Choosing Joy....continued

It is our family’s mantra.  It is printed on the Team Peter bracelets we wear.  But what does it look like to choose joy when things are not joyful, when things are hard, when joy is the farthest thing from your mind.  I try to remind myself to choose joy in the middle of behavioral struggles with Peter.   

Much of life is about choices.  Our response to whatever is happening in front of us is a choice.  Sometimes it is easy to choose joy, other times not so much.  We are working hard to build a team around Peter that will help support him and us when he continues to decline.  We still have hope that medical science will find a breakthrough, that the medication he is currently on will slow/pause the progression, that God will heal him.  However, in the meantime in the day to day, we try to choose joy and do what we can to enjoy the present moment (unless it is an unpleasant moment and then we just try to get through it J ).  Sometimes choosing joy looks like getting through the current moment and hoping and trusting in the promise that joy comes in the morning.  

 

Some of the time Peter is himself, the kind but mischievous/smart and stubborn/funny and determined boy.  I will be honest I struggle as I watch my boy slip away.  He is still there and I cherish the moments that he is, when his smile reaches his eyes, when his laugh is the sound filling my ears, when he is jumping for joy, when he does the hard work and accomplishes a difficult task.  

 

There are other times when it is clear that his Batten Disease is slowly eating away the cells that help make who he is.  Times when he struggles to control his emotions, struggles to control his body, struggles to remember a concept, a word, a memory, the question he had, times that he just struggles.  Sometimes we are able to easily distract or regulate him.  Other times we are not.  Sometimes when he digs in it is something insignificant that we can easily navigate around other times it is not.  Sometimes it happens in the privacy of our home at time when we are not on a schedule; other times it happens when he wakes up in the early morning hours or in a public place or when we have somewhere to be.   

 

As a part of Peter’s team we have  video meetings with a Nationwide Children’s Psychologist.  She is for Peter but she is meeting with us because we are the ones day to day trying to navigate Peter’s behavioral ups and downs.  Today in our meeting she mentioned something a concept called dropping the rope.  Basically when we find ourselves in a tug a war, dropping the rope and stepping away.   There are times when choosing joy looks like dropping the rope and being thankful that Joe and I are in this together and can switch out when we need to.  We also can choose joy because of the amazing support system in our families and Team Peter. 

 

Thanks for helping us Choose Joy.  Most of the time life is okay, it is manageable, there is laughter and joy.  When it is not we have our faith, we have the ability to lean into God, each other, and our support system, which helps make the not okay, manageable. 

It takes a village....

It takes a village and we are beyond grateful for ours. 

Our village, the community of Loudonville over the last week has literally cheered us on. On May 10th High School and Jr High School athletes and elementary students spontaneously ran behind Peter and Joe as they made a lap around the track. But as a friend said my eyes which were already leaking started pouring when I watched what was happening. Several days prior Peter played the drums with the Loudonville High School Band and received a standing ovation. In both occasions tears flowed and the moments were captured to be recalled on the tough days. 

 Three and a half years ago Loudonville Ohio was someplace I had never heard of. I didn’t know there was a Nazarene Church here. Nor did I know that it had the best public Library around. Or that there street fair is pretty amazing. 

 Two years and one day ago (May 12,2022) I had no idea that the CLN3 could be combined to write a death sentence for my oldest son. I knew nothing of Batten disease or that genetic, progressive, neurological , and terminal were synonyms for the disease. 

 Over the last three years Loudonville has become more than a place where I am a pastor. Loudonville has even become more than a home. Loudonville and the people that make up this community have become our people, our village, our tribe. 

 Over the last two years we have ridden the roller coaster of emotions of learning that our son has an incurable, terminal disease. Most days we have settled into choosing joy, trying to make the most of every moment all while trying to lead lives as close to normal as possible. We still butt heads over rules, attitudes, boundaries, and all the normal stuff parents and an eight year old boy disagree on. We just have the added factor of his diagnosis. How important is whatever we are butting heads over? We offer snuggles, give the choice of going to his room to snuggle his animals, we compromise a little quicker, we distract from the argument and try to find easier and happier ground. 

We have the privilege and curse of knowing his days are limited. The question mark is when will the next decline happen. The other question mark is will science and clinical trials beat that next decline or will our sweet little boy continue to slowly slip away, losing abilities, and memories as cells die off. 

As I watched the community rise to clap for my 8 year old percussionist Batten disease was the furthest thing from my mind. As elementary students raced behind Joe and Peter and cheered him on, delayed clinical trials and symptoms were not on my mind. In those moments overwhelming gratitude for the village I knew nothing about filled my heart and mind. In those moments my tribe (both those present and those I know are cheering us on from afar) lifted us up and will continue to carry us on the tough days.  

Thank you Team Peter for cheering us on. Thank you Team Peter for lifting us up. Thank you Team Peter for causing tears of joy instead of tears of grief.

 A couple of important shouts outs – Mrs. Syler his amazing para-professional. She has been a part of Team Peter before it began. She has been Peter’s eyes at school. She is his school mom. She deals with the pendulum swing of his emotions day to day while trying to get him to do his work. Mrs. Abbie has been his TVI from the beginning as well. She is a champion for Team Peter. She helped make the connection to get Peters book published. Mrs. Syler and Mrs. Abbie push Peter to work hard and give grace on the days that he can’t. There are so many others who have been a huge part of Team Peter, our church congregation, his classroom teachers, the LHS cross country team, to name a few. These and the other members of Team Peter make the hard days a little less hard and the good days even better. 

Team Peter, today and everyday choose joy.

The Librarian of Congress and Peter

Peter’s life may not look like what we thought it would, but it has been full of life, amazing adventures, and once in a lifetime experiences. Born in South Africa he had 32 stamps on his passport by the time he was 5. He has been apart of numerous Work and Witness trips (short-term missions trips). He has met Mickey Mouse on his Make-A-Wish trip. He has scored a goal in soccer. Earned a yellow belt in karate. Performed in a piano recital. He wrote a book and has hosted a book signing. Now he can add a few more experiences to the story vault. Peter’s loves story and often wants people to tell stories of memories of experiences. My aunt lives in the DC area so we took a Spring Break trip to visit. Peter’s book was accepted to be a part of the Library of Congress. During our trip we thought it would be cool for Peter to visit the Library of Congress. During our visit we not only got to see his book, but were also able to see one of the reading rooms, see a small portion of the massive card catalog at the Library of Congress. Peter was able to meet leaders from the National Library Services for the Blind (NLS). One of them being a young man who is blind and who works in their music department. Peter also had the honor of meeting The Librarian for the Library of Congress, Dr Hayden, in the ceremonial room. She signed his brailled copy of his library record and read his book with him. She also encouraged him to continuing writing and to find ways to make it a series of books. We also learned that Peter is likely the youngest author to have a book in the Library of Congress. Peter’s life has been and will continue to be full. Full of life, love, laughter, and stories. If and when Battens continues to take away his abilities we will be able to remind him of the stories of the amazing experiences that have filled his life and all of the people that love him and have supported him. To be honest when Peter first had the idea of writing a book we never imagined that it would lead to meeting the Librarian of Congress. We also never imagined that Peters book would be a source of inspiration, courage, and hope for others who are blind or disabled that we have been told that it is. Another lesson learned from my courageous 8 year old. Follow your dreams you never know where they may lead. The years that he will live may not be long but his days will be full of love and adventure.

Rare Disease Day 2024

Today is Rare Disease Day.  A day when we celebrate and call attention to those who have been diagnosed with a Rare Disease.  If I am honest two years ago I would have wondered why a day for that why not just a disease day.  Now almost two years into our journey with Battens Disease I understand the importance of it. We bring attention to the Rare Diseases because they are Rare and therefore not as widely known or understood which means that there are less people pushing for a treatment or cure or even that have a basic understanding of it.  It also means that there are less people to fund and do the research for a treatment or cure, less people who would need the treatment or cure and therefore less money available to fund the research. 

Supporting our stripes with our
Rare Disease Warrior 

 

The FDA does not make easy it for drugs to make it to the market and the guidelines for drug testing  for children is even more stringent.   Without thinking too hard I can tell of you numerous drug trials I have heard about in the last 2 years that showed promise or had proven results in treating one of the various forms of Battens Disease but the research or the trial was stopped or paused due to lack of funding or due to the pharmaceutical company changing direction.  One of those being the trial Peter was supposed to be starting at the end of March.  We do not know exactly what happened only that the trial has been delayed.  For how long we don’t know.  Will the trial still happen, we don’t know.  

 

The time it takes for a drug to go from an experimental drug to available at the  pharmacy can be 10 – 15 years.  And while we do not focus on this fact, the reality is we may not have 10-15 more years with Peter.   There are protocols that exist to speed up the process or to change some of the requirements but for reasons unknown to me those protocols and or changes are not being consider when it comes to treatments and cures to rare juvenile disease.  Time is not on our side.  I have no idea how quickly Peters cells are dying off but I know that it is happening and it heart breaking that I cannot do anything about it. 

 

Even more heart breaking and frustrating is that there is a drug already out there that has been proven to slow the progression in those with CLN3 .  It is a drug that is already approved for treatment of another disease. But it has not been approved for Battens Disease.  So for us to access it off label for Peter it would cost over $15,000 A MONTH! 

This is just a small taste of why Rare Disease is important.  Why advocacy is important.  It is important to  focus on hope and making the most of each moment, it is also important to bring to light where change needs to take place.   It is important to shed to light on why Rare Disease exists.  

 

Living on...blessed time.

 Living on borrowed blessed time. 

 

Recently we had Peter’s six month check up with his Batten Specialist team at Nationwide.   Peter enjoys this appointment because he gets to prove how fast and smart he is.  During the 3 hour appointment Peter is seen by our neurologist, a social worker, an occupational therapist, speech therapist, physical therapist, and a psychologist.  Everyone except for the social worker has a variety of tasks, tests, or measures that Peter has to do in order to document how he is doing and how much he is progressing.  While Peter is doing his tasks Joe and I are answering questions and talking with his team about how he is doing and what we are seeing.  We are very blessed that we are able to have all of these happen in one appointment instead of having to do them each individually.  Peter also loves this appointment because he gets a sushi date afterwards.  

 

We are happy to report that Peter remains stable in all the areas he was tested.  He does have some behavioral and sensory issues, which we discussed with the team.  He has an upcoming appointment with an eye doctor at Nationwide who has had other Batten’s patients.   He is not showing any signs of seizure activity and he did not show decline in his cognitive ability.  We are thankful for this. 

 

We had an IEP meeting with Peter’s school team this month.  The main topic was the transition from 3^rd to 4^thgrade which involves a change in buildings and staff.   Peter continues to do well in school, staying with his peers in everything except for reading and he is working towards being able to read braille.  He has an amazing team behind who help him to be able to learn, grow and succeed and we are very thankful for them.  

 

I will be honest I often feel like we are living on borrowed time before the next decline in Peter’s health.  While we were driving home from the appointment with Peter engaged in an audiobook Joe and I were talking about the appointment and the upcoming trial.  I expressed that I felt like we were on borrowed time and through the conversation had a shift in thinking.  Instead of it being borrowed time maybe a change in perspective was in order.  What if instead, I thought of it as blessed time.   We are blessed to have this time with Peter when he is able to mostly function as a normal 8 year old.  None of us are guaranteed tomorrow or good health.  But we don’t usually spend our days waiting for something to decline in our health even though it could.  Worry doesn’t fix anything, in fact it mostly makes things worse.  So I am trying hard to live on blessed time and focus on the joys of each day.   Our faith, our family and friends, our church, and community help to make that possible.  Thank you to each of you for your support, your prayers and for walking with us through this journey with Peter.  We are hopeful about the upcoming trial and trying to enjoy as much blessed time as a family as we can.  

 

Today whatever you are facing, consider how God may be able to provide a change in perspective to allow you to choose joy and have peace.  

Mountains and Molehills

 “Don’t make a mountain out of a molehill”  is a saying often repeated.   

Lately we have been struggling with Peter because he often latches on to something that to us seems like a small and insignificant thing.  However to him for whatever reason it is a big deal, usually one he cannot move past until it is correctly and fully resolved to his liking
.  When we are at home and/or not under time constraints and/or other responsibilities it is a bit easier to navigate.  Sometimes we are easily able to distract him from whatever he is hyper focused on.  Other times nothing will distract him.  Sometimes it a simple thing; fixing the seam of his sock, finding a stuffed animal, Lego, or rock that he suddenly remembered.  These easy wins are nice and allows us to continue with life or whatever we were trying to do.  Other times he struggles to describe what it is that he wants, needs or express what needs to happen for him to move on.  These times are frustrating, often involve tears.  In these moments we are thankful for God’s grace and the patience to walk through these things.  Thankfully at this point these are not daily occurrences and we work hard to try to keep them from happening where we can.  Peter’s sensory issues seem to be increasing as well.  Again thankfully at this point they are relatively easy to manage.   

 

Life continues to be full and busy.  We had a wonderful Christmas break.  We were able to celebrate Christmas with my parents and as a family before Christmas.  We had a wonderful Christmas eve service with our church family and then flew to Florida to be with Joe’s family.  We had a wonderful visit filled with crafts, games, Lego, stories, and adventures (some of Peter’s love languages).  We came back and jumped right back into life.  

 

Next week we have an IEP meeting for Peter. One of the main topics being his transition to 4^th grade and a new school building.  We are amazed at Peters continued learning.  He is working hard at tactile reading.  Even with all that is going on in his brain and his body he is still working so hard, learning and growing.  In two weeks he has his check up with his Batten’s team which happens every 6 months. 

 

This journey that we are on with Peter has helped us have perspective on the mountains and mole hills of life.  The mountain of his diagnosis sometimes seems insurmountable but then Peter gives us a hug and a snuggle or giggles with joy over something silly.  Or someone on Team Peter supports us in consistent or unexpected ways.  Watching Peter and Jacob grow together continues to be a joy.  The other day they snuggled together in their bean bag chair and I said a prayer that they would always share a special and significant bond. 

 

Speaking of Jacob he is growing with leaps and bounds.  He is generally an easygoing happy baby unless he is teething, tired or hungry.  He is incredibly curious and doesn’t miss much (except his nap occasionally J ).   Our family is blessed.  Thanks for reading, praying, loving and supporting us.  

 

We are hopeful for the trial that is coming this Spring.  Pray with us for lots of moments of joy and for the ability to keep things in perspective.   

Hope is a tricky thing

Hope is a tricky thing when your child is facing a degenerative, incurable, terminal disease.  Hope allows you to dream, to plan for a future, to hear your sons dreams of going to college become an inventor/scientist/mechanic/engineer/construction worker and author and dream with him of this. Reality keeps you grounded in the fact that high school graduation may or may not be in the cards for him.  Hope latches on to every report regarding breakthroughs in science regarding neurological disorders.  Reality knows that other parents before us have done the same thing and still there is no treatment or cure. 

Hope is a tricky thing in the everyday living of life.  Hope is present in the everyday moments; each day that is a good day, gives hope for another good day.  A good day is when there is more laughter than tears, when joy spills out in giggles and feet that bounce up and down off the ground in uncontainable excitement.  The memory of these days helps and gives you hope on the days when tears, frustration, and struggle reign for most of the day.  Most days are a mix of joy, laughter, snuggles, frustration, whining, and struggle, which is probably true for most people.  Right now, Peter is doing well, the disease does not seem to be progressing much and for that we are thankful.  We hope this continues to be true for a long time, but we also know that disease progression is always on the horizon.

 

Hope is also a tricky thing when it comes to scientific breakthroughs and clinical trials.  On 

Wednesday 11/15 we have a Zoom call that will give us more information about a clinical trial for Battens CLN3.  We will learn where the sites will be and how far we will need to travel every 6 months.  We will learn more details about how to enroll, when it will start and other important details.  We will also learn more information about if efforts to convince the FDA that a placebo arm for this trail is not needed were successful. 

 

The week before our call CLN2 Batten’s parents were dealt a devasting blow that the pharmaceutical company that was working on a promising drug for CLN2 has pulled funding from that project.  So, while we are hopeful of things on the horizon, other parents are grappling with hopelessness of another closed door.  For us even if Peter is a part of the half of the study that receive the placebo, the treatment is still (hopefully) close to being an approved treatment.   Being an approved treatment means that it is accessible to CLN3 families as an approved treatment and should be covered by insurance and not an out-of-pocket expense. 

 

Hope is a tricky thing, but when our hope is in Christ we can be assured not of easy answers or cure, we can be assured of His presence with us and His peace that passes all understanding and His grace and mercy which are new every morning. It is our desire that we can continue to find light, joy, and hope in each day no matter what the circumstances.  This is only possible because of our faith in Jesus Christ, and the reality that our hope is found in Him.  Scientific breakthroughs may or may not happen, clinical trials get canceled, test results show disease progression, death and the journey towards it is a natural part of life, if we place our only hope in those things, we will be disappointed.  But the day to day living out of our faith finds us placing our hope first in Christ as well as placing hope in these human things for a chance at more days to choose joy with our Batten’s warrior.  

 

A side note: Joe and I are doing fine.  Most times we are able to focus on the present and make the most of the moment in front of us.  This blog just is a snapshot of some of the things bouncing around in our head.