NEBS VESTSWHAT IS BEST Gauri Pendharkar RCP CPFT

NEBS & VESTS…WHAT IS BEST? Gauri Pendharkar, RCP, CPFT Respiratory Therapy Coordinator Adult Cystic Fibrosis

Respiratory Therapy for CF patients For Healthy Lungs and maintaining good Lung Function : Good respiratory therapy practices at home A daily Respiratory Therapy Regimen: Inhaled Medications (nebulizer treatments, MDI, Podhaler) Airway Clearance (Vest, Pep, OPEP, Percussors, Manual CPT etc. ) Exercise

MEDICATIONS Classes of Inhaled medications Bronchodilators Ø Albuterol, Atrovent, Xopenex, Duoneb, Combivent Mucolytic Agents ( Mucus thinners) Ø Hypertonic Saline (3% or 7%), acetylcysteine [Mucomyst] (10%, 20%), dornase alpha [Pulmozyme] Antibiotics (Tobramycin, Colistin, Cayston) Inhaled Steroids (Advair, Flovent, Symbicort)

Does Order Matter? Bronchodilators Hypertonic saline Pulmozyme Mucomyst AWC – Flutter, Acapella, Aerobika, Manual CPT (with or after above) Antibiotics Inhaled Steroids

#1 Bronchodilators Open and relax the airways Ø Remaining medications can get into lower airways Ø Irritants such as hypertonic saline better tolerated Examples: Ø Albuterol Ø Atrovent Ø Duoneb/Combivent (combination of Albuterol and Atrovent) Ø Xopenex

#2 Hypertonic saline Mobilize secretions by drawing water into dehydrated airways Either 3% or 7% solution Efficacy: Prolonged single dose effect of HTS on Muco Ciliary clearance Ø lasts at least 4 hours Trimble et al. (2018)

#3 Pulmozyme/Dornase Alpha Extracellular DNA makes mucus thicker and stickier Cuts sputum’s DNA Ø thin sputum, easier to expectorate Standard dosage is 2. 5 mg recommended to be taken daily Dornase alpha vs placebo Ø Improved lung function Ø Decreased pulmonary exacerbations Yang et al. (2006)

#3 Mucomyst Not commonly used due to irritation 10% or 20% concentration Helps reduce viscosity of the mucus Reduce irritation by always using with a bronchodilator

#4 Airway Clearance Move thinned secretions out of the airways to ease expectoration. Multiple modalities: HFCWO (Vest) OPEP devices (Acapella, Aerobika, Flutter) Percussive Ventilation: IPV, Metaneb Percussors: ( Elecro Flo, G 5) Huff coughing Active Cycle of breathing Autogenic Drainage

#4 AWC: Percussive Vests

Comparison of 2 commonly used Vests Sine waveform Technology (used in HR vests) vs triangular waveform pulse technology (Respirtech) A Controlled, blinded, randomized, short term crossover study. Comparable amounts of sputum expectorated using both waveforms Kempanien et al, (Chest 2007)

#4 AWC: Other devices for AWC OPEP IPV

#4 AWC: Percussive Devices

#4 AWC: Huff Cough & Postural Drainage

#4 AWC: Active Cycle of Breathing

#5 Inhaled Antibiotics Prescribed based on the type of bacteria cultured in sputum Most antibiotics are for gram negative rods, mainly to target pseudomonas aeruginosa Nebulized or a dry powder Insurance : This is an important factor in determining what antibiotics the Patient will get per their Insurance coverage and approval.

#5 Inhaled Antibiotics: Tobramycin Aminoglycoside used to treat Pseudomonas Aeruginosa Nebulized as aerosol or dry powder inhaler (DPI or Podhaler) Aerosol: 300 mg twice a day DPI: 28 mg (4) capsules twice a day 24 week study with intermittent administration of Tobi well tolerated improved PFT decreased the density of Pseudomonas in the sputum decreased risk of hospitalization Ramsey et al. (NEJM 1999)

#5 Inhaled Antibiotics: Colistin Also known as Colistimethate or Polymixin E Commonly used to treat Pseudomonas infections. Standard dosage: 150 mg in 4 m. L NS A double-blind placebo-controlled study one million units BID x 3 months vs a placebo inhalation Colistin treatment superior Significantly better clinic symptom scores Maintenance of PFTs Inflammation parameters Jensen et al. (1987)

#5 Inhaled Antibiotics: Cayston (aztreonam) Monobactam antibiotics Usual dose 75 mg three times daily, at least 4 hours apart Only to be used with an Altera nebulizer Rapid inhalation time Two Phase III randomized placebo controlled trials Significant delay in the need for inhaled or IV antipseudomonal antibiotics Significant improvement of respiratory symptoms Plosker, (2010)

Inhaled Steroids Common Inhaled Steroids are Advair (discus or HFA) Symbicort Flovent

Nebulizers Pari LC Plus Pari Sprint Sidestream Erapid, Altera

Compressors Pari Vios Pro Travel- Pari Trek S Omron Respironics Mini-Elite

Metered Dose Inhalers (MDIs) Bronchodilators Inhaled Steroids Combivent, Albuterol, Xopenex Advair, Flovent, Symbicort A Spacer should be used along with an HFA to get more deposition of the medication in the Lungs

Cleaning and Disinfecting of Nebulizers Daily cleaning and disinfection in recommended for all neb cups Prevent contamination of the equipment by germs Prevents further respiratory infections Cold and hot disinfection methods CFF. org is a great resource to learn about this and there are videos explaining the process.

Neb Cleaning: Hot Method

Neb Cleaning: Cold Method Soak in 70% Isopropyl Alcohol for 5 minutes Soak in 3% Hydrogen peroxide for 30 minutes For Altera and e. Rapid nebulizer: Follow the manufacturers instructions

Exercise & Yoga Exercise is important for better overall health Provides energy and improves flexibility A Study of Yoga showed improvement in Chest wall excursion Sit to stand test Russell, et al. (2015)

Exercise & Yoga Russell, et al. (2015)

Exercise & Yoga Russell, et al. (2015)

So what really IS best? Patient adherence to treatments is the weakest link Unless it is consistently done, ANY therapy is futile. Barriers to successful treatments Patient understanding of therapies and their importance. Financial burdens/Insurance (medicines, equipment not covered) Time (job, young children). Pick modes of therapy that are easy to do and do not put an extraordinary stress on patients. Reduce treatment times as much as possible Podhaler or MDI Portable devices to use at work, travel etc. A discussion with your treatment team can help you plan an individualized regimen

References Jensen T, Pedersen SS, Garne S, Heilmann C, Høiby N, Koch C. Colistin inhalation therapy in cystic fibrosis patients with chronic Pseudomonas aeruginosa lung infection. June 1987 Kempainen, R. R. ; Williams, C. B. ; Hazelwood, A. ; Rubin, B. K. ; Mila, C. E. 2008 Comparison of High-Frequency Chest Wall Oscillation With Differing Waveforms for Airway Clearance in Cystic Fibrosis. The Free Library (March, 1), https: //www. thefreelibrary. com/Comparison of High-Frequency Chest Wall Oscillation With Differing. . . -a 0187427765 (accessed January 31 2018) Plosker GL. Aztreon 1: Plosker GL. Aztreonam lysine for inhalation solution: in cystic fibrosis. Drugs. 2010 Oct 1; 70(14): 1843 -55. doi: 10. 2165/10484070 -00000 -00000. Review. Pub. Med PMID: 20836577. Yang C, Chilvers M, Montgomery M, Nolan SJ. Dornase alfa for cystic fibrosis. Cochrane Database of Systematic Reviews 2016, Issue 4. Art. No. : CD 001127. DOI: 10. 1002/14651858. CD 001127. pub 3 Aaron T. Trimblea, , , A. Whitney Brownb, Beth L. Laubec, Noah Lechtzind, Kirby L. Zemane, Jihong Wue, Agathe Ceppea, David Waltzf, 1, William D. Bennette, Scott H. Donaldson Hypertonic saline has a prolonged effect on mucociliary clearance in adults with Cystic Fibrosis. Journal of Cystic Fibrosis. Bonnie W Ramsey, M. D. , Margaret S. Pepe, Ph. D. , Joanne M Quan, M. D. , Kelly L. Otto. M. S. , A. Bruce Montgomery, M. D. , Judy Williams-Warren, M. P. H. , Michael Vasiliev-K, B. S. , Drucy Borowitz, M. D. , C. Michael Bowman, M. D. , Bruce C. Marshall, M. D. , Susan Marshall, M. D. and Arnold L. Smith, M. D for the Cystic Fibrosis Inhaled Tobramycin Study group Intermittent Administration of Inhaled Tobramycin in Patients with Cystic Fibrosis. Scott P Russell, PT, DPT, CCS, CGS; Adupa P. Rao, MD, Lynn Fukushima, George Salem, Ph. D Yoga Improves Posture and Physical Performance in Adult Persons with Cystic Fibrosis.