Summer Adventures 2018

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

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

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

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

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

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

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

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

Update on Bennett: Asthma

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

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

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

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

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

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

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

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

Here's what happened:

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

An update on Bennett

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

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

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

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

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

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

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

But, weight gain feels so elusive to me.

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

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

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

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

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

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

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

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

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

Why getting the flu is so scary for Bennett

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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


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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

Moving along well - 12:47pm post

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

Hoping this is all over soon,

Admission for DIOS - 6:55am post

We have been unexpectedly admitted again.  For GI issues again.  I feel like I'm in shock.

It all started several months ago, Bennett started showing returning signs of a bowel blockage.  Bowel blockage is a common complication in CF.  I feared another stricture but knew compaction of stool is more commonly the issue.

Since there were only slight changes in the frequency of his poops,  his doctor and I decided get Bennett an X-ray about 6 weeks ago.  Everything seemed to look fine on the X-ray so we decided to see if symptoms improved.  If they didn't we were going to do the barium enema in February to see if possibly his returning GI symptoms were due to another stricture forming.

While traveling over the Christmas holiday, Bennett had two episodes where he couldn't make it to the bathroom in time.  His GI symptoms were quickly becoming disruptive.  So, his doctor and I agreed to move the barium enema test up to next week - to Tuesday, in fact.

On Monday or Tuesday of this week, Bennett started complaining of abdominal pain.  Abdominal pain in CF is not that uncommon so I worried a little but figured it would subside. Besides, he's 8 years old so his complaints about ailments aren't always very clear to me.  I couldn't really tell whether or not to worry.  By Thursday's complaints, I decided a bowel blockage might be emerging so i began the at home regimen for such a thing: large doses of Miralax.  I just kept hoping to make it to Tuesday.

But, late Thursday night, upon returning home from of hanging out with some close girl friends, I found Brian with Bennett who was on the potty doubled over in what Bennett described as "9 out of 10" pain.  At that point, I realized we wouldn't make it until Tuesday.

I feared a complete or nearly complete blockage, likely due to a stricture (meaning scar tissue in the bowel may have completely closed up). I got in the car at 1am and drove to the ER.  Brian stayed home with the kids that night.

After a 10+ hour day in the ER yesterday, we are now fully admitted to the GI floor at Children's Medical Center.  The good news is the doctors were able to confirm that there is not a stricture forming from our last surgery.  The bad news is Bennett was diagnosed with Distal Intestinal Obstruction Syndrome (DIOS) - a blockage in his ileum where the small intestine and large intestine meet.

DIOS is relatively common in people with CF, particularly those who were born with meconium ileus, which includes Bennett.  Food gets stuck in the bowel and can't move through.  It's like constipation in that it's stuck poop in the bowel.  DIOS is different because it happens quickly, rather than gradually as constipation does.  Unfortunately, the bowels of many CF people just doesn't have the hydration it needs to move the food through easily.  People with CF are always concerned about bowel issues such as this.  There are not a lot of good medicines for improving the CF bowel yet.  So, there's not a lot you can do to prevent it.

Right now, we've been told Bennett will likely be in all weekend.  They will keep doing gastrografin enemas every day until they can break up the blockage...or until surgery becomes the only option.

Bennett had to undergo two enemas yesterday.  I can't even describe how awful that was...two in one day was literally traumatic for him.  I told the docs that when we do another this morning, they have to give him medicine to relax him and to give him pain medicine.  I refuse to allow that to happen to him again.

I feel like I'm in total shock that we're going through this again.  I feel like...that feeling you might have if you've accidentally driven your car into a telephone I'm still just trying to stand up and shake it off.  But questions inside my head keep arising: "what am I doing here?" "is this really happening right now??"

Bennett had been doing so well.  And then boom, almost without any warning, we're in the hospital looking down the barrel at surgery again.

We knew, since Bennett was born with meconium ileus, he was at risk for DIOS.  But like most of these things, we hadn't really been prepared for what we would deal with.  This is a whole new learning curve and one I didn't want to learn right now.  We just returned from traveling, and Christmas holidays - we're still getting over two surgeries and a colostomy from exactly one year ago.  Besides, we had things planned for this weekend.  But CF doesn't care.

I will not be surprised if Bennett doesn't walk away from this situation with PTSD.  It's' hard to believe that one day you're fine and the next day you're absolutely not.  ....and this is your life.  This was supposed to be the easy part of CF, the early years.  And yet, we're being hammered with these things over and over and over again.

Today, we face another day of addressing this DIOS.  Bennett is showing some small improvements so we are cautiously optimistic.  But we've been told we'll be in the hospital through the weekend, maybe in to next week.  We'll see how today goes. 

"Sometimes, I wish I was never born."

We had just finished our 1 hour of math and reading at the kitchen table that morning.  Bennett had just finished crying (some of the time actually screaming) through it, an increasingly common behavior for him at the time.

In so many ways, homeschooling had seemingly been going so well since we began homeschooling in May.  But now, in the middle of October, it was becoming increasingly unbearable for the both of us.  I kept trying new things - slowing down the work, giving him more support, giving him more independence, giving him encouragement, being patient with him at all costs - but nothing seemed to help.  He had the skills to read and to do the math being asked of him.  But it was like he was shutting down on himself.

After that particular morning's work was deemed complete and his crying had stopped, I made an effort to engage Bennett in a deeper conversation of how he was feeling about himself.  I sensed a deep sadness that I wanted to relieve.

I asked, "Bennett, what do you like about yourself?"

Still sitting at the kitchen table, Bennett looked down, shuffled his feet for a minute and then asked, "you want to know what I like about myself or what I hate about myself?"

I paused to think of what to say.  Those words were like a dagger in my heart.  I tried to move on. But I knew his question was really a statement.

I said, "I want to know what you like about yourself."

Bennett looked down again, still struggling to find an answer.

He finally replied, "I don't really know what I like about myself...but I know I hate - that I have dyslexia.  And I hate that my body doesn't work right, that I have CF."

And then he said, trying to hold back tears: "Sometimes, I wish I was never born."

I looked across the room at Bennett, this time intently listening to what he was telling me.  All of the sudden, I took greater notice of him sitting at the kitchen table as we spoke.  There he was, sitting  while connected to his feeding pump, which was silently pumping supplemental nutrition into his belly.  He had just had to endure an hour of math and intensive multi-sensory Orton-Gillingham reading tutoring (that all kids with dyslexia must go through to eventually master written language).  For me, our work was done.  But for him, the work had just ruined his day.  And he couldn't even play until his pump was done.

My heart broke for him into a million pieces.  Even when he wants to pretend he doesn't have cystic fibrosis or dyslexia, he can't.  They are always there, a constant reminder of how he's different and constant whisper that somehow he's broken.

Despite that I had spent hundreds of hours trying to address Bennett's learning needs, researching curriculum, evaluating new ways to teach basic concepts, speaking to him about grit and just seemed like that the more that I tried, the more I saw his self-confidence deteriorate, even when I thought we were addressing the core issue at hand.

I brought this to my own therapist the next day and had good ugly cry over it.  There's little more painful for a mama to hear than her young child, so pregnant with potential, articulate he sometimes wishes he hadn't been born.

When I shared this with her, my therapist, always a calming voice for me, agreed that my intuition was right: I needed to stop what we're doing and do something else.  She recommended that I look to maximize what Bennett is good at and to focus on building up his self-esteem, even at the risk of backing off the school work that we were doing.

Should I just stop completely formally teaching him anything for a while?  I wondered to myself, but didn't voice outloud.

Later that day, I chatted with Bennett's play therapist on the phone during our routine weekly check-in.  She shared with me how Bennett seemed to be doing, based on her observations of his play in the therapy room.  I shared with her that Bennett's interest and effort in schoolwork has not improving, quite possibly deteriorating.  I shared that I felt like he was giving up and maybe not even benefiting from what work I was doing with him.  I shared with her Bennett's conversation with me from the day before.

I asked, a bit rhetorically, but this time out loud, "Should I just stop teaching him for a while?"

To my surprise, she replied, "yes. I definitely think you should stop."

My heart skipped a beat.  All kinds of thoughts flooded my mind:  Wait, I can't actually stop school...Can I? I'll be a bad mom. I'm going to screw him up.  Maybe I've already screwed him up?  He's never going to learn how to read and do math.  What if he gets behind?  He probably already is behind.  How do I catch him up if I just stop?  What about Oliver, do I stop teaching him too?  How do I quit teaching one child but try to convince the other child to keep doing formal learning?  Why do we have to deal with both dyslexia *and* cystic fibrosis?  Is this the best thing that just happened to us?  Or is this the worst?

With my internal thoughts swirling, I needed to sit down.  I grabbed my purse, abandoned my Target shopping cart and sat down on the little bench in the children's shoe aisle.  Moments before, I expected this call to be just a brief chat with Bennett's play therapist when, in fact, this became a game changer conversation that would effect everything.

I agreed with her that Bennett stopping formal learning could be beneficial for him.  But, I worried...was this all my fault??  I'm his teacher now.  If he's struggling at this point, is it because of me?  I hid my fear and aching question behind a less personal question.  I asked, "So, if he was in a traditional classroom at school right now, would you be recommending that I actually pull him from school?"

Without missing a beat she responded unequivocally, "yes. absolutely."

Bennett's therapist shared how her observations of Bennett and my feedback about what was going on at home indicated that Bennett needed some time to gain back his emotional health, to see himself in a positive way and to experience success.  She explained that she didn't think we'd need to stop formal education for a really long time but for a long enough period that he could really gain some inner strength.

"How long are we talking about?  Are you thinking 6 months or a year?"

His therapist replied, "I imagine no more than six months.  But I think we'll know when it's time.  He'll start to show us both inside and outside of the therapy office."

On one level, this information felt surprising.  And yet, another part of me felt like this was exactly the what I needed to do.

It hadn't been that long ago that Bennett was going through multiple surgeries and faced an unexpected colostomy.  It certainly made sense that he might need more time to heal from such a traumatic experience.  And while we enjoyed our summer, the reality was that we had been working on school work practically every weekday since the end of the school year last May.

A well-known recommendation in the homeschooling community is that when kids move from traditional school to homeschooling, they should be given a break of 1 month per year the child had been in traditional school.  This break is often referred to as "deschooling" as it's intentional time to disconnect and decompress from the traditional classroom's expectations of learning.  The goal of "deschooling" is to give the child a love for learning again and to allow the child to pursue their own interests.

Not realizing how incredibly important this "deschooling" process is, I chose for us early on to not take a break.  Looking back, it's no wonder we hit a wall.

I decided that day in Target that I would begin "deschooling" both boys until after the Christmas holidays.  I needed to give myself a "deadline" so that when fears crept up within me about my boys "getting behind" in their education, I could remember that I had made the intentional decision to have peace about it for several months.

Interestingly, many families who "deschool" actually begin to love the process so much they decide to adopt the philosophy full time, which is often called "unschooling".  I don't yet know what homeschooling will look like for us after our "deschooling" experience, but, the one thing I do know is that in the 8 weeks since Brian and I started caring more about where our kids are emotionally than where they are academically, I have never felt more peace within our family, I've never felt more satisfied in my role as a mother and I've never seen my kids so consistently balanced.  Finally, life finally feels symbiotic.

Instead of worrying about whether Bennett's reading skills are "on grade-level," I'm more interested in getting to know Bennett deeply and finding out what he is most intrinsically motivated to learn.
Instead of worrying about Oliver's mastery of rote multiplication facts, I am now paying attention to whether or not Oliver is getting enough uninterrupted play time outdoors or how I can feed his love for all things World War II.  There is incredible freedom that comes from being able to get off the conventional educational treadmill.

CF and dyslexia both suck.  But they are the two things that God has consistently used to prompt our family to slow down and to rethink what we are doing and why.  Like strategically placed road bumps on a busy street, they continually force us to ride our breaks and notice the landscape around us.

I remember tearing up in the principals office of the boys' school last May, when Brian and I shared with the principal that we were planning to homeschool.  Even though I knew it was the best decision for our family, I felt like a total failure - like somehow my kids had flunked out of life for needing a different support system for learning.  I expressed to the principal that I wished my boys were typical.  Essentially, I tried to apologize for their being different.  I wanted them to fit in the box.  Deep down, I just wanted to be the same as everybody else.  I didn't like these continued situations that were bumping us off to side-roads.

And yet, now, having found ourselves on a quiet dirt road, away from the busyness and chaos, I keep thanking God for the huge ways CF and dyslexia blesses us.  Bennett's needs are different.  Oliver's needs are different.  That's ok.  In fact, it's very very good.

My response to Bennett's conversation with me several months ago, when he told me he sometimes wishes that he hadn't been born was, "Bennett, I would rather have you here, even if you had to have CF and dyslexia, than to not have you here at all."

And that is true.  But if I could go back to that moment, I would add, "Bennett, how grateful I am that you were born and you are here.  My life is significantly enriched because of you."