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Cystic Fibrosis Newsletter Welcome! Meet the new Pulmonologist! Dr Lokesh Guglani started his medical training in India, where he went to medical school in New Delhi and then pursued further training in the US. He did his General Pediatrics residency training at State University of New York at Buffalo at the Women's and Children's Hospital and is a board-certified pediatrician. During his training, he received the Thomas F. Frawley Research Fellowship award and was also adjudged the best resident in his class, having received the Theodore I. Putnam Pediatric Resident Award. He was always intrigued by physiologic simplicity and yet the mechanistic complexity of respiratory disorders in children and decided to pursue fellowship training in Pediatric Pulmonology. He completed his Pediatric Pulmonology fellowship training at Children's Hospital of Pittsburgh, which is one of the leading pediatric centers for respiratory care, including a pediatric lung transplant program and a large CF center heavily invested in CF research. His research during fellowship training focused on the immune response to chronic lung infection with Mycobacterium tuberculosis. After having rubbed shoulders with some of the best faculty in Pediatric Pulmonary Medicine, he has now joined Children's Hospital of Michigan Pulmonary Division from July 2011 onwards. His interests include lung inflammation, cystic fibrosis research, and lung function testing (including Infant Pulmonary Function testing). October 2011 Volume 25 Issue 1 Upcoming Events: Family Advisory Board Meeting Dates: *10/18/2011 *12/13/2011 CF Family Education Day 11/19/ 2011 Other Cystic Fibrosis Center News: Hello! My name is Ruby Lam and I will be partnering with Deborah Hachey as the CF nurse coordinator. Some of you may have already met me in passing as I have been a nurse with the Pulmonary division for almost 4 years. Deb and I can be reached at the same phone number (313) 745-5267. I look forward to meeting you all! Inside This Issue: Preferred Sequence for inhaled Medications 2 Reminder!!!!!!!!!! Please Join Us! Hypertonic Saline: 3 Cystic Fibrosis Family Education Day Vest Treatments 3 When: November 19, 2011 Cystic Fibrosis Foundation 4 Time: 9:00am Quiz: Test your knowledge! *Flu shots and updated cystic fibrosis information 4 Preferred Sequence for Inhaled Medications Lokesh Guglani MD There are a number of inhaled medications that patients with cystic fibrosis are taking on a regular basis to stay healthy. However, there is uncertainty regarding the correct order in which these inhaled medications should be taken. To get the maximum benefit from these medications and to get the most out of airway clearance therapies, here are some general rules to follow: Start with Albuterol (Ventolin ® or Proventil ®) nebulization or puffs. This can possibly help open up the airways and improve the delivery of the medications that will follow. However, it is important to remember that not all patients with cystic fibrosis show a response to Albuterol (roughly 30% do). Hypertonic saline (Hypersal ®) or DNase (Pulmozyme®) should be used next. This will help make the secretions of thinner consistency, before they can be shaken loose with the vest and then coughed out. The airway clearance treatment should be next. This could be your Vest treatment, acapella/flutter device or manual chest physical therapy for infants and toddlers. This is the mainstay of your airway clearance regimen and its goal is to move as much of the thick secretions from the small corners of the lung out towards the large airways (like the windpipe) from where they could be coughed out. Infants and toddlers do not cough out the secretions but tend to swallow them. Inhaled antibiotics (TOBI®, Cayston®, Colistin etc.) are always taken after the completion of vest treatment session. It is preferable to do some coughing to get as much secretions out before you start the inhaled antibiotic. It is important to use the correct nebulizer device for each antibiotic. For instance, the Altera ® nebulizer cannot be used for inhalation of any other antibiotic besides Cayston®. If using inhaled steroids (Flovent®, Advair® or Symbicort®), then they should be taken as the very last treatment. Remember to rinse your child’s mouth (or give infants and toddlers something to drink) after this last treatment to remove any residue of the medication(s). These are general suggestions and not all patients are taking all of these medications at any given time. Following this sequence can help the patients get the most out of the time and effort spent on these daily treatments. If you have any questions related to your airway clearance regimen or inhaled medications, please feel free to discuss them with your CF doctor at your next clinic visit. October 2011 Page 2 Hypertonic Saline: What is it, and is it for me? Candace Horvath, CPNP You may not have ever heard of hypertonic saline before, or you may have heard about it, but have some questions. It is extra salty water that is sterile, which means there are no germs in it, that is inhaled. It is used as an additional method of airway clearance when you have Cystic Fibrosis How do you use it and how does it work? The solution comes in a 4 milliliter vial and used with a Pari nebulizer just like other inhaled therapies. Hypertonic saline, or Hypersal, is usually prescribed to be used twice per day. This solution should not be mixed with other medications for inhalation. It is also not safe to make your own salty water, because we can not be sure there are no germs in the water or that it is the right amount of salt. It is thought that the extra salty water drawls moister into the airways helping to thin out secretions. A study funded by the Cystic Fibrosis Foundation found that patients using hypertonic saline had better lung function during the study, and had fewer lung infections than those not using it. What are the side effects? Some side effects of the inhalation could include: an increase in coughing, a sore throat, and chest tightness. Hypertonic saline can irritate the airways. Most of the time patients are told to use a bronchodilator, such as albuterol, before Hypersal to help open up the airways. Who should use hypertonic saline? The study on hypertonic saline was used to patients that were 6 years old and older, with a FEV1 greater than or equal to 40% predicted. More studies are needed to know if this medication is useful in younger patients or patients with a lower FEV1. Hypersal is not meant to replace any current medications or treatments a patient is taking. It is another helpful tool in treating cystic fibrosis. Questions? If you have any further questions about hypertonic saline, or are wondering if it is right for you, please to not hesitate to ask one of your Cystic Fibrosis Team members for more information! Vest Treatments Marcy Haase, RT Performing your breathing treatments and airway clearance therapy (CPT) everyday is very important when treating your CF. Many of our patients have a vest machine to perform their CPT . The families and patients have been trained how to use these machines, for many of you that was a long time ago. During our patient’s clinic appointments many of our patients and families have had questions about the vest settings and if they should be changed since their child has aged and/or grown. If your child’s vest settings have not been updated or adjusted within the last 3 years, please consult your CF doctor, nurse or respiratory therapist to ensure that your child continues to receive the maximum benefit. During your clinic visit, feel free to discuss the vest with either your doctor or one of the respiratory therapists with additional questions. You can also call the pulmonary medicine office and speak to the respiratory therapists Julie Nutting, or Marcy Haase (313745-5543) with questions about the vest. October 2011 Page 3 Dept. of Pulmonary Medicine 3901 Beaubien Blvd. Detroit, MI 48201 Phone: 313-745-5541 Fax: 313-993-2948 E-mail: [email protected] October 20—The 8th Annual Lansing Area Wine Opener October 27 - Cooking for a Cure, Saginaw October 28—2nd Annual Run Like Hell 5k, Ferndale November 10—Moonlight and Martinis, Novi November 12 - 2nd Annual Southeast Michigan’s Finest, Troy For additional details, please visit http://detroit.cff.org Test Your Airway Clearance Techniques 1.What are Airway Clearance Techniques (ACT)? a. Means to gain weight 4. You should stop half way through your ACT session to cough. b. Way to administer medication a. True b. False c. Methods to help clear secretions from the lungs d. Use of nebulized/aerosol treatments only 2. All of the following are airway clearance techniques, 5. The purpose of ACT is: EXCEPT: a. Clear the nasal passages b. Prevent spread of CF to others c. Loosen the mucus so it can be coughed out of the lungs d. Wash out the lungs and cure CF a. b. c. d. e. VEST therapy Manual Chest Physiotherapy (CPT) Flutter Acapella Yoga 3. How long should you do ACT in one session? a. b. c. d. 5 min 6. Daily airway clearance will improve your Pulmonary Function Tests (PFT). a. True b. False 20 min 10 min 50 min Answers: 1. c 2. e 3. b October 2011 4. a 5. c 6. a Page 4