Introduction

► CF is an inherited disease of your mucusand

sweat glands.
► It affects mostly the lungs, pancreas,
liver,intestines, sinuses and sex organs.
► An abnormal gene causes mucus tobecome
extra thick and sticky.
► This gene makes a protein that controlsthe
movement of salt and water not workproperly
(retaining salt=thick secretions)>This leads to
mucus plugs.
 Introduction Cont.

Cystic fibrosis is the most common cause of

chronic lung disease in children and young adults,
and the most common fatal hereditary disorder
affecting Caucasians in the US.

CF is a multi-system disorder of exocrine glands

causing the formation of a thick mucus substance
that affects the lungs, intestines , pancreas, and
liver. The standard test for diagnosis is a sweat
test which evaluates the level of chloride excreted
by the body.
Demographic data
Age: 10 years Gender: Male Ward: Pediatric ward
Medical diagnosis: Cystic fibrosis
Subjective data:
Patient said: “I feel dizzy with difficult breathing while movement and mobility”
Patient said: “I suffer from chest pain that is 6/10, localized with no radiation to
another region, suddenly onset and it is consistent”
Objective data:
The patient looks pale. He is lying on the bed (HOB elevated 45 degree), connected
to oxygen facemask with rate 5 L/min. An IV cannula is in patient’s Lt arm (gage 22).
The patient connected to condom for urination. The patient is conscious, oriented
(*3), GCS 15/15. The patient suffers from productive cough (thick in characteristic),
wheezes and audible breath sound are noticed, and crackles are auscultated
bilaterally
Patient is with clubbing fingers … no cyanosis is seen
Wt: 30 kg … Ht: 110cm … BMI: 24
Chest x-ray showed inflammation in both lungs … CT scan suggests bronchiectasis
VS: BP: 105/53 mmHg … T: 37.3 C … HR: 112 bpm … RR: 29 cycle/min (on O2 mask)
… SO2: 93%
Diagnosis
1- Ineffective breathing pattern related to alteration in the lung function and
accumulation of sputum in the respiratory system secondary to Cystic fibrosis
AMB thick mucus production with cough

2- Fatigue related to imbalance between body energy and activities related to

decreased O2 in the body secondary to cystic fibrosis AMB patient’s
verbalization

Planning
1- To improve breathing pattern

Patient will be able to maintain O2 saturation above 92%

2- To enhance patient’s body activity

Patient will be able to demonstrate body activities in the bed and walk over the
room as tolerated
Intervention
1- O2 supply as indicated and as physician order

Rationale: Oxygenation will enhance breathing pattern by increasing O2

saturation in the blood
2- Continuous assessment of cough and sputum associated with cough

Rationale: Cough improves gas exchange with sputum excretion and results in
increasing O2 levels in the blood
3- Administer sputum dissolving medications (Ventolin, Combivent) by
nebulizer

Rationale: sputum excretion will enhance oxygenation levels in the blood

4- Put patient in semi-Fowler position
Rationale: To increase and improve lung expansion results in gas exchange
and O2 saturation in the blood
5- Chest physiotherapy regular and as needed
Rationale: To facilitate mucus and sputum excretion which results in
respiratory improvement
Intervention Cont.
6- Encourage patient to perform deep breathing
Rationale: To ease the excretion of lung sputum
7- Medications like hydrocortisone (corticosteroids)
Rationale: To improve inflammatory symptoms and facilitate respiration
8- Administer antibiotics as prescribed
Rationale: To fight infection resulting in improving the symptoms of
respiratory distress
9- Demonstrate handwashing techniques
Rationale: To prevent eventual infection as long-run management
10- Monitor VS including O2 saturation
Rationale: To assess management plan and interventional nursing actions
11- Encourage patient to demonstrate bed activities as tolerated and as
applicable
Rationale: Bed activities will decrease fatigue symptoms and improve
circulation
Intervention Cont.
12- Assist patient with ambulation around the room
Rationale: To improve fatigue symptoms and facilitate daily activities
13- Connect patient with O2 while movement and mobility
Rationale: To prevent potential decrease in O2 levels in the blood
14- Provide family teaching regarding the care-providing measures
Rationale: To involve the care-givers in the management and decrease stress
levels which may benefit the management plan
15- Assess tiredness regularly and after intervention
Rationale: To prevent potential complications like O2 decreasing
16- Administer medication that improve energy as prescribed by the doctor
(like vitamins)
Rationale: To increase body energy that may facilitate rehabilitation process
Evaluation
1- Goal was met AEB O2 saturation levels are maintained above 92%
2- Goal was not met because long-term outcomes are not achieved