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 101°F (38.3°C) 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 Blockage in sodium and

production of chloride channels in
abnormally thick, exocrine portion of organs

Respiratory Involvement Gastrointestinal Integumentary

Involvement Involvement
Upper Respiratory Tract Lower Respiratory Tract Decreased reabsorption Diminished
of sodium and chloride in absorption of
sweat glands protein

S/S: chronic Respiratory airways

sinusitis, (bronchioles) are S/S: “Salty” Generalized
nasal obstructed Proper skin, Edema
polyposis Hydration, dehydration,
electrolyte excessive
S/S: Dyspnea, sweating,
Impaired replacement,
SOB, barrel febrile
gas chest,
exchange Sweat
tachycardia,
r/t air chloride
wheezing.
trapping s/t Situational test: values
obstruction Chest X-ray: low self above 60
hyperinflati esteem r/t
Oxygen on, physical
Therapy, Abnormal changes
Pulse from
Oximeter,

Bronchospasms

Stasis of
mucus
Ineffective S/S:
airway Coughing
clearance r/t with viscous
increased purulent
discharge,

Chest
physiothera
py,
Mucolytic
 Pulmonary
Infection
S/S: Mucus plugging Focal biliary Blockage of
Cardinal in pancreatic obstruction ducts in salivary
Ineffective signs of duct glands
breathing infection,
pattern r/t fever, chest
recurring lung S/S: dry
Culture and Biliary mouth
sensitivity Pancreatic Fibrosis of
test enzymes are
Anitbiotic Islets of Mouth Care
Therapy, not released Langerhans Portal
corticosteroi to the
Hypertension
ds intestine Diabetes
Mellitus
Malabsorption
Further lung of fat and S/S:
S/S: Hemoptysis, damage and protein Steatorrhea,
Pneuomothorax, fibrosis with weight loss,
Anemia, tissue erosion. stunted
cyanosis, digital Exams: Stool
clubbing analysis,
Duodenal
Lung Imbalanced
Transplantati nutrition : less
on than body
Respiratory requirements r/t
Failure poor intestinal
Pancreatic
absorption of
Enzyme
replacement,
Nutritional
Therapy,

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

S/S: RLQ Pain,

Abdominal
distention,
abdominal
cramps,
Osmotic
nausea and
solutions, stool
vomiting, foul
softeners,
smelling flatus
laxatives,
increase fiber