25 November 2018


Things have been very peaceful with regard to Bennett's CF as of late.  (Peaceful, as in 2 Vest treatments, 3 inhaled medications, 3 tube-feedings and 15+ pills on a daily basis...but you get the point. ;) )

Bennett's GI issues seem to be in check.  And, despite my recent fears about potential lung issues, Bennett blew a FEV1 of 95 (out of 100), which seems to indicate he's doing well.  Bennett is still in the 3rd percentile for BMI but gained .5 lb and .5 inches in 3 months.  We are giving him 3 tube feeds a day so we hope we can increase his weight and height even more.  I'm trying to figure out if we can increase it to 4 tube feeds a day without inconveniencing either one of us.

This morning, I sat down with Bennett to go through a worksheet with him.  I had received a "adolescent transition worksheet" from a friend whose child is seen at Cincinnati Children's Hospital in Ohio.  I learned about this worksheet while in the midst of a conversation with a friend about "transition" in CF Care.

"Transition" is the word that describes moving from pediatric CF care to adult CF care.  Until even as recently a decade ago, most CF patients were being cared for, even as adults, at pediatric clinics/hospitals.  This is because CF has historically been a childhood disease - people with CF didn't usually live in to adulthood.  Thankfully, things have changed.  Projected life expectancy for CF is now 41 and these days, most people with CF are expected to eventually move from pediatric CF care given pediatric hospitals to adult CF care provided at adult hospitals.  The process of moving from pediatric to adult CF care is often referred to as "Transition."

Bennett is still 10 years away from "transitioning" to an adult CF clinic.  But, I tend to see transition in CF as being less about learning how to move from one hospital to another but instead learning how to manage CF as a child to managing CF as an adult.  And that transition happens from birth.

In the same way all children are given "teenage years" to practice being an adult.  I believe, there should be a similar transition phase for kids with CF to practice being an adult with CF.  This is why I was so interested in filling this worksheet out with Bennett this morning.

Transition is still a relatively new concept in CF care.  So, there aren't a lot of resources for transition of CF care available yet.   That's why I was grateful to get my hands on this resource to try out with Bennett, even if it was a bit premature for his needs.  My goal in completing this form together was just to create a path in my own mind, for what transition might look like.

Right now, as his mom, I carry most of his disease (along with his dad).  I know most of the information.  I make most of the decisions and connections.  I address most of the problems.  I'm in charge.  But, one day, Bennett will be the one who primarily carries his disease, who makes the decisions and the connections, who will be addressing the problems and being in charge.  I don't see that happening late in high school or when he goes to college.  I see that beginning to happen now, ever so slowly, so that the both of us can get used to our new roles - me in the background, he in the foreground. 

This transition worksheet, which Bennett and I did after breakfast this morning, allowed Bennett and me to have a really great conversation about his CF care and his ability to advocate for himself.  I saw him completely light up when he shared with me the things he is proud that he could do.  Bennett was very honest about the things he can't yet do.  And, he asked me to start teaching him about the things he doesn't know much about or how to do.

So, at nine years old, what exactly can Bennett do and what does he understand about his care?  What does he still need help with and how can we, as his family, support him to gaining greater independence with CF?

To my delight, Bennett indicated he can describe CF other others.  He can speak up for himself and tell others what he needs.  He can take part in healthcare discussion about himself (I owe a lot of credit to his CF docs who do an excellent job of talking to him directly when we're in the room).  And Bennett indicated he understands the importance of regular exercise and the importance of sleep and rest.  This was all really great for both of us to hear!  There is a lot that Bennett is already doing well in his transition to owning his own CF care.

There are also areas that Bennett acknowledged he needs help with.  Bennett explained that while he can take his own medications, he still needs help to prepare his meds.  He also needs help properly cleaning his breathing/respiratory equipment.

Bennett said he mostly understands why he takes most of his medicines but acknowledged he didn't totally understand why he had to take certain meds or why they were important to take.  In addition, Bennett asked to learn more about how, if he wants to know more about CF, he can get that information.  He suggested: "maybe I could learn how to call the doctors??"  I so love this!  Truly, this is transition conversation has gone well when my nine year old wants to know how he can call his CF clinic to ask questions.

Things I didn't really know about Bennett, but that I learned from this exercise, is that Bennett doesn't totally understand infection control (the reason why people with CF can't be together and why CF clinicians have to gown up and glove at clinic).  In fact, when I tried to explain it to him, his eyes welled up with tears.  It was hearing that his "lungs are sick" that made him start to cry.  I had to quickly caveat it with, "well, they are sick but they aren't.  I mean, you aren't actually sick right now but your lungs are susceptible to others germs so they are kinda sick."  He asked me, sort of offhandedly, for the third time recently, "am I going to die?"

I need to answer this question for him but I wasn't ready to at this moment.  That's a transition conversation I'm not quite emotionally ready to make.  I also skipped the question about whether or not the person with CF understands his/her issues with infertility.

I really appreciated this little checklist for the opportunity it gave Bennett and me to sit down and talk about aspects of his disease that he and I often take for granted that he may already know.  Bennett really loves it when I give him my full undivided attention.  So, we both really enjoyed the intimacy of the moment of sitting down and talking through how well he takes care of himself and identifying ways he wants to do that even more.

I'm incredibly proud of Bennett and the person he's becoming to be.  So few children have to carry such a difficult burden on their shoulders.  And he does it with such strength and grace.

A well-known psychologist once said: "the goal of raising children is getting them to adulthood as fully intact as possible."  Maybe, I'd add, the goal of raising children with cystic fibrosis is getting them to adulthood as fully independent as possible.

I'm grateful Bennett is making it very easy for me to begin that process now.

02 November 2018

NACFC 2018 - My Reflection on the excitement of CF Modulators

NACFC didn't feel quite so overwhelming to me this year since this was my third year to attend. I was more familiar with the format, the expectations, the pace of sessions.

But, there was a moment that took my breath away when I least expected it to.  

It was during Plenary 2.  I was sitting in the middle of literally the hugest auditorium I've ever been in.  I was listening to the newest information about CF research.  A picture of a little baby came up on the screen (see what I'm talking about here at the 53:35 mark).  NACFC Speaker Dr. Felix Ratjen had spent the last hour explaining all the success we've seen with the newest CFTR modulator medications and wondered outloud, "how about if we start [these meds] early on in babies, in patients, before the lungs are damaged?""

What if we give newborn babies, still yet free of CF damage, the newest CF drugs available?

It was a hopeful statement.

Suddenly, I had this flash of memory to the previous plenary, Plenary 1, held the night before, when speaker Dr. Lisa Saiman shared other new clinical information in CF by juxtaposing where we were in CF research the decade before.

"Ten years ago..." Dr. Saiman would end up saying over and over again as she explained how much CF meds have changed in a decade.  As Dr. Saiman flashed back to the decade prior, I couldn't help but do so in my own life.

It was almost 10 years ago that Bennett was born (he just turned 9).  At the time, Brian and I, new CF parents, eagerly listened to and read the newest CF research with expectant hope.  At the time, we felt very encouraged by the newest research and genuinely hoped that the newest meds available 10 years ago would prevent CF from damaging his body. And yet, back in the present, it's hard to believe that research is 10 years ago old and no longer relevant. 

Pulmozyme, a medicine that we use routinely nightly in our house (and have for years), had only barely been out of trials and become FDA approved when Bennett was born.  It felt "new" and "exciting" and now, it's commonplace and part of the standard CF protocol.

I looked up again at that picture of the cute newborn baby up on the screen and re-heard the words "how about if we start [these meds] early on in babies, in patients, before the lungs are damaged?"

All of the sudden, that idea no longer gave me hope.  It brought me grief.

"We're losing years!" I felt some deep place in my heart call out. "We've already lost a decade!"

No longer is my family in that group of CF families who are"newly diagnosed."  Our family isn't even in that group of families with "young children with CF" anymore.  

We're in a new place, a wonderfully joyful place for most parents: a place with a tween!  

But, when it comes to CF, time often passes faster than research can answer questions and faster than medicines can be approved.  So, while birthdays in CF are a really big deal because they are a huge gift that we celebrate, they also mark time that has passed without a cure.  

Bennett is in a really good place, considering.  He has had very good lung health compared to many children with CF.  But we know silent lung damage is happening and we know that, statistically, he's losing 2% of his lung capacity per year.  

More drugs are coming.  It's a very exciting time in cystic fibrosis.  But it's a hard pill to swallow that we've also lost nearly 10 years of potential benefit for those drugs.  

Sitting in the auditorium, surrounded by a sea of professionals quietly listening to the newest information on CF, I felt time compress.

New meds are coming.  
But we won't be the lucky ones with newborns.  
We won't be the lucky ones with young children.  
Fingers crossed, in another few years, we'll be the lucky ones with an older child.  

But I want those early years back.  I want to be the one with the newborn who benefits.  And I want to stop the clock until we have more tools in our arsenal.

Might this be what it feels like for those who have gone before me - parents of adults with cystic fibrosis - whose time compression is marked by many more FDA approvals of CF medicines...or, worse, are marked by the time when there were no new CF medicines at all? How difficult it must be for them to hear of the excitement in the CF community and yet to know their child, whom they've had to say goodbye, will not benefit?

My heart breaks for them.  And it breaks for us, all of those with CF still suffering from this awful life-threatening disease.

Can I be sad for time lost, even as I have hope for the future?  As a mother, I can hardly help but wanting Bennett to have never suffered.

This is a very very good time for the CF community.
But, as good as it is, this NACFC just reminded me: 

The clock is still ticking...

26 October 2018

NACFC 2018 - 16 Things I Learned at NACF 2018

I just returned from being in Denver, Colorado for the 2018 North American Cystic Fibrosis Conference, an annual conference with more than 5,000 scientists, researchers, clinicians, parents and people with CF in attendance this year.

I wanted to do a "brain dump" blogpost so I won't forget all that I learned during my few days there (this post).  But I also wanted to reflect on some inner thoughts that emerged while I was there (next post).

For the first time ever, Brian came with me this year (we tried to steal away some time for a very rare weekend getaway for ourselves).  Brian was able to attend a few of the microbiology sessions and report back what he learned.

The most important point I should share (something have to remind myself each year) is that, in many ways, CF research is moving fast and, in other ways, CF research is slow.  Most of what we learn at NACFC each year, for the most part, isn't necessarily "groundbreaking" or rather, it doesn't often translate in to an immediate behavior change in CF Care.  But, taken in totality, it is helpful to learn to better understand the research trends and to get an idea of what is coming down the pipeline.

Here are the 16 things I learned at NACFC this year:

1.) Next year is gonna be a big year for cystic fibrosis!
Probably the biggest news was announced during the first Plenary.  We are likely less than one year away from the announcement of the "triple combo" - a Vertex drug (a combination of three drugs) that could benefit up to 90% of the CF Community (those who have 1 copy of the F508del).

We know that the "Triple Combo" drug is highly effective in people with a single copy of F508del. Phase II data results came out in February 2019.  Both VX-659 + Tezacaftor/Ivacaftor and VX-445 + Tezacaftor/Ivacaftor achieved similar results.

Data on the VX-659 trial should be available in late 2018.  Data on the VX-445 trial should be in the first quarter of 2019.  Vertex plans to submit approval no later than mid-2019.  

2.) Vertex isn't the only company advancing a triple combo drug.  

Proteostasis Therapeutics has multiple trials going on right now.  Galapogos does as well.  (Learn more about what is happening in this space by watching the NACFC 2018 Plenary 1 here: - starts about the 25 minute mark)

3.) CFTR Modulators are good.  But they aren't gonna make lung decline go away entirely.  

We know that lung decline is still an issue for those who are currently benefitting from CFTR modulators.  So, our community, even with modulators, needs to keep focused on keeping CF lungs healthy.

This slide, which shows evidence of Ivacaftor treatment and stopping Ivacaftor treatment, reminded me both how much I'm grateful for the CFTR modulators and how they are still not a cure.

This slide is a reminder that, CFTR modulators on the market or not, we still have a lot of reasons why we need to better understand respiratory infections and inflammation.

4.) The CFF is putting $100 million dollars towards an infection research initiative because we have so much more to learn about infection diagnosis, treatment and outcomes.

The CF Foundation recognizes that while CFTR modulators are coming down the pipeline, those for whom cannot benefit from this new drug combination have many years left to wait for their drug to come...and even then, there is some portion of the CF community who won't be able to benefit from these drugs just due to medication interference or some other reason.  Many people with CF are being plagued by lung infections and fungal infections.  That's why the CF Foundation has decided to invest $100 million to study these life-threatening infections affecting people with CF.  (Watch more about this initiative and the research that was presented during this Plenary here: - it starts about the 9 minute mark)

5.) Inflammation remains a major cause for lung damage.  There are new anti-inflammatory drugs in the pipeline.

There are new mucociliary clearance drugs also in the pipeline.

6.) The CF Foundation is putting resources towards using "the oldest new idea to improve CF Care": CoProduction (true partnership between the person with CF/families and the clinician/clinical team when making decisions in care)    

So many people enjoyed the last plenary of NACFC 2018 which was led by Maren Batalden.  Watch it here:

Maren talked about the general healthcare ecosystem, calling this slide a "map" of sorts.  Notice patients, professionals and the healthcare system overlap but all work towards health outcomes.

This was one of my favorite slides.  It demonstrates the relationship between the patient and the clinician.  Many times I've heard clinicians explain their interactions with patients as the first image ("communications").  Even at NACFC there was conversation about "educating" the patient and how the patient needed to be further "educated." But, the place where coproduction actually exists is within the last image of "partnering."  It is here that the clinician is no longer in the center of or more important to the relationship.  In partnering/coproduction, the patient and the clinician are equal.  Here they give and take equally.  This is the place where decision-making works best, as both parties respect each other's expertise.

This slide was really great.  It was the results from the CFF Mind of the Community Survey given last year.  It shows that while patients do tend to primarily trust their doctors with regard to CF research/drugs (70%), clinical care (50%) and medical stages of CF (34%), they actually trusted their peers primarily when it came to sticking to a treatment plan (37%), day-to-day life (67%) and CF and relationships (56%).  It's clear that in many important areas people/families with CF trust people/families with CF more than their often-central care team.  For this reason, we must treat CF differently than we have in the past.  We can no longer have a one-way communication relationship, it must be partnership - it must be coproduction.

Alongside Kathy Sabadosa (a CF Mom) and Melanie Abdelnour (a person with CF), Cindy George (CFF's Senior Director of Patient Engagement shared about the partnership experience from the CFF's point of view.  Cindy mentioned that the CFF is putting resources toward improving coproduction, most specifically within the CFF's mental health initiative, with the addition of CF resources and guidelines, by way of the Success with Therapies Research Consortium and through the Quality Improvement Learning Networks such as the CF Learning Network.

I can personally attest that the CFF is putting more resources towards building better partnership with patients and their clinicians in order to improve CF care.  This new focus from the CF Foundation has supported the work I've been a part of with the CF Learning Network (CFLN) for three years now.  In particular, over the last year, I have been working within the CFLN to improve CF Center-level coproduction.

If coproduction is patients and clinicians working together as equals, then "center-level coproduction" is when a group of patients and a group of clinicians work together as a team to improve CF care at the CF Center level (such as improving the wait time in the clinic, beefing up infection control, improving the clinic's communication system, etc).  "Individual-level coproduction" is what we refer to as partnership between a individual clinician and a individual patient at point of care/within the clinic room, which is also very important.  But I have been working more directly in center-level coproduction.

This year, I took the research I have had the privilege to gather through the CFLN and submitted it as a poster which was accepted at NACFC 2018.  It was then accepted as a presentation at the "Quality Improvement Thematic Poster Session."  It was really rewarding to be able to teach others about the good work happening within the CF Learning Network through our participating CF quality improvement teams at 27 CF Programs across the US.

I am really hopeful that next year I can work with more of our patient and family members within the CF Learning Network to submit their own posters.  We need more people with CF and their family members presenting research at NACFC.  I fully believe that, as patients - members of the clinical team, we know a lot about CF and we should be demonstrating that knowledge alongside researchers at NACFC.

7.) There's a company working towards PERT-free food.  

What if you could offer your kid a snack without the need for enzymes?  That's what the gang at GlycosBio are working on.  It's a very interesting concept.

8.) Bowel, bladder and sexual dysfunction is not uncommon in CF.  Better posture and strengthening core and pelvic muscles may help.
Physical therapists are being used more and more CF care.  There are common issues prevalent in CF that might benefit from work with a physical therapist.  For example, bowel and sexual dysfunction is common in CF.  

This is what it looks like for your diaphragm and pelvic floor when you cough.  This is why for many there is loss of bladder and bowel control, the muscles may be trying to do their job but the pressure from coughing can be too much.

Physical therapist Karen Von Berg explained that building good posture and core muscles may help with the stress the muscles often find themselves under due to CF's effect on the body.    

Of course, Karen Von Berg were also reminded us that we should all be sitting at a 35 degree angle when we poop, CF or not. (Credit: Karen Von Berg, D.P.T. The Johns Hopkins Hospital, "Collaborative Approach to GI Management: A Team Perspective")

9.) One way to address DIOS in CF may be massage of the abdomen.  

I don't have a ton of information about this session but I thought it was good.  Karen Von Berg also explained that massage can help DIOS issues.  It's unclear why it works - if it's because massage on the abdomen gets the blood flowing on that area which improves motility or if it's because massaging itself can massage the poop to the right place.  While Karen didn't have a video on how to give massage but she suggested massaging along the bowel: up the ascending bowel over to and down the descending bowel. (Credit: Karen Von Berg, D.P.T. The Johns Hopkins Hospital, "Collaborative Approach to GI Management: A Team Perspective")

 10.) There is still a lot we don't yet know about pulmonary exacerbations.

How do we define a "pulmonary exacerbation?"  Doctors don't agree.  What do we treat it with?  What do we use to treat?  Where should we treat it?  Who should monitor treatment?  When is treatment stopped and when is the pulmonary exacerbation resolved?  How do we classify it as mild, moderate or severe?  How does the patient/family weight in?  These are just some of the questions doctors and researchers are trying to answer.
11.) There is debate within the clinician community on whether IV antibiotics should be given at home vs. in the hospital.
Before I share more, I must outline what the debate between two really great CF doctors was about.  They debated whether research points us to believe IV antibiotics (once started in the hospital and proven to be effective for an individual patient) should be continued at home vs. in the hospital.  

The debate was convincing on both sides.  It seems that the issue is very patient dependent and results, positive or negative, can due to a complexity of things.  Data may tend towards telling us that FEV1 results seem to improve better if you finish a course of IV antibiotics in the hospital.  But we know that FEV1 is just one measure of someone's overall health in CF.  Data also tends towards telling us that quality of life may improve when a course of IV antibiotics are given at home.

What I came away with for my own family was that IV Antibiotics can be given at home but need to include, at minimum, 1-2 weekly labs, weekly clinic visits, PICC line care and good communication with the home care team.

The conclusion of the debate brought about a few important points both doctors agree on. Both doctors agree that the CF community needs to be more aggressive with treatment of exacerbations.  And, if IV antibiotics are to be given at home, there should be shared decision making (partnering between the clinicial team and the patient) and good processes for administering and monitoring.

This debate will likely continue as more research is being done on it.  STOP 3 is a study that might provide some insights as well as ongoing quality improvement projects from clinics. (Credit: Elliot Dasenbrook, M.D. M.H.S. Cleveland Clinic, "Exacerbation Treatment at Home is as Effective as Exacerbation Treatment in the Hospital. Pro")

While I was swayed slightly by the data to stay in the hospital for IV antibiotics as long as possible, I find myself more than frustrated that the the hospital is hugely disruptive to sleep and family life.  I did gain a better appreciation that IV antibiotics given at home may mean people who get IVs at home tend to push themselves too fast to get back in the saddle (they go back to work, school, family life, etc too fast) and may not fully recognize their own need to rest stress-free. (Credit: Don Sanders, M.D. M.S. Indiana University, "Exacerbation Treatment at Home is as Effective as Exacerbation Treatment in the Hospital. Con")

12.) Respiratory virus infections, including RSV and Influenza A, may promote P. aeruginosa biofilm growth which transitions to chronic infection.  
This slide shows the the presence of RSV (which is one of the cold viruses) has a correlation with more PA (bacteria in green).  But, scientists have been trying to figure out why. 

Apparently, when a virus goes in to the epithelial cells (the outside cell of the lung), the epithelial cells create a substance called interferon.  Interferon signals to other cells that an infection is near and helps other cells resist the virus.  But some scientists now hypothesize that interferon stimulates PA to develop a biofilm.  This biofilm is a protective "bunker" that protects the PA from the body's defenses. Possibly this biofilm establishes chronic infection. (Credit: Brian Kocak, University of Pittsburg, "Interferon Signaling Induces Metabolic Shifts In The CF Airway Epithelium To Promote P. Aeruginosa Biofilm Growth")

13.) Microbial ecology (how bacteria, viruses, etc. interact with each other) is important in CF and shouldn't be ignored.
In the session called, "Mucin as a Nutrient Source for Staphylococcus Aureus Invitro & In CF Associated Chronic Rhinosinusitis," it was presented that, in general, pathology (or CF disease) is determined as much by the conditions of the environment inside the lungs as by the presence of a pathogen (bad bacteria/virus, etc).  In other words how sick with CF you are is probably less about whether you have a bad bacteria in your lungs or sinuses but it's more about the interaction of that  bacteria with other bacteria.  (As Brian explained it to me: it's not whether Johnny is a bad kid or a good kid, it's whether Johnny is a bad kid when he gets around the bad kids at school).

As demonstrated on this slide, the healthy lung environment (left side) has plenty of oxygen (O2) and normal food (GLc/glucose).  But when there's a viral infection (such as an infection in the sinuses, also called "rhinosinusitis"), oxygen levels (O2) fall and nitric oxide levels (NO) rise and lactate replaces glucose (GLc).  It is here, in this new environment that bacteria that was harmless suddenly becomes disease-causing.

This session also discussed how chronic rhinosinusitis (an infection in the sinuses) can help promote Staph A. infections (see above).  For this reason and the reason written above, it's becoming more clear that it's not enough to avoid active Staph A. contamination.  We should also be avoiding any respiratory infection at all, even seemingly harmless ones, since chronic diseases are the result of complex microbial interactions.  As Brian explained to me: it takes a village to get a chronic disease, so it's best to avoid the entire village if one can. (Credit: Sarah Lucas, PHD Candidate, University of Minnesota, "Mucin as a Nutrient Source for Staphylococcus Aureus Invitro & In CF Associated Chronic Rhinosinusitis")

14.) Nutrition options in the CF world continues to expand.  
A few years ago, I learned about Kate Farms at NACFC.  We love Kate Farms feeding tube formula at my house and use it nearly every day.  Now, there is another company on the market called "Functional Formularies" which offers organic, whole-food feeding tube formulas and whole meal replacements.

15.)  Physical therapists are being added to many CF teams and they are doing great things with PT.

Bennett has started benefiting from physical therapy.  So, I was very interested to attend this session on physical therapy to learn more about the benefits of physical therapy.  What was funny was when I arrived for the session, I learned it was Bennett's CF physical therapist giving the presentation.  I guess that's when you know you're at the right place when you want to better understand your clinician's advice and find out that it's your clinician who is teaching others - ha! (Credit: Renalli Arcinas, D.P.T., Children's Medical Center Dallas"Putting the Pieces Together: Comprehensive Application of PT Interventions in CF".)
16. There's a new a college scholarship opportunity for siblings of people with CF through Vertex. 
This may not be new news but this was the first time for me to hear that Vertex has a $5,000 scholarship program which includes siblings of people with cystic fibrosis as well as caregivers (yay for Vertex for recognizing CF affects the entire family!).  To apply, click here:

Speaking of Vertex, Brian and had an opportunity to meet Paul Negulescu from Vertex at NACFC.  I asked Paul if he would take a picture with Brian and me as we wanted to show the picture to Bennett when we returned home.  I always tell Bennett when I go to NACFC that "I am going to meet with the doctors and scientists trying to find a cure for CF."  Now that a med that may actually benefit Bennett is likely to be out in the next year or so, I decided to show Bennett the picture of the person who helped create it.  We don't know what the future will hold for Bennett but it feels really great to know there are a lot of people like Paul out there trying to find a cure for cystic fibrosis.

In conclusion, I wanted to provide free online resources for anyone who is interested in reading more about the research provided at the 2018 NACFC.  Click here to learn more:
Plenary 1:
Plenary 2:
Plenary 3:
The list of NACFC 2018 sessions:
The list of NACFC 2018 posters: (see what research is happening in the CF community)
Poster Session Abstracts: (read these!  They are interesting!)
Symposium Summaries:
Author Index: (find out what research your clinician might be involved in these days)
Facebook NACFC QA Sessions:

12 September 2018

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!

04 September 2018

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!  

07 August 2018

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! :))