9/30/ year-old Caucasian female. Lives with her husband and 1 child, who does not have CF

Transcription

1 Sarah Fitz, APN, MSN, ACNP-BC Loyola 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 1

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) C c C c Cc CC cc Cc Inheritance Lungs Pancreas Gallbladder Liver Bowel 2

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) 3

4 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) 4

5 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 5

6 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 6

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 7

8 MIC Time (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? 8

9 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

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, bisacodyl suppositories PRN First sign of constipation, call RN Pancreatic enzyme replacement is key 10

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 medications per day Exchanging one set of problems for another 11

12 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 12

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 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), S from 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 com.proxy.cc.uic.edu/chapter/ / _1/fulltext.html Davies, J. C. (2015). The future of CFTR modulating therapies for cystic fibrosis. Current Opinion Pulmonary Medicine, 21, Donaldson, S. H. & Boucher, R. C. (2006). Pathophysiology of cystic fibrosis. Annales Nestle, 64, 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, 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, Grossman, S. G. & Grossman, L. C. (2005). Pathophysiology of cystic fibrosis: implications for critical care nurses. Critical Care Nurse, 25(4), 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 ). 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