Blue Team Survival Guide (Revised 2/27/2012, JL Goralski MD and JE Pittman MD)

Blue Team Survival Guide (Revised 2/27/2012, JL Goralski MD and JE Pittman MD) Rounds: - Meet either at desk at 5 Children s (weekdays at 8:30AM) or in 5 Children s Conference Room in CICC (weekends at 8AM) - It is usually a pretty large team Attending, fellow, pulmonary home IV nurse (Benjamin Butler), ward team coordinator (Meredith George), dietician (Kelly Baumgartner/Kimberly Stephenson), pharmacist, respiratory therapist (Kelli Johnson), resident, intern and medical students - Useful to know/ present recent PFT s, best in past year, recent sputum culture results. PFT s presented in the following order: forced vital capacity (FVC), forced expiratory flow in one second (FEV1), forced expiratory flow 25:75 (FEF 25:75), all given in percent predicted. When the attending asks for the best in the last year, they are usually referring to the best FEV1. - In your presentation and the daily progress note, comment on the respiratory complaints that brought them into the hospital. i.e., cough continues to improve and is less productive, wheezing is less pronounced, etc. Always make sure to mention how much oxygen the child is requiring. - When you go in the room as a team, the Blue Team intern should lead the discussion with the family/child. This will help establish you as an integral team member in the parents eyes. Admissions: - Look at recent clinic notes- these are usually very detailed, accurate, and useful. - Three different admission order sets in CPOE for Ped Cystic Fibrosis PUL and Meds, Patient care, and Labs - If the child is being admitted following a bronchoscopy, we very often see fever in the first 24-30 hours post-operatively. These fevers do not require a typical sepsis workup. Note also that if the child has had bronchoalveolar lavage (BAL) the CXR will often look like pneumonia in the segment that was lavaged. CXR cannot distinguish between BAL fluid and pneumonia. Usually a CXR is not even ordered postbronch, unless it is needed to confirm PICC placement. Inpatient CF admissions: All need nutrition consult, physical therapy consult, and respiratory therapy consult. Usually obtain sputum cultures on admission if not recently obtained in clinic. Labs that are done on an annual basis include sputum for AFB, liver function testing, PT/INR, Vitamin A, D, and E levels, and IgE. If these have not been done in the last year, order them with the admission labs except for vitamin levels, which are often drawn at the end of admit (levels are often low with acute illness). Annual screening also includes a CXR, audiology evaluation if he/she has been on aminoglycosides, and an oral glucose tolerance test (age >10). Do not order these upon admission, but feel free to bring it up on rounds that the patient is not up to date. Meds usually updated in WebCis; this list reflects what the patient should be taking but it may not be what they are actually using. When in doubt, point out the discrepancy to the attending/fellow. Choosing antibiotics for CF: When choosing an antibiotic regimen for the patient, the pulmonary team generally looks at cultures over the past year and tries to cover all the known bacteria. Older kids usually respond well to regimens that have been used in the past, so check the old discharge summaries. Patients may respond to regimens to which they appear resistant by sensitivities. Pseudomonas and Burkholderia are always double-covered (not with 2 agents of the same class (e.g. ceftazidime and zosyn both B-lactam drugs), but occasionally regimens are designed that include drugs that have been shown to have in vitro resistance. You should try to

come up with a regimen on your own that you think would be adequate coverage, but always run the antibiotics by the fellow before ordering them. Dosing of antibiotics is often different in CF patients (different metabolism/clearance, need to get penetration into mucus) check guide below and speak with pharmacy. Labs are done routinely to screen for toxicity. In general, once on a stable dose of an aminoglycoside, check BUN/Cr twice weekly, trough levels weekly. Depending on the drug, weekly CBC and LFT s may also be necessary. Discharges - It is crucial to keep the brief discharge summary up to date. The home IV coordinators fax this to the home care companies as the preliminary orders for medications, often 2-3 days before the patient is discharged. If you change a dose on a medication that the child will be going home with, update it in the brief discharge summary so the pharmacy can mix the correct dose. - If the patient has been admitted for a CF exacerbation, use the following term as your discharge diagnosis CF bronchopneumonia caused by (pseudomonas, ORSA, etc.) This is the way it needs to be documented for billing purposes. - If at all possible, make sure follow up in pulmonary clinic for CF patients is scheduled prior to discharge - Follow-up visit should typically include PFTs, which are scheduled separately (speak to schedulers or Meredith PFT clinic code is 662). Pulmonary Function Tests (PFT s) or spirometry: - FVC / FEV1 / FEF 25%:75% are reported in both absolute liters and percent predicted for that age/ sex/ race - PFT s results will be in patient s paper chart as well in Pulmonary Results in WebCis (though these usually take 1-2 days to show up); the inpatient fellow is often paged with the results as well - PFT s usually obtained either upon admission or at most recent clinic visit - PFT s usually obtained weekly in CF patients to assess response to therapy - No PFT s in someone who has a pneumothorax! Miscellaneous meds: (more information below) - Pancreatic enzymes (Creon, Pancreaze, Zenpep, Ultrase) - weight based maximums and dosed in lipase units/kg per meal and per day. Maximum is typically 10-15,000 lipase units/kg/day. For example, Creon 12 means that there are 12,000 lipase units in each capsule of Creon. - Phytonadione (vit K) - 5 mg PO at least twice weekly while on antibiotic therapy since antibiotics also wipe out the good gut flora that converts Vit K; dosed daily if PT/INR already elevated on admission. - CF specific vitamins include SourceCF, ADEK, Vitamax, among others. These have the supplemental fat soluble vitamins that CF children have difficulty absorbing from their diet. - Pulmozyme (dornase alfa) inhaled once or twice daily to liquefy sputum to make it easier to cough out - Hypertonic saline (HS) - concentrations of 3%, 5.45%, or 7%. HS helps hydrate secretions and reduces exacerbation frequency. It is usually inhaled 2-4x daily. - inhaled antibiotics you may encounter include TOBI (inhaled tobramycin), Cayston (inhaled aztreonam), and colistin. All these treat pseudomonas; there is currently no inhaled therapy for staph. Be aware that when you say TOBI, that means the drug is being inhaled. Tobra indicates IV tobramycin very different in their toxicities! - In general, CF patients take their inhaled medications in the following order: albuterol, HS, airway clearance maneuver (VEST, flutter, acapella, CPT), pulmozyme, then inhaled antibiotics or inhaled corticosteroids. - NO IBUPROFEN for children receiving renal-toxic drugs. Give Tylenol instead for pain or fever.

Fat Soluble Vitamin Levels: A,D,E,K - CF patients have difficulty absorbing fat soluble vitamins, so they need to take extra CF-specific vitamins. Vitamin levels are screened for at least yearly and take about a week to come back. - INR obtained on admission (best surrogate for measuring functional deficiency of Vitamin K) if INR is elevated, Vit K may need to be dosed daily. IgE Levels: - Levels usually obtained on admission to screen for Allergic Bronchopulmonary Aspergillosis (ABPA), but takes several days to weeks to come back - > 500 is abnormal in CF (>1000 in asthma) but the trend is more important than any single value, so check back to their last value - If very high may treat with steroids/ antifungals for ABPA Chest Physiotherapy (Chest PT) - Usually done 4x per day while hospitalized +/- albuterol pre-treatment - Order Respiratory therapy consult - Can be manual or device driven (VEST, acapella, flutter, Metaneb, Duet) - needs to be coordinated with inhaled drugs to be most effective Central lines: - If a patient has a Port or gets a new PICC line, need to put order in CPOE to use it - Search CVAD, choose CVAD with Flushes (Pt GT 5kg) - Choose the appropriate line and complete the rest of the screens Asthma - Any child admitted to the PICU for asthma gets a pulmonary consult; most are transferred to Blue team before discharge if there is room - A new asthma order set and protocol is available. This will standardize care for asthma across services and locations (ED, PICU, Floor). This order set is not applicable for children under the age of 2, children admitted with viral bronchiolitis or croup, or those with a medical history of CF/chronic lung disease/ cardiac disease/ or airway anomalies (see attached protocol). - In transfer note, document history of asthma, current home asthma medications, previous hospitalizations or history of intubation, allergic/infectious triggers, PICU interventions (continuous albuterol therapy, IV SoluMedrol, IV magnesium, terbutaline drip and/or heliox). - Review and revise orders in CPOE, dump all the PICU-specific stuff (turn q2 hours, rhythm strip printouts q12, etc.) - Start on home controller medicines if not already started (Qvar, Flovent, etc.). Some kids are not on a controller medicine at home...they will be when they are discharged. Order oral prednisone if not on already. 1-2mg/kg, 60mg/day max. Usually 5 days total steroid therapy but guidelines are for 3-14 days of oral steroid therapy, so you can adjust depending on how the kid looks. - New asthma order set includes: Ability to do q2 albuterol on the floor x 3 then q3h x 4 Standard is high-dose MDI albuterol (4-8 puffs) instead of nebulized medication Pediatric Asthma Score Assessment (to follow treatment response, determine when to wean) Protocol-driven weaning of meds -Can go home when stable on home treatment and after asthma education completed. Prior to discharge, all patients must have an asthma action plan in place. Administer flu shots for those hospitalized during flu

season. Should have follow up appointment with pulmonary scheduled before discharge (typically in 1-2 months) - Asthma education on the Blue Team is provided by Kelli Johnson, RT or Lisa Alarcon, RN. For weekend discharges, RT s may help with asthma education. TRACHEOSTOMY -After a new trach is placed, the child remains in the PICU for 7 days post-op, until the first trach change (must be done by ENT to avoid creating a fistulous tract). - When you accept a tracheostomy patient, ensure the following is by bedside at all times. If anything is missing, inform the nurse. - Sign indicating size/type of tracheostomy tube, suction depth, and the day of the week for the scheduled trach change - A spare trach in the size that the child needs - A spare trach one size smaller. This is for emergency situations where you cannot get the trach back in. - Suctioning should only be done to the depth of the end of the trach tube. Any further and you are likely to cause granulation tissue and bleeding. During your time on Blue Team, you should develop proficiency in suctioning and changing trachs. - If a rapid response is called on a child with a trach due to respiratory distress or desaturations, try suctioning first. If no response, change the trach it may be plugged even if you don t get any secretions back. If you can t get the same size trach back in, use the smaller one and notify ENT. If all else fails, the tracheotomy site can be occluded with a finger or gauze/tape and the child bagged from above. - Trachs are often colonized with bacteria, so in general, don t send a trach aspirate unless there is clinical concern for infection (fever, increased WBC, change in trach secretions, etc.). - Don t be afraid of trachs! They are the most stable airways on the floor! Peds PFT Orders Charge Code Test Name CPT Code 57220014 Arterial Blood Gas Draw 36600 57230013 Airway Resistance PD 94360 57230047 Carbon Mon Diffus Capacity PD 94720 57230054 Flow Vol Loop Pre/Post PD 94060 57230070 Lung Vol Body Box PD 94360 57230088 Lung Vol N2 Washout PD 94240 57230096 Max Insp/Exp Musc Press PD 95831 57230104 Maximum Voluntary Ventilation 94200 57230179 Respiratory Flow Vol Loop PD 94375

57230179 Spirometry Pediatric 94010 57230195 94060 94060 Reading List: Link to Website with miscellaneous readings on Pediatric Pulmonology: http://pediatrics.med.unc.edu/education/uncpeds/intranet-folder/rotation-readings/pulmonologyreadings/pediatric-pulmonology-reading-list#cystic fibrosis

Medication Guidelines for Pediatric Cystic Fibrosis Patients Megan Young, Pharm.D. Pediatric Pharmacy Satellite Hours: 0700-2330 Pediatric Pharmacy #: 966-6679 Blue Team Pharmacist Pager #: 347-1375 After 2330, call Central Pharmacy for questions: 966-8761 Oral Antibiotics Amoxicillin/Clavulanic Acid: 40 mg/kg/dose BID (max single dose = 2000 mg) 500 and 875 mg tablets; 200 and 400 mg chewables; XR tablets 1000 mg ES suspension 600 mg/5ml (less clavulanic acid). Cefpodoxime: 5 mg/kg/dose BID (max single dose = 400 mg) Cefuroxime Axetil: 20 mg/kg/dose BID (max single dose = 1 gram) If using suspension: 30 mg/kg/dose BID 250, and 500 mg tabs; (suspension 125 and 250 mg/5ml nonformulary, tastes terrible) Cephalexin: 25 mg/kg/dose QID (max single dose = 1 gram) 250 and 500 mg capsules; 125 and 250 mg/5ml suspension Ciprofloxacin: 20 mg/kg/dose BID (max single dose = 1 gram) 250, 500, and 750 mg tabs; XR tablets 500 and 1000 mg; 250 and 500 mg/5ml suspension ** Do not give oral suspension via a G-Tube or NG tube Clarithromycin: 15 mg/kg/dose BID (max single dose = 500 mg) 250 and 500 mg tablets; XL tablets 500 mg; 125 and 250 mg/5ml suspension Clindamycin: 10 mg/kg/dose TID (max single dose = 600 mg) 150, and 300 mg capsules; 75 mg/5ml suspension Dicloxacillin: 25 mg/kg/dose QID (max single dose = 500 mg) 250, and 500 mg capsules Doxycycline: 5 mg/kg initial dose, followed by 2.5 mg/kg/dose BID (max single dose = 100 mg) 50 and 100 mg capsules, capsules may be opened Levofloxacin: < 5 yr: 10 mg/kg/dose q 12 hours (max single dose = 750 mg) > 5 yr: 10 mg/kg/dose q 24 hours (max single dose = 750 mg) > 50 kg 750 mg q 24 hours

* Consider continuing q 12 hour dosing even if > 5 yr 250, 500, and 750 mg tablets; 25mg/mL liquid Linezolid: < 12 yr: 10 mg/kg/dose q 8 hours (max single dose = 600 mg) 12 yr: 10 mg/kg/dose q 12 hours (max single dose = 600 mg) * Consider continuing q 8 hour dosing if > 12 yr but less than 45 kg 600 mg tablets (may cut tablets in-half for 300mg doses); 20 mg/ml suspension Minocycline: 4 mg/kg initial dose, followed by 2 mg/kg/dose BID (max single dose = 100 mg) 50 and 100 mg capsules, capsules may be opened TMP/SMX (trimethoprim/sulfamethoxazole): 7.5-10 mg/kg/dose of TMP component BID 80 mg TMP/400 mg SMX tab; 160 mg/800 mg SMX DS tab; 40 mg TMP/200 mg SMX/5mL suspension **May divide TID/QID for nausea with BID dosing** Intravenous Antibiotics Double-cover Pseudomonas, do not combine anti-pseudomonal beta lactams, as they can be antagonistic Initial doses for Tobramycin and Amikacin should be based on patients previous doses; see pharmacist or WebCis consult note for past dosing information Amikacin doses can be calculated from old gentamicin and tobramycin pharmacokinetics If patient has not received aminoglycosides in the past, use the following guide for dosing Amikacin: 20 mg/kg/dose q 12 hours Aztreonam: 50 mg/kg/dose q 6 hours (max single dose = 2 grams) Cefepime: 50 mg/kg/dose q 8 hours (max single dose = 2 grams) Ceftaroline: 600 mg IV q12 hours (minimum weight = 30 kg no pediatric dosing) Ceftazidime: 75 mg/kg/dose q 8 hours (max single dose = 2 grams) Ceftriaxone: 50 mg/kg/dose q 12 hours (max single dose = 2 grams) Cefuroxime: 75 mg/kg/dose q 8 hours (max single dose = 1.5 grams) Ciprofloxacin: 10 mg/kg/dose q 8 hours (max single dose = 400 mg) Clindamycin: 13.3 mg/kg/dose q 8 hours (max dose/day = 4.8 grams) Colistin: 1.6-2.3 mg/kg/dose q 8 hours (max single dose = 100 mg) **Monitor renal function closely** Imipenem: 20 mg/kg/dose q 6 hours (max single dose = 1 gram) **May need to decrease dose to 10-15 mg/kg/dose q6 hours due to nausea and vomiting** Levofloxacin: < 5 yr: 10 mg/kg/dose q 12 hours (max single dose = 750 mg) > 5 yr: 10 mg/kg/dose q 24 hours (max single dose = 750 mg) > 50 kg 750 mg q 24 hours

Linezolid: < 12 yo: 10 mg/kg/dose q 8 hours (max single dose = 600 mg) 12 yo: 10 mg/kg/dose q 12 hours (max single dose = 600 mg) * Consider continuing q 8 hour dosing if > 12 yr but less than 45 kg **Bacteriostatic** Meropenem: 40 mg/kg/dose q 8 hours (max single dose = 2 grams) **Reserve use for resistance to other antibiotics** **May need to decrease dose to 25-30 mg/kg/dose q 6 hours due to nausea and vomiting** Oxacillin: 50 mg/kg/dose q 6 hours (max single dose = 2 grams) Piperacillin/Tazobactam: 100 mg/kg/dose q 6 hours (max single dose = 4 grams of piperacillin) Telavancin: 10 mg/kg q24 hours (minimum weight = 40 kg no pediatric dosing) Ticarcillin/Clavulanate: 100 mg/kg/dose q 6 hours (max single dose = 6 grams of ticarcillin) **Infuse doses > 3 g over 1 hour** Tobramycin/Gentamicin: 6 mg/kg/dose q 12 hours ** If aminoglycoside naïve and > 16 yr, start 5 mg/kg/dose q 12 hours Vancomycin: 25 mg/kg/dose q 8 hours Nontuberculous Mycobacterium Antibiotics Please start only ONE drug per day to assess for Nausea / Vomiting from each drug Recommended Regimens (vary by physician preference) Cefoxitin: 50 mg/kg/dose IV q 6 hours (max single dose 2 grams) For convenience at home may receive 3 g IV q8h Amikacin: 15 mg/kg/dose IV q 12 hours (if on > 2 drugs) **** Want measured peaks of 20 30 **** Tigecycline: > 8 years: Day 1: 2 mg/kg/dose IV OTO (max single dose 100 mg) Day 2: 1 mg/kg/dose IV q 12 hours (max single dose 50 mg) **Data currently supports q 12 hour dosing research is being done on q 24 hour dosing. If using for indication other than NTM dosing interval is always q 12 hours Clarithromycin or Azithromycin*: * May substitute linezolid, fluoroquinolones, or imipenem Clarithromycin: 30 mg/kg/dose PO QHS (max single dose 1 gram) May divide as 15 mg/kg/dose PO q 12 hours for N/V

Azithromycin: 500 mg PO q 24 hours (> 40 kg) 250 mg PO q 24 hours (if < 40 kg) Antifungals (lots of drug interactions) Fluconazole: 12 mg/kg IV/PO q 24 hours (max single dose = 800 mg) 50, 100, 150, 200 mg tablets; 50 and 200mg/5mL suspension Itraconazole: 5 mg/kg PO q 12 hours (max single starting dose = 200 mg) 100 mg capsules, 50mg/5mL solution ** Give with food, acidic juice, or cola Give capsules with food, give suspension on an empty stomach (suspension has better absorption than capsules) Posaconazole: 10 mg/kg PO q12 hours (max single dose = 400 mg) 40mg/mL suspension Voriconazole: IV Loading Dose < 12 yr: 8 mg/kg q 12 hours x 2 doses IV Maintenance Dose < 12 yr: 7 mg/kg q 12 hours IV Loading Dose > 12 yr: 6 mg/kg q 12 hours x 2 doses on day 1 IV Maintenance Dose > 12 yr: 4 mg/kg q12 hours PO Loading Dose: 8 mg/kg/dose q 12 hours x 2 doses on day 1 (Max single dose = 400 mg) PO Maintenance Dose: 7 mg/kg/dose q 12 hours (Max single dose = 200 mg) PO available as 50 and 200 mg tablets; 40mg/mL suspension

Fluconazole Itraconazole Posaconazole Voriconazole Renally Dose Adjust Yes No No No, but do not use IV formulation if CrCl<50 Drug Interactions PPIs: May decrease fluconazole levels PPIs: May decrease itraconazole levels PPIs: May decrease posaconazole levels PPIs: May increase voriconazole levels May increase tacrolimus levels May increase tacrolimus levels May increase tacrolimus levels May increase tacrolimus levels Meals Capsules: food increases absorption Capsules: food increases absorption Best absorption with a high-fat meal Food may decrease voriconazole absorption Solution: food decreases absorption. Take on empty stomach Solution: food decreases absorption. Take on empty stomach Give 1 hour before or 1 hour after a meal Steady State Day 5 of therapy Day 14 of therapy Day 5 of therapy Day 5 7 of therapy in the absence of a loading dose Day 2 of therapy following a loading dose Goal Trough Concentrations (30 min prior to dose) N/A Itraconazole + hydroxyitraconazole > 1 and < 10 mcg/ml > 700 ng/ml 1 5.5 mcg/ml Recommend Adjustments N/A < 1 mcg/ml increase dose by 50% < 700 mg/ml increase dose by 50% < 1 mcg/ml increase dose by 50% > 10 mcg/ml decrease dose by 25-50% > 5.5 mcg/ml decrease dose by 25-50% (may consider alternative agents if clinically significant toxicities) Synergy for Staphylococcus aureus: Rifampin: 10 mg/kg/dose PO q 12 hours (max single dose 600 mg) Pharmacokinetic Monitoring for Aminoglycosides and Vancomycin Peaks and troughs of aminoglycosides are monitored for efficacy and safety, beginning with the 3 rd or 4 th dose. The trough is drawn immediately before a dose; the dose is given and followed by a flush; peak is drawn 2 hours after hanging the dose. All peaks and troughs must be obtained via peripheral sticks. Desired parameters are as follows: Tobramycin: trough < 1 mg/dl; extrapolated peak 12-15 mg/dl Amikacin: trough < 5 mg/dl; extrapolated peak 35-45 mg/dl Usually monitor troughs only for Vancomycin. Trough is drawn immediately before the dose is due. Desired parameters are: Vancomycin: trough 15-20 mg/dl BUN/SCr should be obtained twice weekly. Ensure adequate hydration and discontinue all other nephrotoxic drugs (including ibuprofen at any dose). Patients should also receive an annual hearing evaluation to evaluate for ototoxicity from aminoglycosides

If on vancomycin + aminoglycoside or vancomycin q6h obtain BUN/SCr three times weekly Nebulized Medications Albuterol: 2.5-5 mg nebulized QIDPPD Hypertonic Saline: to transition patient to 7% 3% Hypertonic Saline: 5mL nebulized BID-QIDPPD 5.45% Hypertonic Saline: 4mL nebulized BID-QIDPPD 1mL 0.9% Sodium Chloride + 3mL 7% Sodium Chloride 7% Hypertonic Saline: 4mL nebulized BID-QIDPPD Dornase Alfa (Pulmozyme ): 2.5mg/2.5mL ampule nebulized QDAY - BID Use with PARI LC + nebulizer Nebulized antibiotics are administered via nebulizer after the patient s usual inhaled treatments (including bronchodilators and Dornase) and chest physiotherapy. Amikacin: Doses vary based on patient tolerance (outpatient therapy only) 250 mg nebulized BID, if tolerated increase to 500 mg nebulized BID Aztreonam: 75 mg nebulized TID Patients must supply own medication, Altera device, and cleaning supplies for inpatient use Colistin: Doses vary based on patient tolerance < 6 years: 75 mg nebulized BID > 6 years: 150 mg nebulized BID Tobramycin: 300 mg nebulized BID Vancomycin: 250 mg nebulized BID (outpatient therapy only) Other CF Therapies Pancreatic Enzyme Supplements: Use patient s home brand, OK to use home meds if not available at UNC. Infants: Initiate therapy with 1 capsule of Pancreaze 4,200 or equivalent mixed with one teaspoon of applesauce at beginning of feed. Do not place enzymes in baby bottles. Usual doses: 1500-2500 units lipase/kg/feed. Children: Initiate therapy with 500-2000 units lipase/kg/meal. Usual doses are 10,000-15,000 units lipase/kg/day. Goals: Adjust doses based on degree of steatorrhea and stool frequency: 3-4 stools/day for infants; 1-2 stools/day for children and adolescents. If enzyme requirement is large (>3000 units lipase/kg/meal) consider use of a H 2 blocker (acidity inactivates enzymes). Do not exceed 3000 units/lipase/kg/meal. Doses > 20,000 units/kg/day increase risk of fibrosing colonopathy. Common Pancrelipase Products Product Dosage Form Lipase Units Amylase Units Protease Units Pancreaze 4,200 4,200 17,500 10,000

10,500 Capsule, enteric 10,500 43,750 25,000 16,800 coated microspheres 16,800 70,000 40,000 21,000 21,000 61,000 37,000 Creon 3,000 3,000 15,000 9,500 6,000 12,000 Capsule, enteric coated microspheres 6,000 12,000 30,000 60,000 19,000 38,000 24,000 24,000 120,000 76,000 Zenpep 3,000 3,000 16,000 10,000 5,000 5,000 27,000 17,000 10,000 Capsule, enteric coated microspheres 10,000 55,000 34,000 15,000 15,000 82,000 51,000 20,000 20,000 109,000 68,000 Vitamin D Supplementation Cholecalciferol Age Routine dosing with CF specific vitamins (IU) Step 1: Dose increases (IU) 25OH = 20-30 * Step 2: Dose titration maximum (IU) 25OH = 10-20 * Birth 12 months 400 500 800 1,000 Not > 2,000 > 12 months 10 yr 800 1,000 1,600 3,000 Not > 4,000 > 10 yr 18 yr 800 2,000 1,600 6,000 Not > 10,000 > 18 yr 800 2,000 1,600 6,000 Not > 10,000 * Indicates total vitamin D (IU) intake per day, including Source CF vitamins ** Adapted from 2011 CF Update on Treatment of Vitamin D Deficiency 1 Source CF Softgel/Tablet = 1,000 units cholecalciferol ADEK/SourceCF Vitamins: 1 SourceCF chew tab = 2 ml liquid SourceCF < 1 yr: Liquid SourceCF vitamins 1 ml QDay 1-3 yr: Liquid SourceCF vitamins 2 ml QDay May initiate chewable vitamins (1 QDay) at 2 yr 3-9 yr: SourceCF chewable vitamins 1 QDay > 10 yr: SourceCF chewable vitamins 2 QDay Vitamin K: For patients receiving oral or intravenous antibiotics < 1 yr: 2.5 mg PO additional twice each week > 1 yr: 5 mg PO additional twice a week May require daily vitamin K if PT elevated at admit Salt Supplement: Add supplemental salt to newborn and infant formula and food < 6 months: 1/8 teaspoon per day

> 6 months 1 yr: 1/4 teaspoon per day Ursodiol: 15 20 mg/kg/dose PO BID, (Max: 1000 mg/dose) Azithromycin (Anti-inflammatory) < 25 kg: 10 mg/kg/dose PO Q MWF 25 39kg: 250 mg PO Q MWF > 40 kg: 500 mg PO Q MWF REFERENCES 1. Taketomo CK, Hodding JH, Kraus DM. Pediatric Dosage Handbook, 17 th edition, 2010 11. 2. Gibson RL, Burns JL, Ramsey BW. Pathophysiology and management of pulmonary infections in cystic fibrosis. Am J Resp Crit Care Med 2003;168:918-51. 3. Nelson Textbook of Pediatrics 18 th Edition by Kliegman, Behrman, Jenson, and Stanton; 2007, Elsevier, Inc. 1119. 1182. 4. Olivier KN, Yankaskas JR, Knowles MR. Nontuberculous mycobacterial pulmonary disease in cystic fibrosis. Seminars in Respiratory Infections. 1996;11(4):272-84. 5. An official ATS/IDSA statement: Diagnosis, treatment, and prevention of nontuberculous mycobacterial disease. Am J Respir Crit Care Med. 2007;175:367-416. 6. Zobell JT, Ampofo K, Cash J, Korgenski K, Chatfield BA. High dose ticarcillin-clavulanate administration in pediatric cystic fibrosis patients. Journal of Cystic Fibrosis 2010;9:280-3. 7. Andes D, Pascual A, Marchetti O: Antifungal therapeutic drug monitoring: established and emerging indications. Antimicrob Agents Chemother 2009;53(1):24-34. 8. Hope WW, Billaud EM, Lestner J, Denning DW: Therapeutic drug monitoring for triazoles. Curr Opin Infect Dis 2008;21:580-586. 9. Pascual A et al. Voriconazole TDM in patients with invasive mycoses improves efficacy and safety outcomes. CID 2008;45:201-211. 10. Scott LJ, Simpson D. Voriconazole: a review of its use in the management of invasive fungal infections. Drugs 2007;67:269--98. 11. Tangpricha et al. An Update on the Screening, Diagnosis, Management and Treatment of Vitamin D Deficiency in Individuals with Cystic Fibrosis: Evidence-Based Recommendations from the Cystic Fibrosis Foundation. Ahead of Print. Revised 4/12 - Megan Young, Pharm.D.