WHAT YOU KNOW, THOUGHT YOU KNEW, AND EVERYTHING YOU REALLY DIDN’T KNOW

Cystic Fibrosis

Kari Paca BSN, RN, CPNClinical Nurse II

(Cystic Fibrosis Liaison Level 8/9)

Inspiration

One Republic

Overview of Topics

● Pathophysiology of Cystic Fibrosis● Treating CF Pulmonary Exacerbations● Nutrition Basics ● CF Related Diabetes● Isolation and Infection Control for Patients● How to Work With This Challenging Population● The Future of Treatments

Basics of CF

● Average life expectancy currently is 38 years of age● Genetic, autosomal recessive disorder● Dysfunctional CFTR protein that doesn’t allow for

salt and water to cross the epithelial cell membrane correctly○ Thickened secretions in exocrine glands

● Systems affected○ Airways, sinuses, GI and reproductive

● Major Organs○ Lungs and pancreas

Basic Mutations

Normal I II III IV VNo

SynthesisBlock in

processingBlock in Gating

Altered conductance

Reduced Synthesis

G542D F508del G551D D1152H 3849-10KBc

% of patients with an allele of this class

12 % 87 % 5% 5 % 5%

(Boyle, NACF Conference 2014)

What Happens in the Lungs

● Lack of functioning CFTR● No fluid layer present● Sticky thick mucus that traps white blood cells,

bacteria, fungi, etc. ● Perfect environment for infection● Increased inflammation

● Increased amount of pro-inflammatory cytokines● Studies have shown early inflammation in infant lungs without the onset of

an infection (Courtney et al, 2004)

https://www.nacfconference.org/plen.archive.html

What Happens in the Lungs

● Endobronchitis● Bronchiectasis● Atelectasis● Complications

● Pneumothorax● Pneumonia

Scarring

Mucous Plug

CT Scan 14 Yr old Male

CT Scan of 16 yr old Male

Chest X-Ray Same 16 Yr old Male

Chest CT 6 Yr old Female

CF Exacerbations

● Common Bacteria● Staph Aureus

● MRSA/MSSA● Psuedomonas Aeruginosis● B. Cepacia (several strains)

● Fungus● Aspergillus

● Viruses● H. Flu● RSV

● MAC ● Mycobacterium Avium Complex

Treatments

● Typical stay is 10-14 days (YIKES)● Main focus=airway clearance.

● Increased to 3-4 times per day ● Vest, IPV, EzPap

● Antibiotics● Nebulizers

● Hypertonic Saline, Toby, Pulmozyme, Cayston● Exercise● Monitoring PFTS

● Want FEV1 to be very close, if not back to baseline

GI Tract and Cystic Fibrosis

● Abnormal CFTR protein affects:● Pancreas● Liver● Intestines

● Complications● Malabsorption● Impaired glucose tolerance● CFRD

Nutrition

● Significantly higher caloric needs● Children age 4-6 need 2,000-2,800 cal/day● Teens need 3,000-5,000 cal/day

● Malnutrition is associated with higher morbidity and mortality rates in individuals with CF (Davies et al, 2002)

● High Fat/ High Protein foods ● Does not mean just sugar

● Salt supplementation (usually younger kids)● BMI and lung function are highly correlated● Enzymes before meals, snacks, overnight feeds

CFRD

● Not DM Type I or II● Has components of both● Don’t make enough● Insulin resistance

● Most common comorbidity related to CF● About 16% of CFers have it● Gradual process ● Treatment

● +/- insulin● Not a limit on calories, still watch sugar● Blood sugar monitoring● OGTT

● Annual starting at age of 10 or earlier if suspected CFRD

What to Monitor

● Taking enzymes (time sensitive)● Blood sugar checks● Bowel Movements

● Are they having them?● Are they “normal”?● Distension or tenderness

● Supplements● New Process● Considered a snack for enzyme requirement

Infection Control and Isolation

● CF Glove and Gown● Least limiting● To protect them● Go anywhere with mask (including the fitness center)● Can be in teen lounge as long as no other CFer is in it

Infection Control and Isolation

● CF Droplet● Limiting ● Cannot go outside room to common areas● Can go directly outside wearing mask● Need to wear gown, glove, and mask for PFT● Specific signs for MRSA and B. Cepacia

Infection Control and Isolation

● Droplet● Trumps CF Droplet● When a pt has a virus● Cannot go outside of room period● Wears gown, glove, and mask to PFTs

Ask Your ISS for the in-room Signs!

Strategies to Work With Challenging Patients

● Long stays = more stress● BE A PRIMARY!!● Get Child Life Specialists involved

● Behavioral contract● Psych consults● Make a written schedule of their day with them● Make sure they have the new hospital handbook● Make sure the medical team knows if they always want

bedside rounding...even on the weekend● Meal cards for after hours

What is to Come of CF Treatment

● No Cure● New medications on the horizon

● Ivacaftor● Lumicaftor

● Investigating gene therapy● Incorporating Psychologists in care ● New methods to make it easier to take

respiratory equipment everywhere ● Increased rates of survival post lung-

transplant● 50% are alive at 5 years.

Thank-You!

● CF Team (outpatient) x76181● E-mail me: kari.paca@childrenscolorado.org● www.cff.org ● https://www.nacfconference.org/

Citations

● Boyle, M. (Director) (2014, October 8). Scaling the Mountain: The Journey to Delivering Transformational CF Therapeutics.. North American Cystic Fibrosis Conference. Lecture conducted from Cystic Fibrosis Foundation, Atlanta.

● Courtney, J., Ennis, M., & Elborn, J. (2004). Cytokines and inflammatory mediators in cystic fibrosis. Journal of Cystic Fibrosis, 3(4), 223-231. Retrieved March 13, 2015, from EbscoHost.

● Davies, P., Erskine, J., Hambidge, K., & Accurso, F. (2002). Longitudinal investigation of energy expenditure in infants with cystic fibrosis. European Journal of Clinical Nutrition, 56(10), 940-946. Retrieved March 13, 2015, from Academic Publishing Group.

● WWW.CFF.ORG