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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