27 January 2017

Surgery Date Confirmed

A few days after Bennett's barium enema earlier this month, I called the surgeon's office to find out the date of Bennett's surgery.

When I called, I was shocked to learn the doctor apparently "just left on vacation" and wouldn't be back for several weeks.  Surgery would not be scheduled until he came back.  Ugh.

So, for the last few weeks I've been both grateful that we couldn't schedule Bennett's surgery (out of sight, out of mind) and also full of anxiety knowing it's coming.

Planning surgeries like this is like what I imagine preparing for a hurricane might be like - you think you know what it's going to be like, you think you're prepared...but you know deep inside you have no idea and won't know until it's come and gone.

Thankfully, the surgeon returned this week and we now have an official surgery date.

Bennett will be admitted at to Children's Medical Center in Dallas on February 8 for a colon clean out and prep for surgery.

Surgery will be on Thursday, February 9.

Bennett is expected to be in the hospital for 4-5 days.

When it's all said and done, Bennett's last surgery went really poorly.  Based on the information we have, we believe this past year's issues seem to have all stemmed from complications from Bennett's surgery last January.  So, it's no wonder that I have a tremendous amount of anxiety going in to a similar surgery as last year's.

I have spent the last few weeks in therapy processing my feelings about what happened last year and working to prepare myself for these next few weeks.  Through this work, I have come to better appreciate the trauma our family has gone through with Bennett's CF.  Acknowledging the weight of years of issues, surgeries and complications has given me a greater respect for what we are having to face and for what others who experience similar situations also face.  Many times, including last year's surgery, I downplayed our experience.  "It's our normal, so it's normal, right?" I thought.  I didn't ask my family to come help us.  I didn't reach out to friends to support us as much as I could have.  I tried to minimize our needs, my needs, because I thought it's just our normal. But just because something is normal doesn't mean no-big-deal.

This time, I've asked family to step in to help.  I'm leaning on my husband for more support.  Rather than trying to distract them during this time, I've asked to have more access to the kids. I want them to be near.  I need them to be near.  I hope to be more honest with friends about ways they can help.

I guess what I really am learning to say is:  This is awful.  I don't want to go through it.  I wish God didn't call me to this moment.  But I know he will give me the strength to do this once again.  I need others.  I have the greatest admiration of my son who, more than anyone, feels similarly.

08 January 2017

Barium Enema and next steps for Bennett's Stricture...

Until most recently, Bennett had been doing really well with his health.  His lungs are staying healthy and his GI symptoms were mainly staying at bay.  

However, as of a few weeks ago, Bennett started showing signs his intestinal stricture is causing him problems again.

I contacted his GI doctor to ask if we can move forward with addressing it once again (I had placed everything in hold in September).  The GI doctor was very responsive, despite she was still out of the office for the holidays, and quickly put him on the calendar for a sigmoidoscopy with dilation.  

But, as the week marched on, Brian and I began talking about Bennett's stricture, asking ourselves what options exist for addressing this stricture once and for all.  I reached out to the pediatric surgeon who has been working with Bennett's GI doctor to see if he thought surgery would be an option.

Brian and I haven't been ready for surgery.  But, we rationalized, if Bennett is going to have to undergo a 3-day colon prep and be put him to sleep again, maybe we ought to do surgery, a more definite way to address the stricture.  

Upon hearing our thoughts, the pediatric surgeon confirmed surgery is a viable option for Bennett.  The GI doctor also agreed she believes surgery makes sense.  So, after two weeks of great hesitation, 

Brian and I have decided to move forward with surgery.

To prepare for surgery, a barium enema was ordered.  On Friday, Brian and I took Bennett to Children's Medical Center for the barium enema in hopes it will give the surgeon more information about the placement of the stricture and to confirm there are no other hidden strictures.

This was Bennett's 4th barium enema.  He had one in preparation of his last surgery almost exactly one year ago.  It is a very uncomfortable procedure.  I knew it would be really hard for him to do again.  The procedure consists of a tube being placed in the bottom and clear barium liquid pushed up through the bowel while x-rays are taken.  Not only is it scary for a child, the barium often causes cramping pain.  I was most worried this time about additional pain caused by his stricture.  Thankfully, the X-ray tech was very responsive and decided to place numbing gel on the end of the device that would provide the barium in order to prevent any additional pain that might be caused.

Brian was able to join Bennett and me for the procedure since my sweet friend Kelly offered to pick Oliver and Avonlea up from school and watch them until we made it back to Waco.  This made a world of difference in regard to how traumatizing this procedure was.  Although the procedure was still really hard for Bennett, having both mommy and daddy there made for distraction.  In addition, not only did Brian's being there help me to be calmer, having Brian there allowed me to talk to the radiologist and nurses without the feeling like doing so required leaving Bennett alone.  

We explained to Bennett his only job was to be as calm and relaxed as possible.  The X-ray tech explained the importance of taking deep breaths and breathing through it.  The entire procedure took about 15 minutes once we began.  Bennett did a very very good job despite it all and gave us some great images.

Here is a carton drawing of what a colon looks like.  Bennett's stricture is in his "rectosigmoid" which means in the rectum and sigmoid area.  I wasn't even sure where the stricture was until I saw it on film.  It turns out, it's hard to miss:

Can you find the stricture??  Although we haven't received the official radiology report from the barium enema and we haven't yet heard from the pediatric surgeon on when surgery will be scheduled, we did appreciate hearing the Radiologist say in person that he thinks the stricture is probably less than 1cm (or 10mm).  

Seeing this picture made Brian and I grateful we have made the decision to resect the colon - to cut the stricture out and reattach/fix the colon to itself.  

Upon finishing the procedure and leaving the hospital, to our surprise, Brian and I noticed it was snowing outside.  Experiencing snow in Texas is a rare, a bit like seeing a rainbow after a thunderstorm.  As we were walking out, I wondered aloud to Bennett that maybe God had given him the opportunity to experience snow as a gift to him for his great bravery.  Bennett's face lit up.

I don't know why God chose to send snow in that moment.  But what I do know and what I wanted Bennett to know in that moment is that God is there always - both in the difficult moments and in the magical ones.

04 January 2017

Understanding the CF Foundation Patient Registry

One of the things that the Cystic Fibrosis Foundation is most known for is its Patient Registry.

For more than 50 years, the Cystic Fibrosis Foundation Patient Registry has been collecting data about CF and about those who are born with it.  This aggregated data is used to further CF research.

Most people with cystic fibrosis in the US provides their data to be collected through the Patient Registry but few really know much about it.  So I thought I'd share more about what I know about it.

I have had the unique opportunity to be involved in the CFF's patient registry work over the last year and a half as I have am co-leading CF Foundation's Patient and Family Research Advisory Committee (PFRAC).
Pictured above: Laurie (CF Mom and PFRAC member), David (CF Dad and my PFRAC co-chair) and Aliza (CFF)
This past year at NACFC, our committee launched our first and primary initiative, the Insight CF Registry Research Project, a new way for people with CF and their families to indicate what community and disease trends they want to know more about using data from the CF Registry.

The CF Foundation Patient Registry is a collection of data on the health of CF patients.  There are two pieces of inclusion criteria for the Registry: you must be seen at CF Care Center and you must consent to participate.

(Image from the CFF 2014 Patient Registry Report)

Registry data is collected, alongside of the patient's medical record, by individual CF Care Centers and is managed by the CF Foundation.  To see specific types of information you can find in the CF Foundation Patient Registry, click here.  Although it's changed over the years, currently, data is collected at diagnosis, clinic visits, hospitalizations, and with IV treatments.

(Image provided by CF Foundation)

This might be my inner nerd coming out but I really love seeing how the CF Foundation Patient Registry has evolved in the last 50+ years.

The CF Registry was originally started in the 1960's by Dr. Warren Warwick at the University of Minnesota.

The 1980's, the CFF began to maintain and update the Registry under the leadership of Dr. Bob Beall.  At that time, the Registry was maintained on paper and data was gathered through annual questionnaires.  The types of data that was entered in to the the registry during this time included demographics, treatments and exacerbations.

The first year of analytical data came in 1986.

Two years later, in 1988, the CF Foundation published its first Registry Report.  (Registry Reports are available free online but typically are 2 years behind due to the time it takes to collect and analyze the data.)

In 1994, the CF Foundation began to ask for data to be transferred onto floppy disks.  Also this year, the registry began gathering more data more frequently.  At this time, the registry began collecting data quarterly (rather than annually) and asked for the following items added in to the Registry: height, weight, pulmonary function tests, microbiology cultures.

In 1999, the Registry began creating center-specific reports so that CF Care Teams could monitor how they were doing at their specific center in comparison to other Care Centers.

In 2002, separate year-end reports were created for clinical and general CF community.  Before that, reports were just created for the clinicians to read.

In 2003, the Registry began working on a web-based platform.  Additional variables were added between 1994-2003 but the variables were mainly more details about the categories listed.  The big difference between measures gathered in 1994 and 2003 were about how often the data was collected.  For example, before 1994, the Registry collected one height and one weight measurement per year.  Starting in 1994, clinicians could add up to four measurements per year.  In 2003, clinicians could enter as many heights and weights as "encounters" (when an individual is seen in clinic or in the hospital).

In 2006, some metrics on CF Care Center outcomes were made publicly available online.  This was also the year in which the Registry began collecting detailed information medications used within the CF community (mucolytics, inhaled antibiotics, prochodilators, corticosteriods).

In 2010, registry information began being collected through an enhanced web-based platform and began collecting information on CFTR Related Metabolic Syndrome and CFTR-related diagnosis.  In addition, expanded data began being collected in microbiology, genotype results and infants under 2 years old.

Today, data collected on CF patients is now collected at diagnosis, clinic visits, hospitalizations/home and IV treatment and is collected annually.

Before I knew much about the Patient Registry, I used to think the Registry was a big online database.  It's actually not.  It's a collection of data over time but it's not currently collated in a way that would easily allow for a non-clinicians/non-researchers to hop on a computer and crunch the numbers.  That might come with time but, for now, it's a very arduous HIPAA-compliant process with careful access given mostly to researchers.

People with CF and their families can get a copy of their own personal data from the registry at any time by requesting a copy from their CF Care Center.  People with CF and their families can sometimes get their CF Care Center's Patient Registry data if the CF Care Center is willing to give it to them (just ask your Care Center to find out).  But, since the Registry contains a lot of data and is very complicated, getting access to it in any form is not typically as easy as clicking the "print" button.  This is why most people with CF and their families really don't have access to Patient Registry data outside of the Patient Registry Reports published by the CF Foundation every year.  Personally, I find these reports to be really interesting and enjoy reading them every year.

So, how exactly is data from the Patient Registry used?  In addition to the data that is pulled on behalf of researchers around the world who are looking at trends within the CF Community and with the health of those with CF, every year, a chosen group of clinicians and researchers meet to decide what information within the CF Patient Registry should be pulled out and researched further.

This year is the first year where people with CF and their families have the opportunity to drive the CF research agenda.  Now, not only does this specific group of clinicians and researchers have a chance to identify what needs to be researched further but so now does those who are living with this disease day in and day out.

From now until the end of January, the CF community (people with CF and their families) can visit to submit questions about CF and CF trends they want to know more about.

Some of the kinds of questions the Insight CF Project is looking for include:

* Do people with CF in different regions of the United States have different infection rates and health outcomes?
* Do Hispanics with CF have the same expected survival rate as non-Hispanics?
* Do all people with CF eligible for transplant get a referral? If not, are those not referred different than those who are referred?
* What are the factors that make it more likely that people with CF will recover lung function lost during an exacerbation?

After a submission period of about 3 months, the Patient and Family Research Advisory Committee will identify the top submitted questions to put back to the CF community to rank.   Once those questions are ranked as being questions the CF community strongly wants answered, a multi-stakeholder committee (including people with CF and their families) will be formed to study this question further.

I'm really passionate about this side of CF research because, previous to now, so much of CF research (at least from the patient/family perspective) has been focused on "finding a cure."  While that side of research is absolutely critical, I think, just as important is recognizing that there may be many things that we can do right now to extend the lives of those with CF if only we knew to do it.  The Patient Registry allows us to look more closely at what we are currently doing and evaluate ways to do it better.

I am grateful for the foresight of Dr. Warwick and others from the CFF who created and still maintain the Patient Registry.  I am proud that it is a pillar of our CF care.  And I am incredibly excited about the potential that still exists to better use the Patient Registry.

The advantage of people with CF and their families getting more involved in research (clinical trials, tracking their own data, connecting to the Patient Registry work, getting better access to the data) is that doing so helps patients and families be better partners on our CF Care Teams and can improve mental health by empowering us with new ways to fight a disease that often feels like it's in control.

I want to know what I can do right now to improve Bennett's life.  Patient Registry is pregnant with possibilities.  Now, we just have to discover what the CF community wants to know first.

What data in the patient registry do you wish you knew more about?  Submit questions here: and follow the InsightCF project on Twitter at @InsightCF and #InsightCF.  

P. S. Thanks to Aliza Fink the CF Foundation's Director of Epidemiology allowed me to ask her a million questions regarding the Patient Registry and who is a fantastic advocate for people with CF and their families in this particular research space.  For more information on the Patient Registry, read the CF Foundation's blog posts The Patient Registry: Where We've Been Where We're Going and Turn Your Curiosity Into a CF Research Project.

01 January 2017

Our Christmas

Our little family had a really great Christmas.  A few weeks ago, we took the kids to travel the Polar Express for the first time, pajamas and all.  It was a lot of fun.

The kids loved riding a train and getting to "visit the North Pole."  Oliver, who is 9 years old, is becoming increasingly cautious about the magic of Santa but was still this year very open to enjoying things like this.  Brian and I recognize magical experiences in childhood happen for such a short period so we really enjoyed taking the children on the Polar Express. Plus, it's always fun to pretend you are a character in a book!

Each of the children were given a Polar Express golden ticket in which the Train Conductor punched a hole and wished us a safe trip.  While on the train, we were entertained by dancing men and women in white uniforms who served us hot chocolate and chocolate chip cookies.  Then, we read the book at the heart of it all: The Polar Express. Shortly after we arrived at "the North Pole" and Santa came aboard and gave each of the children their very own silver bell.  (It was dark by that point so I don't have a good picture.)

Thankfully, I was able to capture a better picture of the kids with Santa a bit later.

Avonlea and Kelcie, her ballet teacher
Beyond visiting Santa Claus, December brought us lots of fun celebrations...including Avonlea's first ballet recital, which she loved.  I was hesitant to put Avonlea, who had just turned 3, in dance class in September.  As much as I wanted for her to enjoy ballet, our family's time is so limited due to Bennett's treatments, appointments and needs that I didn't know how we'd do it.  But I learned the classes were provided at the school during the school day so it doesn't add one more thing for our family to have to do. Then I wondered if Avonlea would enjoy it and whether or not it'd be worth paying for.  Sensing my hesitation, the sweet owner of the Waco Ballet, Sara, who knows Bennett's story reassured me she wanted Avonlea to be a part of the class and would do whatever she could to make it work for us.  I am so glad she did because Avonlea has absolutely loved it.  I couldn't be any more more thankful for Sara and Kelcie giving us such a fun opportunity to see our little one shine on stage.

 Oliver had a stage of his own at his school's Biography Fair a few days before the Christmas break.  Each of the third graders were asked to choose someone on which to write a biography report and do a presentation.  Oliver chose composer John Williams who is known for his work on Star Wars, Superman, Indiana Jones, Jaws and much more (including writing the Olympics theme song - who knew?!). Oliver did a great job presenting to his peers, parents and teachers.  The children were asked to dress up as their character but Oliver freaked out when I told him we needed to make him look grey-haired and bald.  So, he dressed up as John Williams as a young man instead - ha!

Oliver Christmas, Brian and I took the kids up for a week to visit Brian's parents in Missouri.  We had a wonderful time despite the 3+ stitches Brian had to get at Urgent Care shortly after this picture when he accidentally ran into a pole at the Incredible Pizza Company.

As if getting stitches wasn't enough excitement, things really got real when Bennett's AffloVest stopped working shortly after arriving in Missouri the day before Christmas Eve.  (Apparently there is a known defect in our model but no one had told us.)  

Thankfully, we were able to do some hand percussion lung therapy but beyond it being a lot of work, our not being used to doing it brought us a lot of anxiety.  Were we doing it well enough?  Were we getting all of his lungs, hitting all of the right places?  Bennett is a skinny kid so it's easy to cause him to be sore, which was worrisome to us.  Thankfully, Brian spent much time on the phone trying to find ways to fix our Vest (which was only received last year) or get a new Vest.

At one point, someone tried to rent us a Vest from a local medical durable equipment company but that fell through because they didn't have one small enough for Bennett.  I was glad since "borrowing" equipment that might have been exposed to those with respiratory infections is always anxiety-producing due to infection control.  We were fully prepared we might have to wait until after Christmas to receive a new Vest.

But, lo and behold, on Christmas Eve, just minutes after I had finished giving Bennett his morning CPT by hand, a box showed up at the door.  It was our AffloVest that had been overnighted to us.  I was so happy and filled with thankfulness I cried.  It took several people working overtime to make this Christmas miracle happen.  We made sure to contact each person to let them know their time away from their family to help ours was immensely appreciated.  Families with children with special needs feel so vulnerable at times like these.  It means a lot when people help, even when they don't have to.

Thankful for magical memories, tiny ballet feet, the music of John Williams, extended family we love, stitches for a noggin that got knocked and new Vest this Christmas,
(p.s. Happy New Year!)