9/30/2017

1

Sarah Fitz, APN, MSN, ACNP-BCLoyola University Medical Center

36 year-old Caucasian female

Lives with her husband and 1 child, who does not have CF

Previously worked as a CPA, but had to quit due to worsening illness

Diagnosed as an infant d/t failure to thrive

Genetic testing revealed F508del mutation

Referred to CF center, followed with CF clinic throughout life

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2

Autosomal recessive

Cystic fibrosis transmembrane conductance regulator

CFTR – protein

CFTR – gene

Chloride channel disruption

Classes I - VI(Fitz, 2015)(Nissim-Rafinia, et al., 2007)(Donaldson & Boucher, 2006)(Grossman & Grossman, 2005)

Cc cc

CC Cc

Inheritance

C c

C c

Lungs

Pancreas

Gallbladder

Liver

Bowel

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3

Very low percentile on growth chart

Pancreatic insufficiency at a young age – required enzymes early on

D/t ongoing slow weight gain, pt had PEG tube placed early in adolescence, continued enzyme therapy

In early teenage years had a few pulmonary exacerbations but was able to complete high school

Pt was being followed at CF center

More frequent exacerbations

Declining PFT's

Declining exercise/functional capacity

Lumacaftor helps bring the protein to the cell surface

Ivacaftor helps the CFTR protein function

Approved for F508del, probably helpful for many others

Requires PA

(Davies, 2015)

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More frequent – every few months to several weeks

Less responsive to treatment, sometimes requiring longer treatment

Having trouble managing treatments/exacerbations at home

Pseudomonas aeruginosa strain 1

Pseudomonas aeruginosa strain 2

Stenotrophomonas

MSSA

Gram negatives Pseudomonas Achromobacter H. flu Stenotrophomonas Burkholderia cepacia

Fungus Mycobacterium Aspergillus

(Gibson, Burns, & Ramsey, 2003, p. 926)(Chmiel et al., 2014, p. 7)

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Gram positives MRSA MSSA Enterococcus VRE

Can resistance be passed along?

CF airway and antibiotic use

MIC - chronic infections

(Sherrard, Tunney, & Elborn, 2014)

Pseudomonas strain 1 intermediate to piperacillin/tazobactam, susceptible to colistin

Strain 2 susceptible to piperacillin/tazobactam and colisin

Stenotrophomonas – treatment of choice is SMX/TMP

MSSA – should be covered by above antibiotics

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Previous exacerbation was treated with IV piperacillin/tazobactam, intermediate dosing with little improvement after several days.

With continuous IV pip/tazo, improved

Regimen Continuous IV pip/tazo Inhaled colisin PO sulfamethoxazole 800 mg/trimethoprim 160 mg, 2 tabs Q12H

Inpatient hospitalization

Aggressive airway clearance Sodium chloride 3-7% BID Dornase alfa Albuterol 2.5 mg/Ipratropium nebulized every 4 hours Vest therapy or high frequency oscillation IS, flutter valve

Nutrition

Central access

Home care setup

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7

Vest

Flutter valve

Incentive spirometry

Postural drainage

High frequency oscillating nebulization

At least a 2 drug regimen is preferred, especially for treatment of pseudomonas

If possible, multiple routes are helpful in terms of targeting desired organism

(Flume et al., 2009)(Chmiel et al., 2014)

Mimimum inhibitory concentration Listed in your culture results with antibiotic susceptibility profile Can be helpful, but in vivo versus in vitro results can vary

Area under the curve

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8

Time

MIC

(Craig, 2014)

Intermittent

Continuous

Extended infusion

Drawing levels Very helpful Must be used correctly Difficult with home health

Very important in the effort to reduce antibiotic resistance

Can be challenging in terms of treating CF exacerbations –often the lab results don't predict the patient's response.

Is the organism cultured causing the clinical presentation?

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Inhaled antibiotics

Alternating months

Data difficult to obtain

Thick, sticky secretions in the gut as well

Pancreatic insufficiency

Caloric needs can be very high!

Early intervention – NG, PEG, G/J, J tube

Several formulations

Usually patients have a preference or favorite

Use formulation from home if possible

Dosing is based on the lipase amount

Dose based on symptoms (sometimes outside of the normal dosing range)

With meals, snacks, TF. Anything with ANY amount of fat – need enzymes.

9/30/2017

10

Pt presented to ED c/o abdominal pain for 24 hours, worsening in severity over the past 8 hours.

Pt normally takes miralax but had some diarrhea so stopped taking it. Now pt states she has not had a BM in 48 hours.

Abdomen is distended, bowel sounds are hypoactive in both lower quadrants.

Pt is quite nauseated but no vomiting. Hasn't felt like she could eat. Held down meds this morning.

When possible, management is medical first.

Polyethylene glycol, PO or NG

Hold narcotics if possible, or limit

NGT for decompression may be needed, NJ for golytely

Oral nalaxone if narcotics necessary

Need one!

Target 1-3 BM's per day, depending on patient history

This patient was advised to target 3 per day.

Docusate/senna, miralax TID, lactulose PRN, bisacodylsuppositories PRN

First sign of constipation, call RN

Pancreatic enzyme replacement is key

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11

Usually high number of adhesions

Operative time can be extended

High bleeding risk

Intraoperative antibiotic lavage

Required multiple transfusions perioperatively

Primary graft dysfunction

Need to follow antibodies

Extubated POD 2

Challenging pain control, improved once chest tubes discontinued

N/V with restarting TF regimen

Significant improvement in mobility, exercise tolerance

Free of O2

Now taking 20 - 30 medications per day

Exchanging one set of problems for another

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Challenging for such young patients

Want to conform with peer group

In this patient's case, raising a child, household duties, returning to work

Formerly felt sick, now feel better

Lifelong illness

Sick role

Peer group

Self-expression/Self-perception

Education

Work

Reproductive health

Romantic relationships

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13

Chmiel, J. F., Aksamit, T. R., Chotirmall, S. H., Dasenbrook, E. C., Stuart, Elborn, J. S., LiPuma, J. J., Ranganathan, S. C., Waters, V. J., & Ratjen, F. A. (2014). Antibiotic management of lung infections in cystic fibrosis: part I. The microbiome, MRSA, gram-negative bacteria, and multiple infections. The Annals of the American Thoracic Society, 11(7), pp. 1120-1129.

Columbo, C., Ellemunter, H., Houwen, R., Munck, A., Taylor, C., Wilschanski, M. (2011) Guidelines for the diagnosis and management of distal intestinal obstruction syndrome in cystic fibrosis patients. Journal of Cystic Fibrosis, 10(S2), S24-28.

Craig, W.A. (2014). Introduction to pharmacodynamics. In A. Vinks, H. Derendorf & J. Mouton (Eds.), Fundamentals of Antimicrobial Pharmacokinetics and Pharmacodynamics (pp. 3-22) Retrieved from https://link-springer-com.proxy.cc.uic.edu/chapter/10.1007/978-0-387-75613-4_1/fulltext.html

Davies, J. C. (2015). The future of CFTR modulating therapies for cystic fibrosis. Current Opinion Pulmonary Medicine, 21, 579-584.

Donaldson, S. H. & Boucher, R. C. (2006). Pathophysiology of cystic fibrosis. Annales Nestle, 64, 101-109.

Fitz, S. M. (2015, October). Cystic fibrosis in the adult patient. Presentation at ISAPN Midwest Conference, Lisle, IL.

Flume, P. A., Mogayzel, Jr., P. J., Robinson, K. A., Goss, C. H., Rosenblatt, R. L., Kuhn, R. J., & Marshall, B. C. (2009). Cystic fibrosis pulmonary guidelines. American Journal of Respiratory and Critical Care Medicine, 180, 802-808.

Gibson, R. L., Burns, J. L., & Ramsey, B. W. (2003). Pathophysiology and management of pulmonary infections in cystic fibrosis. American Journal of Respiratory and Critical Care Medicine, 168, 918-951.

Grossman, S. G. & Grossman, L. C. (2005). Pathophysiology of cystic fibrosis: implications for critical care nurses. Critical Care Nurse, 25(4), 46-51.

Nissim-Rafinia, M., Kerem, B., Kerem, E. (2007). Molecular biology of cystic fibrosis: CFTR processing and functions, and classes of mutations. Hodson, M., Geddes, D., & Bush, A. (Eds.), Cystic Fibrosis (pp. 49-58). London: Edward Arnold Ltd.

Sherrard, L. J., Tunney, M. M., Elborn, J. S. (2014) Infections in chronic lung diseases 2: antimicrobial restistance in the respiratory microbiota of people with cystic fibrosis. Lancet, 384, pp. 703-714.