Care of the
Pediatric
Patient with
Cystic Fibrosis
OBJECTIVES
 Introduction
of patient/PMH
 Cultural Considerations
 Reason for Hospitalization
 Primary/Secondary Medical Diagnosis
 Developmental Considerations &
related theory
 Exceptions to the norms
 Nursing dx and Care plans
 Teaching and discharge planning
 Research
Introduction
 T.R.D
is a 9 y.o. Caucasian male patient
 Admission date: 28 Oct14
 Diagnosed with CF exacerbation/MRSA
 Secondary dx: Positive Rhino Virus
 Isolation:
 Contact & Droplet
 Full Code
 Allergies:
 Claritin, Chloraprep, Seasonal Allergies,
Cestazidine, Ceftazidime, Tape,
 Tubes/Equipment: RAC (20 gauge), CVAD
R chest 22 gauge, GT feeding tube
Past Pertinent Health History


History of CF: dx at 3
y.o.,
Multiple
hospitalizations:




Cholecystectomy at
2 years
Bronchoscopy at 4
y.o.
G-tube feeding: pt.
will only eat certain
food.
Family/Psychosocial:
under full custody of
grandparents
(always at bedside)
Cultural Considerations
 Divorce
household
 Under
full custody
of grandparents
since childhood
 GT
tube feeding
 CVAD
R chest
Reason for hospitalization
 Sent
home from school d/t excessive
coughing since Florida vacation.


PFT poor results during clinic admission.
c/o LL back flank pain
 Admission
Vitals: 36.8*C (PO), HR: 112, RR,
24, BP 120/94
 Primary
dx: CF tune
up/exacerbation/MRSA

Secondary dx: Positive Rhino Virus
 Extravasation
of Contrast dye
Pathophysiology of Cystic Fibrosis






Inherited disease that
affects many organs and
lethally impairs pulmonary
function.
Error in Chloride transport
which results in
 Increased viscosity of
mucous gland
secretions
Affects multiple body
organs: lungs, pancreas,
liver, salivary glands, testes.
Common complications:
pneumothorax, arterial
erosion, hemorrhage
Main cause of death:
RESPIRATORY FAILURE
Diagnostic test: SWEAT
CHLORIDE TEST
Organs affected in CF
Treatment Plan
 Goals:
Treatment of Infections, Airway
Clearance, Increased Oxygenation,
Prevention of malnutrition:






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Antibiotics Therapy
CPT, Bronchodilators, Nebulizer
Supplemental O2 PRN
Continued G-Tube feeding, Pancrelipase
Routine Vitals infection
Tylenol: PRN
Hot compress: RUE extravasation
Treatments continued….
Clinical Manifestations of CF
 Often
Malnourish; Failure to thrive: appear
thinner and smaller
 Vitamin deficiencies: especially fat-soluble
vitamins (A,D,E,K)
 Possible development of DM as pancreas
declines
 Pulmonary manifestations
 CXR: persistent infiltrates and increased
anterior posterior (AP) diameter.
Relationship of Secondary dx
 Dx
of MRSA on
admission day
 Dx of Rhino Virus:
7th day of
hospitalization
Developmental Stage and
Developmental Theory to Care

Personal/social/cognition:



Erikson’s Psychosocial
Theory: 4th stage
Industry vs. Inferiority (612 years old)
Enjoys working with
others; tends to follow
rules; forming social
relationships takes on
greater importance.



Norms meet?
Why or why not?
Application of theory to
care and
communication


Speech/language:


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Give similarities/differences
Understand concepts of
space
Fine motor/adaptive:



Likes to play & compete
More sociable
Looks after own needs
Likes school, answer questions
Gross motor:


Always on the go; jumps
Likely to overdo; hard to quiet
down
Exceptions to the Norms


Appears lethargic “slept remainder of the
day”  Grand parents (day prior admission)
Vitals:


BP (141/83 mmHg); HR: 112 bpm
Pain: 6/10 Face Scale

Poor eating habits/wt. gain

Infections: MRSA/Rhinovirus

Extravasation of CT dye: RUE
Nursing Diagnosis #1:
Acute/Chronic Pain
 Supporting
Data:
--r/o hx of pancreatitis;
-- extravasation of CT dye
--LL flank pain: 6/10 hx of pancreatitis
--RUE swelling/pain: 6/10 (CT dye
extravasation) gallstone
-- HR: 112 bpm; BP: 141/83 mm Hg
 Meds:
Phytonadione,
Ursodiol(prophylactic), Warm compress,
Tylenol PRN, Child life specialist
(bedside/CAT scan)
Acute Pain: Holistic Nursing care
and Interventions

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Assess pain
characteristics
q4h/PRN (use pain
scale: 6/10)
Observe non-verbal
cues
Respond immediately
to c/o of pain
Prophylactic
treatment: Ursodiol
(gallstones)
Distraction Technique
Expected Outcome:
Pt will have decreased
swelling of RUE and pain
level will be in satisfactory
level (2/10 face scale)
 Evaluation: Not met:
Pt. level went down but still
4/10 on FACE scale. BP still
remains elevated during pt.
care.

Nursing Diagnosis #2: Ineffective
Airway Clearance/Impaired Gas
Exchange
 Supporting
data:
Hx of CF, coughing
 Medications:
Albuterol, CPT (qid), Flovent, Advair,
Dornase, Pantanase
Ineffective Airway clearance/Impaired Gas
Exchange Holistic Nursing Care &
Interventions

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Monitor RR, breathing
effort & areas of
cyanosis q4hrs
Assess O2 Sat and
administer
supplemental O2 PRN
Auscultate lung sounds
q4hr
Continued pulmonary
treatment QID
Flovent & Advair
bid(2puffs)
Expected Outcome:
Pt.’s SpO2 will be
maintained at >/= 92%
RA throughout
hospitalization.
Evaluation: Met
Pt. SpO2 was 100% RA
before end of shift.
Nursing Diagnosis #3: Imbalance
Nutrition less than body
requirements/GI
 Supporting
Data:
Decreased appetite, pt. refused to eat,
NPO, fever, constipation, increased
metabolic need due to CF, constipation
 Meds:
5 cans of ensure (115 ml/hr)night
time feeding via GT tube, pancrelipase,
vitamins, Miralax, Pt. gaining wt.
Imbalanced Nutrition: less than body
requirements Holistic Nursing care
and Interventions
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Assess daily wt.
Monitor attitudes towards
eating/food
Pancrelipase enzymes
Tube feed at
115mL/hr(night time feed)
Multivitamins BID
Miralax for constipation
High fat, calorie, protein
diet

Expected Outcome:
Pt. will maintain or gain
more than admission body
wt. before discharge.
Evaluation: Met
Pt. gains 0.2 kg during 9th
day of hospitalization

Nursing diagnosis #4: Infection
 Supporting
data:
Positive Rhino Virus, MRSA, mucus in the
lungs, Isolation(contact/droplet), RAC,
Central IV (portacath), GT tube feeding
 Medications:
CPT, Tobramycin, Meropenem,
Levofloxacin, Linezolid
Infection Holistic Nursing Care and
Interventions



Assess for signs of
infection q4h

Encourage &
promote hand
washing and other
aseptic interventions
 (PPE),
Contact/Droplet
Isolation

Antibiotic treatment
Expected Outcome:
Pt. temperature will stay
within normal parameters
(36.4*C-37.4*C)based on
age during hospitalization.
Evaluation: Met
Pt. temp remain within
accepted parameters:
36.3*C 1330; 36.9*C 1500
before and during end of
shift.
Nursing Diagnosis #5: Anxiety
Supporting data:
--Pt. verbalizes fear of telling pain “I don’t want to tell
the doctors I’m on pain because the more I get hurt”
--Patient uncooperative with tx/interventions(refused
to eat)
--CT dye extravasation

Meds:
Sertraline, Adderall
 Alternative Therapy: pt. playing games with Child
life volunteer, interacting/playing with RN, Child
life volunteer (at bedside & CT scan procedure)

Anxiety Holistic Nursing Care and
Interventions
 Acknowledge
pt.’s
anxiety
 Coping strategies
 Reduce
stimuli
sensory
 Maintain
manner
a calm

Expected Outcome:
Pt. will be more open to
discuss feelings and fears
(pain level) about medical
condition during
hospitalization

Evaluation: Partially
Met
Pt. more truthful about pain
level measures, however still
uncertain pt’s openness to
discuss feelings
Discharge Teaching & Planning

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Continue to monitor and assess pt. pain level
Continue to monitor patients breathing pattern or
any signs of decreased SpO2 (cyanosis etc)
Prevent exhaustion (too much playing, moving
around)
Maintain adequate nutrition/feeding pattern
Continue to monitor patient vital signs and
document abnormal parameters and administer
PRN meds (Tylenol)
Continue to assess and monitor pain level:noting
any discrepancy between facial and verbal
response.
Encourage more open communication with pt.
through play method
Research
Name of Journal:
Journal of Pediatric
Nursing
Title of Research:
Distraction
Techniques for
Children Undergoing
Procedures: A
Critical Review of
Pediatric Research
Conclusion
References

Gulanick , M., & Myers , J. (2011). Nursing care plans: Diagnosis,
interventions, and outcomes . (7 ed., pp. 1-991). St Louis, MO: Elsevier
Mosby

Hockenberry, M. & Wilson, D. (2010). Nursing care of infants and children,
(9th ed.). St. Louis, MO: Elsevier Mosby

Ignatavicius, D. D., & Workman, M. L. (2013). Medical surgical nursing
(7th ed.). ST. Louis, MO: Elsevier Mosby.

Koller, D., Goldman, R. D., (2012). Distraction techniques for children
undergoing procedures: A critical review of pediatric research. Journal of
Pediatric Nursing, 27, 652-681.