Care of Mother & Child at Risk (NCMA 219)

FINALS WEEK 13 Mr. Neeco Andreus M. Martin SN 2 – Y2 – T6B

ALTERATIONS WITH INFECTIONS, INFLAMMATORY AND IMMUNOLOGIC RESPONSES/CELLULAR ABERRATIONS

OVERVIEW
- Decreased appetite, poor weight gain, and
I. ALTERATIONS WITH INFECTIOUS, slow growth.
INFLAMMATORY AND IMMUNOLOGIC CLASSIFICATIONS OF JUVENILE IDIOPATHIC
RESPONSE ARTHRITIS:
1. Juvenile Rheumatoid Arthritis
2. Allergic Rhinitis
3. Asthma
II. CELLULAR ABERRATIONS
• BASIC CONCEPTS ON ONCOLOGY
1. Leukemia
2. Lymphomas
3. Willms Tumor
4. Brain Tumors
• BASIC CONCEPTS ON CANCER
MANAGEMENT AND NURSING CARE
JUVENILE RHEUMATOID ARTHRITIS (JUVENILE
IDIOPATHIC ARTHRITIS

- Juvenile idiopathic arthritis (JIA) is the name

replacing JUVENILE RHEUMATOID ARTHRITIS
(JRA) in the research literature and now in
clinical practice.
- JIA is a chronic autoimmune inflammatory
disease causing inflammation of joints and
other tissue with an unknown cause.
- JIA starts before age 16 years with a peak
onset between 1 and 3 years of age.
- Twice as many girls as boys are affected.
- The reported incidence of chronic childhood
arthritis varies from 1 to 20 cases per 100,000
children with a prevalence of 10 to 400 per
100,000 (Cassidy and Petty, 2011).
CLINICAL MANIFESTATIONS
- The outocome of JIA is variable and
unpredictable
- The disease, even in severe forms, is rarely life
threatening but can cause significant
disability.
- Chronic and acute uveitis can cause
permanent vision loss if undiagnosed and
not aggressively treated
SIGNS AND SYMPTOMS
- Swollen，sun. and painful ioints in the knees
hands, feet, ankles, shoulders, elbows, or
other joints, often in the morning or after a
nap.
- Eye inflammation.
- Warmth and redness in a joint.
- Less ability to use one or more joints.
- Fatigue.
1. SYSTEMIC ARTHRITIS
- is arthritis in one or more joints associated
with at least 2 weeks of quotiidian fever, rash,
lymphadenopathy, hepatosplenomegaly,
and serositis
2. OLIGOARTHRITIS
- Is arthritis in one to four joints for the first 6
months of disease
- It is subdivided to persistent oligoarthritis
if it remains in four joints or fewer or
becomes extended oligoarthritis if it
involves more than four joints after 6 months
3. POLYARTHRITIS RHEUMATOID FACTOR
NEGATIVE
- affects five or more joints in the first 6
months with a negative rheumatoid factor
4. POLYARTHRITIS RHEUMATOID FACTORS
POSITIVE
- Also affects five or more joints in first 6
months, but these children have a positive
rheumatoid factor
5. PSORIATIC ARTHRITIS
- Is arthritis with psoriasis or an associated
dactylitis, nail pitting, or onycholysis or
psoriasis in a first – degree relative
6. ENTHESITIS – RELATED ARTHRITIS
- Is arthritis or enthesitis associated with at
least two of the following:
o Sacroiliac or lumbosacral pain, HLA –
B27 antigen, arthritis in a boy older
than y6 years, acute anterior uveitis,
inflammatory bowel disease, reiter
syndrome, or acute anterior uveitis in
a first – degree relative
7. UNDIFFERENTIATED ARTHRITIS
- Fits no other category above or fits more
than one category
Care of Mother & Child at Risk (NCMA 219)
FINALS WEEK 13 Mr. Neeco Andreus M. Martin SN 2 – Y2 – T6B
DIAGNOSTIC EVALUATION
- Juvenile idiopathic arthritis is a diagnosis of
exclusion; there are no definitive tests.
- Classifications are based on the clinical
criteria of age of onset before age 16 years,
arthritis in one or more joints for 6 weeks or
longer, and exclusion of other causes.
- Laboratory tests may provide supporting
evidence of disease.
- The ESR may or may not be elevated.
- Leukkocytosis is frequently present during
exacerbations of systemic JIA
- Plain radiographs are the best initial imaging
studies and may show soft – tissue swelling
and joint space widening from increased
synovial fluid in the joint.
- Later films can reveal osteoporosis, narrow
loint space, erosions, subluxation, and
ankylosis.
THERAPEUTIC MANAGEMENT
- There is no cure for JIA.
- The major goals of therapy are to control
pain, preserve joint range of motion and
function, minimize effects of inflammation
such as joint deformity, and promote normal
growth and development.
- Outpatient care is the mainstay of therapy;
lengthy hospitalizations are infrequent in this
era of managed care.
- The treatment plan can be exhaustive and
intrusive for the child and family, including
medications, physical and occupational
therapy, comfort measures, dietary
management, School modifications, and
psychosocial support.
MEDICATIONS
NONSTEROIDAL ANTIFLAMMATORY DRUGS
(NSAIDS) are the first drugs used NAPROXEN,
IBUPROFEN, TOLMETIN, INDOMETHACIN,
CELECOXIB, MELOXICAM, AND ASPIRIN are
approved for use in children. NSAIDS must be taken
with food.
- Aspirin, once the drug of choice, has been
replaced by other NSAIDs because they have
fewer side effects and easier administraion
schedules
METHOTREXATE is the second – line medication
used in children who have failed with NSAIDs
alone. It is started in combination with an NSAID.
It is effectivem with acceptable toxicity, which
requires monitoring of complete blood cellc
counts and liver functions.
- Patient educations about possible side effects,
including discussion with teens about birth
defects and avoiding alcohol is essential.
- Prolonged use of systemic steroids is
associated with significant side effects,
including cushing syndrome, osteoporosis,
increased infection risk, glucose intolerance,
cataracts, and growth suppression
BIOLOGIC AGENTS that work by several mechanism
to interrupt and minimize the inflammatory process
are used in children with severe or progressive
arthritis.
- Biologic agents may be used in combination
with methotrexate. The food and Drug
Administration (FDA) has approved
etanercept, adalimumab and abatacept use in
children with JIA
NURSING CARE:
1.) Relieve Pain
- The aim is to provide as musch relief as
possible with medication and other therapies
to help children tolerate the pain and cope as
effectively as possible
- Non – pharmacologic modalities such as
behavioral therapy and relaxation techniques
have proved effective in modifying pain
perception and activities that aggravate pain
- Opioid analgesics are typically avoided in
juvenile arthritis; however, for children
immobilized with refractory pain, short –
term opiod analgesics can be part of a
comprehensive plan that uses multiple pain
relief techniques (Connelly and Schanberg,
2006)
2.) Promote General Health.
- A well – balanced diet with sufficient calories
to maintain growth is essential
- If the child is relatively inactive, caloric intake
needs to match energy needs to avoid
excessive weight gain, which places
additional stress on affected joints
3.) Encourage Heat and Exercise
- Heat has been shown to be beneficial to
children with arthritis
- Moist heat is best for relieving pain and
stiffness, and the most efficient and practical
method is the bathtub with warm water
ALLERGIC RHINITIS
- Allergic rhinitis is inflammation of the inside
of the nose caused by an allergen, such as
pollen, dust, mold or flakes of skin from
certain animals.
- Allergic rhinitis is caused by the immune
system reacting to an allergen as it it were
harmful.
- This results in cells releasing a number of
chemicals that cause the inside layer of your
nose (the mucous membrane) to become
swollen and too much mucus to be
produced.
- Common allergens that cause allergic rhinitis
include pollen (this type of allergic rhinitis is
known as hay fever), as well as mold spores,
house dust mites, and flakes of skin or
droplets of urine or saliva from certain
animals.
Care of Mother & Child at Risk (NCMA 219)
FINALS WEEK 13 Mr. Neeco Andreus M. Martin SN 2 – Y2 – T6B
CLINICAL MANIFESTATIONS
- Allergic rhinitis typically causes cold-like
symptoms, such as sneezing, itchiness and a
blocked or runny nose.
- These symptoms usually start soon after
being exposed to an allergen.
- Some people only get allergic rhinitis for a
few months at a time because they're
sensitive to seasonal allergens, such as tree
or grass pollen.
SIGNS AND SYMPTOMS
- COUGH, ITCHY NOSE, RUNNING NOSE,
SNEEZING, REDNESS, ITCHING, WHEEZING,
SHORTNESS OF BREATH, EDEMA,
LACRIMATION, VOMITING, LOOSE MOTIONS
TREATMENT/MANAGEMENT
It's difficult to completely avoid potential
allergens, but you can take steps to reduce
exposure to a particular allergen you know or
suspect is triggering your allergic rhinitis.
- over-the-counter medications, such as non-
sedating antihistamines
- by regularly rinsing the nasal passages with a
salt water solution to keep the nose free of
irritants.
- VA stronger medication, such as a nasal spray
containing corticosteroids may be
prescribed.
ATOPIC DERMATITIS (ECZEMA)
- Eczema or eczematous inflammation of the
skin refers to a descriptive category of
dermatologic diseases and not to a specific
etiology.
- AD is a type of pruritic eczema that usually
begins during infancy and is associated with
an allergic contact dermatitis will a nereakary
tendency latopy)
AD manifests in three forms based on the child's
age and the distribution of lesions:
1.) Infantile (infantile eczema)
- Usually begins at 2 to 6 months of age;
generally undergoes spontaneous remission
by 3 years of age
2.) Childhood
- May follow the infantile form; occurs at 2 to
3 years of age; 90% of children have
manifestations by age 5 years
3.) Preadolescent and adolescent
- Begins at about 12 years of age; may
continue into the early adult years or
indefinitely
CLINICAL MANIFESTATION
Distribution of Lesions
1.) Infantile form
- Generalized, especially cheeks, scalp, trunk,
and extensor surfaces of extremities
2.) Childhood form
- Flexural areas (antecubital and popliteal
fossae, neck), wrists, ankles, and feet
3.) Preadolescent and adolescent form
- Face, sides of neck, hands, feet, face, and
antecubital and popliteal fossae (to a lesser
extent)
APPERANCE OF LESIONS
1.) Infantile Form
- Erythema, Vesicles, Papules, Weeping,
Oozing, Crusting, Scaling, Ofterm symmetric
2.) Childhood Form
- Symmteric involvement, clusters of small
erythematous or flesh – colored papules or
minimally scaling patches, dry and may be
hyperpigmented, lichenification (thickened
skin with accentuation of creases)
- Keratosis pilaris (follicular hyperkeratosis)
common
3.) Adolescent or Adult Form
- Same as childhood manifestations
- Dry, thick lesions (lichenified plaques)
common Confluent papules
Other Physical Manifestations
- Intense itching
- Unaffected skin dry and rough
APPEARANCE OF LESIONS
African american children likely to exhibit more
papular or follicular lesions than are white children
may exhibit one or more of the following:
- Lymphadenopathy, especially near affected
sites
- Increased palmar creases (many cases)
- Atopic pleats (extra line or groove of lower
eyelid)
- Prone to cold hands
- Pityriasis alba (small, poorly defined areas of
hypopigmentation)
- Facial pallor (especially around nose, mount
and ears)
- Bluish discoloration beneath eyes (“allergic
shiners”)
- Increased susceptibility to unusual cutaneous
infections (especially viral)
THERAPEUTIC MANAGEMENT
- Hydrate the skin
- Relieve pruritus
- Reduce flare – ups or inflammation
- Prevent and control secondary infection
Care of Mother & Child at Risk (NCMA 219)
FINALS WEEK 13 Mr. Neeco Andreus M. Martin SN 2 – Y2 – T6B

The general measures for managing AD focus on UNCONTROLLED GROWTH OF ABNORMAL

reducing pruritus and other aspects of the CELLS
disease. 1.) BENIGN
Management strategies include: - Well differentiated
- Avoiding exporuse to skin irritants or - Slow growth
allergens - Encapsulated
- Avoiding overheating - Non – invasive
- Administrating medications such as - Does NOT metastasize
antihistamines, topical immunomodulators, 2.) MALIGNANT
topical steroids, and (sometimes) mild - Undifferentiated
sedatives as indicated - Erratic and uncontrolled growth
- Enhancing skin hydration and preventing dry, - Expansive and invasive
flaky skin are accomplished in a number of - Secretes abnormal proteins
ways, depending on the child’s skin - METASTASIZES
characteristics and individual needs. 3.) BORDERLINE
Nursing Care: ETIOLOGY
- Assessment of the child with AD includes a 1.) PHYSICAL AGENTS
family history for evidence of atopy, a history - Radiation
of previous involvement, and any - Exposure to irritants
enviromental or dietay factors associated - Exposure to sunlight
with the present and previous exacerbations - Altitude humidity
- The skin lesions are examined for type, 2.) CHEMICAL AGENTS
distribution, and evidence of secondary - Smoking
infection - Dietary ingredients
- Controlling the intense pruritus is imperative - Drugs
if the disorder is to be successfully managed 3.) GENETICS AND FAMILY HISTORY
because scratching leads to new lesions and - Colon Cancer
may cause secondary infection - Premenopausal Breast Cancer
- Fingernails and toenails are cut short, kept LEUKEMIA
clean, and filed frequently to prevent sharp
edges. Gloves or cottong stockings can be - LEUKEMIA is cancer that starts in the tissue
placed over the hands and pinned to that forms blood.
shirtsleeves - Most blood cells develop from the cells in the
- One piece outfits with long sleeves and long bone marrow called stem cells
pants also decrease direct contact with the - In a person with leukemia, the bone marrow
skin. If glovs or scoks are used, the child makes ABNORMAL WHITE BLOOD CELLS
needs time to be free from such restrctions. The abnormal cell are leukemia cells. Unlike
- An excellent time to remove gloves, socks, or normal blood cells, leukemia cells don’t die
other protective device is during the bath or when they should
after receiveing sedative or antipruritic - They may crowd out normal white blood
medication cells, red blood cells, and platelets. This make
BASIC CONCEPTS OF ONCOLOGY it hard for a normal blood cells to do their
work
- CANCER is a complex of disease which The four main types of leukemia are:
oocurs when normal cells mutate into 1.) Acute Lymphoblastic Leukemia (ALL)
abnormal cells that take over normal tissue, 2.) Acute Myelogenous Leukemia (AML)
eventually harming and destroying the host 3.) Chronic Lymphocytic Leukemia (CLL)
A large group of diseases characterized by: 4.) Chronic Myelogenous Leukemia (CML)
- Uncontrolled growth and spread of
abnormal cells ETIOLOGY: THE RISK FACTORS OF LEUKEMIA
- Proliferation (rapid reproduction by cell GENETIC DISORDERS:
division) - Down syndrome
- Metastasis (spread or transfer of cancer cells - Klinefelter syndrome
from one organ or part to another not - Patau syndrome
directly connected) - Ataxia telangiectasia
CHARACTERS OF NEOPLASIA - Shwachman syndrome
- NEOPLASIA is the uncontrolled, abnormal - Kostman syndrome
growth of cells or tissues in the body, and the - Neurofibromatosis
abnormal growth itself is called a neoplasm - Fanconi anemia
or tumor - Li – Fraumeni Syndome
Care of Mother & Child at Risk (NCMA 219)
FINALS WEEK 13 Mr. Neeco Andreus M. Martin SN 2 – Y2 – T6B
ETIOLOGY: THE RISK FACTORS OF LEUKEMIA
1.) RADIATION EXPOSURE
- Nontherapeutic
- Therapeutic Radiation
2.) PHYSICAL AND CHEMICAL EXPOSURES
- Benzene Drugs such as Pipobroman
- Pesticides/ Cigratte Smoking
- Embalming fluids
- Herbicides
3.) CHEMOTHERAPY
- Alkylating agents
- Topoisomerase II inhibitors
- Anthracyclines
- Taxanes
MANIFESTATIONS:
- The symtoms of Acute Leukemia develop
very quickly (within a few days or weeks),
whereas, Chronic Leukemia can go
unnoticed for years and is usually found in
a routine blood test
The following conditions can develop in
Leukemia Patients
- Anemia (a deficiency of red blood cells and
hemoglobin)
- Thrombocytopenia (A low blood platelet
count)
- Enlarged liver or Spleen (leukemia cells build
up in the liver or spleen)
- Leukopenia (a low white blood cell count)
Other symptoms:
- Leukemia can also cause vomiting,
confustion, loss of muscle control and
seizures
- Swollen lymph nodes
- Fever or Chills
- Night Sweating
- Joint and Bone Pain
FOUR MAIN TYPES OF LEUKEMIA
1.) CHRONIC MYELOID LEUKEMIA (CML)
- This ttype affects the lymphoid cells created
in the bone marrow
- It is classified as chronic leukemia, because
the affected cells carry out some of their
normal function initially, making it difficult to
detect.
2.) ACUTE MYELOID LEUKEMIA (AML)
- The more severe form of the disease is acute
myeloid leukemia, which is characterized by
faster progression of the disease
- This is the most commonly incident type
among adults.
- If detected early, statistics show that 20% to
40% of patients survive for at least 60 months
3.) CHRONIC LYMPHOCYTIC LEUKEMIA (CLL)
- This type almost never occurs among
children and ha a very high incidence rate
among people age more than 60
- Men are more likely to be affected by it, than
women
- Progression of this disease is slow
- If the disease has affected the B – Cells, then
life expectancy can be anywhere between 10
to 20 years, if treatment begins early.
However, thos with T cell chronic
lymphocytic leukemia have a very low life
expectancy
4.) ACUTE LYMPHOCYTIC LEUKEMIA (ALL)
- The most common form of cancer in children
is acute lymphocytic leukemia. One fourt of
all cancers is children belong to this type
- It has a high incidence rate among adults,
older than 45 years of age. Chemotherapy is
the established treatment method for this
disease
- Before chemotherapy and other cancer cure
methods were invented, a patient with acute
lymphocytic leukemia could survive for 4
months at the most
- However, thanks to modern treatment
methods, about 80% of the affected children
are completely cure. Adults have been seen
to have a 40% chance of complete cure
- The prognosis for this type will vary,
depending on the stage of disease
progession, but children in the group of 3 to
7 seem to have the highest chance of
complete recovery.
DIAGNOSTIC
1.) Physical Exam
- Your doctor will look for physical signs of
leukemia, such as pale skin from anemia and
swelling of your lymph nodes, liver, and
spleen
2.) Blood tests
- By looking at a sample of your blood, your
doctor can determine if you have abnormal
levels of white blood cells or platelets, which
may suggest leukemia
3.) Bone Marrow Test
- Your doctor may recommend a procedure to
remove a sample of bone marrow from your
hipbone. The sample is sent to a laboratory
to look for leukemia cells
TREATMENT
Most treatment plans for acute lymphoblastic
leukemia have 3 steps. These are induction,
consolidation, and maintenance
1.) Induction
- Killing of leukemia cells in the blood and
bone marrow. Treatments include
chemotherapy. Induction usually lasts for 4
weeks
2.) Consolidatoin Therapy
- Killing of leukemia cells that may be present
even though they don’t show up in tests. If
these cells are not killed, they could regrow
and could cause a relapse. Treatment include
chemotherapy and may include stem cell
transplant (replacement of damage bone
marrow cells with healthy one)
Care of Mother & Child at Risk (NCMA 219)
FINALS WEEK 13 Mr. Neeco Andreus M. Martin SN 2 – Y2 – T6B
3.) Maintenance Therapy
- Preveneting any remaining leukemia cells
from growing by using low doses of
chemotherapy and intravenous treatment
(the infusion of liquid substance directly into
a vein)
LYMPHOMAS
- Pediatric lymphomas are the third most
common group of malignancies in children
and adolescents
- The lymphomas, a group of neoplastic
disease that arise from the lymphoid and
hematopoietic systems, are divide into
Hodgkin lymphoma (HL), and Non –
Hodgkin lymphoma (NHL)
- These disease are further subdivided
according to tissue type and extent of
disease
- Whereas NHL is more prevalent in children
younger than 14 years of age, HL is
prevalent in adolesnce and the young
adult period, with a striking increase
between ages 15 and 19 years
1.) HODGKIN LYMPHOMA
- Is a neoplastic disease that originates in the
lymphoid system and primarily involves the
lymph nodes
- It predictably metastasizes to non – nodal or
extralymphatic sites, especially the spleen,
liver, bone marrow, and lungs, although no
tissue is exempt from involvement.
It is classified according to FOUR HISTOLOGIC
TYPES:
1.) Lymphocytic predominance
2.) Nodular sclerosis
3.) Mixed cellularity
4.) Lymphocytic Depletion. Accurate staging
of the extent of disease is the basis for
treatment protocols and expected
prognoses
CLINICAL MANIFESTATIONS
- Asymptomatic enlarged cervical
supraclavicular lymphadenopathy is the most
commont presentaion of HL
Other systemic symptoms may be manifested:
- Cough
- Abdominal discomfort
- Anorexia
MODALITIES OF THERAPY
- The primary modalities of therapy are
radiation and chemotherapy
- Each may be used alon or in combination
based on the clinical staging
- Radiation may involve only the involved
filed (IF), an extended field (EF) (involved
areas plus adjacent nodes), or total nodal
irradiation (TNI), depending on the extent
of involvement
Prognosis
- Long term survival for all stages of HL is
excellent
- The goal for pediatric HL’s curative treatment
is to provide minimal morbidity with the
highest quality of life
NURSING CARE MANAGEMENT
- Preparation for diagnositc and operative
procedures
- Explanation of treatment side effects
- Child and family support
- Because this is most often a disease of
adolescent and young adults, the nurse must
have an appreciation of their psychologic
needs and reactions during the diagnostic
and treatment phases
2.) NON – HODGKIN LYMPHOMA
- Non – Hodgkin lymphoma occurs more
frequently in children than HL
Histologic classification of childhood NHL is
strikingly different from that of HL, as
demonstrated in the following statements:
1.) The disease is usually diffuse rather than
nodular
2.) The cell type is either undifferentiated or
poorly differentiated
3.) Dissemination occurs early, more often,
and rapidly
4.) Mediastinal involvement and invasion of
meninges are common
- NHL exhibits a variety of morphologic,
cytochemical, and immunologic features,
similar to the diversity seen in leukemia
Classification is based on the histologic
pattern:
1.) Lymphoblastic
2.) Burkitt or Non – Burkitt
3.) Large cell. Immunologically, these cells are
also classified as T cells; B cells; or non – T,
non – B cells (lacking immunologic
properties)
or
- The clinical staging system used in HL is of
little value in NHL, although it has been
modified, and other systems have been
developed
Symptoms
Care of Mother & Child at Risk (NCMA 219)
FINALS WEEK 13 Mr. Neeco Andreus M. Martin SN 2 – Y2 – T6B
DIAGNOSTIC EVALUATION
- Because the clinical presentaion of most
children with NHL is widespread
disseminated disease, thorough pathologic
staging is unnecessary
- Clinical manifestation depend on the
anatomic site and extent of involvement
- These manifestations include many of those
seen in Hodgkin disease and leukemia, as
well as organ symptoms related to pressure
from enlargement of adjacent lymph nodes,
such as intestinal or airway obstruction,
cranial nerve palsies, and spinal paralysis
- Recommendations for staging include a
surgical biopsy of an enlarged node,
histopathologic confirmation of disease
with cytochemical and immunologic
evaluation, bone marrow examination,
radiographic studies (especially
tomograms of the lungs and GI orgnas),
and lumbar puncture
THERAPEUTIC MANAGEMENT
- Aggressive use of irradiation and
chemotherapy
- Similar to leukemic therapy, the protocols
include induction, consolidation, and
maintenance phases, some with intrathecal
chemotherapy
Antineoplastic agents
- Vincristine, prednisone, L – asparaginase,
methotrexate, 6 0 mercaptopiurine,
cytarabine, cyclophosphamide,
anthracyclines, and teniposide or etoposide
- Chemotherapy is the main component of
treatment for NHL in childrent
- The prognosis is excellent for children with
NHL, in developed countries, more than 80%
of children with NHL are now cured with
modern therapy, even patients with widely
disseminated disease
WILMS TUMOR
- Wilms tumor, or nephroblastoma, is the most
common malignant renal and intra –
abdominal tumor of childhood
- The incidene is estimated to be 8.0 cases per
million children. Approximately 500 new case
are diagnosed each year in the United States,
with 6% involving both kidneys
- Occurs about three times more often in
African Americans than in East Asians in the
United states
- The pek age at diagnosis is approximately 3
years and occurrence is slightly more
frequent in bots than in girls
- The majority of patients with Wilms tumor
are diagnosed at younger than 5 years of
age, with 1% to 2.5% having a familial origin
- Unfortunately, there is no method of
idetifying gene carries at this time
CLINICAL MANIFESTATIONS
- Abdominal swelling or mass:
o Firm
o Nontender
o Confined to one side
- Hematuria (less than one fourth of cases)
- Fatigue and malaise
- Hypertension (occasionally
- Weight loss
- Fever
DIAGNOSTIC EVALUATION
- In a child suspected of having Wilms tumor,
special emphasis is placed on the history and
physical examination for the presence of
congenital anomalies, a family history of
cancer, and signs of malignancy (e.g
weight loss, size of liver and spleen,
indications of aneia, lymphadenopathy)
- Most children brought to the practitioner
because of abdominal swelling or an
abdominal mass
- Specific test include
o Abdominal ultrasonography and
abdominal and chest computed
tomography scan; hematologic
studies; biochemical studies; and
urinalysis
o Radiographic studies
- If a large tumor is present, an inferior
venacavogram is necessary to demonstrate
possible tumor involvement adjacent to the
vena cava
Care of Mother & Child at Risk (NCMA 219)
FINALS WEEK 13 Mr. Neeco Andreus M. Martin SN 2 – Y2 – T6B

- A bone marrow aspiration may be performed determines the location and extent of the
to rule out metastasic, which is rare in tumor
children with Wilms tumor - Other test that may be used include CT,
STAGING OF WILMS TUMOR ANGIOGRAPHY, ECG, AND LUMBAR
PUNCTURE
- CT, or MRI is routinely performed before a
lumbar puncture procedure to identify
intracranial abnormalities that may cause
a contraindication to the procedure
- Lumbar puncture is dangerous in the
presence of increased ICP because of the
possibility of brainstem herniation after a
1.) STAGE 1 – Tumor is limited to the kidney
sudden release of pressure
nad completely resected
- The definitive diagnosis of a brain tumor is
2.) STAGE 2 – Tumor extends beyond kidney
based on brain tissue specimes obtained
but is completely resected
during surgery
3.) STAGE 3 – Residual non hematogenous
THERAPEUTIC MANAGEMENT
tumor is confined to abdomen
- Treatment may involve the use of surgery,
4.) STAGE 4 – Hematogenous metastases;
radiotherapy, and chemotherapy or a
deposits are beyond stage 3, namely, to lung,
combination of these treatment modalities
liver, bone, and brain
- The optimum treatment is complete
5.) STAGE 5 – Bilateral renal involvement is
surgical resection of the primary tumor
present at diagnosis
with preservation of adequate neurologic
THERAPEUTIC MANAGEMENT
function
- Combined treatment with surgery and
- Radiation therapy is an integral part of
chemotherapy with or without radiation is
treatment for many brain tumors but can
based on the histologic pattern and clinical
cause significant neurocognitive side effects
stage
as well as endocrinopathies
- It sometimes includes radiation therapy
- Because rapid brain development occurs
- Treatments depend on the stage of the
during the first 3 years of life, radiation
cancer, because this type of cancer is rare, a
therapy, particularly craniospinal radiation, is
children’s cancer center that has treated this
avoided in children younger than 3 years of
type of cancer might be a good choice
age.
NURSING CARE MANAGEMENT
- Chemotherapy may be used as primary
- Nursing care of the child with Wilms tumor is
treatment or in an effort to delay radiation
similar to that of children with other cancers
therapy until patients are older and may
treated with surgery, irradiation, and
experience fewer neurocognitive side effects
chemotherapy
NURSING CARE MANAGEMENT
BRAIN TUMORS - If a brain tumor is suspected in a child
admitted to the hospital for cerebral
-Most common solid tumor in children and
dysfunction, establishing baseline data with
are the second most common childhood
which to compare preoperative and
cancer
postoperative changes is an essential step
- In children, the use of reference terms benign
- Vital signs, including blood pressure and
or malignant is generqally avoided because
pulse pressure (the difference between
any tumor, despite its nature, can be fatal or
systolic and diastolic pressures), are take
associated with significant morbidities in the
routinely and more often when any change is
developing brain of a child
noted
DIAGNOSTIC EVALUATION
- Any sudden variastions are reported
- The signs and symptoms of brain tumors are
immediately
directly related to their anatomic location and
- Observation of symptoms of Cushing triad –
size and, to some exten, the child’s age
a hallmark sign of increased ICP, which
- Diagnosis of a brain tumor is based
includes bradycardia, hypertension, and
subjectively on presenting clinical signs,
irregular respirations – is a. Crucial role of the
objectively on neurologic test, along with
nurse
surgical confirmation of the histologic
- It is also important to note a change in vital
diagnosis
signs during or after diagnostic procedures
- A number of tests may be used in the
- A routine neurologic assessment is
neurologic evaluation, but the most common
performed at the same time as vital signs,
diagnostic procedure is MRI, which
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FINALS WEEK 13 Mr. Neeco Andreus M. Martin SN 2 – Y2 – T6B
and head circumference should be measure
for infants and very young children
- The child is observed for evidence of
headache, vomiting, and any seizure activity.
The location, severity, and duration of the
headache are noted, as well as its relationship
to activity, time of day, and any associated
factors
- Behaviros such as lying flat and facing away
from light or refusing to engage in play are
clues to discomfort in nonverbal children
- The child’s gait is observed at least once
daily. Head tilt while talking or performing an
activity as well as other changes in posturing
should always be documented
TREATMENT MODALITIES
AIMED TOWARDS:
- CURE: Free of disease after treatment —>
normal life
- CONTROL: Goal for chronic cancers
- PALLIATIVE CARE: Quality of life maintained
at highest level for the longest possible time
- SURGERY: Surgical removal of tumors; most
commonly used treatment
- Preventive or prophylactic
- Diagnostic surgery
- Curative surgery
- Reconstructive surgery
- Palliative surger
1.) CHEMOTHERAPY
- Use of antineoplastic drugs to promote
tumor cell death, by interfering with cellular
functions and reproductin
2.) RADIOTHERAPY
- Directing high – energy ionizing radiation to
destroy malignant tumor cells without
harming surrounding tissues
TYPES:
1.) TELETHERAPY (external)
- Radiation delivered in uniform dose to tumor
- External beam irradiation and uses a device
located at a distance from the patient.
- Produces x – rays f varying energies and is
administered by machines a distance from
the body 31% to 39 inches (80 to 100 cm)
2.) BRACHTHERAPY
- Delivers high dose to tumor and less to toher
tissues; radiation source is placed in tumor or
next to it; in brachytherapy, the radiation
deviced is placed within or close to the target
tissue. Radiation is delivered in a high dose
to a small tissue volume with less radiation to
adjacent normal tissue, but required direct
tumor access
- 2 types of Brachtherapy
o Temporary and Permanent
3.) IMMUNOTHERAPY
- Use of chemical or microbial agents to
induce mobilization of immune defenses
4.) BIOLOGIC RESPONSE MODIFIERS (BRMs)
- Use of agents that alters immunologic
relationship between tumor and host in a
beneficial way
5.) BONE MARROW PERIPHERAL STEM CELL
TRANSPLANTATION
- Aspirating bone marrow cells from
compatible donor and infusing them into the
recipient
6.) GENE THERAPY
- Transfer of genetic material into the client’s
DNA
NURSING MANAGEMENT
- Promote measure that relieve pain and
discomfort
o Pharmacoogic and non –
pharmacologic intervention
- Promote measure to maintain intact skin
- Promote measures that maintain oral
mucosa
- Promote measure to prevent injury from
abnormal bleeding
o Monitor platelet count; avoid aspiring
products, etc
- Promote measures to enhance body image.
o Take an honest gently, caring
approach; encourage client to
express and verbalize feelings
- Promote measures that address preventing
complication of cancer therapy
- Instruct client and family about the disease
process and treatments; provide necessary
information for self – care
- Help client and family cope effectively
- Promote measure to reduce social isolation
BRAIN TUMORS
CARE OF CLIENTS RECEIVING CHEMOTHERAPY
CLASSES OF CHEMOTHERAPY DRUGS:
1.) ALKYLATING AGENTS:
- Action: create defects in tumor DNA
- Ex: Nitrogen Mustard, Cisplatin
- Toxic Effects: reversible renal tubular necrosis
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FINALS WEEK 13 Mr. Neeco Andreus M. Martin SN 2 – Y2 – T6B
2.) ANTIMETABOLITES:
- ACTION: Phase specific
- Ex: Methotrexate; 5 fluorouracil
- Toxic Effects: nausea, vomiting, stomatitis,
diarrhea, alopecia, leukopenia
3.) ANTITUMOR ANTIBIOTICS:
- ACTION: Non – phase specific; interfere with
DNA
- Ex: Actinomycin D, Bleomycin, adriamycin
(doxorubicin)
- Toxic Effect: damage to cardiac muscle
4.) MIOTIC INHIBITORS
- ACTION: Prevent cell division during M phase
of cell division
- Ex: Vincristine, Vinblastine
- TOXIC EFFECTS: Affects neurotransmission,
alopecia, bone marrow depression
5.) HORMONES:
- ACTION: Stage specific G1
- EX: Corticosteroids
6.) HORMONE ANTAGONIST:
- ACTION: Block hormones on hormone –
binding tumors ie: breast, prostate,
endometrium; cause tumor regression
- Ex: Tamoxifen (Breast); Flutamide (Prostate)
- TOXIC EFFECTS: Altered secondary sex
characteristic
EFFECTS OF CHEMOTHERAPY
Tissues:
- (fast growing) frequently affected
Examples
- Mucous membranes, hair cells, bone marrow,
specific organs with specific agents,
reproductive organs (all are fetal toxic: impair
ability to reproduce)
CHEMOTHERAPY ADMINISTRATION
Routes of Administration:
- Oral
- Body cavity (intraperitoneal or intrapleural)
- Intravenous
- Use of vascular access devices because of
threat of extravasation (leakage into tissues)
& long term therapy
Types of Vascular Access devices:
1.) PICC LINES
- Peripherally inserted central catheters
2.) TUNNELED CATHETERS
- Hickmam, Groshong
3.) SURGICALLY IMPLANTED PORTS:
- Accessed with 90o angle needle – Huber
needles)
NURSING CARE OF CLIENTS RECEIVING
CHEMOTHERAPY
- Assess and Manage:
o Toxic effects of drugs (Report to
physician)
o Side effects of drugs: manage nausea
and vomiting, inflammation and
ulceration of mucous membranes,
hair lose, anorexia, nausea and
vomiting with specific nursing and
medical intervention
- Monitor lab results (drugs withheld if blood
counts seriously low); blood and blood
product administration
- Assess for dehydration, oncologic
emergencies
- Teach regatding fatigue, immunosuppresion
precautions
- Provide emotional and spiritual support to
clients and families
ALTERATIONS IN NUTRITION AND GI, METABOLISM,
& ENDOCRINE
1.) CLEFT LIP/ PALATE
- A cleft is a gap or split in the upper lip and/or
roof of the mouth (palate). It is present from
birth
- Result when the maxillary processes fail to
fuse with the elevations on the frontal
prominence during the sixth week of
gestation
- Approximately 30% of children with cleft
and/or palate will have another congenital
anomally
- The cause is believed to be multifactorial,
involving combination of environmental and
genetic influeces
- The defect may be unilateral or bilateral and
may occur alone or in combination with a cleft
palate defect
- Varying degress of nasal deformity may also
be present
- Clest palate defects are less obvious when
they occur without a cleft lip and may not be
detected at birth
- Clefts of the hard palate form a continous
opening between the mouth and nasal cavity
and may be unilateral or bilateral, involving
just the soft palate or both the soft and hard
palates
ETIOLOGY FACTORS
- Smoking during pregnancy
- Maternal use of alcohol
- Use of medications such as anticonvulsants
and steroids during pregnancy
DIAGNOSIS
- Diagnosed at birth or during the newborn
assessment, but may be diagnoseds in utero
- Successful imaging of the face via
transabdominal ultrasound can be
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FINALS WEEK 13 Mr. Neeco Andreus M. Martin SN 2 – Y2 – T6B
performed as early as 13 to 14 weeks’
gestation
- Use of three – dimensional ultrasound or
magnetic resonance imaging, if available,
allows for a clearer picture of the defect and
enhances the ability to detect isolated palate
prenatally
MANAGEMENT
- Directed toward closure of the cleft(s),
prevention of complication, and facilitation
of normal growth and development in the
child
- Cleft lip repair typically occurs at most
centers between 2 and 3 months of age
- Most physicians adhere to the “rule of tens”.
The infant must be 10 weeks, weigh 10
pounds, and have a hemoglobin of 10
The two most common procedures for repair
of CL are the:
1.) Tennison – Randall triangular Flap (Z-
plastly)
2.) Milliard rotational advancement
technique
- Cleft Palate Repair typically occurs between 6
and 12 months
- There is concern that early CP repiar
interferes with skeletal growth of the
midface, but postponing palate closure
beyond the child’s first words may result in
increased speech disorders.
The most common techniques to repair CP
include the:
1.) Veau – Wardill – Kilner V – Y push back
procedure
2.) Fulow Double – opposing Z – plasty
- Approximately 20% to 30% of children with
repaired CP will need a secondary surgery to
improve velopharngeal closure for speech
- Secondary procedures may include palatal
lengthening, pharyngeal flap, sphicter
pharyngoplasty, or posterior pharyngeal wall
augmentation
THE MAIN TREATMENTS ARE:
1.) SURGERY
- An operation to correct a cleft lip is usually
done when your baby is 3 to 6 month and an
operation to repair a cleft palate is usually
done at 6 to 12 months
2.) FEEDING SUPPORT
- You may need advice about positioning your
baby on your breast to help them feed, or
you might need to feed them using a special
type of bottle
3.) MONITORING HEARING
- A baby born with cleft palate has a higher
chance of glue ear, which may affect hearing.
Close monitoring of their hearing is
important and if glue ear affects their hearing
significantly, a hearing aid may be fitted or
small tubes called grommets may be placed
in their ears to drain the fluid
4.) SPEECH AND LANGUAGE THERAPY
- A speech and language therapist will monitor
your child’s speech and language
development throughout their childhood
and help with any speech and language
problems
5.) GOOD DENTAL HYGIENE AND
ORTHODONTIC TREATMENT
- You’ll be given advice about looking after
your child’s teeth, and they may need braces
if their adult teeth don’t come through
properly
POST – OPERATIVE CARE FOR INFANT INCLUDE:
- Assess vitals signs frequently and maintain
the infant’s airway
- Measure intake and output
- Prevent aspiration through proper
positioning during and after feeding
- Prevent the infant from rubbing the suture
line on the bedding by positioning the infant
in a supine position
- Maintain soft elbow immobilizers
- Maintain the suture line or steri – strips
placed over the incision, Place antibiotic
body ointment on the incision site as ordered
- Medicate the infant as prescribed to contorl
pain and to minimize crying and stress on the
suture line
- After cleft palate surgery, avoid the use of
metal utensils or straws, which may distrupt
the surgical site
HIRSCHSPRUNG DISEASE
- Also known as congenital aganglionic
megacolon
- Is a congenital anomaly in which inadequate
motility causes mechanical obstruction of the
intestine
- The disease occurs in approximately 1 in
5000 live births, and is more common in
males than females
- Occur as a single anomaly or in combination
with other anomalies such as congenital
heart defects, down syndrome, and urinary
tract anomalies
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FINALS WEEK 13 Mr. Neeco Andreus M. Martin SN 2 – Y2 – T6B
- Is the congenital absence of ganglion cells
(nerve cells) in the wall of a variable segment
of rectum and colon
- The absence of autonomic parasympathetic
ganglion cells in the colon prevents
peristalsis at that portion of the intestine,
resulting in the accumulation of intestinal
contents and abdominal distention
- In the most cases, the area lacking ganglion
cells is liited to the rectosigmoid region of
the colon
CLINICAL MANIFESTATIONS
- Abdominal distention
- Feeding intolerance
- Billous vomiting
- Failure to pass meconium within the first 24
to 48 hours after birth
- Enterocolitis (inflammation of the intestines)
is a complication of Hirschsprung disease
that can be fatal if not recognized and
treated early
Symptoms of enterocolitis
- Fever
- Foul smelling or bloody diarrhea (frequent,
watery stools)
- Abdominal pain
- Vomiting
DIAGNOSIS
- History of bowel patterns
- Radiographic contrast studies
- Rectal biopsy for presence or absence of
ganglion cells
- The rectum is small in size on palpation and
does not contain stool
- Abdominal radiographs generally show a
distended bowel with dilated bowel loops
throughout the abdomen
- Water – soluble contrast studies reveal a
transition zone between the normal and
aganglionic bowel
INTUSSUCEPTION
- Intussusception is the most common cause
of intestinal obstruction in children between
the ages of 3 months and 3 years
- More common in boys
- Occurs when one segment of the bowel
telescopes into another segment, pulling the
mesentery with it. The mesentery is
compressed and angled, resulting in
lymphatic and venous obstruction
- As the edema from the obstruction increases,
pressure within the area of intussusception
increases
- When the pressure equals the arterial
pressure, arterial blood flow stops, resulting
in ischemia and the pouring of mucus into
the intestine
- Venous engorgement also leads to leaking of
blood and mucus into the instestinal lumen,
forming the classic currant jelly – like stools
- The most common site is the ileocecal valve
(illeocolic), where the ileum invaginates into
the cecum and then further into the colon
CONSERVATIVE TREATMENT
- Consists of radiologist – guided pneumo –
enema (air enema) with or without water –
soluble contrast or ultrasound guided
hydrostatic (saline) enema, the advantage of
the latter being that no ionizing radiation is
needed
- Intravenous fluids, NG decompression, and
antibiotic therapy may be used before
hydrostatic reduction is attempted
- Laparoscopic surgical repair is commonly
performed
ACUTE APPENDICITIS
- Is an inflammation of the vermiform
appendix, the small sac near the end of the
cecum, and is the most common cause of
emergnecy in children
- The condition occurs most often in children
and adolescents ages 10 to 19 years
- Almost always results from an obstruction in
the appendiceal lumen
- It can be cause by a fecalith (hard fecal mass),
parasitic infections, stenosis, hyperplasia of
lymphoid tissues, or a tumor
Signs and Symptoms
- Guarding
- Rigidity
- Nausea
- Vomiting
- Onset of pain before vomiting
- Anorexia
- Rebound tenderness following palopation
over the right lower quadrant
As appendicitis progresses
- The child remains motionless
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FINALS WEEK 13 Mr. Neeco Andreus M. Martin SN 2 – Y2 – T6B
- Usually in a side – lying position with knees
flexed.
- Sudden relief of pain usually means that the
appendix has perforated
DIAGNOSIS
- Appendicitis in young children can be diffuclt
because their pain may be less localized and
their symptoms more diffuse than in the
older child
- The presence of an elevated white blood cell
count (above 10,000/mm3). Increased
neutrophil ratio, and an elevated C – reactive
protein combined with the symptoms
- A white blood cell count greater than
15,000/mm3 in a patient with appendicitis is
a strong indicator that the appendix has
perforated
- Abdominal ultrasound is preferred as the
initial screening tool in the diagnosis of
appendicitis
- CT scans are more sensitive and may be used,
especially when the appendix cannot be seen
well on ultrasound or the results are
inconclusive
TREATMENT UNCOMPLICATED APPENDICITIS
- Involves immediate surgical removal
(appendectomy), generally through a
laparoscopic appendectomy
- Preoperatively, the child is kept NPO
- Intravenous fluids and electrolytes, and
antibiotics are administered
PostOperative
- Antiobiotics may be administered generally
discharged within 24 to 36 hours as long as
they have adequate oral intake, are afebrile,
and receive effective pain relief with oral pain
medication
TREATMENT PERFORATED APPENDIX
- Laparoscopic appendectomy is generally
performed
PostOperative
- May have a nasogastric tube to decompress
the abdomen
- Will remain NPO until signs of bowel function
return. Bowel function is best indicated by
the passage of flatus or stool
- The child will also have a peripheral or
temporary central line for administration of
intravenous fliud and medication
- After surgery for a perforated appendix, the
child will receive antibiotics for several days,
Morphine is generally given for pain
ESOPHAGEAL ATRESIA
(EA)/TRACHEOESOPHAGEAL FISTULA (TEF)
- Congenital esophageal atresia and
tracheosophageal fistula are rare
malformation that represent a failure of the
esophagus to develop as a continous
passage and a failure of the trachea and
esophagus to separate into distinct structure
- These defects may occur as separate entities
or in combination, and without early
diagnosis and treatment, they pose a serious
threat to the infant’s well – being
- Cause of EA/TEF is unknown
- In esophageal atreasia, the foregut fails to
lengthen, separate, and fuse into two parallel
tubes (the esophagus and trachea) during
fetal development. Instead the esophagus
may end in a blind pouch or develop as a
pouch connected to the trachea by a fistula
- Often associated with a maternal history of
polyhydramnios
SYMPTOMS
- Excessive salivation and drooling
- Often accompanied by three classic signs for
this defect: cyanosis, choking, and
coughing
- Sneezing may also be noted
- During feeding, fluid returns through the
infan’ts nose and mouth
- Aspiration places the infant at risk for
pneumonia
- Depending on the type of defect, the infant’s
abdomen may be distended because of air
trapping
DIAGNOSIS
- Esophageal atresia is suspected based on the
presence of polyhydramnios and a small or
absent fetal gastric bubble noted on prenatal
ultrasound
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FINALS WEEK 13 Mr. Neeco Andreus M. Martin SN 2 – Y2 – T6B
- After the child is born, radiopaque catheter is
inserted into the hypopharynx and advanced
until it encounters an obstruction
- Chest radiographs are taken to ascertain
esophageal patency or the presence and
level of a blind pouch
- Sometimes fistuals are not patent, which
makes them more difficult to diagnoses
- The presence of gas in the stomach or small
bowel is indicative of a coexisting TEF
TREATMENT
- Involves connecting the two ends of the
esophagus and ligating the fistla if present
- If primary repair of the esophageal atresia is
not possible in the neonatal period, a
gastronomy tube is placed for feeding and
the fistula is ligated
- Esophageal atresia is a surgical emergency
PREOPERATIVE SPECIFIC INTERVENTIONS
INCLUDE:
- Have sunction readily available to remove
any secretions that accumulate in the
nasopharyngeal airway
- Place the infant with the head of the bed
slightly elevated to minimize aspiration of
secretion into the trachea
- Use of continous or low intermittent suction
to remove secretions from the blind pouch
- Withhold oral fluids, and provice
maintenance intravenous fluids
- Constantly monitor the infant’s vital signs
and overall condition
AFTER SURGERY
- Administer intravenous fluids and antibiotics
- Monitor strict intake and output
- TPN may be needed until gastrostomy or oral
feedings are tolerated
- Monitoring and assessment of feeding
tolerance are ongoing
- Feedings are introduce slowly and in small
amounts
PYLORIC STENOSIS
- Hypertrophic pyloric stenosis (HPS) occurs
when the circumferential muscle of the
pyloric sphincter becomes thickened,
resulting in elongation and narrowing of the
pyloric channel
- This condition usually develops in the first 2
to 5 weeks of life, causing projectile
nonbilious vomiting, dehydration, metabolic
alkalosis, and growth failure
- The precise etiology is uknown
- Hypertrophic pyloric stenosis (HPS) occurs
when the circumferential muscle of the
pyloric sphincter becaomes thickened,
resulting in elongation and narrowing of the
pyloric channel
- This condition usually develops in the first 2
to 5 weeks of life, causing projectile
nonbilious vomiting, and growth failure
- The precise etiology is unknown
- Pyloric stenosis is not a congenital disorder
SYMPTOMS
- Usually become evident 2 to 8 weeks after
birth, although onset may vary
- Initially, the infant appears well or
regurgitates slightly after feedings.
- The parents may describe the infant as a
"good eater" who vomits occasionally.
- As the obstruction progresses, the vomiting
becomes projectile.
- In projectile vomiting, the contents of the
stomach may be ejected up to 3 feet from the
infant.
- The vomitus is nonbilious and may become
blood tinged because of repeated irritation
to the esophagus.
- The infant generally appears hungry,
especially after emesis; irritable; fails to gain
weight; and has fewer and smaller stools.
- The child may present with dehydration and
metabolic alkalosis depending how long the
child has been vomiting
- On physcial examination, peristaltic waves
may be observed across the abdomen as the
stomach attempts to move contents past the
narrowed pyloric canal
- An olive – sized mass in the right upper
quadrant may be evident
DIAGNOSIS
- Abdominal UTZ
- Blood tests will determine if the child is
dehydrated
TREATMENT
- Surgery is performed as soon as possible
after the infant’s fluid and electrolyte balance
is restored
o Laparoscopic Pyloromyotomy is the
preferred surgical method to correct
pyloric stenosis
NURSING CARE MANAGEMENT
- Includes meeting the infant’s fluid and
electrolyte needs
- Minimizing weight loss
- Promoting rest and comfort
- Preventing infection
- Proving supportive care for parents
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FINALS WEEK 13 Mr. Neeco Andreus M. Martin SN 2 – Y2 – T6B

IMPERFORATED ANUS - Also known as gluten – induced enteropathy,

gluten sensitive enteropathy, and celiac
sprue, is a permanent intestinal intolerance
to dietary wheat gliadin and related proteins
that produces mucosal lesions in genetically
susceptible individuals
- As a result, children develop steatorrhea
(bulky, fould – smelling, fatty stools);
deficiency of fat – soluble vitamins A, D, K,
and E (the vitamins are not absorbed because
- Imperforate anus includes several forms of
the fat is not absorbed); malnutrition; and a
malformation without an obvious oepning
distended abdomen from the fat, bulky
- Frequently a fistual leads from the distal
stools. Because Vitamin D is one of the fat –
rectum to the perineum or genitourinary
soluble vitamins, rickets or loss of calcium
system
from bones may occur
- The fistula may be evidenced when
- Hypoprothrombinemia may occur from loss
mecomium is evacuated through the vaginal
of vitamin K. In addition, children may have
opening
hypochromic anemia (iron – deficieny
DIAGNOSIS
anemia) and hypoalbuminemia from poor
- Based on the physical finding of an absent
protein absorption
anal opening
SYMPTOMS
- Abdominal distention
- Vomiting
- Absence of meconium passage, or presence
of meconium in the urine
- Additional physical findings with an anorectal
malformation are a flat perineum and the
absence of a midline intergluteal groove
MANAGEMENT
- Surgical
o Anoplasty, colostomy, posterior
sagittal anorectoplasty (PSARP), or
other pull through with colostomy,
and colostomy (take down) closure
- After the defect has been identified, take
steps to rule out associated life – threatening
defects, which need immediate surgical
intervention
- Provided no immediate life – threatening
problems exist, the newborn is stabilized and
kept NPO for further evaluation
- IV fluide are provided to maintain glucose
and fluid and electrolyte balance Typically, children are seen
- The current recommendation is that surgery - With impaired growth
be delayed at least 24 hours to properly - Chronic diaarhea
evaluate for the presence of a fistula and - Abdominal distention
possible other anomalies - Muscle wasting with hypotonia
CELIAC DISEASE (MALABSORPTION SYNDROME) - Poor appetite
- Celiac disease is a sensitivity or abnormal - Lack of energy
immunologic response to protein, The first evidence may be growth failure and
particularly the gluten facor of protein found diarrhea
in grains (wheat, rye, oats, and barley) - Children ages to 5 to 7 years
- When children with the disorder ingest o Abdominal pain
gluten, changes occur in their intestinal o Nausea
mucose or villi that prevent the absorption of o Vomiting
foods, especially fat, acroos the intestinal villi o Bloating
into the bloodstream o Constipation
o Extraintestinal manifestations
▪ Including iron deficiency
anemia
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FINALS WEEK 13 Mr. Neeco Andreus M. Martin SN 2 – Y2 – T6B

▪ Short stature
▪ Pubertal delay
▪ Dental enamel defects
▪ Alopecia
TREATMENT
- To continous the gluten – free diet for life
because there is some suggestion that these
children are more pron to GI carcinoma later
in life if they do not continue the diet into
adulthood
- In addition to his, children need to have
water – soluble forms of vitamins A and D
administered
- Both iron and folate may be necessary as well
to correct any anemia present