66. MEDICAL MANAGEMENT
3.Protease inhibitors (PIs),
â–ŞProtease inhibitor drugs block the action of
protease enzymes. This prevents protease
enzymes from doing their part in allowing HIV
to multiply, interrupting the HIV life cycle as a
result. This can stop the virus from
multiplying.
â–ŞEx. ritonavir (Norvir), lopinavir/ritonavir
(Kaletra)
67. MEDICAL MANAGEMENT
4.integrase strand transfer inhibitors
(ISTIs).
 The INSTI mechanism of action is to prevent HIV
integrase from incorporating proviral DNA into the
human host cell, thus inhibiting the HIV-catalyzed
strand transfer step.
 Ex. raltegravir (Isentress), dolutegravir (Tivicay)
68. MEDICAL MANAGEMENT
• Children are prescribed a regimen involving
multiple drugs, such as one PI plus two NRTIs.
• In addition, many children are given prophylactic
therapy for PCP (such as trimethoprim-
sulfamethoxazole [TMP-SMZ]) beginning at 6
months of age.
69. MEDICAL MANAGEMENT
• If a child develops tuberculosis, a
combination of antituberculosis drugs, such
as isoniazid or rifampin, is used.
• In addition to routine Immunizations,
children with HIV should also receive
Pneumococcal and HPV vaccines.
Attenuated vaccines should be used with
caution.
70. ALLERGY/HYPERSENSITIVITY DISORDERS
Allergic diseases occur as a result of
abnormal antigen–antibody responses.
Symptoms can be chronic and minor, such as
those that occur with seasonal rhinitis, or
acute and severe, as in an anaphylactic
reaction.
71. 4 TYPES OF HYPERSNSITIVITY REACTIONS
Type I: Immediate
 Involved Cell: IgE
 Mechanism: IgE attached to surface of mast cell triggers release of
intracellular granules from mast cells on contact with antigens.
 Effect: Allergies, asthma, atopic dermatitis, anaphylaxis
Type II: Cytotoxic
 Involved Cell: IgG or IgM
 Mechanism: Antigen–antibody reaction leading to antigen destruction;
complement is activated
 Effect: Hemolytic anemia, transfusion reaction, erythroblastosis fetalis
72. 4 TYPES OF HYPERSNSITIVITY REACTIONS
Type III: Immune Complex Disease
 Involved Cell: IgG or IgE
 Mechanism: Antigen–antibody complexes precipitate; complement is
activated, leading to inflammatory response.
 Effect: Rheumatoid arthritis, systemic lupus erythematosus
Type IV: Delayed
 Involved Cell: T-lymphocyte
 Mechanism: T cells combine with antigen to induce inflammatory
reactions by direct cell involvement or the release of lymphokines.
 Effect: Contact dermatitis, transplant graft reaction
73. ASSESSMENT
• History taking. A family history is
important because there seem to be
familial tendencies with allergic
diseases.
• Common signs and symptoms;
o Rhinitis
o Urticaria (swelling and itching)
o Rash
o Dennie Line
o Allergic shiners
74. ANAPHYLACTIC SHOCK
• Anaphylactic shock is an immediate,
life-threatening, type I hypersensitivity
reaction that occurs after exposure to
an allergen in a previously sensitized
child.
76. SYMPTOMS
 low blood pressure, shock, loss of consciousness
 • Skin: hives, swelling, itch, warmth, redness, rash
 • Stomach: nausea, pain/cramps, vomiting, diarrhea
 • Other: anxiety, feeling of impending doom,
itchy/red/watery eyes, headache,
 cramping of the uterus
77. MANAGEMENT
•Main goal is to reduce child’s
exposure to the allergen and
modify the child’s response to
the allergen.
78. MANAGEMENT
•Main goal is to reduce child’s
exposure to the allergen and
modify the child’s response to
the allergen.
79. NURSING MANAGEMENT
• Environmental control – involves limiting exposure to
allergens in a child’s environment.
- Avoid offending foods, medicines, insects etc.
• Health education to patient and family members on
prevention, the recognition of the signs and symptoms
as well as the steps to take when the said condition
occurs.
• If indicated, explain the importance of the need to carry
an epinephrine self-injector for children with history of
anaphylaxis.
80. MEDICAL MANAGEMENT
• Immunotherapy/Hypo sensitization – produce a state of
increased clinical tolerance or “sustained
unresponsiveness”
- is done when the child’s allergy symptoms cannot be
controlled by avoidance of an allergen or
conventional drug therapy.
 Epinephrine, injected intramuscularly, is the standard of
care for the treatment of anaphylaxis regardless of the
cause (Muraro, Lemanske, Castells, et al., 2017).
81. URTICARIA AND ANGIOEDEMA
URTICARIA
- or hives, refers to macular wheals
surrounded by erythema arising from the
chorion layer of skin; they are intensely
pruritic (often described as having a burning
sensation).
82. URTICARIA AND ANGIOEDEMA
ANGIOEDEMA
- edema of the skin and subcutaneous tissue.
This occurs most frequently on the eyelids,
hands, feet, genitalia, and lips—areas where
skin is loosely bound by subcutaneous tissue.
83. TREATMENT
- Immediate:
•Administration of intramuscular epinephrine
•Oral anti-histamines
- Long-term:
•Corticosteroids
•Cyclosporine (an immunosuppressant)
•Omalizumab (Xolair) (monoclonal antibody) –
usually reserved for older adolescents or
adults.
84. ALLERGIC RHINITIS
- associated with an IgE-mediated
inflammatory response to allergen
exposure. It is a risk factor for the
development of asthma.
85. ASSESSMENT
- associated with an IgE-mediated
inflammatory response to allergen
exposure. It is a risk factor for the
development of asthma.
86. ASSESSMENT CONT.
• Common symptoms of allergic rhinitis
include;
oCongestion
oSneezing
oNasal engorgement
oprofuse watery nasal discharge
87. ASSESSMENT CONT.
• Common symptoms of
allergic rhinitis include;
oCongestion
oSneezing
oNasal engorgement
oprofuse watery nasal
discharge
88. TREATMENT/MANAGEMENT
• Avoidance of offending allergen
• Use of pharmacologic agents:
oAntihistamines
oLeukotriene inhibitors
oCorticosteroids
• Immunotherapy
89. PERENNIAL ALLERGIC RHINITIS
• Allergic rhinitis becomes perennial (year-round)
when the allergen is one that is present in the
environment year-round, such as house dust
mites or pet hair.
• In addition, serous otitis media can accompany
the disorder as a long-term consequence.
91. ATOPIC DERMATITIS
• Atopic dermatitis is a highly pruritic, chronic
inflammatory skin disease that is often the first
manifestation of allergic disease.
• Many children with this disease will often
develop allergic rhinitis and asthma.
• A complex inflammatory process that involves
an epidermal barrier defect.
92. TREATMENT
• Treatment is primarily aimed at reducing the
amount of allergen exposure, if such allergens can
be identified. Second major consideration in
treatment is aimed at reducing pruritus so children
do not irritate lesions and cause secondary
infections by scratching.
• Use of elimination diets to identify food allergens.
93. TREATMENT
• Regardless of the mode of hydration, the skin
should still be wet or moist when applying
lubricants.
• Antihistamines – to reduce itching.
• Use of steroids
oLow -potency steroids can be used for
maintenance.
oHigh-dose topical steroids can be used
intermittently for exacerbations.
94. TREATMENT
• Topical calcineurin inhibitors tacrolimus and
pimecrolimus are also useful in atopic
 dermatitis management.
• Relapsing atopic dermatitis and severe refractory
cases of atopic dermatitis may require long-term,
anti-inflammatory therapy with intermittent use of
systemic anti-inflammatory or immunosuppressive
treatment.
95. DRUG ALLERGIES
• A drug allergy is the abnormal reaction of
your immune system to a medication. Any
medication — over-the-counter,
prescription or herbal — is capable of
inducing a drug allergy. However, a drug
allergy is more likely with certain
medications.
98. TREATMENT
• Discontinue using the drug and notify
prescriber.
• Administration of antihistamine, ex.
Diphenhydramine.
• In case of anaphylaxis, epinephrine
injection can be given.
99. FOOD ALLERGIES
• Are an abnormal immune response
caused by exposure to a particular food
protein. They can be IgE-mediated, cell-
mediated, or mixed reactions, although
IgE-mediated (type I hypersensitivity)
reactions account for most food reactions
(Robison & Pongracic, 2012).
101. TREATMENT
• Eliminate offending foods from the diet.
(Hypoallergenic diet)
• Health education: Urge parents to become
conscientious shoppers and read labels
carefully to be certain the foods they are
buying do not contain products to which their
child is sensitive.
102. STINGING INSECT HYPERSENSITIVITY
Children may have severe hypersensitivity
reactions to stings from bees, wasps, hornets,
or yellow jackets (Yavuz, Sahiner,
Buyuktiryaki, et al., 2013). Although a serum
sickness reaction may occur, the usual
reaction to these stings is an immediate type I
hypersensitivity reaction (anaphylaxis).
103. TREATMENT
• Hyposensitization by immunotherapy
• Acute anaphylaxis – administration of epinephrine
injection.
o Health education: importance of learning self-
administration of epinephrine.
• Avoid stinging insects;
o Not using scented preparations
o Not going barefoot
o Use of insect repellant
104. CONTACT DERMATITIS
• Contact dermatitis is an example of a
delayed or type IV hypersensitivity
response; it is a reaction to skin
contact with an allergen (a substance
irritating to the child only with prior
sensitization).
106. DIAGNOSTIC
oPatch testing – to identify allergens
causing contact dermatitis.
â–ŞCorticosteroids should be temporarily
discontinued as it may alter result.
107. TREATMENT
• Removing identified allergen.
• Apply dressing moistened with water
saline or Burow’s solution.
• Application of hydrocortisone lotion
• Baths with baking soda mixture in water.