1. Care of the Pediatric Patient with Cystic Fibrosis
Good Morning everyone! Today we are going to discuss on how to care for a patient with cystic Fibrosis.

2. 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

3. 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

4. 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)

5. Cultural Considerations
Divorce household
Under full custody of grandparents since childhood
GT tube feeding
CVAD R chest

6. 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
On the morning of 26 Oct 14 patient TRD was admitted in their local clinic due to extensive coughing.
Besides the fact that my patient had an exacerbation of his CF, another reason for his admission was his c/o LL Back pain. According to his grandparents TRD had a hx of pancreatitis, and they were worried that it is recurring again due to his complaint of pain.
human rhinovirus (HRV), a member of the family Picornaviridae, has been described. Although some studies have suggested a substantial pathogenic role for these viruses (3), controversy still exists (4).
Previous studies of respiratory virus infections in CF patients provided conflicting results on the potential effect of rhinovirus. Smyth et al (15), who evaluated respiratory exacerbations in CF patients, identified rhinovirus as the leading agent, with exacerbations following a more benign course but resulting in greater use of intravenous antimicrobial drugs (15). Olesen et al. (4) studied 75 CF patients for 1 year and used PCR to investigate the presence of 7 different viruses in sputum or laryngeal aspirates. The group reported that viral infections did not reduce lung function or increase respiratory symptoms. Rhinovirus was by far the leading agent identified throughout their study

7. 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

8. Organs affected in CF

9. Treatment Plan
Goals: Treatment of Infections, Airway Clearance, Increased Oxygenation, Prevention of malnutrition:
Antibiotics Therapy
CPT, Bronchodilators, Nebulizer
Supplemental O2 PRN
Continued G-Tube feeding, Pancrelipase
Routine Vitals infection
Tylenol: PRN
Hot compress: RUE extravasation

10. Treatments continued….

11. 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.

12. Relationship of Secondary dx
Dx of MRSA on admission day
Dx of Rhino Virus: 7th day of hospitalization

13. Developmental Stage and Developmental Theory to Care
Personal/social/cognition:
Likes to play & compete
More sociable
Speech/language:
Give similarities/differences
Understand concepts of space
Fine motor/adaptive:
Looks after own needs
Likes school, answer questions
Gross motor:
Always on the go; jumps
Likely to overdo; hard to quiet down
Erikson’s Psychosocial Theory: 4th stage Industry vs. Inferiority (6-12 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

14. 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

15. 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)

16. Acute Pain: Holistic Nursing care and Interventions
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.

17. Nursing Diagnosis #2: Ineffective Airway Clearance/Impaired Gas Exchange
Supporting data:
Hx of CF, coughing
Medications:
Albuterol, CPT (qid), Flovent, Advair, Dornase, Pantanase

18. Ineffective Airway clearance/Impaired Gas Exchange Holistic Nursing Care & Interventions
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.

19. 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.
Pancrelipase (creon): digestive enzyme that is use (sprinkile) in his meal to help digest food since his pancreas is block and not able to produce the enzyme body needs.
Miralax: to help prevent constipation

20. Imbalanced Nutrition: less than body requirements Holistic Nursing care and Interventions
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

21. 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
The role of bacterial infections in CF is well established, and most treatments focus on eradication or suppression of bacterial infections (mainly those caused by Pseudomonas aeruginosa)
Tobramycin: aminoglycosides/antibiotic tx: interferes with protein synthesis of susceptible bacteria
Meropenem: inhibits bacterial cell wall synthesis
Levofloxacin, Linezolid

22. 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.

23. 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)
Sertraline: antidepressant, OCD adjunct, Anxiolytic ( daily PO)
Adderall: CNS stimulant for ADHD and Narcolepsy. Affect chemical reaction in the brain and nerves that contributes hyperactivity and impulse control.

24. Anxiety Holistic Nursing Care and Interventions
Acknowledge pt.’s anxiety
Coping strategies
Reduce sensory stimuli
Maintain a calm manner
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

25. Discharge Teaching & Planning
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

26. Research
Name of Journal: Journal of Pediatric Nursing Title of Research: Distraction Techniques for Children Undergoing Procedures: A Critical Review of Pediatric Research

27. Conclusion

29. References
Gulanick , M., & Myers , J. (2011). Nursing care plans: Diagnosis, interventions, and outcomes . (7 ed., pp ). 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,