System Failure

In the world of cystic fibrosis, the older you get, the more meds they add to your list of things to do.  The body gets sicker, the list of medicines and treatments grows.

Until recently, I really struggled with getting in all the things I have to do to keep Bennett healthy on a daily basis.  For example, Bennett takes 8 medicines three different ways, all by 8am.  It didn't help that that my husband's job demanded that he be gone alot, so it was just me a lot of the time.

I felt so frustrated at myself.  It's easy, as a CF mom, to get signals that somehow, by failing at the incredibly burdensome treatment regimen, I was failing, not just my son but as a mom.  If mom guilt is bad, I'd argue CF mom guilt is even worse.

In general, I work really hard to give myself grace and recognize I'm only doing what I can.  But who wants to hit the bar lower than ideal when your kid's life depends on it?


Several months ago, feeling the pressure that I wasn't doing everything Bennett needed, I said to one of Bennett's CF docs, "Tube feeds ARE a priority. I just have 40 other things that are a priority before that."

I remember thinking right after I said that: Do I *really* have 40 priorities more important that feeding Bennett?!

Then, all of the sudden, a small but very honest voice inside me responded to myself's question with a huge litany of fears and concerns - some I didn't even know existed.

I tried to write them all down.  Here's what I came up with as "top priorities that come before feeding my son his g-tube feeds":
* making sure Bennett doesn't vomit
* making sure Bennett doesn't get C.Diff again
* making sure Bennett gets exercise so his lungs don't get sick
* making sure Bennett doesn't stop eating by mouth
* making sure Bennett doesn't get acid reflux
* making sure Bennett's emotional needs are being met
* making sure my other children's emotional and physical needs are met
* making sure my own emotional and physical needs
* making sure we have insurance coverage for the formula
* making sure we have feeding bags
* making sure we have feeding bag extensions
* making sure the feeding pump battery is charged
* making sure I know where the feeding pump backpack is
* making sure we have enzymes or Reliazorb at home for his tube feeds
* making sure I don't give a tube feed before a Vest treatment
* making sure I put the Liquidgen in with the tube feeds
* making sure I don't freeze the Liquidgen on the top shelf of the refrigerator
* making sure I don't leave the Liquidgen out on the counter to spoil
* making sure I throw the Liquidgen away after it expires in 14 days
* making sure we can slow our family schedule down so we can give tube feeds

That's only 20 "priorities" but, I guarantee if I sit here long enough, I could list 20 more...

My point is that failing to be proficient with the CF treatment regimen is never as simple as "I don't want to care for my son" or "I don't know the importance caring for my son."  The challenge of doing what the doctors want me to do comes down to organization, time and addressing a list of bigger priorities that is a mile long (some, I don't always even consciously know).

Getting all of the things done that people with CF have to do isn't as easy as just checking them off a list.

It's not about willpower.  It's about barriers.

Emotional barriers, physical barriers, insurance barriers, etc.

And something I've recently come to more fully appreciate, it's about system barriers.

Success at home with the CF treatment regimen requires success with aligning the systems we live in.

This is why I have gained much greater success with the treatment regimen lately (I'm now up to being able to give Bennett 3 tube feeds consistently daily).  But it's taken a tremendous amount of oversight, self-reflection and effort.  It's also taken a good deal of money and required I change our physical surroundings.

I think we're set up from here to move forward more easily.  But, I can't help but think about so many of our CF family friends who don't have the time, money or ability to master their entire environment to improve how they take care of CF at home.

For a very long time, it may looked like, on paper, that I hadn't been working diligently to give Bennett tube feeds daily.  Until recently, I was able to get in less than 10% of Bennett's tube feedings (1-2 feeds per week).

Every time I would come to clinic, I would cringe.  At times, I felt scolded and shamed.  It wasn't that my CF team wanted me to feel that way.  The reality is they feel the same pressure as I do - to not hit the bar lower than ideal when Bennett's life depends on it.

But, having said that, there have also been times when I didn't feel my efforts were as fully appreciated - how diligently I was working towards improving that 10%, even as that number wasn't changing.

It's because I was working incredibly hard to change, not the number/behavior, but the system.  For me, what's the point in getting in 100% of tube feeds consistently for 2 months and then burning out completely?  I want 100% for the long term.

CF is a time-bomb.  Time is of the essence.  But CF is a also marathon, not a sprint. I wanted to find a way to take care of Bennett at a pace that works for him, for me and for our family - and in a way that we can continue to support over time. 

As humans, we live in a variety of systems. It's easy to tell myself that I have full control over my life.  But the reality is I live and work in a series of systems that create pressures on me to behave in certain ways.

I have come to realize that recognizing that these systems exist helps me to appreciate why I can totally believe in the value of something and fully want to do it but still struggle to get it done.  It also helps me understand how I can better align what I want with what can do.

For simplicity sake, I love thinking about the 4 primary systems we live in: the micro-system, the meso-system, the exo-system and the macro level system.


The micro-system (individual) is the system I have the most control over - where I place my hair brush, which color I prefer, my choice of hobbies, etc.  This is the place were I made the decision I *want* to give Bennett tube feeds.

The meso-system (social environment) is the system in which my family and friends exist.  This is where, for example, the pressures of my children's needs and my husband's desires exists.  This is the system that I was struggling with when it came to Bennett's tube feeds.

The exo-system (physical environment) is the name that describes the system where pressures are placed on me by my husband's job, my children's school, my co-workers, our church, etc.  This is also where I struggled for a long time to align Bennett's tube feeds.

The macro-level system (community environment) describes the limitations and opportunities that come from my location of where I live and my larger community.

When I want to change something about Bennett's care, I have to evaluate what is and isn't working in a system.  And I have to prioritize the particular system's need for balance.

When I couldn't figure out Bennett's tube feeds, I recognized that it wasn't that I could make a change to my micro-system (I *wanted* to give Bennett tube feeds).  The change that had to happen was within our meso-system (the system of our family) and our exo-system (our home).

It took Brian and me more than a year to make these changes, changes propelled by more than just the need to give Bennett his tube feeds, but most definitely by our need to take the best care of Bennett's health.

We realigned our meso-system.  We withdrew the boys from school and began homeschooling last year to slow down our family life and give us more control over our schedule.  I spent time working with each of the children to be more independent in their self-care and in Bennett's care.

We realigned our exo-system. We purchased a new home, specifically with Bennett's CF care in mind.  (I cannot even express what I huge difference this has made!)  Now I can keep more supplies on hand, have them readily available and identify when I need to restock.  A new home layout also allows Bennett to have more space to do his treatments where it feels functional and less in our face or as a mess on the counter.

Even though my micro-system was fully on board with giving Bennett tube feeds, I made changes there too.  I started using FolioHealth, a new pilot tracking platform which helps me accurately track how successful I am being with the treatment regimen and identify patterns that prevent me from getting it all done.  (Another game changer!!!)

And I designated someone in my life who checks in with me regularly about Bennett's care to help me recognize when Bennett's CF regimen is too much for me to handle emotionally and encourages me to find a way to get a break.

Change cannot be made in a vacuum.  We live in the midst of complex systems.  When CF families like mine are asked to do "one more thing", it must mean that everything else must move to make room for that one thing.  Considering we are asked to do "one more thing" several times at every quarterly appointment, that means we are trying to make room for up to (or even more than) a dozen new things per year.

I am tempted to feel like a failure when I can't do that.  But I have to remember that it's not me that's a failure, it's the failure of a system.  I just have to change my system somehow.

It might not have looked on paper for a long time that I cared about improving Bennett's nutritional health but the very opposite is true.  Now that we've got our working systems in place, hopefully the paper will accurately reflect all our hard work!

First Day of School 2018

Today was a momentous day in the Gamel household, the start of our newest adventure as a family.  It was the first day back to school - at a brand new school - for Oliver and Bennett.  (Avonlea had her first day back to school at her school a few weeks ago.)


We are all so grateful for such a wonderful year homeschooling last year.  A year of homeschooling was like water to all of our souls.  We were able to rest.  We were able to gain back family time.  And the boys were able to gain back self-confidence after having had challenges the year before with both cystic fibrosis and dyslexia.

A year and a half ago, I struggled to figure out what the best school option for the boys would be moving forward after a difficult year.  Should I homeschool?  Might another school in our area be able to better meet the boy's needs and the unique needs of our family?  I contemplated opening my own school after discovering a fantastic school model in Austin called "Acton Academy" that I would have loved to replicate in Waco.  I was even awarded a grant last fall and attended a 3-day think tank in New Orleans with 4.0 Schools for people around the US who want to start their own schools.  But, alas, I struggled to feel settled that I could run a school, take care of 3 young kiddos and deal with Bennett's ever-unpredictable cystic fibrosis.

Ultimately, our family chose to homeschool last year and we were very happy with that decision.  The only downside was that my boys, having been used to attending school daily, missed learning within a community of their peers.  Homeschooling was so good in so many ways.  But, since we didn't join a co-op our first homeschooling year, we found it could also be lonely.

Having experienced the beauty and flexibility of homeschool but also the community and connection of traditional school, Brian and I wished there was another option - something in between homeschool and traditional school.  This is where that little school in Austin called "Acton Academy" looked so interesting to me.

Well, low and behold, this summer, I heard from a friend that there was someone in Waco who was creating a school called "Acton Academy."  I couldn't believe it.  I called up that someone, who now is a friend of mine, and shared our story.  It was then that I knew, our boys would be 2 of the 22 founding students of the school now officially called "Acton Academy Waco."

Oliver, First Day of 5th Grade

Oliver, 10 years old

Bennett, First Day of 3rd Grade

Bennett, 8 years old
The boys were very excited to go to school this morning.  This new school seems like a great match for what they need.  It's a school that is focused on helping each child discover their abilities and talents, in addition to mastering their academics.  CF brings Bennett a lot of challenges but also brings him a lot of gifts.  Dyslexia does this for both boys, as well.  I look forward to seeing how Acton Academy will help the boys discover, in a self-directed way, how to use their strengths as leverage over their weaknesses.  



Some things I love about Acton are that it offers a mixed-age classroom, gives no homework (yes! let them play!), focuses on building character, uses socratic discussions, uniquely integrates technology, offers alot of freedom to the students and is overall very intentional.

I don't believe there is one school or one school model for all children.  Just as children are different, so are their needs for different school models and communities.  Brian and I consider it a great privilege to have a choice in the way we educate our children.  We realize so many families never get that opportunity.  Brian and I are evaluating each child and each school year as we go.  I'll homeschool in a heartbeat again in the future if any of my children need me to.  But, I'm also just as happy to kiss them goodbye and wish them a really great day at school.  



Of course, I can't fail to mention that there's another little Gamel experiencing a new adventure this year.  Avonlea started Kindergarten three weeks ago.  It's hard to believe our sweet baby is a kindergartner.  But it makes it easier when we see how much she loves it!  

Summer Adventures 2018

It's been a while since I've posted so I thought I'd share pictures of our family's adventures this summer:

In June, Bennett entertained us as a Oompa Loompa in the Waco High School sponsored children's theatre production of, "Willie Wonka and the Chocolate Factory."


Bennett really loves singing, dancing and making people laugh so it's no surprise that theatre is right up his alley.  We did have one day at theatre camp where a little boy a few years younger than Bennett accidentally pulled out Bennett's g-tube while he and Bennett were playing chase.  It was pretty stressful because I didn't have a necessary tools to put his g-tube back in quickly.  We worried we would have to go to the ER to have them help us.  But, fortunately, we were able to get the syringe we needed to put it back in.  I figured Bennett would be so shook up after this situation, he might not want to return back to the theatre camp afterwards.  But, he did want to go back.  I cherished this moment because, as Brian pointed out later, it was an example of how Bennett has developed the emotional resources to withstand the interruptions CF has in his life.  His resilience is admirable.  Despite being in tears and afraid he was going to the hospital one moment, the next moment, he was wanting to get back to theatre.


Recognizing that the kids are beginning to appreciate theatre productions more and more, Brian and I decided to take the kids to their first Broadway musical, "The Lion King" in Dallas this summer.  The boys loved it.  Avonlea did too but the play started her bedtime so she was anxious for it to be over towards the end.  The funniest moment for me was, at climax of the play, during one of the moments when the entire theatre was silent, Avonlea drew a deep breath, sighed loudly and with resign said outloud "I like the movie better."  Obviously, she's still learning the value of live plays.


In July, our family traveled to Florida and enjoyed some time at the beach.  Oliver says he would live at the beach if he could.




Of course, our biggest adventure this summer was moving to a new home.  We moved after eight very special years in our home in Waco.  It was hard to leave the home in which we brought Bennett and Avonlea home from the hospital and from the only home Oliver remembers.  But, the good news for everybody is we moved just about 15 minutes away and we now have more space for our once-babies to grow into teenagers (which is happening faster than we'd like to admit).


Speaking of growing, Avonlea turned 5 years old this summer.  She's very proud to be so big and has declared that now she's five, she now should be eligible for a puppy. :)


Summer 2018 has been good to us.  And, I might be super excited about the next adventure this fall... (EDIT: oh no, I'm so sorry if this blogpost was misleading - no, I'm not pregnant!  It's not that kind of adventure news!! Our little family is very much complete! :))

Update on Bennett: Asthma



Bennett went to his quarterly CF appointment this week.  Overall, he received a good report, I think?

Bennett continues to have no GI issues at all, which is fantastic.  The main issue we are still dealing with is improving his overall nutrition.  Bennett is in the 4th percentile for BMI.  He should be in the 50th.  His height is staying on the growth curve but his weight continues to struggle to stay on it.

It's very defeating to watch his weight not be able to keep up with his height, even as he eats really well.  But, it's just a reminder that people with CF need a significant amount of calories to keep themselves healthy.

We continue to work towards more g-tube feeds per day, but the treatment burden for daytime g-tube feeds is really burdensome and challenging (think 3 30-minute feeds while at home in addition to 15 minutes of prep work each time, not including all of the other meds and treatments Bennett has to do + life).  Nighttime feeds, which many people with CF do instead of daytime feeds, is very disruptive to Bennett's and our family's sleep so, for now, we work for daytime feed success.

Avonlea and Bennett graciously obliged to my picture taking effort.  Avonlea was just about to devour a Hostess cupcake.  Bennett was drinking a Capri Sun.  Snacks are a bit part of making 2-3 hour CF clinic appointments manageable.  Oliver is missing from this picture because he was at camp for the morning.

Bennett's respiratory health check this time was an interesting one.  Although his lungs sound healthy and he is showing no respiratory symptoms (such as a cough or runny nose, etc.), Bennett's PFT (pulmonary function tests) results were not very good Tuesday.

Since age 4, Bennett has been doing a PFT test at every quarterly appointment.  One year ago, he blew a 96% FEV1.  At this appointment, he blew a 80% FEV1.  It's expected that people with CF will see a 2% FEV1 drop, on average, per year due to lung damage.  But, that 2% is a far cry from the 16% drop we saw this week. Typically, significant drops in FEV1 typically indicates evidence for lung infection.

When we saw a drop in Bennett's FEV1 during his last CF appointment 3 months ago, we hoped it was a fluke.  The doctor prescribed an oral antibiotic in hopes that it might address any possible lingering infection.  Upon our discussion, at that appointment, as to why Bennett's FEV1 was lower than normal, it occurred me that Bennett had not had time to do his albuterol treatment that morning (since we were rushing to get out of the door as we live 2 hours away from the CF Clinic).  Bennett's pulmonologist agreed that missing an albuterol treatment could potentially have a negative impact on his numbers.  She suggested that at our next appointment that we should get a PFT without albuterol and then get another with albuterol to see what difference it makes.

Here's what happened:

Turns out, albuterol makes a world of difference for Bennett.  He had a 11% change in FEV1 with albuterol and a 31% change in FEF 25-75% with albuterol.  This was significant, the doctor explained, because it means that the restriction of air Bennett has in his lungs (aka the reason his PFT numbers don't look good) is likely due to muscle constriction, not mucosal constriction.  In layman's terms, Bennett's lung issues may be being caused by a case of asthma, not infection.

This blew my mind.  Here we were afraid of a lurking infection and all the while it may simply be something much less worrisome - asthma.  This makes sense given the fact that Bennett has stayed very healthy and is showing no respiratory symptoms.  And yet, not knowing asthma might be the culprit, we were coasting towards having to treat an infection we could only believe might be causing silent damage to his lungs.

Bennett was prescribed an inhaled steroid in hopes that will help him gain back his full lung capacity.

As soon as we came home, I did some research to better understand any connections between asthma and CF.  Turns out there is one.

As this study reports, "patients with cystic fibrosis (CF) have a higher prevalence of asthma than the background population."  The study goes on to say, "when CFTR function is absent in new-born animals, airway smooth muscle dysfunction and airway obstruction develops independently of airway inflammation and infection....This supports a link between CFTR dysfunction and asthma...asthma has been suggested as a CFTR-related disorder, that may occur in CF heterozygotes when unknown additional factors further reduce CFTR function or interact with CFTR on asthma."

I find this interesting for several reasons.  It makes me wonder if people who are carriers of CF (people, like Brian and me, who carry 1 gene for CF) are at a higher risk for asthma.  It also makes me wonder if the CF community, particularly parents of young children with CF, should be more aware of the high numbers of asthma in children with CF and the potential role asthma might play in the person's with cystic fibrosis health.  Although I knew other CF families who had been diagnosed with asthma, I had not been aware, until now, what kind of impact it could have.  For me, it begs the question - should everyone with school-aged children with CF try a pre and post PFT with albuterol to see how they respond?

Bennett loves making the chicken "run" during his PFTs.  This chicken image moves across the screen while doing in-room Pulmonary Function Tests to help children blow out their breath a designated amount of time.

This experience of recognizing asthma in Bennett really surprised me because had we not found it, we would have been talking about IV antibiotics during this appointment.  IV antibiotics have not been completely ruled out.  If Bennett's PFTs don't improve in the next few weeks with his daily use of an inhaled steroid, we might be eventually looking at being admitted to the hospital for powerful meds to help out his lungs.

But, considering Bennett has not cultured anything other than MSSA, considering that his lungs sound clear and considering he is showing no respiratory symptoms (no runny nose, cough, etc) and has not been sick, I am feeling like his drop in PFT numbers is probably due to asthma.

Only time (and a PFT test in August) will tell.

The best thing the CF Foundation is doing that you've probably never heard of...


If you are in the CF community and haven't heard of it yet, you will soon enough.  It's making it's ripples and those ripples are mighty.

It's called the CF Learning Network (CFLN).


I just returned from our most recent CF Learning Network Community Conference in Minnesota this past weekend, which was really fantastic, and I thought it might be a good time to share about the CFLN so others know what great things are going on in our community.

The CF Learning Network is a network of CF Care Centers, patients and family members and quality improvement experts working together to improve the way we do CF care.  The CFLN has been a 3 year pilot, funded by the CF Foundation, to find better and faster ways of improving health outcomes for those with CF.

The CF Learning Network is one of 9 networks run by the Anderson Center at Cincinnati Children's Hospital and was brought to the attention of the CF Foundation by my friend and fellow CF mom, Erin Moore.  Erin saw other disease communities making impressive progress and wanted those with CF to have the same benefit.  Fortunately, the CF Foundation agreed.

The goal of the CF Learning Network is to learn together to find ways to improve CF.  If one CF Care Center has better data - such as, if someone knows how to improve lung health well,  the work of the Network is to help that Care Center identify what specific things they are doing in order that other CF Care Centers can replicate their process and improve lung health well too.  It's not about finding which CF Care Center is best (using data for judgment)...but it's about finding ways so that everyone can do it best (using data for learning).  If one of us improves the way we do CF care, we all should improve the way we do CF care.

As my friend Erin often says, "improved CF Care shouldn't happen by chance."  Improved CF care should happen systematically.  That is what the CF Learning Network is trying to do - systematically replicate good practices to improve CF Care faster.  This is done by using specific tools called, "quality improvement skills."  These quality improvement (QI) skills can easily be learned and used by both clinicians and patients/caregivers to learn, replicate and improve the way we do things.

In addition to the use of quality improvement skills to make good CF Care replicable, another important focus of the CF Learning Network is to improve "co-production."  Co-production is a buzz word that refers to patients/family members working together with clinicians to improve care.



Every team within the CFLN has been asked to have at least one Patient and Family Partner on their team - every CF Care Center that participates in the CFLN must work with one person with CF or a family member to work alongside their Quality Improvement (QI) team to make decisions.  My own role in the Network is to both support our Patient and Family Partners in their unique positions and to learn how teams can improve that partnering experience for everyone involved.

The CF Learning Network is working hard to improve the asymmetry of the patient/clinician relationship, not just when co-producing on a Care Center-level but also on an individual co-production level when caring for a patient in the exam room.

For so long, health care has been paternalistic. Doctors/clinicians were once thought of as the all-knowing "experts" who "give health" to patients/family members.  But, times have changed.  Now, health care is patient-centered.  Thanks to the internet, patients and caregivers have access to more information than ever before.  And now, clinicians are become more and more aware that there is no one more engaged in their own health than the patients/family members.

So, the CF Learning Network is capitalizing on this new way of respecting each other and working together. No longer are patients/family members and clinicians working on two opposite sides of the health care system, we are one.  We are on one team - two experts (clinicians, the experts of CF and patients/caregivers, the experts of their own experiences) with two separate sets of skills and knowledge sets - working together for the same goal.

I have been involved since the early design phases and have watched the CF Learning Network grow from a small group of patients/family members and clinicians to almost 30 Care Centers involved in the work.

Why is it important for the CF community to know about the CFLN?  Because it's a game changer.  It is the hope of the Network that as more people participate in a new way of doing CF care, our ability to improve the lives of those with CF will only get easier and better.  Excellent medicines for CF are coming.  But until then and even after, we've got to figure out better way to manage this disease and it's symptoms in whatever form it continues to exist.

CF Moms Pam, Erin and me - we've been working on this Network stuff for a very long time!

This past weekend was our 3rd CFLN Community Conference.  We spent 3 days listening to presentations, learning what others are doing well and discussing ways to improve together what we are already doing.  There are common measures/goals the entire CF Learning Network has decided to focus on and everything that we do within the Network is to improve those particular measures/goals.

Things we often talk about within the CF Learning Network are the burden of care for CF, the quality of life of those with CF (and their families), the new challenges that come with growing life expectancies and new medications coming on the market.  We've dawned a new day in CF care and I am grateful to be a part of a new way of addressing those changes.

If you'd like to know if you're CF Care Center is participating, click here: https://www.cflearningnetwork.org/partners/.  If your CF Care Center doesn't yet participate, let them know you would like them to do so in the future when the Network opens up participation again. 

The CF Learning Network has historically been called the "CF Care Model of the Future." But I am so grateful so say the future is here.

An update on Bennett

We were able to escape the flu this year.  In January, Brian and I decided to keep Avonlea from preschool for about six weeks and we decided to stay away from the public as much as possible.  This turned out to be a good decision.  Our entire family stayed very healthy throughout the winter.

We even pushed back Bennett's quarterly CF appointment until Spring to prevent Bennett being needlessly exposed to the flu.  So, Bennett finally had his CF Clinic appointment today. 


To our surprise, Bennett's lung function went down.  It is so weird when this happens because he's showing no respiratory symptoms.  Our CF doc thinks the lower lung function is either a fluke (Pulmonary Function Tests, known as PFTs, can be difficult for kids to get just right)...or she thinks it's due to a typically common bacteria called MSSA (Methicillin-sensitive Staphylococcus aureus).  Because we've seen his lung function drop before, Bennett will be given a round of oral antibiotics to treat MSSA, which worked in the past.  There's a chance that this drop in lung function could be due to some other bacteria or fungus in his lungs.  But we won't know this until we get back our routine throat culture results next week.  In general, people with CF have a 3% drop in lung function each year.  But, it feels pretty worrisome when it happens without explanation.

Today's lower lung function is just a signal that CF is working behind the scenes against us. Eventually, if we can't get Bennett's lung function back up, Bennett will require IV meds and a lengthy hospitalization.  We aren't there yet.  But we're inching closer each time this happens.  It's so frustrating to have CF slowly take away Bennett's ability to breathe and not to really be able to do much about it.  There are meds to treat.  But the meds really just delay the inevitable.

I don't feel hopeless.  But I do feel helpless.

Bennett's lungs have been so healthy.  He's eight years old and so many children with cystic fibrosis never get to age 8 without an exacerbation or without a serious lung infection.  So, we feel tremendously blessed that he's this healthy this far in to his life.  But, I still feel sad about today's drop in lung function.

To add to my feeling a bit bummed about today's appointment, Bennett continues to grow on his growth curve (height and weight) but he's still underweight and under where we all want him to be.  He needs the extra weight to help him grow taller and to help him address future health issues he's inevitably going to run up against.  Good nutrition is really critical.

But, weight gain feels so elusive to me.

Twice daily treatments, 4 nebulized medicines, every-other-day cleaning and sterilizing of equipment, 3-4 enzymes 7 times a day (every time Bennett eats), 2 meds given at night, 4 meds given in the morning, 1 med given 3X a week, 1 capful of Miralax in 4oz Gatorade given daily, 2 gummy vitamins...

and that doesn't even include the routine for gaining weight...

30 minutes 2X daily on the feeding pump with Reliazorb cartridges, along with 15ml of liquid fat given 3X daily

It seems so simple.  But it's a mess to organize.

I have to have the feeding bags, extensions, the feeding pump, syringes, the Reliazorb medication, the Liquidgen liquid fat and the formula.  Only three of those items insurance will pay.  In addition to the 15 minutes it takes two times daily to set up the feed, Bennett has to find an hour in his day to be sitting on the pump.  Of course the feeding pump has to be charged and Bennett has to be able to tolerate the forced feeding, which often causes him to feel nauseous, overwhelmed by acid reflux and gastrointestinal issues.  Very often, by the time we get one thing in, we find ourselves running up against something else with CF that we haven't gotten in.  I end up having to prioritize his CF care to evaluate which of the things gets "bumped" for the day.

Every day I set out to do all the things on the list of things to do for CF.  And every day, I feel the struggle of managing CF, as well as everything else within my power to manage in my role as a parent, wife, friend, daughter and healthy-self.

What's equally hard is knowing in the back of my head that CF doesn't quit.  CF doesn't go into remission.  In fact, it just gets harder, throwing bigger punches as time goes on.

To prevent feeling discouraged, we work to celebrate the joys we have right now.  Delaying CF's erosion of the body is still a huge win.  Bennett's lung function went down but his lungs still sound great.  Bennett hasn't gained as much weight as he needs.  But he didn't lose any and is continuing on his growth curve.

I am confident we'll get that lung function back up and will make strides in his weight gain.  We made it through a pretty yucky flu season.  We'll make through this hiccup as well.

Why getting the flu is so scary for Bennett

Since returning home from the hospital two weeks ago, Bennett has been doing really well.  He feels great and is showing no more GI symptoms.  He's on a capful of Miralax a day, which seems to be just perfect for him.  So, now, we are turning our attention on trying to keep him healthy during the flu season.


The flu is always scary when you have a child.  But, considering this is potentially the worst flu season in history and considering the fact that we already deal with an underlying chronic disease like cystic fibrosis, I literally wake up every morning thankful that we have escaped it one more day.

I know I can't do much to prevent getting the flu.  The flu is practically everywhere.  But we're certainly trying to be as safe as possible over here.

There are four main reasons why the flu is particularly bad for Bennett (and all those with cystic fibrosis):

1.) The flu risks serious complications in people with cystic fibrosis, including death.  CF already causes the body to be inefficient in providing the body nutrients and working properly.  The added stress of the flu and lack of appropriate fluids can cause the body to struggle even more.  This can affect the liver and the heart in ways that are particularly serious.

2.) The flu risks Bennett losing weight he can't afford to lose.
Bennett has struggled with gaining weight since he was a baby.  He is consistently underweight and too low for the BMI he needs to have to be healthy.  Getting sick only taps his body more and uses up the limited resources he has.

3.) The flu risks a hospitalization and getting sick with something else.
Our recent hospitalization only further motivates us not to want to land in the hospital.  Thankfully we made it out of our last hospitalization with no flu or additional sickness.  But the hospital is full of bugs that can make Bennett very sick.  So, we want nothing to cause us to be forced back in to the hospital again.

4.) The flu risks damaging Bennett's lungs in a way that he may never fully recover.
This is the part of the flu that most healthy people don't ever have to think about.  Most of the time, our bodies repair themselves without any issue.  But, in CF, it's different.  The word "fibrosis" in cystic fibrosis means scarring and thickening, which is what happens to the lungs when they continue to try to repair themselves. Many times it's a cold or the flu that can bring upon an infection that gets stuck inside the CF lungs.  CF lungs can continue to struggle with it for a very long time, causing damage over time.  In addition, CF lungs are susceptible to lots of rare "opportunist" infections that look for opportunities to nestle themselves in the lungs when the person is already fighting another infection.  It becomes a problem on top of a problem on top of a problem.  So, while the flu may be well over, a new issue in the lungs may rear it's ugly head.

What we are doing at our house to prevent the flu:

It turns out that homeschooling the boys has been really beneficial with regard to their exposure to germs this winter.  In fact, realizing Avonlea was still a risk of bringing it home, we decided to pull her out of preschool for a few weeks to minimize our chances of Bennett getting sick.  Based on the reports I'm getting from inside the classroom, it seems like this has been a good decision so far.

We are minimizing getting out in public as much as possible.  But if we have to get out, we get out during the week, particularly in the mornings, when places are less frequented.

We spend a lot of time outside, playing on bikes and playing in the sun (when it's warm).

We've already gotten the flu shot (something we do every year).  And we're poised to get Tamiflu if necessary.

We are asking anyone who comes over to our house or to play to make sure they haven't been around anyone who is sick in the last 48 hours.

We are washing our hands, using hand sanitizer, not getting too close to strangers and regularly talking about how to prevent getting sick.

However, I feel pretty helpless right now.  It's such a fine balance.  While staying away from sick people is beneficial, staying away from people, in general, can begin to feel depressing.  So, it's a balance between not becoming hermits but also staying safely away from those who may pose a risk to Bennett.

Thankfully, our friends and teachers have seemed to understand.  It helps to talk to other CF parents online who articulate they are dealing with the same fears.

It's hard to be afraid of invisible germs you can't see.  You can feel like you're crazy.  And yet, when I talk to people who have had the flu this year, they remind me I'm not.

It always feels good when someone says to me, "you're doing the right thing" or "that's smart."  It makes me feel better about leaning on my own motherly intuition to keep my child safe.

One of my new favorite crowdsourced websites right now is Flu Near You, a website where you can track flu activity near you.  It's not quite as robust as it needs to be but it's a great way to capture what's going on with the flu on the ground.

Reports say the flu season may be peaking right now so I'm hopeful we're headed out of this flu seasons soon.  Of course, reports are we still have another 11-13 weeks.  And even then, we may still get it.  But I'm trying to do whatever I can to use the knowledge I have to keep Bennett's lungs in the best condition they can be for as long as can be.  That's my job.  I'm his mom.

Rest


We were discharged and arrived home at 9:30pm last night.

This morning, four out of the five of us didn't wake up until 10:30am.  It was so evident that each of us had much-needed sleep to recover.  It felt good to wake up feeling rested.

Since Brian had to go to work this afternoon, we spent the day as a family trying to make up what we lost over the last two days.  We unpacked.  We took down our Christmas decorations.  We shared a meal around the dinner table.  We rode bikes.  We played Barbies.  We rested.

Throughout the day, I found myself reflecting on our recent hospitalization - moments would flicker in my mind - no doubt an effort to process it all.

Today, I reflected on our super fun ER nurse who was with us the 10+ hours while we were there.  He was so on top of everything and made our time there as comfortable as possible.  He asked for things on our behalf even before I did.  (Really, all of our nurses were wonderful this hospitalization.)

I thought back to the Radiologist who made a really bad gastrografin enema just a tiny bit better by her attentiveness to details and compassion.

I laughed to myself today when I remembered how random it was that Bennett and I accidentally ran into our GI doc in the hallway after Bennett's first X-ray early Friday morning.  Although typically I will contact our GI doc to let her know when we go to the hospital, I hadn't yet had a chance to do so since we had come overnight.  From that point on, even though she wasn't on call at the hospital, she stayed in contact with all the doctors involved in our case and helped decide what should be done for Bennett.  In my disappointment of being admitted, she called and helped me better understand his situation.  I rested in the trust I had in her, that she knew Bennett's case well, that she knew we didn't want to be there and that she would advise the docs on whatever she felt was best for him.

I have continually been touched by the many friends and family who have reached out to us via text and social media letting us know that we're not alone, that they are praying for us and that they are cheering us on. 

This afternoon, I received a sweet email from Bennett's CF pulmonologist who had heard from her colleague that we were in the hospital and just wanted to check in.  She even included an email to Bennett to let him know she cared.

I really hate CF.  But these little moments of support and love are a bit like a mother's kiss - Nothing can take away the situation.  But connection of people who care does make it feel just a little bit better.

For everyone who continues to ride this journey with us, thank you.

This is what crazy is made of - 3:45pm post

(Bennett's face captures his excitement of eating for the first time in two days.
I put his spaghetti in a styrofoam cup, which is weird unless you consider so is the smell of hospital dishes.)

Last night, upon leaving the hospital, Brian and I called our parents to let them know Bennett might need surgery.  Both of our mothers actively discussed plans to possibly come to Waco to help us.

And yet, 24 hours later, we're being told we will be discharged tonight.

As quickly as things went south, things have gone north again.  Don't get me wrong.  I'm happy.  In fact, I'm thrilled.  But this is what crazy is made of.

Normal life...serious condition...hospitalization...possibly more a serious condition...then back normal life.

As quickly as we can accommodate to one thing, we end up having to accommodate to another.  Sweet Oliver cried over dinner tonight because we'd not be staying the night after all.  It was just last night that he was begging not to have to leave our house and to go the hospital in Dallas.  These schizophrenic changes have an affect on each of us.

Nonetheless, being discharged tonight is excellent news.  This afternoon, Bennett's IV blew (aka started swelling his hand) so they took it out.  He begged that we not put it back in.  They said they wouldn't replace it as long as his bowel clean out was done.  About that time, Bennett's colon showed signs it was fully cleaned out.




The docs asked for one more X-ray to confirm.  If it looked good, they told him, he could eat.  So, Bennett has started eating.  If he can tolerate his food, which everyone expects he can, we'll pack up and go back home tonight.

We're really glad this is over.  But, part of me is asking, "Am I crazy? Did it really just happen!?"

Moving along well - 12:47pm post


Bennett started responding to yesterday's enema around midnight so the team started him on GoLightly, which is used to clean out the colon.

At 6am this morning, Bennett was given a belly X-ray.  The X-ray showed marked improvement so Bennett's 8am enema was cancelled.  Surgery has also been taken off the table.

About an hour ago, three Gastroenterology med students, 2 residents, 2 fellows and an attending came into our room to give us an update.  The attending explained that his X-ray looks so well they believe we are on the downhill slide.



Yesterday's x-ray (left) vs today's x-ray (right); black insight the belly is trapped air between the stool

Bennett has been given the green light to start clear liquids.  Once he has nothing in his bowels, they will let him start eating again.  They have started enzymes (without food).  Since Bennett's bowels being cleaned out is what we are waiting on, there is a good chance that we may be discharged tomorrow.

The attending said part of the complication with Bennett's situation is that he didn't present as a classic DIOS case.  DIOS cases usually show symptoms happen quickly (within a few days).  But, for Bennett, he was presenting symptoms several weeks ago.  So, the team felt very confused on whether or not his issues were a stricture, constipation or DIOS.

Bennett is feeling better now that his belly isn't full.  Thankfully, Bennett has felt good most of the time here.

Bennett did share his thoughts this morning when Brian took Oliver and Avonlea downstairs.  He told me, "I was telling Daddy last night about how I'm scared about the future.  I'm scared because I don't know what's going to happen with CF."

I just listened but I wanted to grab him close and reveal to him: that's the *exact* feeling I'm feeling right now, Bennett.

He went on, "It's like you're at home and then you go to the hospital they say you can't eat and you have an enema.  The hardest part of the future is not knowing.  I feel like, it's a maze...like God can see it on the top.  But we can only see down at the bottom."

We prayed last night, all five of before Oliver, Avonlea and I left to sleep at my sister's house.  We prayed that God would give Bennett peace, that the treatments would work...and that Bennett wouldn't have surgery or an enema the next morning.  Part of me cringed because I didn't want him to be heartbroken if God didn't answer his prayer.

But Bennett reminded me this morning that God had heard his prayer.

Brian told me that he shared that when Bennett told him how he felt scared about the future with CF that Brian responded to him, "even though we don't know what's going to happen in future, we know that God sees the future and is already there preparing good things for us."  He said he told him, "That's why we can wake up each day and look forward to it because each day is a gift that God gives us, it's a present we can unwrap."

I'll be honest, I haven't enjoyed unwrapping the days where CF is involved.  And hearing Bennett say that he is scared of the future is hard to hear because I know he doesn't even know what statistics say the future holds with CF.  He only knows his short life experience with it.

But it's good to hear him talk about his fears.  And it's good to hear my husband remind me God is there.  And it's good to remember that yesterday is over.  Tomorrow has not come.  Today is what we have.  And today is looking pretty good.

Hoping this is all over soon,