Slideshow Widget

New diagnosis (but this time not for Bennett)

Wednesday, April 20, 2016



A few days after Bennett came home from the hospital in February, I gathered up all my strength and took Oliver overnight for a 3-day psychoeducational assessment in Dallas.  (Psychoeducational assessments are psychological tests, like a QI test, for example, used to analyze the mental processes underlying a child's education performance.)

My mother suggested I give myself and our family a break and reschedule the assessment for another time.  Surely a traumatic two-week hospital stay would be enough to warrant cancelling the appointment, she said.

But I explained to her that as much as I needed rest and truly didn't want to go, I knew I needed to move forward for Oliver's sake.  Rescheduling the appointment would only delay getting Oliver the help he might need.  I had been willing to move heaven and earth for Bennett over the last two weeks.  What I did for one child, I wanted to do for the other.

Not only did my mother end up supporting my decision, but she even offered to help me fill in the gap by staying in Waco to care for Bennett and Avonlea while Brian worked and I went with Oliver.

Although CF has been our family's biggest battle, Brian and I have watched our oldest child suffer quietly for a long time.  I was ready for some answers.

Play therapy for separation anxiety and severe sleeping issues began at age 3 for Oliver.  We tried medication at age 6.  By 1st grade, Oliver began loathing school and showing academic concerns. In 2nd grade, we changed from a traditional school model to a Montessori school model to see if that helped his enjoyment of learning at school.  But his struggle in school, his separation anxiety and his inability to sleep in his own room all continued.

We needed answers.  We decided to have Oliver undergo psychoeducational testing so we could get a better handle on what might be going on.  We were blown away by the results.

Oliver has been identified as having dyslexia (likely dysgraphia too) and as being gifted.  We were able to see on paper what we already knew in our gut: Oliver is very bright but he also really struggles.

Reading is challenging, writing is challenging, spelling is challenging, focusing is challenging, and organizing is challenging for Oliver.  You'd never know because he is able to use his great strengths to hide his great weaknesses.   Oliver's input of data is effortless but his output is fractured.

Oliver has a fantastic ability to verbally understand above his level.  But, if asked to demonstrate what he knows, he can't always do that in a traditional way.  This is what it's like to have a learning disability - to have a part of the brain that works so efficiently and then have another part that struggles so much.  When not supported, it can be a frustrating existence.

Oliver's issues have been ongoing for years.  I have shared some about it here, here and here.  I have thought long and hard about sharing our newest understandings about Oliver publicly.  But the reality is he is already decided to be public about it (if you don't believe me, ask his dental hygienist and his hairdresser).  And, I'm OK with that.

I think there is something important about being open about these struggles.  Shame grows in the dark corners.  There is nothing to be ashamed about with regard to having CF, dyslexia, learning challenges, anxiety, depression...the list could go on.  These are very human things.

But what I have found fascinating about receiving Oliver's new diagnosis is how similar it is to receiving Bennett's diagnosis.

With Bennett's diagnosis, I went through shock and then came to acceptance.  I spent many months late at night ferociously educating myself on his condition.  I immediately began to implement therapies and identifying experts in the field.  I joined communities of people dealing with CF so I could connect quickly to allies who understood what I was going through and could guide me through the next step of the journey.  I began to learn new vocabulary and ask thoughtful questions.  I hit roadblocks and had to learn to overcome them.  I grieved.

Oliver's diagnosis has been no different.

The first year of Bennett's diagnosis was the hardest for me.  CF has a large learning curve.  It took me a full year to understand his diagnosis and become confident in my ability to coordinate his care.

I am hopeful it won't take me so long to come to a similar place with Oliver's learning disability diagnoses.  But I am thankful that I can look back on our journey with Bennett and remind myself things will naturally improve as our family acclimates to this new diagnosis.

Like CF, dyslexia looks different in every child. Like CF, dyslexia is often unseen and unknown. Like CF, many people know about it but don't know what it's like to live with it.

We have already placed Oliver with a skilled dyslexia specialist who is working with him one-on-one.  We have a great psychologist and a fantastic psychiatrist working with us and him to find ways to support him with issues of anxiety.  I have identified some really great moms who have children with Oliver's diagnosis than have been hugely helpful.  And Oliver's school and sweet teachers are working very hard to support him in the classroom.

Things will continue to only improve as Oliver becomes a stronger advocate for himself and can tell us all better what he needs.  I'm just in the thick of it right now - at the beginning of it all and it's hard.

Parenting is hard.  Having two children with special needs is hard.

I don't always know what is best.  I don't always have the answers I want.  I don't always know how to balance being a mom, being an advocate and being a support to my child's growing independence.  I don't always know what is normal, not-normal, acceptable, not-acceptable, good or best.  And living in that space of figuring it out is like being blind in the dark crawling on my knees feeling my way around.

But Brian and I embrace the gift that children with special needs provide.  Having special needs regularly reminds us to be appreciative when things are easy, to be kind to others even when things are different for them and to see God's creation as being beautiful and wonderful in all ways.

Life is a beautiful mess and having children who struggle - one who struggles to stay healthy and another who struggles to process the world in the same way most of us do - reminds me of these things daily.

It's probably most difficult for me when the variety of systems in which we live and work daily don't always account for and even support these differences.  But while I'd much prefer not to see Bennett or Oliver struggle, I recognize there is a gift embedded in each challenge.

I often tell the boys: "you do hard things well."  And they do.  Our family does hard things well.  But, just to be totally transparent, we do hard cries well too. :)

VLC 2016: State of the Foundation

Thursday, April 14, 2016

I apologize this post got lost in my drafts and never posted!  This is the second (and last one) of my VLC 2016 posts...  


The CFF's Volunteer Leadership Conference (VLC) is an annual networking gathering of CF community parents, volunteers and CFF staff.  Each year, I attend to learn more about what the Cystic Fibrosis Foundation (CFF) is up to and to hear the latest on the CFF's effort to find a cure for CF.  

Each year I have attended VLC, I have tried to blog about my experience so others who couldn't attend could still gain the valuable information.  In a previous post, I shared the "State of the Science" in CF and where we are in our quest to find a cure for CF.  In this post, I want to share the "State of the Foundation" and where the Foundation stands.  

One of the reasons I am so active in CF Foundation work is because, as a fundraiser for the CFF, I consider it my responsibility to pay attention to how that money is being used to improve the lives of those with CF.  Here are some of the ways the CFF is using our money well:



There are three main new things to highlight about what's going on in the CF Foundation these days.

The first new development is the additional support to Care Centers.  The second development is Compass.  The third thing I wanted to highlight is the variety of ways the CF Foundation is trying to listen to patients voices.

At the end of the blog, I have posted pictures of some of the people I met this year at VLC.  Although I appreciate the knowledge I gain at VLC, it's getting to meet the people that I love most.
  

Let's look more closely at the CFF's investment in and additional support for Care Centers.  The CFF says they are working to recruit more physicians in to CF care.  There are three programs recently developed to help them to do this.  The first is a program called PACE which is an effort to recruit adult pulmonologists.  The second program is called DIGEST which is an effort to recruit pediatric and adult GI specialists.  The third program is called ENVISIO which is an effort to recruit pediatric and adult endocrinologists. In addition to recruiting more physicians to CF care, there is also a push to recruit more pharmacists, physical therapists and opportunities for mental health care coordinators in the clinic.  This is great news for our CF community.  We need more doctors educated in CF care.


The second development I wanted to share is that there is a new collection of services available for the CF community called, "Compass."  The CF community has a lot celebrate these days.  But as more people live longer with the disease, it has become apparent that living longer with CF brings up new issues.


The CFF is working with a renewed focus on patient assistance services.  Compass has been created to provide resources life transitions, transplants, living and food expenses, transportation to and from care, housing, pregnancy and adoption, scholarships, funeral assistance, hospice, mold remediation, fitness programs and much more!


Compass has four main goals - to help with insurance issues, to identify financial resources, to help with legal issues and to find other resources related to life with CF.


People with CF often struggle with health insurance issues.  Compass can help with evaluating insurance plans, troubleshooting insurance issues, understanding medicaid and medicare and getting help with insurance appeals.


Compass can help with financial resources, such as medication co-pays, out of pocket expenses and applying for social security and disability benefits.




The great thing is Compass is for anyone regardless of insurance status or income level.  Compass is for people with CF and their families (even the care teams).  Compass is always free and confidential.


The third development I wanted to share was how the CF Foundation is working to better listen to the voice of people with CF and their families.  There will be a peer-to-peer mentoring pilot program for adults beginning in May of this year (the CFF just hired someone to oversee it).  There will be a small online interactive conference created by and for people with CF in September of this year.

There is also going to be a Pilot Learning Network kicking off this year, which I am intimately involved in, that will begin facilitating better collaboration between clinicians, patients/caregivers and the CF Foundation.  I'll share more about this soon but it's connected to the Design Meeting I attended last year: see here and here.



There was so much more to this year's conference but I thought I would just summarize my favorite parts.  To read more about the CFF's 2016 VLC, check out this storify: https://storify.com/cf_foundation/vlc-2016.  Or, listen to a recording of VLC here: https://www.cff.org/Take-Action/Events/Volunteer-Leadership-Conference/


I thought I'd share just some of the pictures I took of amazing CF parents and CFF staff members I had a chance to spend time with in DC.  These are my friends Cathy and Erin, both CF moms.


This is Dr. Bruce Marshall, VP of Clinical Affairs at the CF Foundation.  A really great guy, Bruce works daily to improve CF care at the CF Care Center level.  I have had the privilege of working with Bruce on developing the CF Care Model of the Future and working together to improve ways clinicians and people with CF and their families can better collaborate to improve CF care.


This is Dr. Preston Campbell, the CF Foundation's new President and CEO.  Not only is Preston a really smart guy, he is compassionate and approachable...oh, and, if you can't tell, he's really tall!


This is Carin, a CF mom whom I first met online.  Yay for connected CF Mamas.


This is Amy, my local CFF Executive Director.  I have known her since Bennett was born.  She's amazing - dependable, caring and "in it" for all the right reasons.  I am so thankful the CF community has Amy and even more thankful to consider her a friend.


This is Andy, the only person with CF who was allowed to attend VLC all weekend.  (Due to cross infection issues, only one person with CF is allowed to indoor CF Foundation events.)

Here are my impressions of Andy:
a.) he looks so dang healthy (you'd never know he has CF),
b.) he's super funny,
c.) he's a vulnerable guy who, in his effort to communicate support for mental health issues in CF care, is honest about his own struggles depression,
d.) he's an sweet daddy of two children and
e.) it's clear he's on a mission and is using every day to improve the world around him (particularly for those in the CF community)!  

It was nice to meet Andy in person for the first time, introduce myself and for him to say, "oh, I know you!"  It feels really great to have an online community that feels as real as in person when you actually get to confirm that you each exist in the flesh.


I love these women!  Noreen (left) is a CF mom of a young adult with CF.  Maureen (purple shirt) is a CF aunt and a HUGE advocate for the CF community.  Kelli (to my left) is a CF mom of a teenage boy.  She led VLC the first year I attended.  And Mary (far right) is a CF mom of two young boys with CF.  


While it can seem like there are lots of CF mamas at the conference, CF dads are represented as well.  Jarrod, on the left, is a phenomenal CF dad.  He runs the non-profit 2 Lungs, 1 Fight and recently wrote a blogpost about VLC on the CFF's blog here: https://www.cff.org/CF-Community-Blog/Posts/2016/My-3-Takeaways-From-the-2016-Volunteer-Leadership-Conference/ To the right of the picture is Darryl, a dad of a young boy with CF.  Even though Darryl's little boy is young, Darryl is an on-fire CF dad who is going to do whatever it takes to get his son a cure.  I feel honored to know so many amazing people who are working tremendously hard to find a cure for their children and are, in the process, doing the same for my son.

"Training the Ninjas"

Saturday, April 9, 2016

Since Bennett's surgery in February, we have been focused on addressing Bennett's residual bowel issues through pelvic therapy.

When we were in the hospital for Bennett's latest rectal prolapse surgery/colon resection, one of the pediatric surgeons I really like had a very early morning heart-to-heart talk with me.  She explained that, as a pediatric surgeon, she was highly concerned that Bennett has had so many rectal prolapse surgeries.  "Four surgeries and the child is only 6 years old?  I'm really concerned something else is going on" she told me.

In that conversation, she pushed me to look further and not settle for believing this is purely CF issue. It was both alarming and reassuring to have this particular conversation.  It felt good to know that somebody else feels like this isn't ok nor is it normal.  But it felt scary to see how clearly uncomfortable she was with how he is doing.

When Bennett left the hospital, our follow-up plan was to allow him to heal, to do some bloodwork and to begin pelvic therapy.  Everyone agreed that his body was going to take some time to get back to it's normal again.  The bloodwork would reveal celiac or other markers of bowel inflammation.  The pelvic therapy was to evaluate and teach Bennett's pelvic muscles to work correctly.

Thankfully, the bloodwork showed Bennett does not have Celiac disease and no markers for bowel inflammation.  That's why, for now, our attention has been on utilizing his pelvic therapy sessions with a physical therapist.

I wrote about Bennett's pelvic therapy during a previous post.  But here's an update since then:

During his second pelvic therapy session, Bennett was given electrodes (stickers with little metal buttons on him) which were connected through wires to a computer.


The physical therapist let Bennett connect the electrodes to give him a sense of confidence during the process.


Then, Bennett helped place his own electrodes to his bottom and when watched the computer screen.  As he squeezed his muscles, the computer graph would go up and down.  It didn't take long for him to get the hang of it.  The therapist would ask him to squeeze for 1 second and then relax, then squeeze for 1 second and relax.  After about 10 of those, she would move to squeezing for 10 seconds and then relaxing for 10 seconds.  The goal is to strengthen his pelvic muscles and give him greater control over the area.  (The therapist was wearing blue gloves, which was intriguing to Bennett.  When he inquired about them, the therapist offered him to wear one too, which he did and enjoyed.)


The way biofeedback works in pelvic therapy is that the computer shows when the body is acting or responding.  Halfway through the session, Bennett had a ah-ha moment.  He stopped and said, "oh, I know what we are doing! We are big and it is small" (meaning we, the people, are big and it, the sphincter muscles, is small).  "We are training, like we are the masters...training the ninjas."  I wanted to laugh so hard.  What a sweet child-like way to put in to words what we were doing.  Yes, we were retraining the muscle.  And yes, it was as if we were masters simply training the ninja.


At our third pelvic therapy session, the physical therapist used a different screen.  This time, she used the electrodes to work on "opening" and "closing" the sphincter muscle.  This was represented on the screen with circles.  When Bennett "opened" his sphincter muscle, it caused the circle to get bigger.  The opposite happened when he closed his sphincter muscle.  It was a great visual for a kindergartner to use to connect what he was doing physically to what the therapist wanted him to do.

At the end of the session, the therapist had Bennett sit on the potty.  She noticed he doesn't have good posture on the potty.  Although he is trying to help himself be more comfortable and "help the poop come out," she says his posture needs to be changed so he can help him be more comfortable and to minimize the strain on his body.

The therapist asked me about the height of the stool is that Bennett uses on the potty.  She also pushed to make sure he is relaxed on the potty and sitting more stable.  It is her believe that these things influence his issues with rectal prolapse.

Overall, I think pelvic therapy is good.  It seems like these kinds of things would be obvious - open your body and poop comes out.  But, from what I understand, the body's elimination process is quite complex so things can easily get mixed up.

Something that came up during our last pelvic therapy session is that the therapist is concerned that his poops are not quite right.  She has suggested that we bulk up his poop with fiber.  She's also wondered if his bowels are not fully emptying itself when he has to go.

This, on top of a comment from his kindergarten teacher last week about how much class he misses each day due to being in the bathroom at school, recently emphasized even more the conversation I had with the pediatric surgeon several months ago to really push to find out what's going on with Bennett's GI issues.

The CF GI doctor, Brian and I are considering putting Bennett through a more tests to investigate what is going on.

It's SO hard to want to do more tests when our entire family is tired of the pokes, the pricks, the tests and the results.  But we also don't want to miss it if there is something we can do to improve his health and quality of life.

Bennett's 2016 Video :: This is Bennett's Story

Monday, April 4, 2016

Things with Bennett are going much better these days.  It's been more than 10 weeks since Bennett's most recent surgery and difficult back-to-back hospitalization.  Although we are trying to help Bennett gain the weight he lost through that experience and even though Bennett is still needing pelvic therapy to address his GI issues, he seems relatively back to normal.

Thankfully things have calmed down for our family, just in time for things to gear up for Bennett's Brigade 2016!

To kick off this year's CF Foundation Great Strides season, here's our newest Bennett's Brigade video.

Listen to 6 year old Bennett share, from his perspective, what it is like to be 6 years old and have CF...and what he wants most of all from those who have asked, "how can we help?":


Join Bennett's Brigade and help us find a cure for Bennett:


Nothing is more evident of how loved Bennett is than when I see a list of 7 different Bennett's Brigade teams, led by dear friends in 4 different states, who will walk in honor of Bennett this Spring, just exactly as they did last year.  

If you're in Missouri, Texas, Georgia or North Carolina and want to register to walk with or to donate to a Bennett's Brigade team around the US, click the link below:

Columbia, MO- April 30, 2016
Waco, TX - May 7, 2016
Kansas City, MO - May 7, 2016
Atlanta, GA- May 21, 2016
Raleigh, NC - May 21, 2016
Dallas, TX - May 21, 2016

All funds donated go to the CF Foundation in honor of Bennett.

Want a Bennett's Brigade t-shirt to show your support? 
T-shirts are $15 and come in child to adult sizes!
Click here to tell us your size: http://goo.gl/forms/CxzZYO94GW 


T-shirts must be ordered by noon CST on April 15th so if you want one in 2016, now is the time to do it!
 

Happy Easter!

Sunday, March 27, 2016



He is Risen!

Baylor's Heisman's trophy winner RGIII always says, "know your why."  
As the pastor at our church said to us this morning, 
"Today is our why.  Today is why we do what we do."

"Because He lives, I can face tomorrow, 
Because He lives, all fear is gone,
Because I know He holds the future,  
Life is worth the living,
just because He lives!"
-Christian hymn

 Happy Easter,

VLC 2016: Most Current Cystic Fibrosis Science Update from the CFF

Wednesday, March 23, 2016

Two weeks ago, I had the privilege of attending the Cystic Fibrosis Foundation's Volunteer Leadership Conference (VLC) in Washington DC.  

I was one of more than 500+ parents, volunteers and CF staff who attended this conference that offered us an opportunity to network, share our experiences, gain new insights from other members of the CF community, learn more about what the Cystic Fibrosis Foundation (CFF) is doing and hear the latest on the CFF's effort to find a cure for CF.


This blogpost (one of two) will summarize the parts the state of the science in our effort to find a cure for CF.  


For those who have 2 copies of the F508del gene mutation, Ivacaftor (Kalydeco) and Lumacaftor (Orkambi) is currently available on the market.  But, as you can see from the slide above, it reaches only about 30% of the normal CI- transport.  This is an improvement from the less than 10% most people with CF have.  But, fortunately, there are three potential medications coming down the pipeline that might help.  The first is VX-661 (whose trade name will be Tezacaftor).  The second is VX-152.  The third is VX-440.  In the lab, it looks like the combination of Ivacaftor (Kalydeco) + VX-661 + VX-152 might offer the greatest benefit to people with CF.  But, the Ivacaftor + VX-661 and VX-440 may also be a good option. 


For people like Bennett who have only one F508del mutation, Ivacaftor + VX-661 + VX-152 may still be the best option, however, the drug benefits will be lessened from potentially 80% normal CI- transport to possibly 50%.  

Thankfully, VX-661 might be available as early as late 2017 or early 2018.  And it is hoped there will be a Phase 2 study in people with CF evaluating VX-440 or VX-152 in combination with Ivacaftor + VX-661 as early as the second half of 2016.  It is still unknown when that study will begin.  Check out this Vertex Research Pipeline: http://www.vrtx.com/research-development/pipeline

So when might these 2nd generation correctors be available to patients with CF?  Dr. Boyle, from the CFF, said probably in 3-4 years ("maybe between 2020 and 2022").


Although it was not spoken about in depth, Dr. Bill Skach reminded us that CF Foundation is working on finding a cure for every class of CF gene mutations.  (Check out the CFF's pipeline here: https://tools.cff.org/research/drugdevelopmentpipeline/)



The CFF's goal is to find a cure for 100% of those with CF.  Ninety-five percent of people are expected to benefit from Kalydeco and a corrector medication.  There are a remaining 5% who will not benefit from Kayldeco + a corrector drug but there are efforts to help them as well, including the 3% who have nonsense mutations and the remaining 2% without nonsense mutations.


A new initiative has been started for those in that last 5% of CF mutations who will not benefit from corrector and potentiator drugs.  This initiative is called the "Nonsense and Rare Mutation Initiative" where 40 million dollars has already been committed to find a cure for these people as well.


A few of the other projects that the CFF is working on include a "New Technologies Research Conference" where they are focusing on gene expression, editing, deliver and stem cells" (which, by the way, there will be a gene-editing plenary at the North American CF Conference this fall).  The CFF is expanding their CF Foundation Therapeutics Labs with a focus on nonsense drug discovery, stem cells and gene editing.  And the CFF is working to develop the next generation of CF researchers will more training opportunities and career development.  


According to Dr. Bill Skach, the CFF is funding more grants, funding more labs and putting in more resources, towards a cure than ever before...all while also putting more resources towards expanding the programs to help those with CF live a healthier and happier life.


Dr. Boyle gave an overview on the top 5 Emerging Topics in CF Clinical Research.  They include: a worldwide effort to enroll clinical trials, an explosion of CFTR modulator trials, new anti-inflammatory clinical trials, continued work with the FDA and a new focus on lung transplantation.



The CFF is working to improve enrollment for CF Clinical Trials by supporting and working with other CF Clinical Networks.  This is good for everyone in our 70,000-person worldwide community.


There has been an explosion of CFTR modulator trials.  This means that there may very soon be competition for Vertex.  This is such amazing news as more and more labs are knocking on the CFF's door to get a piece of the Finding-A-Cure-For-CF pie.


VX-661 is already involved in clinical trials.  The VX661-108 trial, evaluating those with 1 F508del mutation and one residual function mutation (typically those who are pancreatic sufficient) is likely to complete in 2017.  The VX661-106 trial, those with 2 F508del mutations, may finish later this year.  The VX661-109 clinical trial is still working to build it's site capacity.




Inflammation is part of the devastating cycle of CF and it's damage on the lungs.  So, it's really great to see there are some potential anti-inflammatory medications in clinical trials.  


The fourth topic of Emerging Topics in CF Clinical Research is that the CFF is working with the FDA.  The CFF has put out a guidelines paper on CFTR modulator development, has a partnership with the FDA Pulmonary Division, holds a join workshop with the FDA, CF researchers, parents and families and surveys physicians and patients.


The CFF recognized there was a recent negative FDA ruling on Kalydeco and residual function mutations.  It was not a surprise as it was based on mainly lab data.  They are evaluating the best path forward for residual function mutations, negotiating other potential challenges with FDA and is look at the use of placebo/role of in-vitro testing in FDA approvals.


The last Emerging Topic in CF Clinical Research presented by Dr. Boyle was that the CFF is taking on lung transplantation.  Lung transplantation is the last resort for many with CF at end-stage disease. The CFF wants to increase support during that period of time.  


The CFF recently held a CF Lung Transplantation Summit that began evaluating how to improve pre-transplant, transplant and post-transplant care.  Dr. Boyle said, "we know lungs are rejected faster than kidneys and hearts.  We need to find out why."

In the next VLC blog post, I'll share more about the good things the CF Foundation is doing to improve care for people with CF!