Not just the prison guard...but also, the prisoner



Everything hit me all at once last Friday.  Feelings of depression and fears of panic.  "What's the point in getting up today?" I thought as I pulled my covers off and tried to start my day. 

I was on week 2 of full isolation, having seen no one and having gone no where.  "I can't keep doing this with no end in sight," I said to myself. 

My days were bleeding into one another.  Every day felt like the one before it.  The color was draining from them. 

COVID19 had obliterated my calendar, manipulated my routines, and separated me from many of the things that motivate me each morning.  Left behind in the rubble was only the promise of a quarantined life inside my home with children at my feet all day and an unknown future in a world that will never be the same.

There are a lot of things I've had to give up since choosing to self-isolate on behalf of Bennett during this pandemic.  But, being a big supporter of mental health, one thing I haven't given up is meeting with my therapist. 

Thankfully, on the same day I woke up feeling the weight of how my life has forever changed, I climbed in to my SUV in the garage (currently the only place I can cry without my children hearing me) and fired up my laptop in time to see my therapists face through her online video portal. 

I cried throughout my session as I explained to my therapist how trapped I felt.  Not only was I grieving the stress I had recently undertaken by self-isolating but I was grieving the fact that I had come to accept that our situation won't end for a really long time.  However long it takes for the world to feel safe enough to venture back out again, it will take even longer for those of us with family members with health conditions.

My therapist asked how I was caring for myself in the midst of being quarantined.  I shared how I hadn't been very focused on myself.  Instead, I was focused on taking care of everyone else: making sure we had stored up enough food, protecting the children from accidental interactions with neighbors while outside, making sure the children were not traumatized in this process, making sure I could work, making sure I could do school with them each day, making sure the bills were paid.

I admitted that I had not thought about my own needs during this time.  I had not thought about how to fill up my own bucket.  I had not thought about relaxing.  I had not thought about how to center myself and calm my own heart.

My therapist asked me what I do to relax and I told her how creativity is often how I find myself.  "But," I explained, "it's really hard to be creative when you're stressed."  My therapist nodded and then wondered aloud: maybe the reason I wasn't giving myself time to do art right now is because it is precisely in those quiet moments that I might start to feel overwhelming feelings.  If I can stay busy, I can keep scary feelings at bay.

Then my therapist said, "I think you should remember.  You are not just the prison guard - monitoring who and what comes in and who and what goes out, taking care of the prisoners by preparing food and cleaning up.  It's important to remember that you too are a prisoner in this."

"What do prisoners do all day to get through their sentence?" she asked rhetorically. "They read.  They take classes.  They pick up a new hobby."

The image of a lone prisoner inside their jail cell crocheting popped in to my head.  It was a terrible and beautiful image.  There's tension there: a prisoner doing art.

A prisoner doing art is someone who has accepted their reality and decided to make the most of it. 

I felt a sense of peace at the thought of focusing on the beauty and gifts that can be found inside a prison cell, rather than focusing on the prison cell walls themselves that keep me away from beauty and the gifts on the outside.  It's a small change of perspective.  But it was the very one I needed as I was beginning week 3 of self-isolation, knowing I very well have months more before my family will likely return to life as normal.

In that moment I resolved that I can't just focus on surviving.  I have to focus on thriving.

Everything has changed.  It's scary and I'm afraid just like everyone else.  But there is a lot of opportunity for beauty in this moment in my life.  I've been given a gift of time.  I've been given a gift of discovering more about myself.  I've been given a gift of trusting God.  I've been given a gift of getting to know my children on a deeper level.  I've been given a gift to connect to friends I don't usually have time to connect to.  There are so many tissue flowers I can stick up on my prison cell bars, if I decide to do so.

Instead of feeling trapped, I want to feel secure.  Instead of feeling loss, I want to feel gain.  The world isn't going on without me.  My world is where I am.

Tomorrow starts 4 weeks on self-isolation for my family.  I am still having moments of waking up at 4am for no reason and not being able to go to sleep.  I'm still having feelings of overwhelm and helplessness.  But I now feel more capable of what to do in those moments. 

Instead of focusing on what I've lost, I'm focusing on what I have.

How We're Doing CF Care At Home During COVID-19

Bennett uses a home spirometer.

My family has been self-isolated in our home for nearly two weeks now, an effort to keep Bennett safe from COVID-19.  And I am fairly confident it will be many more weeks, possibly months, before our family will be able to safely venture out again.

Besides being homebound and having to go back to homeschooling, one of the most significant changes that COVID-19 has brought to our life is the temporary halt to going to the CF Clinic in person for Bennett's routine care.

Thankfully, we just had a CF appointment a month ago and Bennett is doing well.  But, depending on how long COVID-19 goes on, we could find ourselves, like many others, having a CF Clinic appointment from our living room.

Since the COVID-19 virus is overwhelming hospitals and risks the health of people with CF, the CF Foundation is advising people with CF to stay home, even for routine CF care visits.  (CF doctors are currently working on providing routine CF care through telemedicine/telehealth.)

I'm not totally sure how telemedicine is going to work (how exactly does a doctor listen to your lungs through a computer, for example?).  But I'm looking forward to trying it out.  I most definitely want to keep Bennett well and *not* go to the hospital right now.

With that in mind, I've been trying to consider ways to help Bennett stay well at home during COVID-19 and to prepare for telehealth CF Clinic visits.  Here's what I'm doing these days:

1.) We aren't slacking on CF care one bit.  I am hopeful that Bennett won't get sick with COVID-19.  But, just in case he does, I want to make sure his body is in the in optimal condition to fight off infection.  Therefore, we are doing our CF treatments religiously and being home is allowing me to increase his g-tube feedings so increase his weight.  I also vigilant to make sure Bennett is getting outside to run and play and use his lungs as much as possible.

2.) I'm tracking Bennett's daily symptoms using Folia.  Foliahttps://www.foliahealth.com/, a free app for caregivers of children with special needs, allows you to track anything you want.  I track coughs, nausea, stool consistency, tummy aches, headaches and how adherent he is to his treatment regimen so that I can give a consistent report to my clinic about how he's doing.  This is particularly helpful to have should Bennett get sick.

3.) I'm using a scale to capture Bennett's weight over time.  I am weighing Bennett once a month just to make sure he's getting good nutrition while quarantined and not losing weight.

4.) I'm using at home spirometer to capture his PFTs.  PFTs are typically taken at the CF Clinic every three months when well and more often when sick.  Right now, I am using a home spirometer once a week to get some baseline data and to teach Bennett how to get used to a home spirometer which is slightly different than in the clinic.  My goal is to have home PFT baseline data to use so I can compare should he get sick.  I trust my CF Clinic's PFT machine more, but in absence of clinic PFT data, I appreciate having our home spirometer.

5.) I'm using a thermometer to check for fever as needed.  Since we're fully isolated and I am not worried about Bennett getting COVID-19 right now, I am checking for fever only as needed.  However, if he was being exposed to anyone coming or leaving the home, I would check his fever daily.  We know a very early symptom of COVID-19 is fever.

6.) I will use our home oximeter to capture Bennett's oxygen levels if he gets sick.  Low oxygen levels isn't typically a problem for Bennett.  But an oximeter can be helpful when breathing issues arise.  I will check Bennett's oxygen levels if he was to show signs of being sick.

7.) I have a stethoscope to listen to his lungs...but it's unlikely to ever be much of a benefit to me as one typically needs a trained ear to put it to good use.  Nonetheless, I like having it.  Maybe it just makes me feel good to know I could listen to his lungs if I wanted to.  The reality is that in the worst case scenario, it might allow me to have a doctor friend listen to his lungs if I was really worried about something in the middle of the night.  But, in general, I don't think it'll do much for me.

8.) I'm staying on top of medication and formula refills.  Since the pandemic can make the most random things scarce quickly (think toilet paper), I try to stay on top of my medication refills for fear one morning I'll wake up and find one of Bennett's medicines is out of stock or on backorder.

9.) I'm keeping Miralax stocked.  People with CF are at risk for bowel obstruction.  In fact, it's probably the number one thing that could send Bennett to the ER so I always keep Miralax stocked and use it (under doctor's guidance) whenever I think he might be getting backed up.

10.) I change out Bennett's masks and nebulizer cups every three months.  I typically change out Bennett's nebulizer cup and masks at each CF Clinic appointment.  This time, I've written it on my calendar to change 3 months since our last appointment.  And I've written another date 3 months from that to remind myself.  I typically keep 2.5 days worth of masks and cup on hand because that's how many can fit in my sterilizer.

11.) I change out Bennett's g-tube button every six months.  It's easy to forget to do but I try to remember to change Bennett's g-tube button two times a year so that it doesn't become an emergent situation.

12.) I'm stay connected to the CF community online.  Staying connected to the CF community makes me feel much less alone and allows me to get quick answers to things without needing to bother my CF Clinic.  If I need something that an expert, such as a doctor, needs to advise me on, typically the CF community will encourage me to ask my doctor.  But often I just need a quick answer from someone who has been there before and the CF community is there 24/7.

13.) I'm staying connected to my CF Clinic.  I know my CF Clinic is busy right now.  I also know they will likely just get busier in the coming weeks.  But that won't change my communication with them.  I will continue to reach out to them via email or MyChart as I need and will continue to let them know of any changes I make at home.  CF Care is a partnership and I want to make sure I'm being a full partner with my team in Bennett's CF Care, even if my team can't get back to me as quickly as they typically have in the past.

14.) I'm always looking for at home protocols for CF Care from other CF families.  Sometimes other CF Clinics put out printed at-home protocols.  I really appreciate being able to learn from how other CF Centers coach and support their patients.  I try to share what my clinic does with other families.  I'm always looking for good at-home resources.

Although these are things I'm doing, there are some things I can't do and things I don't know how the clinic with will do them outside of a hospital/clinic either:

I don't know how CF Clinic throat swabs will work with regard to CF telemedicine.  But I'm hoping to eventually be able to throat swabs at home, especially since we're 2 hours away from our CF hospital.  I suppose if we can do drive-through COVID-19 tests, drive through throat swabs could one-day be an option?  I'll wait to hear from my clinic on that.

I also don't know how bloodwork will be done at home.  Some people have talked about having home nurses do the bloodwork.  But with COVID-19, I don't want to even risk that.  So, we'll see.  Typically Bennett doesn't need bloodwork but once a year so hopefully we'll be back in the clinic for that.  The same goes for an Oral Glucose test (OGTT).

And, how about X-rays?  X-rays will always require a clinic visit.  Thankfully, we grabbed ours last month at clinic so we have a recent one completed.  But, I'd probably opt for a small clinic or urgent care near home at this point if we had to get an x-ray...anything besides going to the hospital.  In absence of sickness, x-rays are typically done annually for CF patients.

I'm curious if there are other things I've missed about doing CF care at home and CF Clinic via telehealth during COVID-19.  If you know of anything I should be thinking of, please let me know!  I love crowd-sourcing and learning from others!

Dear Teachers: Thoughts from an COVID-19 At-Risk Family Just Trying to Survive


I received the first email from one of my children's teachers today regarding school work that will soon be sent home.  The email was a "heads up, it's coming next week" email from Oliver's teacher, which I appreciated.

I replied to the email with thoughts from a mother dealing with a child with special needs during this national emergency.

I wanted to share it on my blog because I hope other teachers will read it.  The point of the email: Educators, be compassionate with your expectations, our children and families are just trying to survive right now.

****

Dear Teacher,
One thing I wanted to share with you, and all educators, who are currently making lessons plans is right now is: please be mindful that although children are at home, many are under duress in the current situation. Adults' schedules have changed.  Adults' financial situations may have changed. This naturally causes stress among the adults in the home and therefore is stressful to the children.

In our particular case, since Oliver's little brother's underlying respiratory condition cystic fibrosis means COVID-19 could be fatal to him, our family is now fully quarantined (and plan to be for at least 6 weeks).  This means that we are having to ration food and supplies to make sure we can make it as long as possible without having to risk exposing Bennett to COVID-19.  My children spent much of this morning just grieving - grieving over their Spring Break trip which was cancelled, grieving the loss of their normal schedules, grieving the loss of being able to see their friends and teachers at school and grieving loss of not being able go about life as normal.  

Oliver will have no problem with any of the assignments you give him.  I know he will welcome them from his favorite subject!  But I would very much appreciate if you would share this sentiment with other teachers.  Children in distress will struggle to learn and retain new information.  We know this is brain science.  So many of America's children are in distress right now.  

It's also worth noting that, although children may not currently be in school, they are most certainly learning.  These days they are getting a front row seat to learning "supply and demand," learning how viruses work in the body, learning about countries around the world and learning how pandemics affect economies, etc.  There is so much learning happening, even in the absence of worksheets.  

If teachers would be compassionate towards families in crisis and children in crisis when they send work home, it would be so greatly appreciated.

That said, we look forward to hearing from you and to the work you will send.
Thank you,
Breck Gamel

Figuring Out How to Keep Bennett Safe Through the Coronavirus


It's been a hard to know when and how...but self-quarantining seems nearly impossible to escape at this point.  Due to cystic fibrosis, Bennett is part of the at-risk population extremely vulnerable to the coronavirus. If Bennett were to get COVID-19, it would likely, at best, compromise his long term lung health and, at worst, risk his life. (Side note: I realize that most children are being mostly spared from the symptoms of this illness, but doctors are unclear whether or not children with CF will be affected; we can only assume they will be as they are highly vulnerable to respiratory issues)

Although Waco has yet to see any positive coronavirus tests yet, we know it's just a matter of time. Spring Break is this week.  Baylor students and many in our city who have been traveling will return this weekend.

For this reason, we have made the very difficult decision to self-quarantine for a time.  The plan is to mainly stay at home with a few outings here and there but to eventually stop going out completely, as appropriate.

Questions I've had to recently ask myself are: what does it mean to self-quarantine?  Do we just not go out?  Do we stop going to school?  Do we stop having people over?  How far is too far and how far is just right?  There are no good answers.  It's such a personal decision.  Even CF doctors around the nation aren't consistent with their recommendations.  Nobody really knows what it is best.

The decision to self-quarantine was a slow progression.  It came last week with cancelling of a large conference that I was set to attend in 3 weeks.  Then, I decided to cancel our Spring Break trip to the Alamo.  On Monday, I decided to cancel our two nights in a hotel in San Antonio.  On Tuesday, I decided to cancel all of our plans next week.  I don't know what we'll do after that.

Although it's been difficult to know where to draw the line, each day seems to reassure me the line I'm drawing is the right one.  It's getting worse out there as the virus spreads.

The goal is to keep Bennett well.  Is it even possible to do that?  I don't know. Is my goal of staying home until it passes too lofty?  Possibly. Is it worth all the trouble to try?  Yes, I think so, to the extent that I reasonably can.

One of the things that reassures me that self-quarantining could be valuable for Bennett (outside of the fact, I'm not really sure I have much choice, considering the alternative) is: we've done it before during flu season and it worked.

Here's the thing about the flu, though.  We get our flu shot every year.  We have tamiflu should we get it.  We have neither of these options with the coronavirus.  So, if self-quarantining helped with the flu, it's really our only shot with the coronavirus.

I don't really know what our self-quarantining will look like for us over time.  But here are the things I've done to try to keep our family safe (on behalf of Bennett):

* Stock up on items to the extent it's reasonable (peanut butter, noodles, canned food, etc.).
* Filled all of Bennett's prescribed medicines for the month
* Make sure we have a working thermometer and cold/flu relief medicines
* Make sure Bennett has all of his durable medical equipment at home
* Stay home as much as possible; stop going out unless necessary
* No big groups, such as church, birthday parties or ballgames; eventually we'll limit even friends
* When able, have needed items delivered

Preparing for a self-quarantine is one thing.  Experiencing it is another.  It's very easy for everyone to get cabin fever and even to feel depressed.  So here are some of the things I learned during our first quarantining experience that helped us cope being at home (and a few other tips I plan to put in to place this time around):

* Get emotional buy-in from everyone, kids included; explain the situation and make sure everyone is on board.
* Set a daily routine/rhythm so that everyone knows what to expect day
* Get up on time, get dressed and make your bed every morning, even if you don't go anywhere (it will make you feel good)
* Set a bedtime for everyone and stick to it; good sleep hygiene is really important to prevent depression
* Develop excitement in the family by finding things to look forward to each day (this is especially important with young children who want to know, "what are we going to do tomorrow?!"); try to make each day slightly different
* Give each family member as much space as possible during the day (it's easy for everyone to become irritable without appropriate space)
* Set a certain period of the day for quiet alone time (necessary for introverts)
* Set a certain period of the day for connection time (necessary for extroverts); ideas include: read a family book together as a family, put together a puzzle as a family or play a board game each night; make a fort in the living room
* To address kid boredom, pull out old toys or suggest playing with old toys in a new way
* Organize.a closet or drawer, kids love playing with new stuff or using old stuff for crafts; besides, at least when all of this is over you'll have a clean house!
* Encourage everyone to keep learning (play educational games online; learn how to play an instrument, how to cook, how to paint, etc.  Youtube has instructional videos for all kinds of pasttimes; learning will keep everyone stimulated)
* Use videoconferencing to connect with people, especially the kids' friends
* Bury yourself in a book
* Enjoy the outdoors (there's no coronavirus in the woods)
* Get out safely; you don't even have to go anywhere!  Get in the car, roll down the windows and just drive; the change of scenery can be very helpful
* Exercise daily; exercise is important for everyone so make it fun and regular
* At the beginning of the quarantine, create a list of everything you want to do.  Come back to it once the newness wears off.

We don't know when this will end.  I'm not even quite confidence it's fully gotten started.  But I do know that, at at the end of the day, every person and every family should do whatever they feel most comfortable with doing for the people they love.

I know I don't really have much control in keeping Bennett well from a highly contagious respiratory virus that is rapidly affecting the world.  Bennett may get the coronavirus despite my best attempt.  But, it won't be for lack of trying.  And that is what I need to know to bring me peace.

Infection control with COVID-19: What cystic fibrosis has taught me




Social media and the news circuit is filled with almost hourly updates of the spread of COVID-19 in the US.  It can be very scary for anyone, but especially for those of us who have loved ones in the most vulnerable populations.

COVID-19 scares me.  But, let's be honest, so does everything when you have CF.  The flu. Colds. Coughs. Sick people. Hospitals. Large groups of people. Flying. Pets. Dirt. Hot tubs.  I've gotten very used to being scared of things I can't see: bacteria, viruses, illnesses.  Those are kryptonite to those with cystic fibrosis.

When you live with CF, the common rhinovirus can take away your lung function and put you in the hospital, so you learn to live life with an elevated level of fear.  You also learn very quickly ways to keep yourself safe.

I thought today how strange it feels that the rest of the world is now talking about "infection control," something so commonly talked about in the CF Community.  Since people with CF can make other people with CF sick, "infection control" is super important.  At the hospital, people with CF are restricted to stay in their rooms without special permission because of "infection control."  People with CF can't even be in the same classroom at school because of "infection control."

As annoying as this is, when you have CF, I have found myself quite thankful for it today as I hear about COVID-19.  While these things are not fail-safe, they are second nature for our family.  For that reason, I thought I should share some of the things we do nearly daily to keep Bennett safe from respiratory viruses, in hopes others might feel comforted there are things they can do to keep themselves safe too.

Here are just some of the things we do in our family to keep Bennett safe from dangerous respiratory viruses:

1.) Wear a mask in the hospital (masks actually don't keep you safe but it does signal to others to keep their distance)
2.) Stay 6 feet apart from others who might get you sick
3.) Sanitize surfaces touched by a potentially sick person (Lysol wipes are our friend!)
4.) Stay out of hospitals/clinics/pharmacies at all costs
5.) Consider withdrawing from school when sickness is circling
6.) Drive, rather than fly
7.) Stay away from crowds
8.) While in the hospital, insist doctors and nurses wear gloves and a plastic gown when they care for you (we ask for them to wear masks during flu season)
9.) Don't be afraid to ask clinicians sanitize stethoscope or any other medical device before it's used on you
10.) Sanitize nebulizer cups and plastic breathing masks before treatments
11.) Increase breathing treatments and chest therapy when sick
12.) Prioritize sleep when susceptible to being sick
13.) Stay hydrated when susceptible to being sick
14.) Pay attention to good nutrition when susceptible to being sick
15.) Make sure meds are not missed
16.) Use good hand hygiene (I keep hand sanitizer on me all the time)
17.) Don't share toothbrushes, eating utensils or drinking cups; wash clothes, towels and sheets more frequently
18.) Don't shake hands, hug or kiss when sick
19.) Don't share common objects with those who are sick or might be sick (such as pens, door handles, computers)
20.) Refrain from being together in enclosed poorly ventilated places such as a car.
21.) Stay away from anyone coughing or sniffling and ask others to identify you as soon as they start feeling symptoms so you can stay away
22.) Quarantine sick family members to one room or part of the house

Here's what the CF Foundation recommends for infection control for people with CF.  It is not written with COVID-19 in mind. https://www.cff.org/Care/Clinical-Care-Guidelines/Infection-Prevention-and-Control-Clinical-Care-Guidelines/Infection-Prevention-and-Control-Clinical-Care-Guidelines/

Also, here are some tips for those trying to stay well from any respiratory virus: https://www.cff.org/Life-With-CF/Daily-Life/Germs-and-Staying-Healthy/How-Can-You-Avoid-Germs/

The CF Foundation just put out an announcement about COVID-19 today. See here.

It is my desire for the healthcare and research community to learn more about infection control.  While we know some about how viruses spread, there is a lot we don't know.  I am very hopeful that this new spotlight on how respiratory viruses spread and how to keep yourself safe will bring insights and understandings that will benefit CF community.  While I am confident COVID-19 will eventually be a thing of the past, the CF community will have to continue to think of infection control until there is a cure for CF.

Trikafta is what we're afta!


Excitement in the CF Community is through the roof!  Yesterday, the FDA approved Trikafta, a triple combination medication that is believed to eventually benefit 90% of people with cystic fibrosis!  This is EXCITING NEWS!!!!  This is no cure.  But CF clinicians and patients both say this is a GAME CHANGER!!!

Adults in our CF community who have been in clinical trials with this drug have been talking for months about the significant changes they've seen personally.  This drug, for many people, has meant fewer hospitalizations, increased lung function and healthier feelings overall.  It's almost hard to wrap my brain around!

In order to benefit from Trikafta (pronounced "Try-Kaft-Uh"), the FDA says one must have two specific CF gene mutations: F508del and a nonsense mutation.  Bennett has both.

The FDA also says one must be 12 years old.


Bennett just turned 10 last month.

This means that he will now need to wait two more years to benefit, unless the FDA approves the drug for his age group before then.

To give perspective on what that might look like - there are currently 3 other drugs already on the market for people with cystic fibrosis (none of which benefit Bennett), the latest medicine, Symdecko, was approved for adults ages 12+ in February 2018.  It took more than a year, in June 2019 before it was approved in children ages 6-11.  So, our wait could be a while.

So, we're in that gray space.  The space of almost but not quite.  GET EXCITED!!!!! But wait...

It means fewer hospitalizations, increased lung function, healthier feelings overall are to come for Bennett...but not yet.

As a mama, these dual feelings are like what one might feel like, if having waited in line for hours to get on a much anticipated roller coaster ride, the ride stopped working, just as it's finally time to get on it.  It's elation met with disappointment.  It's a muffled joy.  It's hope deferred.

Sure, comfort comes in the form of reassuring thoughts: "they'll get the ride up and going.  It will be your turn really soon."  But the wait feels particularly difficult to endure now.  The build-up so intense. Pinned up hope aches for a place to go.  It's so close now.  But, knowing it's not our turn just yet gives way to fears: could this thing I've waited for so long actually be illusive?

I have thought many times today and yesterday about a blog post I wrote several years ago when the first CF drug, Kalydeco, came out on the market.  I felt a similar feeling then too.  But, as I reminded myself then and do again today - rescue helicopters are going to come back for my loved one too.  I just have to hold on and wait a bit longer.

To be honest, waiting can be a gift - because it gives me great empathy for the 10% of people with CF with two nonsense mutations who have NO drug available right now from which they benefit.  They don't get the privilege of waiting for their son to turn 12 so they can have a medicine too.

I think of my friend and CF adult, Emily, who is fighting tooth and nail to move science forward faster, in time that she herself would benefit.  Her work and the work of her nonprofit Emily's Entourage has given us all hope that one day, sooner than later, 100% of people with CF will have something that will slow down this awful disease.  But until then, she painfully waits.

The CF Foundation continues to work on this as well, regularly reminding us: we won't stop until it's done.  And the job isn't done until ALL people with CF have a drug that makes a meaningful difference on their health...and even still, until there is a cure.

So, while our family waits a little longer, we will know this is the same ache many still feel in the CF community.  And while we wait, we will experience the anticipation of getting access to a bottle of hope called Trikafta.


Bennett moves his cart of medicines and machines to his room for bedtime treatments.

I tried to explain to Bennett last night news about this drug.  I tried to manage his expectations, just as I try to manage my own.  I explained that if this drug works, like people are saying it does, it might mean he wouldn't have to go to the hospital as often.  It might mean he could stop some of his medicines at some point.  He might even be able to stop doing his twice-daily Vest. 

Doctors don't yet know what this drug will really be able to do for a person.  Drugs affect people in different ways.  And different clinicians are more or less comfortable testing a drug's potential.  It will take a series of trials and errors to find out just how this drug will help Bennett, once he gets there.  And researchers will begin studying the drug on a larger scale once more people have access.

Bennett asked if he would still have to do his pump.  I told him I didn't know.  But I hoped this new medicine might allow him to stop having to get so many tube feeds.  He is very excited about the idea that anything, even remotely like a cure, might be within reach.

For now, we head in to flu season, praying for God's continued protection.  And we will just keep doing his CF meds as normal. 

We can't stop now.  For Bennett, hope is on its way.  Trikafta, we can't wait to get our hands on you!!!!!

**Note: To all those in the CF Community who have come before us, thank you.  Thank you for your diligence in fundraising...thank you for putting a laser focus on drug development...thank you for continuing to persevere until a cure is found.  We are a part of a larger story that has been happening long before we were here and it feels amazing to be able to experience this particular chapter of the story.  Also, to our extended community who loves and supports Bennett, I hope you can take joy in this celebration as well!  You have contributed financially and physically to see a cure for Bennett.  It's happening, guys!!!  So, thank you!

Double Digits!?!


Today, Bennett turned double digits! Bennett is 10 years old! It's hard to wrap my brain around.  He's halfway grown. 



When I met him for the first time in the NICU, his body swollen and near death, I didn't have any idea the amazing adventure I would end up going on with him as his mother.  Over hills and through dark caves, splashing across creeks and sliding down rocks, I would experience the pains and joys of 10 years of caring for Bennett with cystic fibrosis.  Each adventure would reveal a jewel, a tiny gift from the the experience, a sliver revelation of who Bennett is inside.  I recognize this adventure is not over.  But, ten years in to it, I am incredibly grateful for the trip so far.  How lucky I am to be his mother.


So, who is Bennett, at age 10?  Bennett is playful, outgoing and loves to be with people.  He very tender-hearted and sensitive to other's feelings.  He lives in the moment and is always up for something exciting.  Bennett is brave, so very brave.  He is resilient and strong.  Bennett is compliant and easy to please.  He is wise.  Bennett rarely complains.  He is great friend to others and incredibly lovable.  Bennett is passionate about superheros, has a weak spot for gummy bears, and loves surprises.  In his free time, he enjoys playing with Lego men, singing to The Greatest Showman soundtrack with his sister and dressing up, especially if it makes you laugh.


Today, on Bennett's birthday, I decided to take the children to get flu shots.  I hated that it fell on Bennett's birthday but I felt it was important enough for us to get the when we could.  Bennett asked to be the first one to get his flu shot, as an example to his sister and to get it over with.  He was a rockstar.


Ultimately, the whole crew got their flu shot...despite little sister's brief protest.


Avonlea felt a little better about the process of getting a flu shot today when she learned she could give her doll her own flu shot too.  (I guess she reasoned:"if you can't beat 'em, join 'em.") She started by cleaning the area.


We're thankful for Hillcrest Pediatrics for being so kind to let us have a syringe so baby doll could get her shot....

...and a band aid that matched her mama's. 


Tears all gone - just smiles and the confidence that we're doing everything we can to keep Bennett healthy!


After flu shots and errands, we celebrated Bennett with a Pizookie from BJ's.  Here's to double digits, Bennett!  May we find a cure for cystic fibrosis and, maybe one day, even celebrate you in the triple digits!  Happy Birthday!!

Bluebonnet Pictures 2019

One of the best parts of living in Texas comes every Spring when the bluebonnets and other Texas wildflowers pop up alongside the highway and in random fields.  The beauty lasts for only a few weeks but when you see them, you know Spring has sprung!
It's a societal norm in Waco to drop off your children in a random field, place them among the flowers and to tell them to say "cheese".  So, we did it this year just like we have almost every year since we've been in Waco.  Here are this year's pictures and, if you keep scrolling, you'll see how much these nuggets have grown.

Oliver, age 11

Bennett, age 9 

 Avonlea, age 5


 April 2019 (Bennett, 9; Avonlea, 5; Oliver, 11)


April 2015 (Oliver, 7; Avonlea, 21 months; Bennett, 5)


April 2014 (Oliver, 6; Avonlea, 9 months; Bennett, 4)


April 2013 (Bennett, 3; Oliver, 5)


April 2012 (Bennett, 2; Oliver, 4)


April 2010 (Oliver, 2; Bennett, 7 months)


An update on Bennett - March 2019


Bennett had his quarterly CF appointment last week so I thought I'd share how he's doing health-wise.  I also thought I'd share how he's doing all around:

First of all, Bennett has blueish green hair these days.  It's starting to fade but after asking consistently for at least a year to dye his hair, I finally relented, shortly after family photos were taken.  Santa brought Bennett semi-permanent hair dye.  The directions said it would wash out in anywhere between 5-25 washes.  What the directions didn't say is how often a 9 year old typically washes their hair when they don't want the dye to come out. Ha!


On one hand, I'm so glad I finally gave in to his very simple request.  A friend of mine made the point that allowing him to change his hair color was a great way to give him control over his body, which I hadn't thought about but I agree.

On the other hand, I didn't realize that, in order to put the blueish green hair dye on blonde-ish brown hair, you have to bleach it first.  Bleach: what a terrible thing for CF lungs to inhale!  Nonetheless, I was already committed at this point when I realized it, so I grinned and beared my fears and went forth.  I told him, though, I didn't imagine a time when we'd be doing this again. ;)



Last week, Bennett went to see his CF Team at Dallas Children's Hospital for his quarterly CF appointment.  Bennett gained two pounds in 3 months so his GI doc was very happy.  Gaining weight is very difficult for people with CF.

We are currently giving Bennett 3 tube feeds daily, that's over 1500 calories and he's eating on top of that, which is wonderful.  His ability to gain weight is a good sign he is staying healthy.  He's still low in BMI so he needs more calories and more weight gain.  But, for now, he's at least on track with his growth.


Bennett's lung health is also looking good.  His PFTs were 92%, down from 95%.  But, considering that he hasn't gotten sick this winter so far, his Pulmonologist gave a good report and said his lungs sound good.  Bennett's throat culture results shows he continues to be free of psuedomonas and MRSA, for now.  We're starting back up nasal rinses and Flonase due to headaches, which may indicate sinus build up.  But we are hoping we can hedge that off early.

  

Bennett is doing well in school.  This picture shows him sitting next to the board he made to demonstrate his work in robotics.  You can see he's wearing his feeding bag.  Bennett is very open about his CF at school.  He sorta has to be open, considering he requires a tube-feeding each day at lunch.  We are grateful that the school is more than willing to help us daily keep him on track with his weight gain!  

Oliver and Bennett (and most recently, Avonlea) started a new school this year and it's going super well.  They are growing in incredible ways.  Acton Academy is a bit of a blend of homeschooling and a gifted/talented program.  Their school is very hands-on and self-directed, which requires a lot of motivation to learn on their part.  Thankfully, since the school has designed the learning process to be so much fun, my kids have very little problem with motivation.  In fact, they beg me to drop them off at school at the first possible moment and to pick them up at the very last moment of the day.

Their school year is split in to 7 different sessions.  In addition to the core subjects of Math, Reading and Writing, each session at school has a project-based focus.  During the first session, they focused on entrepreneurship.  Their second session was focused on learning about Ancient Greek culture.  Their third session was all about Robotics.  Their current session is about Plants and Gardening (they were making plant cell models out of Play-doh earlier this week).



As naturally awesome as this session is, gardening at school has presented challenges for Bennett due to CF.  Many of the bacteria, yeast, molds and allergies that Bennett's CF lungs are susceptible to getting live in soil and decaying things.  

These bacteria, yeast, molds and allergies can easily get kicked up into the air when dirt is moved around.  And Bennett can easily breathe in these tiny particles into his lungs, which can cause havoc in his body.  If and when Bennett gets a bacteria in his lungs, it will likely require weeks on IV antibiotics and/or inhaled meds and may include weeks in the hospital.  So, we are very hesitant to risk this happening.

I hated having to explain to the teachers (who are actually called Guides at Acton) that Brian and I believe there's no amount of playing in the dirt or near the dirt that is good for Bennett.  Out of abundance of caution, we also don't let Bennett play outside when the lawn is being mowed, either.  

Not all families with CF believe it's necessary to withdraw children's playtime in the dirt - "children with CF shouldn't live in a bubble" is often an argument - but, based on what we know about the risks, Brian and I feel more comfortable letting him learn a bit more hands-off during this session.

It's disappointing that Bennett can't play in the dirt.  But, we reason that we work too hard to keep him well, to do something that we know has a risk to undermine all of the work we've done so far.  I'm thankful that the kid's school partners with us and is willing to do whatever it takes to keep Bennett's lung health optimal for as long as possible.



The last thing I thought I'd share is that we just started Bennett in twice-weekly sports training class for 7-11 year olds to see if he can improve some of the issues that showed up on Bennett's most recent physical therapy assessment.  

Although this new sports training class is an added expense and means "one more thing" to Bennett's week and our family's schedule, we are trying to focus on Bennett's long-term health goals and believe that doing so will, not only build his core muscles and improve his lung health, but also teach him that being physically active is caring for himself.  

Thankfully, Bennett really enjoys working out and has made new friends.  A new friend said to his mother other night about Bennett; "he's a lot of fun." 

I'm so glad to have received a good report on Bennett's health and to breath a sigh of relief for another 3 months.  Brian and I are incredibly proud of Bennett's resilience and his willingness to try new things!  

Transition

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.