31 October 2015

Happy Halloween 2015

Happy Halloween

...from our littlest Tango dancer, our bravest Spanish superhero, Zorro, 
and everyone's favorite, our playful Pinata!

A big thank you to my friend Margaret who helped sew lace on a black skirt and the top of a red leotard.  Finding am already-made toddler flamenco dancer costume was a bit challenging.  :)

Zorro was an easy costume to pull together.  Oliver's favorite part of his costume was his sword.  The mustache idea was his own. :)

I made Bennett's pinata costume by hot gluing strips of felt on a an oversized one-piece pair of pajamas.  It took forever but it was well worth it!  He loved seeing people laugh when he told them he was a pinata.  Brian and I thought this costume so well reflected his personality!

We have had quite a few rainy days in Texas lately so we were worried that Halloween might be cancelled.  Thankfully, it cleared up a few hours before go time.  All three kids loved trick or treating this year.  Oliver enjoyed explaining each of the kids' costumes.  At one point he said, "I'm Zorro.  He's a pinata.  And she's a Spanish princess."

Bennett, the most social of the three kids, really enjoyed thanking people for the candy and telling them "happy halloween."  At one point in our trick or treat outing, we came across a house in which a neighbor, dressed as a witch, was standing outside making scary hallowing noises.  Bennett, confident that he did not need to be scared, walked up to the neighbor lady and said, "I really like your voice.  You make really good scary noises!"  It made the neighbor's day.

Avonlea cracked us up tonight.  Earlier in the day, Brian and I had practiced with her how to knock on a door and say "trick or treat." But all that practice went right out the window when Avonlea got up to the first house.  Tightly clutching her pumpkin bucket (which she called her "purse"), Avonlea went up to the first house, knocked with her tiny fist and then shouted loudly: "Open the door!!"

Thankful for moments like these,

29 October 2015

2015 Pumpkin Patch Pictures

Excuse me while I insert some of our annual pumpkin pictures for the Grands... ;)  

See how much the kids have grown through our annual pumpkin patch pictures!

 Pumpkin Patch - 2015 (Oliver, almost 8; Bennett, 6; Avonlea, 2)

 Pumpkin Patch - 2014 (Oliver, almost 7; Bennett, 5; Avonlea, 1)

Pumpkin Patch - 2013 (Oliver, almost 6; Bennett, 4; Avonlea, 3 months)

Pumpkin Patch - 2012 (Oliver, almost 5; Bennett, 3)

 Pumpkin Patch - 2011 (Oliver, almost 4; Bennett, 2)

Pumpkin Patch - 2010 (Oliver, almost 3; Bennett, 1)

Pumpkin Patch - 2009 (Oliver, almost 2; Bennett, in Mommy's tummy)

Pumpkin Patch - 2008 (Oliver, almost 1)

Pumpkin Patch - 2007 (Oliver, in Mommy's tummy)

26 October 2015

Raising money for a cure for CF in memory of Sonny

This past August, my good friend Casey (below in red), along with his sister Laci (in pink shoes), held the 15th Annual Sonny Rose Memorial Softball Tournament in Jonesboro, Louisiana.  This year's fundraiser benefitted the Cystic Fibrosis Foundation in honor of Bennett.

Casey and I went to high school together.  He's a great guy and a strong supporter in Bennett's fight to win out against CF.  Casey and his sister know what it's like to lose a sibling so they don't want Avonlea and Oliver to ever have to experience that feeling.  Casey and Laci lost their big brother, Sonny, to a car accident about 15 years ago.  

Here is a sweet childhood picture of Casey (in red), Sonny (in blue) and Laci (in pink).  

Recently, I asked Casey to help me remember who Sonny was as a person.  Here's what he shared with me:

"Sonny was a great brother, a loyal friend and a fun guy to have around.  He was an entertainer and the life of every party.  He loved sports, especially baseball.  Sonny had a big heart and loved kids. He would be honored knowing that we are raising money, in his name, to help find a cure for Bennett."

Sonny, left, and Casey, right

Our family was bummed not to be able to travel to Louisiana for the tournament since school had just started for the boys.  But we're hoping to be there next year.  

This year's fundraiser raised $2400 for Bennett's Brigade.  First place went to "Beauties and Beasts". Second place went to "Dirty Dozen." Third place went to team "Old School".

Thank you to Casey, Lacey, Casey's family and everyone who participated in this year's softball tournament.  My heart is incredibly grateful for the gift of love that was been given in the name of Sonny to Bennett and all those suffering from Cystic Fibrosis. 

Deeply thankful, 

22 October 2015

Mental Health and Cystic Fibrosis

Terminal diseases are depressing.  Chronic diseases are anxiety-producing.   Cystic Fibrosis is both.

My final blog post on what I learned at this year's North American Cystic Fibrosis Conference is about the big announcement by the Cystic Fibrosis Foundation regarding mental health:

Across the country, CF Care Centers will soon begin screening (and, for some, treating) CF patients and their families for anxiety and depression.

(This post is my third of three blog posts about my experience at the 2015 North American Cystic Fibrosis Conference.  To read my first post, click here.  To read my second post, click here.  This specific blog post addresses items that were presented in the 3rd plenary at NACFC.  Watch this plenary yourself, click here.)

A recent international study showed that CF caregivers and people with CF have a depression/anxiety prevalence of 2-3X higher than that of the general community. 

Out of caregivers and those affected by CF, CF moms scored the highest in anxiety and depression.

This study also showed that if a caregiver had a higher anxiety/depression score, the child was likely to as well.

CF Foundation announced at NACFC that they believe mental health is an important unmet clinical need.

The CFF has begun working to address this issue by:
* creating mental health guidelines
* developing manuals on how to create referral networks
* writing handouts on psychotherapy
* encouraging local CF Care Centers to establish "mental health coordinators" in their CF Clinics
* implementing strategies for prevention/screening/case management for those needing interventions and
* offering trainings for CF clinicians on cognitive behavior therapy and psychopharmacology.

There was naturally a lot of excitement in the room when this announcement was made.  I think everyone was relieved to see a plan in place for addressing an issue that most already recognized: CF can take a huge toll on one's mental health.

I am so appreciative that the CFF is being forward-thinking in this area and bringing awareness to this issue.  Hard work was put in to this study and its' coordinating plan to address what to do with so many in our community struggling to cope.

I'm a huge proponent for psychotherapy and the use of medication to treat mental health issues. I'm so glad that the CFF is encouraging conversations between doctors and patients/caregivers regarding this issue.  

But I have two concerns on this topic I think are worth mentioning:

1.) I'm concerned that surveying and referring CF caregivers and patients to mental health resources may lend itself to be a "quick fix" to a much bigger problem that has yet to be addressed: the cognitive burden CF brings to a family.

The fact that CF moms, who very often (although not always) carry the caregiving burden for the child with CF, have the highest rates of anxiety and depression gives us some insight in to the fact that this issue goes beyond the disease itself.  As a CF community, we have to address the fact that while CF treatment may be keeping us healthier, we have a "quality of life" issue that must be addressed.  

So much of CF care today is filled with a day-in and day-out, over-and-over-again monotonous time-intensive treatment regimen - paired with a lack of confidence, at times, that what is being done is right or best - and punctuated by infections/flareups/exacerbations that require significant logistical changes.  As people with CF grow up, more and more medications and treatments are added over time.  This takes an emotional toll both on the person with CF and their family.  

It is the most helpless feeling to do all of the things we do for CF and not really know whether or not it matters (Should I be doing 2 treatments daily or 3?  Am I sterilizing my equipment the right way? Should I treat this fungus in my lungs or leave it alone?  Is it better to do IV meds at home or in the hospital?)

What I wish CF clinicians understood about CF is that simply referring patients and caregivers to a therapist or simply prescribing a pill is actually, inadvertently, simultaneously adding to our cognitive burden.  It's one more appointment to arrange, one more pill to remember to take, one more co-pay to make.

I wonder how significantly beneficial to the mental health of those in the CF community it would be if we addressed the cognitive burden of Cystic Fibrosis.

2.) I'm concerned that if there is not mutual trust between the patient and the healthcare provider then surveying people with CF and their caregivers for mental health will not be ultimately beneficial.  

It's well-known among patients/caregivers that some patients/caregivers are not always fully forthcoming or honest with their healthcare providers.  This isn't because they don't want to be.  Many times this lack of honesty is due to a lack of time to talk further in the clinic.  Sometimes it's due to the patient feeling unheard.  Other times it's because the patient/caregivers fear that they have to say would upset the clinician.  Whatever the reason, we know patients struggle with this issue.

Trust is fundamental to bringing value to the patient/caregiver screenings.  If patients and caregivers are not already feeling a sense of trust with their CF Care Teams, if they are already not open about their lives with their healthcare providers, they will not likely share their issues with depression and anxiety.

How might we address building greater trust between patients/caregivers and those who care for them?

My suggestion would be for their to be more opportunities healthcare providers and for patients to work together outside the clinic, to get to know each other as humans first.  Maybe this shared trust could happen through working together on a patient and family advisory council.  Maybe NACFC provides opportunities for clinicians and caregivers to get to know each other better.  Maybe social media provides a place for clinicians and patients to interact.  However it happens, if patients and healthcare providers can learn to talk to each other about normal human things first, it will be easier to talk about more challenging things like feelings of depression, anxiety (even thoughts of suicide).  

Clinicians, in caring for their patients' well-being, are often concerned about patient "adherence" (adherence describes whether or not a patient is taking the medications/treatments that have been prescribed for them).  It was mentioned at NACFC that improving a patient's mental health will likely improve their adherence to their medications.

However, I'd like to flip this and suggest the otherwise may be true: 
Listening to patients/caregivers and truly understanding the reasons why CF patients/families struggle to do their meds may give greater insight in to not only "adherence" issues but also why mental health issues in the CF community exist at all.

I am proud of the way the Cystic Fibrosis Foundation works to relieve the pain and suffering of families with CF.  How might we work together to maximize these efforts?  Let's look to relieve the cognitive burden of CF and build a greater mutual trust between healthcare providers and their patients/caregivers.  I believe doing so will greatly improve the mental health of everyone fighting this unrelenting disease.

18 October 2015

My NACFC 2015 "Nuggets"

This post is my second of three blog posts about my experience at the 2015 North American Cystic Fibrosis Conference, the largest international gathering of CF clinicians, researchers, patients* and parents.  To read my first post, click here.

There is way more information than I could easily share on my blog so I highly recommend watching the NACFC plenary sessions that were recorded live: Plenary 1 (future of personalized medicine)Plenary 2 (about clinical trials)  and Plenary 3 (about mental health).  I also recommend reading fellow CF mom Rebecca's blog for other NACFC medical/science updates.

To easily share what I learned at NACFC, I am sharing them in "nugget" form:

Nugget #1: We have seen an increase of MRSA in CF lung cultures in the last 20 years.  There are currently limited treatment options for MRSA. A trial funded by the CFF called STAR-TOO shows there is benefit to early intervention for newly acquired MRSA.

Nugget #2: The Inhaled Vancomycin Therapy (AeroVanc) study for those chronically infected with MRSA is preparing to start Phase III trials in 2016.  If it succeeds in Phase III clinical trials and approved by the FDA, it will be the first inhaled antibiotic to treat MRSA.

Nugget #3: There may soon be a new way of doing pediatric PFTs.  It's called the Multiple Breath Washout (MBW) and Lung Clearance Index (LCI).  It may eventually come to a pediatric CF Care Center near you.  MBW/LCI was selected as the primary outcome for efficacy in the European Ivacaftor/Lumacaftor study in younger children with CF ages 6-11.  It was also chosen to be the primary outcome in the Saline Hypertonic In Preschoolers (SHIP) study.

Nugget #4: There looks to be a new standardized way of evaluating "silent" CF disease progression in very young children.  It's called Pragma-CF.  This is the work of ArestCF, the Australian Respiratory Early Surveillance Program.

Nugget #5: We are in the age of personalized medicine, particularly with regard to Cystic Fibrosis.  Some people benefit from drugs while others do not. The CFF has recognized this and are actively looking for more tools to figure out the right therapies for each person.

Nugget #6:  Scientists believe personalized model systems (such as intestinal organoids, nasal cells and stem cells) may eventually help predict clinical benefit of new therapies.

Nugget #7: Novel biomarkers may have the ability to give us more information on patients' disease progression.  Doing so may eventually accelerate clinical trials and streamline therapies.  An example of this is the biomarker of mucus.

Nugget #8: The life expectancy of those with Cystic Fibrosis has increased over the last 30 years.  Evidence-based medicine is part of that success.  This slide shows when medications have been introduced to regular CF care and how they correlate:

Nugget #9: Thankfully, this year there are more medications/treatment protocols being evaluated to treat CF than ever before:

Nugget #10: This year is also the year that patient participation in clinical trials for CF is higher than ever before (even higher than was projected).

Nugget #11: The way the CF community does clinical trials needs to be adjusted.  In a recent CFF Caregiver study regarding clinical trials, it was found that 77% of the patient/caregivers report symptom relief as the primary goal of pulmonary exacerbation treatment.  However, respondents thought doctor's primary goal of antibiotic treatment was equally recovery of lost lung function and relief of symptoms.  The study found that overall, patients/caregivers are willing to participate in clinical trial studies, trust their physicians more than study researchers, expressed legitimate concerns regarding treatment duration and consider symptom resolution a high priority.  According to the CF Foundation, understanding the needs of the CF community will hopefully help CF researchers make clinical trials more conducive to successful research.

Nugget #12:  The CF Foundation just turned 60 years old.  It used to be called the "National Cystic Fibrosis Research Foundation." (Just a random fact I thought was interesting.)

Nugget #13: CEO Bob Beall has officially stepped down from leading the CF Foundation.  Dr. Preston Campbell has taken over as CEO.  But, thankfully, Bob Beall will continue to work at the CF Foundation, he will just no longer be leading as CEO.  Bob explained, "this is a train that you can't get off of."  

If you've heard about the biggest "nugget" from NACFC regarding Cystic Fibrosis and mental health, I haven't forgotten about it.  I'll post my thoughts in a few days on the topic in my third and final blog post about NACFC 2015.

15 October 2015

NACFC 2015 - What was it like and what did I learn?

Despite that our family has been dealing with Cystic Fibrosis for six years now, this past weekend was the first time I had ever attended the North American Cystic Fibrosis Conference, the largest international gathering of CF clinicians, researchers, patients* and parents. (*Due to infection control issues, only 1 person with CF is invited to the conference; however, understandably, some people with CF choose to come anyway.)

There is way more information that came out of NACFC than I could easily share on my blog so I've decided to narrow my thoughts to the next three blog posts.  But for those interested in learning more, I highly recommend checking out fellow CF mom Rebecca's blog for medical/science updates, watching the CF Foundation's recorded plenary sessions (probably the best part of the conference) Plenary 1 (future of personalized medicine)Plenary 2 (about clinical trials) and Plenary 3 (about mental health) and following on Twitter the hashtag #NACFC.

I decided maybe one of the best first ways to share about NACFC experience is to create a FAQ page...of frequently asked questions I would ask myself if I wasn't already myself. ;)  So, here you go:

1.) What was NACFC like?
NACFC was interesting.  This year's conference was held in Phoenix, Arizona (what a cool place to experience - the desert and mountains, so weird!)

The conference was big (~4000 people) but well-run.  It was exciting to see so many people connected to a disease that affects so few.  I heard that this year was even bigger with regard to outside interest in NACFC, as more and more companies are seeing pharmaceutical success with treating the disease.

I had been warned that this conference was a medical conference and therefore might be above an average person's comprehension level.  Although there were sessions "over my head" (or simply not interesting to me), most sessions were understandable and relevant to Bennett's care or to the care of other CF families I know.

In general, there were parts of the conference that I thought were fantastic (like the plenaries, the pulmonary journal we received, the exhibit hall and many of the sessions).  There were other parts that I thought were disappointing (some of the sessions could have been improved with regard to content and delivery).  But, overall, I thought it was good.  My favorite part, by far, was the opportunity to connect with people in the hallways and over meals.

This picture is the first "Facebook CF Mamas" group gathering that I know of.  Out of the current 3,000 CF moms on the Facebook group "CF Mamas," 13 of us were able to find each other at NACFC to have lunch and say hello to each other "in real life."  It was a really fun experience for us all as many of us have shared our stories and used each other as a resource online for many years now.  Maybe this group will grow at NACFC next year.

2.) What did you learn at NACFC?
The main thing I learned this year at NACFC is just how much more left we have to learn.

For example, I went to a session on infection control, which I was excited to attend.  But, when I left the session, I was only even more convinced that we have much to learn about our current infection control practices and how well they do (or don't) work.

I went to a session on assessing for liver disease.  But when I left the session, what I had gained was that researchers don't necessarily know what's best with regard to appropriately assessing liver disease.

Researchers shared that there is little information on whether antibiotic treatment specifically targeting staphylococcus aureus results in clinical improvement.  This still needs further study.

It was discussed that biomarkers might help identify early CF lung disease.  But that still needs to be studied.

Research is being done on pulmonary exacerbations.  But currently it's stated that "much of the current accepted treatment approaches to manage pulmonary exacerbations in CF lack quality evidence."

For me, I think that before NACFC, I may have been under the impression that we already knew all of these things - well, that the researchers and clinicians did.  I think that I falsely thought that I could just come to NACFC with open hands and the researchers/clinicians would rain down all of their information and insights so I could catch them.

But, what I learned was that we are still a very ripe community for learning.  Through some of my other patient advocacy work, I have learned over half of the clinical care that is given is not actually evidenced-based.  What I learned at NACFC is that CF is is no exception.  Care is given as it is known.  But until something is studied and known for sure, it's simply a hypothesis. What I learned at NACFC is how many hypothesises exist and how much we still want (and need) to know.

Ironically, there was a plenary at NACFC that addressed this very issue: the need for further studies. The plenary spoke about clinical trials and our need to enroll more patients in clinical trials so we can do more studies, not just with new meds but with current treatments so we can improve care.  As we gain more information about how CF works and as we gain more capabilities to use biomarkers and more effective measuring tools such as MRIs to measure lung disease, the more we need to study to know why things work the way they do.  Only then can we change clinical care to be more evidence-based.

Nonetheless, I left a bit surprised that things are more complex than I had first imagined.  It leaves me to wonder: how might CF patients and families better join forces with their CF healthcare providers to create evidenced-based clinical care sooner?

3.) What new products did you learn about at NACFC?
Ooh! I got to see a few really cool products coming out to the CF community!!

I loved getting to see the tiniest new Hill-Rom Vest coming out soon!  I love how it fits a baby doll!  I don't know exactly what age this would benefit but Bennett started at 10 months with the size up from this one.  So, I'd have to say this new size fits children even smaller.

Even more, I loved getting to see the Alcresta enzyme for g-tube feedings!  Yay for making my life easier!!  I won't post a picture, for fear I'll compromise their pending FDA approval.  But I will share that it isn't just the Alcresta enzyme that's so wonderful - it's how the enzyme will be administered that is novel.  Ultimately, the enzyme will attach to the feeding tube and will continuously be supplied during a tube feed so that it works over time.  Hooray for no more waking up our children to take enzymes and no more crushing enzymes that we really aren't supposed to!  (I'll post a pic as soon as I hear it's officially on the market! If you have a kiddo with a g-tube, you're going to go nuts over this!)

While at the exhibit hall, I was able to check out Hill-Rom's MetaNeb.  It's not yet something that most people have at home but it may be in the next two years.  It's supposed to help mobilize secretions in the lungs by using supplemental oxygen.  Sounds complicated but looks like it could be amazing.  The Hill-Rom rep, a CF mom herself, suggested we ask our hospitals if they have this device to use when we are inpatient if we'd like to try it out.

I had a chance to see the new childsize AffloVest, which is for kids around 7+.  I found it to be really easy but a little heavy considering the other vests are nothing but air.  Since every percussion vest company was represented at NACFC, I had the opportunity to try on both an adult Hill-Rom Vest and AffloVest to see the difference.  (I didn't look in to Respirtech since we don't own one.  But I did see the new color vests they have now, which I love, btw.)  I could feel what people talk about when they say the AffloVest and other Vests work differently.  The Hill-Rom vibrates harder using air.  The AffloVest is softer using vibrations.  The say they are supposed to work the lungs differently.  I couldn't feel how it best they benefit as I don't have CF.  But, wearing them did give me better insight in to their benefits and disadvantages.

Another product I saw but I don't have a picture of is the Aeroeclipse breath acuated nebulizer cup which can be placed together (as could any neb cup and mask) with the Aerobika oscillating PEP device.  Bennett hasn't used either of these yet but from the conversations I had with the CF families I met, many are using and benefiting from PEP devices such as these.  So, we will try it in the next few weeks.  I hope to also speak more to our CF Clinic about their thoughts on these products.

4.) Would you recommend others to go to NACFC?
Yes, I would recommend other CF families to go to NACFC.  But, at this point, I also would say that anyone who is not able to go should not lose sleep over it.

Currently, the best parts of NACFC are live streamed and the conference journal (Pediatric Pulmonology), which is fantastic, is available for free here.  So, you don't have to go to get some of the best parts.

The conference has a way to go before it would be considered "patient-centered."  So, for people with CF and CF families to gain as much benefit as I think (and hope) they will eventually be able to do, it's likely got a few years to go yet.

Nonetheless, I was glad I went and do recommend it to others.

5.) If you could change anything about NACFC, what would you change?
There aren't that many things I would change if I could.  But here are two things I'd love to see improved at NACFC for next year:

1.) I would like to see everyone encouraged to share what they are learning at NACFC on social media.  
NACFC offers important and valuable information to those in the audience and those not able to be.  I'd love for in the CF community to have greater access to what goes on at NACFC.  It would be really great to be encouraged throughout the conference to tweet thoughts and nuggets of info.  Can you imagine if even half of the 4,000 clinicians and researchers regularly tweeted throughout NACFC?  It would be an epic global conversation for all to enjoy!

2.) I would like for people with CF and/or CF parents to be given a real-time platform at NACFC to teach clinicians about their real life experiences of CF.  
Many medical conferences are beginning to recognize the power of including patients in their conferences.  Although the CF community has challenges with cross-infection, involving CF families and people with CF (through the magic of technology) could have a profound impact on the kind of learning that happens at NACFC.  I believe involving people with CF and their families in the planning stages of NACFC is a great first step to moving towards patient-centered care in the CF community.  Clinicians have a CF knowledge to teach us.  But CF patients and their families have a different kind of knowledge to teach clinicians.  I'd love to see that honored.