16 December 2020

A vaccine is amazing, but that's not the medicine I'm anxiously awaiting

For nine months, the world has ached and longed for a vaccine for COVID19.  It has been thrilling this last week to watch the distribution of a medicine that has the potential to change the world back to the way it's supposed to be.  Along with so many others, I look forward to the day when I, too, can have access to this drug.

But, for me, the vaccine, as remarkable as it is, pales in comparison to the drug I'm waiting for.  

The drug I'm waiting for is one I've waited for for over a decade.  It's a drug that has the potential to change my son's world forever and to extend his life expectancy.  The drug I'm waiting for is Trikafta.

Trikfata is the first ever drug to treat the underlying cause of cystic fibrosis to which Bennett will have access.  It's not a cure but it's considered "a game changer."

Any day now, Vertex, the pharmaceutical company who makes Trikafta, is expected to submit paperwork to the FDA for approval for it's triple combination modulator therapy.  At one point several months ago, Vertex said they expected to do so by end of Quarter 4.  I've been on the countdown for months. 

As of today, there are only 10 business days left in Quarter 4.  (Do you see me pacing the floor?!) 

No one outside of Vertex knows when they will submit their application to the FDA.  COVID19 and other issues may force Vertex to wait to submit in 2021.  

Even after they file with the FDA, it will likely be another few months before the FDA approves the drug giving us access.  I have no choice but to be patient.

But knowing access to Trikafta is *so close* but still so far away can feel intolerable for me as a mother of a child with CF.  Like a toddler given permission to have candy but forced to wait until it's unwrapped, waiting at this point feels excruciatingly difficult.  Every day we wait is one more day Bennett is at risk for further lung damage.

It's bittersweet to listen to the stories of people with CF currently on Trikafta (it's already available for adults with CF with qualifying mutations).  Their stories of weight gain, no more coughs, fewer hospitalizations, less treatments, before and after pictures are just amazing.  It's super exciting.  I want my child to experience it too.

Of course, nothing is for certain.  Just like the COVID vaccine isn't 100%, neither are we guaranteed that Trikafta will work for Bennett.  We won't know until we give it a good try.  But all signs seem to point to this drug greatly benefit Bennett.

Bennett will still have CF and there are a lot of parts to CF that Trikafta won't be able to fix.  But it will hopefully stop this progressive disease in its tracks, slowing it's progression and giving Bennett a better quality of life overall.

In March, I watched the world become preoccupied with social distancing, trying to stay well from a respiratory disease.  The world began doing many of the things we in the CF community had been doing for years.  I felt thankful to no longer feel so alone in it.  

Ironically, I feel the same sense of solidarity. Maybe others are looking at the vaccine and I'm looking at Trikafta.  But, either way, there's a sense that we're all in this together: each of us is patiently waiting for our own access to a medicine that offers us hope.

04 November 2020

Top Takeaways from the 2020 North American Cystic Fibrosis Conference

For the first time ever, the annual North American Cystic Fibrosis Conference was held virtually (due to the COVID19 pandemic) in October 2020.  

Although one of the best parts of NACFC is getting to meet with clinicians, researchers and parents in the hallways between sessions (and sometimes adults with CF who come too), one of the hardest parts of NACFC is not being able to go to all of the great sessions going on simultaneously.  

If there was a positive about an all virtual NACFC, it was that, since it was online, I had a chance to watch all of the great sessions I wanted to see.  Content was streamlined this year and most sessions were provided OnDemand for several weeks for attendees.  I've been told that a good portion of the sessions will be placed on the CFF's Youtube page in the next month for all to see.

While I missed getting to see people in person at NACFC, I loved being able to really soak up the information.  I really loved all of the new topics that were discussed this year and those topics that moved forward.  I am regularly in awe of the amazing work that happens each year around the world that is presented each October.

Without further adieu, let me present my top takeaways from the 2020 North American CF Conference:

1.) The CF Gut is getting some serious attention in the research and clinical space.

I really loved Plenary 3 this year about all of the new parts of CF Care that haven't yet been fully explored: nutrition, liver, pancreas and intestine.  

Considering Bennett's CF has been more GI-related than lung-related, I am particularly thrilled that there is a new focus on understanding how CF works in the digestive system.  

There was an excellent video shown during the plenary about the GI system, much like we've had for years about CF and the respiratory system (will post here as soon as it's released).  

My favorite take away, however, was the comment that scientists believe dysmotility and dysbiosis in the GI system are the "bronchiectases" of the lung system.  Basically, this means that scientists believe that  dysmotility (muscles in digestive system don't work properly) and dysbiosis (microbial imbalance) in the CF GI system indicate CF-related damage, just as scientists believe bronchiectases in the lungs indicate CF-related damage.  The great thing now is, upon knowing this, researchers will begin to understand these particular markers in the body so they can begin to address CF-related gut problems earlier.

2.) Addressing mental health in people with CF continues to be a priority, with a special awareness of those taking Elex/Tez/Iva.

I really liked hearing more about mental health in the CF community.  It's clear that while Trikafta is a blessing to our community, for some people with CF, Trikafta has brought a type of existential crisis of sorts: mental health challenges not previously experienced.

There was a very interesting session regarding mental health for those taking Elex/Tez/Iva (brand name: Trikafta).  What I learned is that there are real issues for some people on Trikafta...but so far, it seems those who indicate they are having more mental health problems on Elex/Tez/Iva are those who mental health problems previously existed and were likely exacerbated by the new Trikafta medicine.  

This new insight felt reassuring to me as Bennett will hopefully soon have access to Trikafta and I have worried this med might negatively affect his mental health.  Reassuringly for pediatric patients, most have not indicated new mental health on this med.

For those who are struggling with mental health on Trikafta, there are many clinical interventions that can be explored to improve mental health,  such as changing doses.  But this is an area that will continue to be researched, especially with regard to psychotropic drugs.    

3.) COVID and Trikafta are a double duo of changes to CF Care in 2020.

The CF Community had two significant events that may have forever affected the trajectory of CF Care: FDA approval of Trikafta for people ages 12+ in 2019 and the COVID19 pandemic in 2020.FDA Approval for Trikafta forever changed CF Care in that it will make it so that most of the CF Community will be significantly more healthy over time and will need less intervention in the future to stay healthy.The COVID19 pandemic initiated widespread implementation of virtual CF visits that will likely continue in some form in the future.  

The unfortunate thing about these two confounding events happening at nearly the same time is that, together, they will make data trend origins difficult to identify.  However, there is some data between the two events that does show us that Trikafta is having a significant affect on people with CF.

Alex Elbert from the CFF Registry Team explained in the chart above: "Pulmonary exacerbations started a trend lower at the end of 2019, when nobody in the US heard about Sars-Cov-2 virus.  The drop is a clear evidence of Trikafta effect on the pulmonary exacerbation rate.  The data in 2020 will combine both effects." I thought that was pretty exciting to hear/see!

4.) There is an effort to make sure the diversity of the CF Community is fully appreciated in all areas of CF Care.

There was a great NACFC session called, "Culturally Competent Care" which addressed a variety of issues that clinicians treating CF should and are beginning to think about.  For example, how to address unique needs of transgender patients (such as chest binding) and how to be mindful of systemic bias that exists within the healthcare system.  

I thought the session was enlightening and really refreshing.  One of my favorite quotes was from Susanne Muenzel who said, "Cystic Fibrosis is most prevalent in non-Hispanic White patients. However, as the US population becomes more racially & ethically diverse, minorities represent an increasing portion of patients."

I appreciated hearing the stories of Raeshaun ( and Terry and Michele White (

5.) There is movement on the Path to a Cure for whom modulators are not an option. 

The first NACFC Plenary addressed the issue of curing CF beyond modulators.

There are three different ways currently being researched to do this: mRNA, DNA and gene editing. mRNA delivery would restore CFTR protein irrespective of mutation.  The goal would be restore the CFTR activity to the secretory cells and ionocyte cells. mRNA and DNA therapy would likely be therapy that would need to be repeated (within months).  

Gene editing would not likely need to be repeated.  Gene editing would be focused in the basal cells and may be performed in either specific or mutation-independent manner.

"One we have developed methodologies that work in the airway, the goal is then to begin to apply them whe ther it's the pancreas, the GI tract or other affected organs." said presenter Brian R. Davis, PhD.

CF is an ideal candidate for genetic based therapies.

"The future is quite bright: what it holds is that the Foundation has set the path for evaluating several therapeutics, in parallel, in an effort to develop, in a very time effective manner, the most efficient and safe molecular therapeutic that can be effectively re-dosed." -presenter, Maria P. Limberis, PhD.

It is expected that a clinical trial for nonsense therapy will begin this year and a first mRNA therapy clinical trial is expected in 2021.

6.) We are learning more about how to better address the needs of people with CF-related Diabetes Care.

As patients with CF grow older, they acquire additional health complications.  Diabetes is one of the most common co-morbidity in adult CF patients.

It is thought that modulators may change the course of CF-Related Diabetes disease for CF Patients, especially in those patients who might have early diabetes.  However, we will have to wait to see what the data says.

Endocrinologist Marisa Desimone, MD shared in her excellent presentation, "It's important to remember that very few CF patients have a very truly normal glucose metabolism."  

Desimone explained studies suggest there is a benefit to screening CF patients for CFRD as early as age 6.

Desimone went on to explain that the primary goals in management of CFRD is to improve weight gain, protein anabolism, pulmonary function and survival.  These goals come before the goal of glucose control (which is one way managing CFRD is very different from Type 1 or Type 2 diabetes).  She shared that CFRD complications in people with CF are primarily micro-vasular,

I also learned during this session that insulin is the only recommended treatment for CFRD and that some patients may only need treatment during acute illness.

I was really thrilled to learn that, in the same way CF Teams are beginning to recognize the value of having a GI doctor on the multidisciplinary CF team, there is an increased awareness to the value of having an endocrinologist on the CF team.

7.) Recent large-scale study shows CF Carriers may, in fact, affect your health. 

In this recent study, researchers have shown to have detected an increase prevalence for 57 out of 59 CF-associated conditions.  Just some of the conditions evaluated and found to be related were chronic sinusitis, infertility, bronchiectasis, NTM infections, etc.  

CF conditions previously not associated with CF Carriers included constipation, Type 1 or secondary diabetes, short stature, failure to thrive and scoliosis. 

The two conditions that CF Carriers did not see odds-ratio increased were: osteoporosis and cirrhosis.  

In the picture above, all 59 conditions evaluated are listed.  The blue dots indicate conditions that are increased odds-ratio for people with CF.  Red dots indicate conditions that are increased odds-ratio for CF Carriers.  As demonstrated in the above image, while people with CF had a higher odds-ratio of these conditions, CF Carriers also had an increased odds-ratio.  

Morbidity could impact large numbers of people given that there are greater than 10 million CF Carriers in the US alone.  The burden of disease related to CF Carrier status may be sufficient enough to consider it it's own well-recognized but less severe disease. "In a way being a CF carrier, at least for the population may raise the possibly of increased risk of CF-related conditions," explained University of Iowa CF Director, Douglas B. Hornick, M.D.   

Dr. Hornick says, "Perhaps, it may be time [for genetic counselors] to edit or rethink the recommendation [to CF Carriers] by saying, 'CF may affect your health', rather than does not or categorically does not affect your health." 

01 October 2020

Lego to Bennett's 11th Birthday!

Bennett turned 11 on September 30.  Since this was Bennett's pandemic birthday (seems like everybody had at least one of those!), we decided to hold a small birthday party outside.

Bennett chose to have a Lego-themed birthday.  It's one of his very favorite things to play with.

Party favors included lego-shaped gummies in lego-shaped plastic banks and little bags of lego-shaped hard candy.

Opening presents

Being very gentle when sister presents her present to him.

Make a wish!

If there is a picture to capture Bennett, this just might be it.  Bennett loves to make others laugh and never takes himself too seriously.  He is pure joy.  Happy 11th Bennett!

27 September 2020

Did you hear that, Pongo!? Avonlea turned 7!

These pictures are about two months late but they are too sweet not to share.  The baby in our family, Avonlea, turned 7 mid-July.  We love birthday party themes so this year's theme was 101 Dalmatians.  

Avonlea's birthday couldn't have come at a better time.  We were 5 months in to the pandemic and we all needed something to look forward to.  I was concerned about keeping our family safe from COVID so we decided to invite our neighbors and have a socially distanced birthday party in our front yard with a pinata. I feel pretty safe from COVID outdoors so it made the party much more enjoyable knowing it was unlikely anyone would get sick.

Avonlea is growing up very quickly. She brings our family such joy.  Avonlea is insightful, playful, thoughtful and compassionate.  She loves singing and playing with dolls.  In order to build up the fun of her upcoming birthday, I suggested we find an dalmatians outfit to go with her birthday theme. I offered to buy her a matching bow just for fun.  She requested two bows.  She explained she had very clear plans for her hair on the day of her birthday party: she wanted to wear it in two ponytails to look like two dog ears.  Her creativity is always delightful.

While we look forward next year when we can invite more friends to celebrate Avonlea's birthday and can have the birthday party inside without the risk of COVID, I was very pleased to be reminded that small celebrations can bring big belly laughs and meaningful moments that will last a lifetime.  Happy 7th Birthday, Avonlea!!

10 August 2020

Hair in my face, hanging upside down but still holding on

Up until almost this very moment, I've been single-parenting alone for nearly 6 months without a break. Things changed for the first time this week and it's been a welcomed respite.  Parenting 3 children (including one with a chronic health condition) alone 24/7 for months on end in the middle of a world-wide pandemic has taken every bit of my focus.  We have had very little face-to-face contact with the outside world and the little contact we have had has been short lived, outdoors in the Texas heat.

I've wanted to blog so many times.  I've had so much to say.  But sleep, messes and peanut butter and jelly sandwiches have needed my attention more.

Now that I have time to write, I realize there's no organization to my thoughts. I want to pour them out onto the computer screen like pouring a bucket of random Legos onto the floor.  Pour them out, assess them, and then try to figure out what to make of them.

From time to time, the blog has become particularly quiet.  Sometimes it's been because Bennett's CF has been well-managed and things are peaceful here.  

Other times the blog has been quiet because I've been too busy gripping the handle bars as life flung me around a sharp curve or catapulted me into a deep dive before shooting me straight back up again.

In these moments, I just wish I could get off the ride.  I'm tired of the "ups and downs" and "around and arounds."  I was really hoping I'd be in for a quieter more pleasurable ride, like a ride in a canoe or something.  I wanted beautiful views, not whiplash.

But this ride called "Life" doesn't ask you what you want.  It just says, "hold on! here we go!"

When I think of how much our life changed with cystic fibrosis, I can look back and say, "wow, that's not the trip I would have planned but I do see such sweet parts of it."  In the same way, I am trying these days to pretend like I can "look back" to my life right now as a working single mom separated from friends and family due to COVID19 living in an uncertain world and about to start homeschooling and say, "oh, that's so sweet."  But it's hard to have 20/20 vision when you're just trying to keep your eyelids open.

I don't know where this ride is taking me right now.  Where will life have me in a couple of years?  I'm confident more now than ever before that I have completely no idea.

Exiting the car is not an option.  So, I'm making sure the seatbelt is secure.  I'm reminding myself outloud that God is in control and that I'm not in a freefall.  

Although I can often feel as through I'm suspended in the air - my hair whipping my face, unable to see where I'm going - I know that the truth is, I can lean back into the hard seat, let out a scream and "enjoy" (er, accept) this part of the ride.

Upside down won't last forever. 

29 June 2020

16 Weeks of Living with CF during COVID19

As of today, we've been quarantined for almost 4 months.  So, I thought I'd share a little bit about how our family has been coping with CF during the COVID19 pandemic and our efforts to "re-open" in the community.

For the majority of our time in quarantine, we've been completely self-quarantined.  The CDC has listed cystic fibrosis as one of the diseases that puts a person at high risk for complications of COVID19.  It's still too early to know how exactly COVID19 affects people with CF.

At our most strictest quarantine, we never got within 6 feet of others, got our groceries by delivery only and held playdates online. However, after many weeks in self-quarantine, I began to feel more comfortable considering Waco's COVID numbers were consistently much lower than in other parts of the US.  (For many weeks, we had no more than 5 new cases a day, pretty impressive for a county of 250,000).  As summer approached and I listened to the needs of the children, I began to consider that possibly this moment was the best time (if ever) that we should start thinking about going out and connecting with people again.

The moment I decided to "loosen our belt" really crystalized when Bennett's CF doctor, who was doing a telehealth visit with us at the end of May, suggested that I consider bringing Bennett in to the CF clinic at the end of the summer to do X-rays, PFTs and bloodwork.  She said, "you can do it all at once, right before you have to lockdown again during flu season."  While I agree, my initial thought was: "Oh, wow.  How can we "lock down again" if we've never unlocked ourselves to begin with?"

So, about 4 weeks ago, I started giving serious consideration to restarting hair cuts, dentist appointments and outdoor playdates.  Every decision has been agonizing - deciding what is "safe" and "not safe."  Safe is very relative.  What is worth the risk of my child's health?

I've given up the idea that we can have good mental health and experience completely no COVID risk.  At this point, I'm focused more on mitigating the risk and making any risk as low as possible.

Thankfully, deciding to get haircuts for the children was much easier to make when the kid's hairdresser (whom we love) agreed to do the children's hair outside while wearing a mask.  In 100 degree heat, that's was a pretty big ask.  I'm grateful for a community around us who knows about Bennett's disease and is willing to whatever it takes to keep Bennett safe.

Studies show that ones risk for COVID goes up 19X indoors.  So, I've tried to make sure we stay outdoors whenever we can.

Oliver's orthodontist and the kids' upcoming dentist appointments were harder to decide to do.  But the flip side of waiting out COVID is possibly having braces on longer (which no one should have to endure) and having tooth decay.

COVID numbers in Texas started to radically rise about a week ago.  In one way, it's been scary to feel that COVID is out of control in our state and community.  But, in another way, it's meant no difference for us.  Behaviorally, we've been acting as though it's been out of control for a while.  And we'll continue to, even after the crisis subsides.  I'm grateful that a local law requiring masks was recent enacted.  It has given me the greatest amount of peace at this time.

While we aren't going out of the house much, there are things we've found to do that are safe.  Outside feels freeing.  But, in Texas, the heat is so oppressive right now.

We've been thankful for water toys, ice cream and neighbors who have given us access to their pool when they are out of own.  Last week, we enjoyed horseback riding together (  And, while we couldn't travel to Florida this summer as we had planned, we snuck away for a "COVID19-safe vacation" to an empty home in Louisiana for new scenery.

I'm as anxious as the whole world is for this virus to be over.  I dream of shopping down aisles in a store again, enjoying friends at dinner in a restaurant and no longer feeling anxiety when I get close to people.  There are so many things to miss right now.

I'm afraid our personal quarantine could last at least through next Spring.  And that feels very very long.  But I'm trying to manage these days by focusing only on the day ahead.  

Each day has its hidden gifts, and I'm trying to find and savor them.  COVID19, I've found, is full of opportunities to learn resilience, to be reminded that God is in control and to practice gratitude.

13 May 2020

Mother's Day

This Mother's Day blog post was delayed because of...well, caring for my kids.  But, even though it's three days later, I've decided to post it anyway.

I had a really lovely quarantined Mother's Day.  It was a mix of delight, intention and surprise.  It was a completely different one from last year.

One year ago this week, I sat on the floor, alongside Brian, and shared with our children that their father and I had made the decision to end our marriage.  It was a complete surprise to the children.  Telling them was as painful as I imagined it would be.

This entire last year has been filled with firsts.  Alongside each first came unexpected and, at times, paralyzing grief.

While I haven't written publicly about these experiences out of a respect for others' privacy, I have most certainly wanted to.  I desire to be authentic in all areas of my life.  And, for me, writing is healing.

But I felt it prudent to spend the last twelve months quietly healing with the help of therapy, my faith and the tender care of close friends and family.  Our divorce was finalized last month.

What I can share now is that hope is reemerging once again.  Grief is less evident than it once was.  We are at a new normal and everyone is doing well.

Mother's Day this year was my first as a single mother.  And while it was one I had never intended to experience, it was full of blessings.

My sweet boys, Oliver (age 12) and Bennett (age 10), helped me clean the kitchen before we went to bed the night before so that I could wake up to a clean kitchen on Mother's Day morning.  Six year old Avonlea, in full delight, gave me a manicure and pedicure.  And all day she kept telling me how much she wanted me to have the best day ever.

There were other surprises as well.  Neighbors who regularly check on me made sure I felt loved and seen.  Friends near and far called or texted to let me know they were thinking of me.  I was very overwhelmed by kindness.

The most memorable part of Mother's Day this year, though, was reading four little words that Bennett (who has dyslexia) wrote inside my Mother's Day card:

"I know your trying."

I wanted to simultaneously laugh and cry when I read those words.

I could have interpreted his words as disparaging, a sign of my failure as a mother.  But I knew the opposite was true.  He meant those words to be affirming.

"I know you're trying."

My children have heard me say the phrase "I'm sorry I'm trying" to them many times: when I've burnt dinner, when I've forgotten to follow through with something I promised I would, when I felt stressed and needed to apologize for my behavior.

I'm a mother.  I'm also human.

I do a lot of reminding my children that I'm human.  I want to give them permission to be human too.

Our job on earth isn't to be perfect, it is to be our whole ourselves, imperfections and all.

When my children see my brokenness, I want them to see Jesus in me.  He is who makes things right.

To hear my child accept my fallibility gives me great joy.  It takes away the pressure for me to be supermom or to have everything figured out.  It gives me space for me to just be me, just a human who has been given the awesome and sacred opportunity to walk alongside the journey of three little beings as they figure out their way in the world.  Sometimes, on this journey, I'm able to protect them from the hurts of the world.  Sometimes I'm not.  But at all times I am able to trust them to a God who cares for them even more than I do. 

As I kept reading my Mother's Day card, I noticed that right below Bennett's words "I know you're trying" were three words that Avonlea added to them: "I love you."

I can't think of a better Mother's Day gift than to be reminded that my kids accept me for who I am and love me anyway,

Happy Mother's Day, fellow Mamas.  "I know you're trying" too.

02 April 2020

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.

23 March 2020

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.  Folia, 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!

15 March 2020

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

11 March 2020

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.

04 March 2020

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.

Also, here are some tips for those trying to stay well from any respiratory virus:

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.