Optimizing Respiratory Therapy for Adult Cystic Fibrosis Patients

Creating an effective daily respiratory therapy regimen is crucial for adults with Cystic Fibrosis (CF) to maintain healthy lungs and good lung function. This includes inhaled medications such as nebulizer treatments and bronchodilators, airway clearance techniques like the Vest and Pep devices, as well as exercise. Understanding the order in which to take medications is important for optimizing their effects. Key classes of inhaled medications include bronchodilators, mucolytic agents, antibiotics, and inhaled steroids. Utilizing a combination of these treatments can improve lung function and reduce exacerbations in CF patients.

• Respiratory Therapy
• Cystic Fibrosis
• Adult Patients
• Inhaled Medications
• Lung Function

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1. NEBS & VESTSWHAT IS BEST? Gauri Pendharkar, RCP, CPFT Respiratory Therapy Coordinator Adult Cystic Fibrosis

2. 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

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

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

5. #1 Bronchodilators Open and relax the airways Remaining medications can get into lower airways Irritants such as hypertonic saline better tolerated Examples: Albuterol Atrovent Duoneb/Combivent (combination of Albuterol and Atrovent) Xopenex

6. #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 lasts at least 4 hours Trimble et al. (2018)

7. #3 Pulmozyme/Dornase Alpha Extracellular DNA makes mucus thicker and stickier Cuts sputum s DNA thin sputum, easier to expectorate Standard dosage is 2.5 mg recommended to be taken daily Dornase alpha vs placebo Improved lung function Decreased pulmonary exacerbations Yang et al. (2006)

8. #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

9. #4 Airway Clearance Move the 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, G5) Huff coughing Active Cycle of breathing Autogenic Drainage

10. #4 AWC: Percussive Vests

11. 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)

12. #4 AWC: Other devices for AWC OPEP IPV

13. #4 AWC: Percussive Devices

14. #4 AWC: Huff Cough & Postural Drainage

15. #4 AWC: Active Cycle of Breathing

16. #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.

17. #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)

18. #5 Inhaled Antibiotics: Colistin Also known as Colistimethate or Polymixin E Commonly used to treat Pseudomonas infections. Standard dosage: 150 mg in 4 mL 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)

19. #5 Inhaled Antibiotics: Cayston (aztreonam) Monobactam antibiotics Usual dose 75mg 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)

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

21. Nebulizers Image result for photos of nebulizer cups Pari LC Plus Pari Sprint Sidestream Erapid, Altera

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

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

24. 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.

25. Neb Cleaning: Hot Method

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

27. 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)

28. Exercise & Yoga Russell, et al. (2015)

29. Exercise & Yoga Russell, et al. (2015)

30. 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

31. References Jensen T, Pedersen SS, Garne S, Heilmann C, H iby 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...-a0187427765 (accessed January 31 2018) Plosker GL. Aztreon1: Plosker GL. Aztreonam lysine for inhalation solution: in cystic fibrosis. Drugs. 2010 Oct 1;70(14):1843-55. doi: 10.2165/10484070-000000000-00000. Review. PubMed 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.: CD001127. DOI: 10.1002/14651858.CD001127.pub3 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.