Therapy

    Frequency

Chest Physiotherapy

Hand, Vest, Flutter, Acapella, (Exercise)

BID+

Nebulized:

-Βeta-2 agonist

Albuterol* (may mix with hypertonic saline)

BID+

-Hypertonic saline

7% HyperSal^® solution*

BID

-Mucolytic

Pulmozyme*

q day – BID

-Inhaled Antibiotics

Tobi^®*

colimycin

BID for 28 days qo month

Macrolides

azithromycin

250mg (<40kg)

500mg (>40kg)

po q M, W, F

Infection Control

Disinfection, sterilization nebulizer sets

Hand washing

Each use

Subjective features

·          Fatigue

·          Anorexia

·        Dyspnea

Objective features

·          New or increased cough from baseline

·          Weight loss

·          Lower oxygen saturation from baseline

·          Hemoptysis (either a new onset or any increase from baseline)

·          Change in sputum: color (white to yellow/green,) quantity, and tenacity

·          Decline in forced expiratory volume in 1 second (FEV1) of 10% or more from baseline

·          Crackles in the lungs on physical examination*

·          Fever*

·          Infiltrate on Chest radiograph*

·          Elevated White blood cell count (WBC)*

< 6years

6-12 years

13-17 years

>=18 years

·        New crackles

·        Increased cough

·        Decline in weight-for-age percentile

·        Increased sputum

·        Decline in FEV₁ (% predicted)

·        Increased cough

·        New crackles

·        New P. aeruginosa

·        Decline in FEV₁(% predicted)

·        Increased cough

·        New crackles

·        Hemoptysis

·        Decline in FEV₁(% predicted)

·        New crackles

·        Hemoptysis

·        Increased cough

          Admission

Weekly while on IV’s

Comments

Sputum Culture (CF culture)

X

Acid Fast Bacilli (AFB) culture

if none done in 12 months

Consider if febrile, worsening bronchiectasis, or failure to respond to therapy

Viral Swab RSV/Influenza

if signs/ symptoms

Chest Radiograph

X

Pulmonary Function Testing

if patient able

                X

Treat until pulmonary function returns to baseline or plateau in FEV1

High Resolution CT scan chest

As needed

CBC with differential

X

                X

Liver function

X

                X

BUN/Creatinine

X

                X

Glucose

X

If hyperglycemia, monitor 2º post-prandial glucose for CF-related diabetes

PT

 if having hemoptysis

Administer Vitamin K, monitor until normalizes

Drug Levels:

    vancomycin

    tobramycin

                X

                X

Aim for tobramycin peak 10 times the  Minimum Inhibitory Concentration (MIC) to P. aeruginosa in mcg/L

Characteristic

Acute

Chronic

Onset of clinical symptoms

Rapid

Slow

Bacterial population

Clonal

Polyclonal, polyspecies

Growth

Planktonic

Planktonic & biofilm

Antibiotic treatment goal

Eradication

Suppression, palliation

Consequences of antibiotic failure

Death

Accelerated disease progression

Sputum Culture

Antimicrobial

Agents

Chest Physiotherapy

(Hold CPT only if massive or submassive hemoptysis)

GI

Infection

Control

Other

Antimicrobials

P. aeruginosa alone

Anti PA and tobramycin*

·   Increase CPT with albuterol nebulizer treatments to q4-6º

·                   -Optimize nutrition (1.5-2.0 x RDA)

·                   -No limit of calories/fat even if CF-Related diabetes

·                   -Optimize pancreatic enzymes

·                   -ADEK vitamins

Private

  Room

-Maintain home TOBI cycle

-Continue chronic macrolide therapy

- Physical Therapy

P. aeruginosa with methicillin-sensitive S. aureus

Add nafcillin**

(or use

piperacillin/ tazobactam or

ticarcillin/ clavulenate

for Anti PA with tobramycin)

·   Consider increasing pulmozyme to bid

Private

  Room

P. aeruginosa with methicillin-resistant S. aureus

Add vanc or linezolid***

·    Hypertonic saline 7% 3 ml by neb bid

·    0₂ to keep saturation  > 92%

Private

  Room

Contact

-Consider corticosteroids if:

      -RAD component

      -ABPA

      -palliation of dyspnea

Multi Drug – Resistant

  P. aeruginosa with or without

A. xylosoxidans

B. cepacia

S. maltophilia

Meropenem AND

-tobramycin or

-doxycycline/ tobramycin or

-doxycyline/ trimethoprim/ sulfamethoxazole or

-clarithromycin

OR

Cefepine/tobramycin

Private

  Room

Contact

-Consider addition of inhaled Colistin

(if sensitive)

Pick One:

Pediatric Dose

Adult Dose

Comments

Alternative Dosing

-aztreonam

-cefepime

-ceftazidime

-imipenem/cilastin

-meroperem

-ticar/clav

-pip/tazo

50 mg/kg IV q8º

2 g IV q8º

Useful in PCN β-lactam allergy

50 mg/ kg/dose IV q8º

2 g IV q8º

Continuous Infusion

Load 15 mg/kg then 100 mg/kg/24º

50mg/kg/dose IV q8º

2 g IV q8º

15-25 mg/kg IV q6º

0.5-1.0 gm IV q6º

40 mg/kg IV q8º

2 g IV q8º

Continuous Infusion

125-250 mg/hour for 24º

100 mg/kg IV q6º

3 g IV q6º

100mg/kg IV q 6º

3 g IV q6º

Continuous Infusion

3.375 g infused over 4 hrs, q8º

Add Aminoglycoside:

-tobramycin  

10-12 mg/kg IV q24º

Same

Aim peak 10 times MIC PA to tobramycin

3-15 mg/kg/dose IV q 8º with peak 10-12 µg/mL; trough < 1-2 µg/mL

-amikacin

7.5 mg/kg IVq8º

Same

Peak 25-30 µg/mL; trough < 5 µg/mL

15 mg/kg IV q24º

doxycycline

5 mg/kg/day q12º

300 mg

May be beneficial in combination with TMP/sulfa/ doxy or meropenem/doxy

TMP/sulfa

4-5 mg/ kg of TMP q12º

4-5 mg/ kg of TMP q12º

May be beneficial in combination with TMP/sulfa/ doxy or menopenem/doxy