Cystic Fibrosis Family Night 2019 – C.S. Mott Children’s Hospital (Part One)


– Good evening, we’re going to get started so we don’t keep you too late at the end but please eat your food,
go out and get more food if you want or drinks or
dessert looks really good. Try to stay away from it for me but, so welcome to our family night. We are very excited to have you here and this is kind of an informal so we’re going to be presenting. If you have questions please write it up so in that way you can
address it at the end so we can keep the flow of the talks so you would remember
what you want to ask. I have with me Kathy Enix, our coordinator and I’m gonna ask her this
year to introduce everybody so I won’t mess up any names. – That doesn’t mean I
won’t mess up the names. So another just quick
reminder as we get started, there is a survey that you got with your agenda when registration. As you’re watching if
there’s feedback you have, definitely jot it down. If there’s a question on
there that I didn’t ask that you would like to answer, feel free to put that on there. That feedback helps us create
the events for future years. So I think I’ll start off
with just kind of listing through some of the staff
that have taken the time out to come and join us today
and talk with you all today and we’ll start with Dr.
Nasir, of course you saw her and then Amy Filbrin is here and I’ll just have you raise your hand since we’re all eating. Lindsey Kaverley is also
here speaking tonight and they’re all from the pediatric team. Lauren Kastner is here and her husband Nick Kastner’s joined her and then from the adult team
we have Dr. Simon is here and XiJing Jia should be here as well. We have our adult NP, John Sandefur and Vikas Sood are also
here and our clinic admins, Cheryl Evans out at registration has done an amazing job this year as usual with organizing and
getting everything set up, so thank you to Cheryl. Courtney Bauer is also whenever
admins here helping out. The adult pediatric clinical research team has a table over there if you
weren’t able to visit them, you can stop by afterwards. Dawn Cruz is the manager and
then we’ve got Marissa Lynn, Erica Blunt and Jessica Carey. Pediatric clinic nurses are coming but they are running a little late as they had to finish up phones so Maria Netenofski, Jordan Stifler and Katie Wait are here today and then Ronnie downer
is here, I did see her from the adult team, she’s
the nurse extraordinaire as well as clinic coordinator and then Katie Hall wasn’t
able to be here today as a clinic coordinator
but she had planned on it. Pediatric in-patient
nurses have joined us, Liz Baron, Sue Roebuck and
Diane Brown are all here and then adult in-patient
nurses, Sarah She and Krystal Merrill are here. Our outpatient dietitians,
Courtney Iwanaki, Sandra boom are here and then our pediatric
psychologist Jennifer Butcher should be here a little bit later. Pediatric RT, Sherrod Dagger, our supervisor is here
as well, Cindy Jones. Pediatric social workers,
Julie Lehrman has joined us with her baby and then Haylie Marenick, our newest addition to
the team is here as well and then Kate Thornton, our
in-patient social worker. Maura Pitcher from the
adult social work team and Bryan Nickerson the
in-patient social works. Today we got Chris Tapley
from the pediatric team and Kate Tails and then
adult team, Gomati Kamfadi and Kristen Keith and Sarah Sites and then one thing we want to mention is our adult CF advisory
board has been instrumental on the adult team. I’m not going to list all
their names as their patients but they’ve been wonderful in connecting together
via like a Skype program to be able to meet together regularly so that’s been wonderful. And then we have our pediatric
family advisory board which many of them have
been able to be here today. So Chris and Sarah Barth
I saw, Aaron Boylett, Wendy Carmac and Rebecca Rains, Heather Tremmel and Brandi
Fenner and then Josh Fenner. Alright, so this event
really gets put together with a lot of support
from all of our vendors and I know you’ve all been
around kind of talking to them but if you didn’t get an
opportunity to see them all or if you haven’t got an opportunity yet, feel free at the end
to kind of swing around and say hi again and that
they have lots of information and take-home items as well. And so I just want to kind
of run through who they are so you know who they are and so in order of alphabetized
is a Abvy, Allergen, Kiasi, Cystic Fibrosis Services Pharmacy, Foundation Care Pharmacy,
Genentech, Gilead, Monoghan, Pauri, Repitec, Vertex and Vivas and then of course we’d like to thank our local CF foundation, Chapter for everything they do they
do for the CF community and how willing they are
to work with us as well. So we have Brent Zuber
here and Shelley Frances, Haley Molloy and Valerie Phelps and then I think Shelley
wants to just say a few words before we get started with
Dr. Nasir’s presentation. – Good evening, I’m Shelley Frances. I’ve been with the
foundation for 18 years. Cathy asked me to speak to you tonight just a little bit about
some of the programs that the CF foundation now offers. We have a community voice
program that anyone can go on to our website, family, friends, patients who want to get involved
and help kind of put their, give us their input into what they feel our
initiatives should be going forward so that’s a really good
thing to get involved with and have your voice heard. One of the other things wanted me to talk to you about was CF peer connect. This is a program that
helps connect CF patients who are experiencing similar issues. It used to be 18 and up and now we have lowered
the age to 16 and up. Those patients are all vetted to ensure that they follow their protocols and so you’re not gonna have another kid who is giving your kid bad advice but it also was really helpful because I know a lot of you
have probably seen the movie or heard of the movie Five Feet Apart and it’s something that we
really wanted to expand upon in terms of recognizing that
CF patients need to talk to other CF patients. So they can talk to them
about things like career, going to college, dating even, all of those important life transitions that thankfully we now have a very large and robust adult population
that really needs to be able to have someone to talk
to about those things and you can find both of those
programs through CFF.org. There’s also Compass program
which is really, really, really critical that you
all know about Compass. they can help you
resolve insurance issues, they do a multitude of things and I’ll just give you
one really good example because I know a lot of
people don’t pick up the phone and call Compass because they
either don’t know about it or they don’t know what they do but there was a family in another state who ended up with a 43,000
dollar bill after their insurance or so they thought and for two years, this poor family struggled
with this 43,000 dollar bill and then finally paid it but called, someone mentioned Compass
and they called them and within two weeks, they had gotten all of their money back so please don’t hesitate. They may not always be able to help but they will go out of
their way to try and help you and that number is also on our website or you can stop by, we
have plenty of chip clips with their phone number on it and the last thing I want to talk about is Great Strides. Great Strides is coming up that
fuels all of these programs and drug research and discovery and your awesome patient
care that you receive here at the University of Michigan. So please if you can, join us for a walk. Even if you can’t fundraise
and you just want to come out and see, I know Dr. Nasir said
she will be with us this year at the Ann Arbor walk. Please come out and join
us, we have several teams at the University of Michigan from the different care
centers that participate and they’re among the best in the country. So if you want to come
out and support them, we really appreciate it. But any other questions you have, if you want to know about Compass or any of these other
programs, just see us, we have tons of information we would love to give you, thank you. (audience clapping) Dr. Nasir always does the introduction so I would like to
introduce to you Dr. Nasir, she can now give her talk. – All right, well welcome
everybody, I’m really excited. This is something that I
really look forward to. All our team look forward to. A lot of the people that
are here from the staff and faculty are here
because they wanted to come, I didn’t ask them to
come which is really good and speaks volume of their dedication. So today I’m gonna be talking
about keeping healthy with CF and it’s a title that came
out actually from talking with the family advisory board because I didn’t know what
I was going to talk about and they recommended that
was one of the topics that came in from that. So you can make a difference. This is for the CF patients
and the families as well. We can make a difference in their care and also care of others. I’ll be specifically
talking about adherence because that’s top of the list. I mean if everybody does their
medication as they should, that adds a lot of years to their life. So this is something
that’s very important. You know, the central data
I will talk about it as well as I always do. Exercise advocacy, as
the patient gets older, you really need to be more
active and more you know, working with the political
arena if you will to promote CF agenda and then
touch upon transition as well. So it is a balance between
workload and capacity and we can tell you that we understand but we really don’t
because we don’t live it but we know that how
much work in the average as CF patients put in to stay healthy and we know it’s two to four hours a day, could be more when they are sick. So it’s a lot of work they’re
doing and they have to also, we have to keep in mind that capacity. We want to make sure that we’re not overwhelming them too much. So working with them,
making sure of the you know, time management, trying to
see if there is alternative to different things so they
can be at least managing and going to school and
doing their activities as they would like to, it’s important. So this is a picture
of two of my patients, they gave me permission
to use the picture. Actually, there are a lot older now and it’s just, I asked them to
bring me all their medication that they have at the time and that’s about maybe five years old
to take a picture of it so I can show people here
and you know, overseas when I go to, how many
medications these patients have. They also brought their vest
and they put it on for me. So it’s a lot of medication it’s you know and we understand that but the trade-off is that
they’re living longer, they’re doing a lot better. So it takes a village as they would say. So adherence is an important topic. You know, in a study in CF
patients using refill data, so they just went, got refill
data from different pharmacies at a nationwide claims
database and they looked at it and see how many medications
patients are filling and they said about half of the patients are filling their medications
half of the time or less. So I’ve done a lot of
adherence studies as well and that number, it
depends on the medication that you’re doing, but
there is a that’s very true and that’s from refill data. There is significant decrease
in adherence was noted during adolescent and adult adulthood. So at the beginning, moms are in control and dads are on control so patients do their
treatment a lot more regularly and then they get older and
then we have the rebelling and not doing as good as they should. And then there is a low
and moderate adherence rate associated with higher CF hospitalization. So when patients are not doing
their medications regularly, they will get sicker more often and they’ll end up in the hospital. So when you look at the
frequency of clinic visits that in the first part, you can see that in the younger patient
group there is about 73% of the patient come to
clinic four times a year and I’ll show you some of our data. This is national data but our data and then as we get older it’s about 56% coming in four
times a year as recommended. So you can see the trend here. So our center data, it looks at you know, the four times a year
visits and then you can see that the, it’s hard for me to turn around but you can look at it. So our University of Michigan in patients that are two to six year old were about 79% patient coming
in four times a year and that took years of quality
improvement work that we did. Everybody in the office is involved in it, Courtney is involved in
it as secretarial support. The clinic staff are involved in it, the nurses are on top of it
and we also look at it as well. When a patient reschedule a
clinic or cancel a clinic, we have to make sure
that there’s rescheduled in a timely fashion. And it was comparable
to the national average but we always are looking for, to be one of the top centers. So we really are aiming for the 97, 98%. Now when you, remember
the number here is 79%. Now, when you go to the
patient that are seven to 17, you can see it drop to 71%. Still closer to the national average but the numbers are going
down and then when you go to the older ones, older than 18, you drop to 53%. So as it is you know, and we’re matching with the national
average but it’s dropping and that’s exactly what I
was talking about earlier. This is a beautiful patient of ours, thank you for the pictures. And then you look at the
median weight for height when we use the World Health
Organization for the standards and this is a group that we
really having struggle with it. Babies are not listening to us. We keep telling them to eat
more but they don’t listen. So we keep trying, so parents
are trying very hard as well and our data is getting little better. So we are, you can see like
you know, we were good here and it’s a small number of patients, so very small number of patient
would make a huge difference and then you can see that
we’ve been struggling for the last few years but it
is getting a little better. And we have Courtney and Sandy
working on it really hard to get the numbers higher. So when you look at the
patient that are two to 19, then they start listening to us, right. So our data is better in that sense and we are above national average
which is really important. For us, it’s important for our patients, it’s all about the patients
at the end of the day. And then when we realize that the diet and telling patients to eat
more it’s always very stressful for the patients, for the families and if we can help in any way with that, that would be helpful. So there is a study right
now called Be In Charge and it’s abbreviation for
behavioral intervention for change around growth and energy and this is a study that
came in from Cincinnati, Cincinnati does a lot of
quality improvement work and there is a big group
of psychologists there that work in it as well. So we joined and I’ll
show you in a little bit how many centers are
doing that, not too many, but you know, it’s going and it’s mainly has developed
into a website or mobile app. How about these food trackers or you know, or MyFitnessPal or things like that, how can how can we do that? We use things like that
for children and see that we can help them online on the spot so they can enter data,
we can see the data, we can give them
recommendation on the spot, so this is really part of it. So families complete seven
different themed modules to help improve calorie intake and discuss CF and extra
calories and measure food and all of these things. And we’re doing that project with Courtney in the lead of the program
with Dr. Butcher as well. I’m with them, helping them kind of thing. It includes the diet
tracker which is really good for the patient’s parents to do it and then we can look at
it and give recommendation and always multiple
users can can access it and there is a training that goes with it. So it’s really, I think
it’s a good program and it took a long time, we were part of the
pre-launching for the program and it took a lot of time to
get all the technology together and the CF foundation helped quite a bit, it’s funded by the foundation. So this is kind of a snapshot
of what you would see. So the technology platform. So you have a Be In Charge and that it’s a vegetable garden theme and it tells you different
information about it and then here it shows you the patient and did they enter the
breakfast lunch and dinner or is there a problem. So it’s really a very interactive program that we’re starting. We recruited two patients up till now. So the two sides of the
platform, us and the family. The care team uses the clinician dashboard which is on different dashboards. I can see all the patients that’s enrolled and then we can see
the details of each one and then the families
uses the Be In Charge and the diet tracker so that
way we can help them with it and the patient’s age
is three to 10 years old and like I said, Courtney I don’t see her, so Courtney has recruited
two patients already. So recruited one and then
last week when I was out, she recruited a second one. So would be interesting to
see and we’re trying to see how would be the best and easiest. Also Mrs Violettas is our
parent advisor in that program and that’s really important
because I have my opinion of what would work, but
that might not be correct. So she’s there to help
us out and also helped with developing the program as well. These are the group of
people in the study. So us and then Boston Children, Children of Richmond, Virginia, Cincinnati and then the Stanford group and then Children’s of Colorado as well. So it’s not a lot of
centers which you don’t want to be a lot of centers at this stage because you want to see how you can work the program the best and then you can generalize it. So the idea is hopefully will
be generalized to everybody but we’re very excited to be part of that. So back to our data. So this is for patients,
median BMI for patients that are 20 and older and you can see how much
beautiful their data is and that’s actually I think, part of it is that they have extra dietitians help which is really, Dr.
Simon works really hard to get that going and to
convince the adult side that we do need extra
dietitians for the program. So they’ve been doing
really well with that in addition to their nurses as well. So this is also one of my patients. I don’t know if the parents are here and looking at FEV1
for six to 12 years old and we’re matching with
the median for national, but we’re getting a little better, but the next one is even better. Yeah, isn’t she cute?. Thank you for the picture (chuckles). Well, we asked for some pictures and then the parents send us
so I use all of them here. So this is for patient 13 to 17 which is really the toughest
group for me to work with because they don’t usually, they have their own way of doing things and usually adherence
go down with this group but look at it, you know, the
data is getting better too. Now I have to mention, we have a huge quality improvement project that we started two years ago, I think, about two years ago and
we’re working really hard and every discipline in the
center was working on it. Like nurses, dietician, social worker, of course respiratory therapy and physical therapy are working on it. Social work, I don’t know, psychology. So everybody worked
together to get improvement in the pulmonary function testing and I think it’s paying off. We’re seeing, this is
the data from last year so it would be interesting
to see this year. So exercise and again I use
pictures from my patients. So exercising people with
CF is really important and the level of physical activity and adherence to exercise
program are low in CF patients. A lot of them just you
know don’t want to exercise and parents don’t want to push them because they don’t know
if they can do it or not and then the reason is
lack of exercise tolerance. Maybe they get tired
so okay, you sit down, don’t worry and then also you
know, not wanting to do it and I just want to stay
home kind of thing. So that’s important to keep in mind. Having said that, there
is a lot of my CF patients that are very active and
they bring me pictures and they showed me and
they told me tell me how much activity they are
doing and it’s very exciting. So physical activity is the key element in CF treatment strategies. Physical activity includes
structured exercise which would be the best
but any exercise is good and you know, it’s essential and physical exercise training
may form an important part of the care of the patient and actually there are some organization, we just did a Facebook live on Friday and we had Emily Schuyler
with us in the Facebook live which I’ve never done before, but anyway, I think it was fun. And she was saying that her organization actually The Rock CF
would give walking shoes or running shoes to
everybody that want one. So I wanted to publicize that,
so if your kid wanted to run or do something like this,
you can actually go on to Rock CF website and
she will send your shoes which is encouraging the
patient to do more activities. This is cheerleading or band, I don’t remember which one, band? So exercising people with CF, the benefit from including
physical activity is really great and I have some patient
that would come to me and say in the summer, I do much better, my lung function a lot better
because I run around a lot and I jump on the
trampoline and I do swimming and my numbers, when I say oh
your lung function is better, because I exercise in the
summer more than in the winter. So that is really important. And any activity is good. So then you know, so
exercise is important, we need to really make
sure adherence, exercise, now we need to really, there is a new push on partnering with the
families and the patients. So it’s an oldest, new
idea to improve CF care. There was a session about it
in the national CF meeting that I thought it was very helpful. So the opportunities is you know, have assertion, vision, key strategies, but the important part
is the seven worthy goals and these are the seven worthy goals: So patients and families
are full partner with us so we really need to work together. I love it when patients
come, I’m being sarcastic, when patient come to me and
saying I’m doing everything on my list and I know very
well that they are not. So it’s not for my
health, it’s your health so you need to tell me what’s
happening so I can help out. So it’s full partnering with the patients and with the family. We’re aiming for normal
growth and nutrition. We’re aiming for maintaining
lung function at the best you know, reducing acquisition
of respiratory pathogens and I don’t know who saw the new movie, I have not seen it because I was traveling but you know, it’s very important. It’s Six feet not Five
feet, so just remember that and also important not to
be around other CF guests. Look for complication and
make sure to address it, transplantation and palliative care and then access to appropriate therapies and treatment is very important as well. Okay so co-production
is another terminology that is being used right now with CF and it’s mainly is about the
patients and professionals. It’s a partnering with them. So we need to partner with the
patients and with the family and I’m sorry I keep seeing
patients as people with CF to better use of each other’s assets. So try to talk to me more or
talk to your physician more to see how they can help you and then we can talk to you more and see how we can have the
treatments around your lifestyle and your things that you wanted to do. So health, Canada’s you know,
public improvement continuum. So to have public improvement continuum, you have to communicate,
you have to listen, consult, engage and that
lead to the partnering. So I cannot say I’m
partnering with my patients if I’m not doing all of that. And if your physician is not doing it or your nurse is not doing
it, you know, talk to them. Make sure that you voice your
opinion about this as well because we really need to
work together as partner. All right, so at the end of the day, it’s all about the patient,
all about people with CF. I’m here because of that,
all my team members are here, you are all here because
you care about your children and your adults and you
want to do the best for them and to do the best, we
all have to work together to get the community to understand, to get the health system to understand and to make sure that we are
doing the best for the patient. And better partnering
with the patients lead to a lot of outcome improvement
and as you can see that from all these applications
that’s available. Now the last one or the
one before last actually is the advocacy. Very important at this stage
in the game to be advocate for your child with CF
and your adult with CF. You cannot just sit home and not do much, you have to be, any opportunity you have, you have to do advocacy work and advocacy work can be a lot of things, it doesn’t have to be that
you’re going to the State Capital and working with them but you
could be doing a lot of things like you know, even talking
to people about the movie or educating people about CF, that’s part of advocacy as well. So that’s very important
to empower the the patients and the families and and to coordinate with your caregivers and
with your congress people to make sure that you’re doing well and then just make sure that
they hear what you have to do. And the reason for advocacy
that is really important is this slide, so you can see that patients are getting
older and you can, especially from this slide over here, this is the number of
patients that are under 18 and this is the number of patient above 18 and they are a straight line up. So now I can say that the
adult program is bigger than the pediatric
program and I thought that for so many years but I’m
very excited that’s happening. (audience clapping) And that’s really because of
the improvement and medication that we have for the CF patients. The more partnering we’re
having with the patients and with the families and
also with the importance of focusing on quality of life. You don’t really just what to live longer, you want to live longer,
healthier and doing better. So now goes to transition. So our kids are getting
older, we have to pass them on to the adult side and we
really wanted to do it in an organized fashion and in a way that they are not just moved
from pediatric to adult or they feel abandoned or you know, all of a sudden I’m taking
care of them for 18 to 21 years and I’m passing them without preparation. So that’s very important. So we have a program called CF R.I.S.E which is responsibility independence and two other abbreviations. I try to remember them but maybe Julie can help me over there. Self care and education. So there are two programs. One of them is 16 and above and this is what I’m having here. So it’s mainly a tools that we use because you know, I think some of the adolescents are not listening because maybe they don’t
know enough about CF because we always talk to the parents, not including the children
or the adults, young adults. So we started by going
through the elementary things. How is CF for you, do you know what CF is, so that’s really important. So we have a knowledge assessment. So we’re not telling them okay, you know, we’re gonna you know do
a transition program now, you can go to adult. We start at 16 and we usually
give them the modules, choose a module you like out of the 11 that’s listed
here and work on it. It’s not too long, it’s
maybe a couple of pages and immediately give you a
response if you do it online on your iPhone or iPad or
whatever and then that way, and then if there are any deficiencies then we can work with them to correct it, the team members will address it. And the idea is not to do it once, the idea is to do it more than once to make sure they are okay with that and then the responsibility checklist. Now we’re in pediatrics,
moms and dads do everything for the kids, that’s what
I did with my kids as well and the idea is to get them
to behave like an adult. So responsibility checklist. Make sure they call us
when they need refill, make sure they make
appointments for themselves and that start gradually
makes sure they understand what insurance is about. So that’s really important
and we give one to mom or dad and one to the patient and
see how they answer it. If the kid’s saying I do everything, I call and I do my refills and I do and then mom or dad would
say we do everything. So there is a disconnect here. So we work with them in
gradually moving them to be responsible and it
depends on the age as well, so you don’t want to have a 16
year old calling all the time and doing all the insurance issues so we gradually have to move them there. So having said all of that,
the bottom line is this slide. This slide shows you how
much work we’re doing for CF. I just came yesterday from
an investigator’s meeting for another study going on. We are having more physicians
involved in research. Dr. Jia is participating
with us from adult side, we have Dr. Kevin Lee and
Dr. Filbren working with. So it’s not only me doing the research, it has to be a lot of people because it’s a lot of studies and you can see from there
that so many going on in phase two and then you
have to see whatever makes it to phase three is less but we
have a lot of phase two going. We have some phase one as well. So bottom line is there is
a lot more things coming down the pipe. We have a lot of new,
advanced and new medication so we’re very excited about
it but it’s a lot of work. So shout out for research. Please when Don and her
crew come to you and ask you to participate in a
study, don’t just say no. Try to listen to them and
see if it’s feasible to do it because more studies meaning
more things available for CF patients. And with that thank you. (audience clapping)

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