Appendix C

CONCEPT MAP
Care of a Client with Cystic Fibrosis
Case Scenario: A 5-year-old girl comes to the hospital for the first time with complaints of fever, malaise, and a cough for 2 days. She has a history
of asthma for which she uses a steroid inhaler daily and an albuterol inhaler as needed. She has been tried on various over-the-counter cold and
allergy remedies, but her respiratory symptoms have been worsening over the past several months. Her past medical history is notable for an
episode of rectal prolapse and sinusitis during each of the past two winter seasons. Her mother also reports that her daughter has always been
small for her age. The examination reveals a moderately ill-appearing child whose height and weight are at the fifth percentile for age. Her
temperature is 101F (38.3C) and respiratory rate 32 breaths/min. She has scant purulent rhinorrhea bilaterally, wheezy breath sounds in all lung
fields, and diminished breath sounds on the right side. Heart sounds and capillary refill are normal, yet she has digital clubbing.

Legend:
Pathophysiologic course

Clinical Manifestations

Respiratory Involvement

Diagnostic Tests and Evaluation

Gastrointestinal Involvement

Therapeutic and Medical Management

Integumentary Involvement

Nursing Diagnosis

Risk Factors:
- Both parents carry the CF gene.
(autosomal recessive)
- Race: Caucasian/ white children

Genetic defect of the

long arm of
Chromosome 7

Mutations in CF
Transmembrane regulator
(CFTR)

Exocrine
Gland
Dysfunctions

Increased
production of
abnormally thick,

Respiratory Involvement

Blockage in sodium and

chloride channels in
exocrine portion of organs

Gastrointestinal
Involvement

Integumentary
Involvement

Upper Respiratory Tract

Lower Respiratory Tract

S/S: chronic
sinusitis,
nasal
polyposis

Respiratory airways
(bronchioles) are
obstructed
S/S: Dyspnea,
SOB, barrel
chest,
tachycardia,
wheezing.

Impaired
gas
exchange
r/t air
trapping s/t
obstruction

Chest X-ray:
hyperinflati
on,
Abnormal

Oxygen
Therapy,
Pulse
Oximeter,

Bronchospasms

Stasis of
mucus
Ineffective
airway
clearance r/t
increased

S/S:
Coughing
with viscous
purulent
discharge,
Chest
physiothera
py,
Mucolytic

Decreased reabsorption
of sodium and chloride in
sweat glands

Proper
Hydration,
electrolyte
replacement,

Situational
low self
esteem r/t
physical
changes
from

S/S: Salty
skin,
dehydration,
excessive
sweating,
febrile
Sweat
chloride
test: values
above 60

Diminished
absorption of
protein
Generalized
Edema

Pulmonary
Infection
S/S:
Cardinal
signs of
infection,
fever, chest

Ineffective
breathing
pattern r/t
recurring lung

Anitbiotic
Therapy,
corticosteroi
ds

S/S: Hemoptysis,
Pneuomothorax,
Anemia,
cyanosis, digital
clubbing
Lung
Transplantati
on

Culture and
sensitivity
test

Further lung
damage and
fibrosis with
tissue erosion.

Mucus plugging
in pancreatic
duct

Focal biliary
obstruction

Biliary
Pancreatic
enzymes are
not released
to the
intestine
Malabsorption
of fat and
protein

Fibrosis of
Islets of
Langerhans
Diabetes
Mellitus

Respiratory
Failure

Pancreatic
Enzyme
replacement,
Nutritional
Therapy,
Incomplete
digestion

S/S: dry
mouth
Mouth Care

Portal
Hypertension

S/S:
Steatorrhea,
weight loss,
stunted

Exams: Stool
analysis,
Duodenal

Blockage of
ducts in salivary
glands

Imbalanced
nutrition : less
than body
requirements r/t
poor intestinal
absorption of

Formation of
large, bulky
Risk for impaired
skin integrity r/t
acidic stools
Rectal
Prolaps

Osmotic
solutions, stool
softeners,
laxatives,
increase fiber

DIOS

S/S: RLQ Pain,

Abdominal
distention,
abdominal
cramps,
nausea and
vomiting, foul
smelling flatus