CF Case Study Fitz -...
Transcript of CF Case Study Fitz -...
9/30/2017
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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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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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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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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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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.
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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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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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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.