SOCIAL MEDIA

15 July 2021

The Day We Waited 11 Years For...



This was the moment we've waited his whole life for: On morning of July 1st, Bennett swallowed his first dose of Trikafta, the first medication approved by the FDA for children ages 5-12 that addresses the underlying cause of Bennett's specific type of cystic fibrosis.  


Each pill costs about $270 each.  Bennett literally swallows nearly $900 a day to keep himself healthy.  In total, Trikafta costs about $300,000 a year.

Out of the 8 daily medications Bennett takes, this is the only one that is not prescribed to stop the symptoms of CF.  This one is prescribed to actually stop CF itself.  

Trikafta is no cure.  But we have some real hope that this medicine will significantly slow down the progression of his disease.


Trikafta comes in a pill pack.  It's the combination of three meds (think trifecta): ivacaftor, elexacaftor and tezacaftor.  Bennett is instructed to take two pills in the morning.  He takes an evening pill exactly 12 hours later.  To absorb the medicine, he also has to eat something with fat in it.  Bennett is expected to take this medication for the rest of his life.



The expectation that Bennett would be getting on Trikafta soon has been a family experience.  We've spent lots of time talking about it and looking forward to it.

The night before Bennett's big day, Avonlea (age 7) spent an hour hiding in my bathroom to make Bennett a handmade "Trikafta" sign with which to surprise him the next morning.  

We had known this day was coming for months, even years.  I thought we were ready until a few nights before the medicine arrived.  One night, at dinner during a conversation about our anticipation of Bennett getting on Trikafta, Avonlea reflectively lamented, "I'm going to miss Bennett not doing his Vest anymore."  

It was such a sweet comment.  She loves her brother dearly.  His doing his CF treatments is all she has ever known since she was a baby.

I reassured her his treatments weren't going away, "oh, Bennett will still have to do his Vest.  The medicine will help but he won't be able to stop his treatments and taking his medicines for a while."  

Bennett abruptly put his fork down and curtly responded, "so this is a downgrade?!"

Part of me wanted to burst into laughter and tears at the same time.  It was so funny to me that my Generation Z child was using computer technology language to articulate the impact of Trikafta to his treatment regimen.  And part of me was so sad to think that, as exciting Trikafta is to all of us, the reality of the situation for Bennett would be that he would ultimately be adding *more* treatment to his already burdensome treatment regimen.

I reassured him that it wouldn't be like this forever.  We don't yet know how Trikafta affects the body long-term.  Doctors don't know yet what impact it will be to stop the medications prescribed for addressing the symptoms of CF.  When is too early to withdraw meds?  What's the impact if we take off this med versus that?  Nobody yet knows, not even the researchers.

The night before Bennett was to take his first Trikafta pill, another important conversation came up.  As we were saying prayers before bed like we always do, Bennett began to become emotional.  He shared about his fears of taking Trikafta.  He shared how he likes himself just as he is and how he didn't want to change. He shared how he was afraid of gaining weight, becoming "fat" and even losing his g-tube.  

It occurred to me that even though I've wished CF away all his life, CF is all he knows.  His body has CF and getting rid of CF is, in some way, risking getting rid of his body as he knows it.  I had to really breathe deeply and accept that even in good gifts, there can be losses.

I tried sharing with him why it was ok.  I wanted him to be ok.  But found the conversation to be futile.   He needed time to grief and nothing I said was going to speed that up.

I realized I needed to give him the control of this big change in his life.  As much as I had waited for 11 years for him to start this medication,  I told myself that we could wait a few days or weeks longer.  

I said, "I want you to take Trikafta tomorrow because I think it will make you feel better.  But I'm going to let you decide whether or not you want to take it.  It's your body.  If tomorrow isn't the day, let's wait.  You take it when you're ready.  It's up to you."

That seemed to help.  Bennett seemed to appreciate the space to make the decision that felt right to him.

But, I was pretty quickly reminded that while Bennett is now a pre-teen and has grown up moments, he's still a child who is a sucker for candy.  When I asked Bennett if he wanted me to get him a Snickers bar to take with his Trikafta, he quickly agreed tomorrow would be a great day to start. 

And that was it!  He was all in!



I first learned about the real possibility Bennett getting access to a medicine that could help him back in 2016 (see: http://www.initforbennett.com/2016/03/vlc-2016-state-of-science.html).  Here we are, five years later, with that medicine in our hand.  

I feel incredibly blessed by the gifts of science and the many blessings in forms of donations to the CF Foundation that came from people who care for Bennett and other people with cystic fibrosis.  Thank you to everyone who helped us get to this point.  The fight isn't over but we have a pretty powerful tool in the toolbox that we've never had before!

29 June 2021

Goodbye Waco, Hello Dallas!

The worldwide pandemic changed the world.  Our world included.  

The kids and I faired really well during a year of COVID. No one got sick and everyone was able to make it through a year of homeschooling while I worked from home (thanks to amazing neighborhood friends and a fantastic babysitter).  But our time staying safe from COVID gave me an opportunity to recognize some unmet needs in our family during those months of isolation.

After careful consideration, in January, I began the process of moving the children and myself to Dallas, Texas - closer to Bennett's CF care, closer to more educational opportunities and closer to more family support.  

I was pregnant with Bennett when we moved to Waco twelve years ago. We had expected the move to be temporary and it became permanent, which I loved. Waco is such a great place to live and raise a family.  I am so very grateful for the many years and many friends we made in Waco. We have so many sweet memories. 

Our move to Dallas is not too far from Waco, only two hours "up the road". We plan to visit as much as we can. The kids will continue to spend time with their dad there.  

This season of change for our family goes beyond just moving. Bennett is currently getting access to Trikafta, the medication that we hope will change his life.  And this Fall, Bennett is moving into his last year of middle school while Oliver begins a final year of junior high.  

While we are feeling very positive about these changes, we know grief is also a real part of change. Loneliness has a way of settling as the newness of moving in fades away.  

What keeps me grounded is knowing that I've felt God's prompting throughout the move.  While I was initially hesitant to consider moving away, every curious step I have taken has been met with open doors and a sense of peace.  

I've thought several times about Isaiah 43:19 where God says: "Behold, I am doing a new thing..."

I don't totally know what new thing God is doing in this season of our lives.  But I do know, whatever God is doing, it is good.

09 June 2021

Trikafta Approved!

Joy!  That is what our family feels today!! 

 

This morning, with one eye open and the other still shut, I woke up, rolled over and opened my smartphone.  I clicked a notification to find a link sent to me by another mother of a child with cystic fibrosis. My heart lept: 

It's approved!!!!!! 

A few minutes later, Bennett walked in my room. I was delighted to share with him the good news, "Hey buddy, your medicine has been approved!"  He grinned and gave me a high five!  It was the news we were waiting for.

Trikafta, the first medication approved by the FDA that benefits Bennett's age group and Bennett's genetic mutation by addressing the underlying cause of his cystic fibrosis, is now available for Bennett to take!  We are now within weeks of starting this new potentially life-changing therapy.

If you've prayed for Bennett, walked for Bennett, raised money on behalf of Bennett and/or followed Bennett's blog, THANK YOU!!!  It is our hope that you will celebrate this huge gift with us!!  You are part of his story!  You are part of *this* story!!


We have so much to be grateful for.  Bennett is 11 years old and has stayed very healthy so far.  His lung function currently sits at 89%.  However, he has never had a cough or acquired a lung infection that required IV antibiotics.  We hope the Trikafta will increase Bennett's lung function to 100%, which would be considered normal for any child.

Bennett is in the 40th percentile for his height but he struggles most with his weight.  Despite a g-tube and daily tube feedings, Bennett's weight is in the 13th percentile (his doctors prefer his weight to be at 50 percentile).  So, we hope Trikafta will help Bennett's GI system work more efficiently allowing him to keep the nutrients he takes in.  And, if possible, we hope Trikafta might even allow him to get rid of the g-tube (something he's never lived a day in his life without).  Additionally, we hope Trikafta will extend Bennett's life expectancy.



Trikafta, which has been approved for people ages 12+ since October 2019, affects every person differently.  We don't know if it will work well for Bennett.  There have been some side effects that have been difficult enough for some people that they've had to stop taking the medication.  But clinical trials seem to indicate that children have tolerated the medicine well.  So, we are very hopeful.

I wrote in my blog many years ago about what it feels like when the CF community rejoices at the approval of a drug for those with cystic fibrosis, but a drug in which your child cannot benefit.  This was the case in 2012.  There was so much excitement and so much hope.  But, we couldn't benefit because Bennett didn't have the right gene mutation. 

I am very aware that, today, there are around 10% of people with cystic fibrosis and their families for which today is a painful day.  They are excited for the CF community.  But they know their random genetics mean they must wait longer until they can benefit from a drug that will increase their life expectancy and give them relief from this cruel disease.  They are on a clock, a race for a medication like Trikafta and each day that goes by is another day lost.  This is also the case for so many CF families around the world who do not yet have this medication available to them in their country.  For these families, I just want to say, as exciting as today is, there is a sadness I feel with you, as well.  Your time is coming too and I will be anxiously waiting along with you, working to help us find a medication that benefits your child, your family member, you.  We are in this together, as the CF Foundation's tagline says, "Until it's done."

Lastly, I want to thank the hard working people at Vertex and the Cystic Fibrosis Foundation who worked so hard to make this dream a reality.  Bennett's story began many years before Bennett's born, before we knew we would become a part of the cystic fibrosis community.  Thank you to all the scientists, investors and brilliant minds that set forth this venture philanthropy model of discovery to bring this medication forward.  Thank you to those clinicians and clinical trial participants who tested this medication so we can feel confident about it's safety.  Today's joy isn't only because Bennett has hope like never before.  It's because we realize the gift we've been given through the work and sacrifice of so many before us.

We can't wait to share when Bennett receives Trikafta in hand.  And we can't wait to see what happens.  Trikafta is not a cure.  CF is not going away.  But it's hope that CF will have just a little less control over Bennett's life, and for that, we are eternally grateful!

Today is a very exciting day!!!  We hope you will celebrate with us!!  CHEERS!!!!

18 February 2021

Emergency Preparedness and Cystic Fibrosis



Texas is in the middle of a historic winter storm event right now.  And I'm in the middle of Texas.

For many many Texans, tonight brings another day and night with no electricity and no heat.  On the heels of no heat or electricity, there is an emerging water crisis.  While the upcoming weather looks to return to our normal above freezing temperatures in the coming days, we are holding our collective breaths that our frozen pipes won't destroy our homes, businesses and schools.  This is day 7 of historic low temperatures.  We are weary.  Lest we forget we're all in the middle of a COVID pandemic that has forced us to withdraw from our normal life for nearly a year.  

The children and I are some of the luckiest ones having not lost heat and electricity during this Winter Storm.  I can only assume our power is near a critical city infrastructure and the reason why we have continued to have heat and electricity so far.  But I have gone most of the week fully expecting and prepared to lose electricity and a way to warm ourselves.  

I wanted to share some of the ways I prepared for this winter storm and some of the ideas I've learned from our CF community about how to cope in an emergency while having cystic fibrosis.  While this list won't likely help most of our CF community suffering in Texas right now, I hope it can help my future self and others who randomly find this blog at some point after googling "managing CF during crisis." 

Here are ways to prepare for an emergency when considering cystic fibrosis:

* When there is no electricity and you can't do your percussion Vest, use hand Chest Percussion Therapy (CPT).
* Consider getting an battery-powered Afflovest percussion vest.  Insurance will usually cover some portion of a new medical device every 5 years.  This is a good investment in times like these.
* Keep a list of your medications and access to your medical record/hospital portal on your phone.  
* In anticipation of an event, try to fill all prescription medications.  Even better, ask your doctor to write a 90 day prescription so you never run out of medicine.
* Store up supplies overtime.  While they take up closet space, they can also give you extra time to get supplies if something unexpected happens.
* Boil masks and nebulizers if electricity doesn't allow for baby bottle sterilization
* Remember hand hygiene is so important with CF.  Keep baby wipes on hand.
* Consider asking your doctor for a prescription for an albuterol inhaler in case you can't use your nebulizer to do albuterol.
* The eRapid can be a battery powered way to do breathing treatments.
* In an emergency, if you don't have electricity and need to do your treatments from home, try going to your local fire station.
* Remember to reach out to your CF Clinic for help or just to let them know what you're dealing with.  A CF social worker's job is to help find community resources to help during a crisis. 
* Consider purchasing a generator for backup power at home.
* Use a power inverter to do nebulized medicines in the car. 
* If on oxygen, arrange for extra oxygen tanks on hand.
* Create an emergency plan.  This link offers some really great suggestions, as well as some good scenarios to think through when you're not in the midst of an emergency.
* Connect to your community.  You don't have to fight CF alone.
* Let your utility company know you have a person with a medical condition in your home.  Some utility companies will prioritize your home's access to electricity in a crisis, if they know ahead of time.
* Have a 3-day supply consisting of one gallon of water per person per day (or more!).  Since electrolyte levels are important for people with CF, consider also having gatorade on hand as well.
* When you're in a difficult situation (emergency or not) and need help related to life with CF,  call the CFF's Compass.  They are there to help connect you to communicate organizations and resources.  
* Read about how the CF Foundation suggests families with CF prepare for emergencies.
* Feeding tube feeds can be hard without electricity.  Pumps have batteries but they need to be charged eventually.  One electricity-free feeding tube Bennett loves and uses is the bFed bolus system.

Bennett uses the bFed bolus feeding tube system.  This feeding bag works through gravity, rather than electricity.  It can easily be hands-free with the addition of the "Free Arm."  We really love not having to depend on electricity/batteries to do his feeding treatments.


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 (https://www.instagram.com/spirituallybreathing/) and Terry and Michele White (https://twitter.com/noaacf)

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 (http://www.brazosbluffsstables.com/guided-trail-ride/).  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.