Cystic Fibrosis Center Newsletter - Lifespan...Cystic Fibrosis Center Newsletter 2014 Summer Edition Greetings to all as we usher in another summer in the Ocean State. We have been

Cystic Fibrosis Center Newsletter 2014 Summer Edition

Greetings to all as we usher in another summer in the Ocean State. We have been busy at the Center, working on several

established and newer projects, identified since our last newsletter. In follow up of several

items from my last report:

Thanks to all who have re-signed consent for the CFF patient registry.

For infection control, the new guidelines were published last week. Notification of changes will be shared with you in clinic.

I also plan to send a separate mailing once we have an implementation plan.

We continue work on getting genetic testing done through the DNA Diagnostics Lab at Johns Hopkins in the Mutation

Analysis Program. We identified those who need testing and are working through the process with you. If you think you are

eligible, please contact Pam or ask us at your next clinic visit.

In the Pediatric Program, we will introduce an annual behavioral health assessment. Dr. Kristen Benito, the center’s child

psychologist, will do the assessment. She provides more details in her article.

Our biggest personnel news is the retirement of Dr. Robert Klein, the Pediatrician in Chief for Hasbro Children’s Hospital

and the Chair of the Department of Pediatrics at the medical school. Dr. Klein has been very supportive of the center and

helped me move forward with many initiatives. We all wish him well, as we look forward to new leadership.

“Always bear in mind that your own resolution to succeed, is more important than any other one thing.”

Abraham Lincoln

Annual Health Behavior Assessment From your CF Center Psychologist, Kristen Benito PhD As part of the CF clinic’s mission to provide comprehensive care, we are now providing an Annual Health Behavior assessment for all children and adolescents. The purpose of this assessment is to understand how families fits CF treatments into their lives. We understand that adherence to all of the CF treatments is difficult for many families. Information from this assessment will be important so that the CF team knows how to best support your family in keeping up with your child’s treatments.

What can you expect during the Health Behavior Assessment?

I will meet with your family for about one hour. I will ask you questions about CF treatments and aspects of your life that are related to the treatments, as well as about other behaviors related to your health, such as exercise and sleep. I will also ask you to fill out a short questionnaire.

When will the Health Behavior Assessment take place?

The Health Behavior Assessment will take place during one of your usual clinic visits and will happen once per year.

Will the clinic visit be longer on the day this assessment is completed?

On that day, you will meet with me and then with your physician. You may also complete PFTs. When possible, we will have you meet with other team members (such as Nutrition and Social Work) at the next clinic visit, so the length of your visit will be minimized.

Who can you contact with questions?

You can contact Dr. Benito (401-793-8125) or Pam (401-444-3860) with any questions. You can also ask questions at your next clinic visit. This link, from the CF Foundation, is a good source of information about the challenges and importance of CF adherence:

http://www.cff.org/images/customcontent/HLFeb01GA.pdf.

Rhode Island Hospital / Hasbro Children’s Hospital

Center Director’s Update: Karen L. Daigle MD

Breathe In – Breathe Out - Repeat

Stacie Caddick-Dowty RRT, AE-C

You may have recently heard about a high school student’s research project that studied peppermint oil in a nebulizer to help treat Pseudomonas Aeruginosa. We recently

received the following notice.

Please read below as this practice is NOT recommended by the Cystic Fibrosis Foundation. -Stacie

Dear colleagues, Dr. Rebekah Brown at Vanderbilt notified us that she was receiving questions from patients regarding peppermint oil and Pseudomonas.

The source of the questions appears to the description of a study presented at an Intel-sponsored science fair. For this particular project, a teenager performed an in vitro study and found that peppermint oil had a comparable effect on Pseudomonas as the comparison antibiotic, tetracycline.

http://www.intel.com/content/www/us/en/education/competitions/international-science-and-engineering-fair/bacteria-remedy-isef-2013.html?wapkw=pseudomonas

The web story (which has now been taken down) suggested that a few drops of peppermint oil in the nebulizer may be helpful to patients with CF.

Small doses of peppermint oil taken orally appear to be safe (http://nccam.nih.gov/health/peppermintoil), but we are not aware of data suggesting that it is safe when taken by nebulizer.

Of course, we recommend against the use of peppermint oil in a nebulizer and wanted to alert you that patients may ask you about this issue.

Sincerely,

Preston W. Campbell, III, M.D. Executive Vice President for Medical Affairs Bruce C. Marshall, M.D., M.M.M. Senior Vice President of Clinical Affairs

Born in: Madison, Wisconsin but grew up in Rhode Island

Fond childhood memories: My grandmother braiding my hair in the morning before school. Fishing with my Dad.

Hobbies: Photography, reading, following my 7 year old daughter on her many activities

Favorite travel destination: My front porch on a sunny afternoon

Favorite movie(s): Hook, ET, The Wizard of Oz

Favorite book(s): Snowflower and the Secret Fan, Middlesex, A Wrinkle in Time

Fears/phobias: Public Speaking

Dreams/goals: To see my daughter graduate from college and make a happy life for herself.

Staff Spotlight Kathy Cooney LICSW

Cool off with this refreshing and

berry delicious parfait!

INGREDIENTS – MAKES 2

SERVINGS:

2 – 4 oz vanilla pre-prepared pudding cup 1 cup fresh cut berries (or any other favorite fruit) 1 – 6 oz vanilla yogurt (use a thick and creamy yogurt), divided 1 – 6 oz strawberry yogurt (use a thick and creamy yogurt), divided 1/2 cup granola 1/2 cup sliced almonds

DIRECTIONS:

Prepare 2 – 8oz plastic cups.

Place 1 – 4 oz vanilla pudding in each cup.

Spoon ¼ cup fresh fruit over top.

Spoon ½ container of strawberry yogurt

overtop and smooth surface.

Spoon ½ cup of granola over top.

Repeat with ¼ cup fruit, ½ container of

vanilla yogurt and top off each parfait

with ¼ cup sliced almonds.

Approximately 590 calories and 15 grams of

protein per serving!

Helpful Tips:

Make this recipe the night before, place in

the refrigerator and it is “Ready to Go” for

breakfast.

For those allergic to milk, make your own

instant pudding with soy milk and use a

soy based yogurt.

Substitute salted peanuts for the almonds for

a salty, summer kick. Add a tablespoon of

honey and wheat germ.

Pediatric CF Center Dietitian:

Maria Santini, RD, LDN, CNSC

Education Committee:

Stacie Caddick-Dowty, Pam

Marciniak, Maria Santini and

Karen Swartz.

The committee rolled out the quarterly

education topics in January.

We started with “Airway Clearance” and

“Energy Requirements” and will soon be

moving on to “Order of Therapies” and

the “Ins and Outs of Enzyme Therapy”.

The “Topic of the Quarter” is

highlighted on the CF Bulletin /

Information Board located in CF clinic.

We plan to evaluate our education

efforts with a repeat questionnaire

once all education sessions have been

completed. If you’ve missed a session

or need a little refresher, let your

Education Team know.

Happy Learning!

Transition: Led by Kathy Cooney.

The group has redone the process of

tracking the progress of each patient

through the transfer of care to

the Adult program.

Led by Maria Santini and Dr. Daigle.

We started using the road map for managing

patients with suboptimal nutritional status in

January. Pediatrics...

Ocular Safety of Ivacaftor

Treated Patients: this is a 2 year study of children 11 years or younger being treated with ivacaftor (Kalydeco®).

We enrolled 1 patient.

Fibrosis Colonopathy in CF :

We are now in year 2 of this 10 year observational

study. The aim is to identify risk factors for

developing this unusual problem in the large

intestine. Any patient who has GI or abdominal

problems that might suggest the problem will be

asked to participate. We are monitoring but have

not enrolled any patients.

Adult Care Patients

Pediatric Care Patients

Genotyping:

In the Adult Program, a project was designed to

identify and track testing

Cystic Fibrosis Adult Center News

OGTT Quality Improvement Project

Rachel Drywa, Jeanne Houtchens, Pam Marciniak and Camille White

The Cystic Fibrosis Foundation recommends that everyone who has CF and who does not have diabetes get an Oral Glucose Tolerance Test (OGTT) every year beginning at age 10. The OGTT is a blood test that helps determine whether you have CF-related diabetes (CFRD), impaired fasting glucose (IFG) and/or impaired glucose tolerance (IGT).

Untreated CFRD can lead to weight loss, inability to gain weight, and/or a decrease in lung function. Early diagnosis and treatment of CFRD helps to maintain optimal weight and lung function.

To improve the percentage of adult patients having an OGTT completed yearly, we are now offering OGTTs during clinic visits in our adult program. If you are due for your annual OGTT, we will send a letter with instructions 1 – 2 weeks before your appointment. We ask that you arrive at 8:30 am and plan to stay in clinic for at least 2 ½ hours to complete the test.

Happy Summer Everyone!

Jeanne Houtchens RNP

Just a quick note regarding the changes that have happened over the

last year. As some of you may know, there have been changes to the

direct admission policy on Jane Brown 4 North. Since September of

2013, CF Adults patients are admitted to the medical teaching service

at RI Hospital.

Most of you know that RI Hospital is the major teaching hospital for

Brown University. Consequently most of the hospitals units have

interns and residents caring for patient under the direct guidance of

attending physicians.

Now when you are admitted to the Jane Brown, you will be cared for by

physicians on the teaching service with Pulmonary consultation

(Drs. Corrao, Donat, and Stanchina and Jeanne Houtchens NP).

Because of the change, we can only directly admit those patient who have

been seen in the clinic same day or within the previous week. If you are

sick, we will make every effort to fit you into adult clinic that week to

assess for inpatient admission. As always, you can go directly to the ER

for evaluation for admission.

Our last item that we are working on is medication reconciliation.

It is of the utmost importance that we get your medication list as

the hospital is transitioning to a new medical charting program

called EPIC. Accurate medication lists are important to keep

track of medication interactions, prior authorizations as well as

medication refills. So, if we can ask all of the Adult patients to bring

in your medications or an up to date medication list to your next 2

clinic appointments to that we may import them into your current

medical chart. Any questions or comments, please feel free to

contact myself or Pam.

THANK YOU to all walkers, (including our very own

Julia Estrela, Stacie Caddick-Dowty and Dr. Daigle)

donors, volunteers and sponsors for a successful 2014

Great Strides Season! We are $1.8 Million closer to a

cure at the MA/RI chapter thanks to your dedication and

generosity! Remember fundraising is open until

the end of the year. GO GREAT STRIDES!

Check the MA/RI Chapter web page for chapter news/updates

as well as “upcoming events”.

7/23/14: Sail Narragansett Bay on the schooner Aurora

9/27/14: CF Cycle for Life / Little Compton