Cough it Up, Spit it Out!
Fall 2012
Volume IX, Issue 3
Pediatric Pulmonary Center CF Updates
A Division of Childrens Hospital of Greenville Hospital System (864)454-5530
Special Interest
Articles:
North American CF Conference 2012
• CFTR research
is growing.
• Lots of stories
about our kids,
send me one
about yours!
The NACFC was held in
Orlando, Florida the
second week of October.
We had the privilege of
sending Dr. Jane Gwinn,
Lisa DuBose NP,
Rebecca Peace RT, and
Katie Stogsdill RD from
our clinic.
Individual
Highlights:
This is the largest
gathering of CF
professionals and the
exchange of information is
invaluable. Each provider
came back with new ideas
and strategies to help our
clinic take better care of
• Yummy chicken
dish for a cold
winter night.
Good bye Dr. E 2
your child. There was a
lot of information
regarding Quality
Improvement and we have
already done a couple of
projects related to
evaluating for CF related
diabetes. This year we
plan to look at our
patient’s BMI. The CFF
would like all children to
have a BMI of at least
50%. We are also
considering a project that
reviews annual labs and
chest x-rays, as the CFF
literally ‘grades’ our center
on whether our patients
obtain these screening
measures to alert us of
changes in the patient.
CFF credentials its
centers yearly, and our
ability to stay within their
guidelines falls heavily on
compliance with
appointments and with the
recommendations set
forth in regards to labs,
cultures, lung function and
others. Please help us to
continue to help you care
for your child as the CFF
recommends and do your
best to keep your
appointments.
Dr. James Yankaskas of UNC Adult Program speaks
CF drug pipeline 3
CF living
4
Angels with Roses
5
Our organization6
Our center had the
pleasure of hosting Dr.
Yankaskas of the UNC
Adult CF Program in
September.
Dr. Yankaskas has been
treating CF patients for
roughly the last 30-40
years.
Our CF team was well
represented with this
instruction, as we had the
staff of our office, in
addition to Dr. Mike
Dougherty of Peds GI, Dr.
Elaine Moreland of Peds
Endo, Dr. Robin LaCroix
of Peds ID and Dr. Amy
Treece of GMH adult
pulmonary present.
Dr. Yankaskas reviewed
the aging CF patient and
the needs that are noted.
He also addressed issues
in regards to unusual
bacteria that the patients
can become colonized
with and how to best
intervene.
Dr. Yankaskas also spent
quite a bit of time
discussing the challenges
of an adult CF center. He
stated that UNC was not a
lot different than GMH
about 15-20 years ago
and that with gradual
system organizational
support; he felt that GMH
could potentially have an
adult CF center in the
future. His words of
encouragement and the
presence of one of our
adult colleagues were
very inspiring.
Our center continues to
strive and ask for the
support of an adult
program, but it is you as
the parents who can do
more.
Page 2 of 6
Cough it Up, Spit it Out!
Dr. Elidemir has taken a position in Florida
Dr. Okan Elidemir and his
wife, Anne, left our
practice in late
September.
Dr. Elidemir had the
opportunity to become
Medical Director and CF
Center Director of the
Sacred Heart hospital
which is affiliated with the
Nemours Childrens
Hospital System.
“Dr. E” left with a
saddened heart as he
“CFTR research is
really taking off and
could have some
promising options in the
future for our patients.”
literally built the GMH
Pediatric Pulmonary
division. He was of
course saddened to be
leaving ‘his’ patients.
The move allows their
family to be much closer
to their extended family,
and we all know how
important it is to have
family support.
Dr. E left his patients in
the very capable hands of
Dr. Steve Snodgrass and
the rest of our Pediatric
Pulmonary Division’s care.
We will certainly miss Dr.
E, but don’t feel too sorry
for him, as rumor has it
that their new home is
only a block away from
the beach in Pensacola!
So to Dr. E and Ms.
Anne, we say good bye
and good luck and wear
your sunscreen! 
CFTR Modulator Research is booming!
This summer has been a
busy time for CF
researchers. Most of us
know about Kalydeco for
the CF gene G551D.
Research is now
expanding to see if this
drug could in fact have
positive results when used
in combination with a new
corrector drug “VX-661”.
There is also research
presently with Kalydeco in
combination with VX-809
that is in Phase 2 testing
and will be enrolling in
further trials in the coming
months
and efficacy data must be
obtained PRIOR to any of
these drugs being on the
market.
There is also another
study in Phase 3 of VX809/770 combination
therapy for patients with
CF who have 2 copies of
delta F 508.
There is a possibility that
there will be research
sites that you could enroll
in, but those details have
not yet been released.
This is a very exciting
time, but as exciting as it
is, we still must be patient
and realize that the safety
New website offers info on CF Mutations
The Clinical and
Functional Translation of
CFTR website was
launched in April. It gives
information on more than
160 mutations of CF, with
the goal to better describe
all mutations in the CFTR
gene that are found in CF
persons.
This was done in
collaboration with the CFF
and an international team
of geneticists and
diagnostic experts. The
data includes information
on people from North
America, Europe, Israel,
and will soon include data
from South America and
Australia.
You may visit this website
at: www.Cftr2.org
Please send feedback and
questions to
cftr2@jhmi.edu.
Page 3 of 6
Cough it Up, Spit it Out!
Online Drug Pipeline is now interactive
The CF Foundation has on
its website a presentation
of what drugs are in the
scientific pipeline for
possible use in CF care.
The CF Drug Pipeline
breaks down the various
drugs that are being
studied to treat the various
aspects of CF disease.
The categories are broken
down into: gene therapy,
CFTR mutation,
restoration of airway
surface liquid, mucus
alteration, antiinflammatory, antiinfective, transplantation
and nutrition. There are
phases for each drug
which include: Pre-Clinical
(initial testing in the
laboratory), Phase 1
(human safety trial), Phase
2 (human safety and
efficacy trial), Phase 3
(definitive trail) and then
once the FDA allows, the
therapy is made available
to patients. This last step
typically takes a little
longer with children, as
they want the drugs to be
safe in the adult population
first and then work to
expand its uses to the
pediatric world.
will describe for you what
the drug is for, how it is
used and will direct you to
hyperlinks to give you
more in-depth information,
which typically includes
articles and sites where
the drug is being tested.
The site also allows you to
see what company is
working on the various
drugs, as well as clinical
sites it is being researched
and any news posted
about it from the CF
Foundation.
Caption describing picture or
graphic.
The site is now interactive
and you may click on the
various categories and it
Recipe Corner: Parmesan Cheese Chicken
Ingredients:
Directions:
Nutrition Information:
1/3 cup grated parmesan
cheese
Combine cheese, stuffing,
parsley and garlic. Dip
chicken legs/breasts in
margarine, then roll in
crumb mixture. Place
chicken in a shallow
baking pan lined with
aluminum foil. Sprinkle
with remaining margarine
and crumbs. Bake 60
minutes at 375 degrees.
Makes approximately 4
servings.
Serving Size:
½ cup herb crumb-style
stuffing
2 TBS chopped parsley
1 clove minced garlic OR
1 tsp garlic salt
8 chicken drumsticks OR
4 breasts
½ cup melted margarine
2 drumsticks or 1 breast
Calories per serving: 574
Protein (gm): 59
Fat (gm): 32
Carbohydrate (gm): 10
.
“This is a great main
dish to pair with
vegetables of your
choice, and pretty
economical too!”
Page 4 of 6
Cough it Up, Spit it Out!
Birthday celebrations noted in CF Roundtable
“While we do have a
mean age of survival
presently for CF,
anything is possible, so
take a look at these
birthdays & feel
empowered.”
The CF Roundtable
newsletter is published
multiple times throughout
the year and is touted as a
newsletter for adults with
CF.
Our practice finds it very
helpful, especially the
articles written by Beth
Sufian who runs the CF
legal hotline. This
newsletter is open for
subscriptions and these
can be done online or via
mail. For information:
www.cfroundtable.com.
Now for the birthdays they
have listed in the most
recent newsletter, and
these always get me
excited!
Lawrence Lafary
72
Laura Mentch
59
Debra Radler
50
Marion Rojas
75
Kathy Russell
68
Hal Soloff
81
These people hold for all
of us, hope for what our
children can attain, with
good care and compliance
of therapies.
CF Living, check it out on the web!
Genentech is the proud
sponsor of this new
educational tool.
Genentech, the makers of
Pulmozyme, truly care
about the care of CF
patients and their families.
CF Living is just another
resource they have made
available to help our
patients and families cope
with Cystic Fibrosis. CF
Living is an educational
program that offers
support for people caring
for people with CF or
those living with CF. The
tool allows you to work
closer with our CF team
and helps you understand
better the treatment
options, as well as provide
you with interactive
educational resources to
keep you abreast of new
things. You can enroll
today at:
www.cfliving.com.
Patient Assistance Resource Library available
“This online database
will help families to
understand health
insurance and
coverage.”
The CF Foundation has
launched another new
program to help our families
learn more about Cystic
Fibrosis. The PARL or
Patient Assistance Resource
Library is an online database
with information that can help
guide you and your family
thru the tangled web of
health insurance and
coverage.
There are a variety of
resources available such as:
Sample medical necessity
letters and prior authorization
letters
How to guides to help you
fight thru obstacles
Templates for appeals,
exceptions and
reconsiderations
CF care guidelines
Evidence-based medical
journal articles
The site also offers things
like nutritional supplement
assistance programs,
genotyping assistance
programs and the site is
being updated regularly.
You can review the
information yourself by
going to:
www.cf.org/LivingwithCF/
AssistanceResources/Libr
ary.
If you have questions or
suggestions, please
contact Russell Schuerch
at 1-240-482-2846.
Page 5 of 6
Cough it Up, Spit it Out!
Angels with Roses, Birthday Wishes!
Dustin M.
10/20
Emily S.
2/17
Heath R.
12/5
Drew R.
2/11
Riley K.
12/18
Danielle H.
11/2
Cheyanne P.
2/12
Arey’a L.
2/17
Piper B.
10/3
Cody S.
10/24
Jessica D.
2/05
Reid B.
12/25
Austin R.
12/21
Kaden G.
10/25
Hanna M.
10/17
Kyleigh C.
1/11
Logan T.
1/26
Owen G.
1/20
Adam R.
10/8
DyMond G.
11/1
Benjamin K.
2/18
Kaiden B.
2/13
Georgia K.
11/10
Mason S.
10/21
Hazel H.
12/31
Hunter C.
11/9
Victoria B.
12/30
Camryn P.
2/20
Heather B.
1/21
Kamron K.
10/14
Yael B.
2/26
Jonathan M.
11/14
Buddy the Bear wishes
everyone a Happy Birthday!
Angels with Roses: our kids and parents in their words
Winner of Child Hero of
the Month Award
Hanna M. was asked
about a time when she
was proud of herself. Her
response was, “I used to
be small, but I gained lots
of weight and grew a lot
and that is how I got my
award. I was proud
because it made me feel
special and I was not as
small. That’s what made
me proud.”
Mom offers some insight
Kerry is mom of Kaden
and she reports that
Kaden is a busy little boy
who attends Rock Springs
K5 program. She says he
loves to swim, act out
movies and sword fight
(she left off the karate
chops he likes to dole out
during office visits!). Mom
says Kaden really
struggles with doing his
treatments, but she says
that she and dad, Barry,
are consistent with all
therapies and are
aggressive with any
increase in symptoms.
Good advice, a team
approach is always best,
especially when the child
sees the parents as a
team in terms of treating
their CF.
Play is sometimes best
Zariah’s mom also had
some advice to offer our
parents. She has lots of
family who all help in the
care of Zariah. Mom says
that the most important
thing for her, is that Zariah
gets to play and that her
CF does not get in the
way.
Aaylivia’s mom agrees
with the above statement.
She says that Aaylivia’s
favorite thing is to play.
Emilee’s mom also agrees
that play is important. She
says Emilee loves to climb
and run (especially when
she is in our office!). She
also has some advice,
“Treat your kids like
normal even if they have
CF.”
“Hannah won Child
Hero of the Month
Award. Way to go
Hanna-- We are proud
of you!”
Page 6 of 6
Cough it Up, Spit it Out!
Patience is key
Division of Pediatric Pulmonary
200 Patewood Dr., Ste. A 300
Greenville, SC 29615
PHONE:
(864) 454-5530
FAX:
(864) 241-9246
Freddrick’s mom also
agreed with our last 2
mothers in regards to
play, but she also had
some advice for other
parents. “Just have
patience and don’t be
afraid that [your child] will
get hurt. Keep all the
doctor appointments and
.
make
sure the kids eat
and take their meds, and
love them.”
Go Riley!!!!
We’re on the Web!
See us under Childrens
Hospital
At:
www.ghs.org
Miss Riley has recently
been given an award for
being the most creative in
her art class. She is also
a cheerleader and
performed cheers in her
school talent show.
Mom’s advice, “Take your
We’re also on FACEBOOK
So check it out and ‘like’ us
medicine and do your
treatments and BE
NORMAL!”
Positive Attitudes
Piper’s mom had some
awesome words of
wisdom, “A CF diagnosis
is not the end of the world.
Because of Piper’s great
care team, she is leading
a mostly normal life. The
future can be a scary thing
to think about, but we
have learned not to dwell
on it. If we do our best to
keep her healthy, that is
what is important.
Chest PT is vital!
Dymond’s mom says,
“Stay current with your
treatments and their lungs
will be great. I can’t speak
for weight cause right now
my baby is fighting with
that. Don’t forget to use
the Bopper to help break
that mucus up and spit it
out!”
CF does not define her
Alyson’s mom says, “Keep
everything as close to
‘normal’ as possible.
Treat your child with CF
the same as your other
children. As parents it is
very hard to watch our
daughter cough and get
sick, but we treat her just
like our other 2 kids.
Alyson knows she has CF,
but she thinks it is just part
of who she is. We don’t
think about it as a
‘disease’. We don’t have a
child that is affected by a
disease called CF. We
have a child who happens
to have CF.
www.facebook.com/GHSChildrens
About Our Organization…
Dr. Steve Snodgrass is the Medical Director of the Division of Pediatric Pulmonary of the Greenville Hospital System’s
Childrens Hospital. Our vision is to transform health care for the benefit of the people and the communities we serve. Our
mission is to heal compassionately, teach innovatively and improve constantly. Our CF center is affiliated with Duke
University Medical Center. Our CF Center Director is Dr. Jane Gwinn. We are continually striving to maintain the high level
of care we deliver to our CF families. We have multiple subspecialists involved in our care center. The Childrens Hospital
is here to support our CF families to the best of our abilities.
Editor: Lisa A. DuBose, APRN, BC
Newsletter is made possible by a grant from
Aptalis pharmaceuticals