Seasons Change

 Seasons change, seasons come and then they go.  Spring is springing here in Ohio.  Ahh-choo.  Colors are popping up everywhere.  A fine green dust covers everything between the rain showers.  Watching new life emerge on the trees, hearing the birds song through the open windows, reminds me that we did it.  We survived another winter.  As we gear up for summer and all that means for our family, I have to remind myself that where we are now is just a season and soon it to will pass.   

I have been a bit hesitant to write it down (i.e. type it out) because even though I don’t really believe in some magical power that changes things if you say it or write it, I am afraid putting it out there will somehow jinx it.   How is that for a run on sentence?  

 

Peter is doing good, he is doing great even.  He is doing great at school.  He is growing by leaps and bounds in his reading ability.  In his recent testing he did well and continues to learn new skills, facts, and ways of doing things.  He is loving being a part of both the Ashland University and the Special Olympics Track and Field team.  Over our Spring Break we went back to Tennessee where we had what we call our COVID year from June of 2020 to June of 2021.  During that COVID year our lives were kind of on pause.  We had no idea what the future held in that year.  Some of the struggles we had with Peter in that year we know now were due to Batten Disease.  But at that point we had no idea it even existed.  Our focus was on returning to South Africa and then switched to focus on finding out where our new home would be.  

 

Fast forward through a few season changes, we are now finishing our 4^th year in Loudonville.  In our four years here we have welcomed a dog and Jacob into our lives as well as numerous friends.   We have added a number of diagnosis to our myChart lists.  We have added a family moto of Choose Joy into the mix as well.  Sometimes life is great, fun, and exciting and sometimes it is heavy, hard and exhausting.  

 

There was some time in the fall where we all struggled.  Peter was not sleeping well, he was dysregulated more than he was regulated, he was having trouble controlling his emotions and his body, there were lots of tears and frustrations, things were hard.  After a bit of this we realized this was not going away and I began mentally preparing that this was our new normal.  Around that time we reached out to his team at school and at Nationwide to involve them in the process of how do we adjust to this new normal.  We changed some things, tried some things, the school team changed some things and tried some things and Peter began to do better.  We also made some changes in his before bed routine and a few other adjustments and his sleep improved, which meant our sleep improved.  That in and of itself was a game changer for everyone.   I am relived to write that where he was this fall was not his new normal.  He has been doing so much better, he is able to ask for help or ask for a break, he is being creative and thinking of new ideas, he has returned to being kind and empathic (for the most part…he is still a 9 year old boy J ).  We are very thankful that where he was in the Fall was not our new normal.  In fact our now new normal find us trying to help him be more independent in his daily activities, to do more to further his skills in academics and athletics.  For that we are grateful.  There are still struggles don’t get me wrong, however struggles are a part of life.  It is up to us to Choose Joy when things are going well and when things are a struggle.  

 

So today whatever season you are in remember this to will pass and change.  If the season is good be thankful and soak it up, if the season is hard look for ways to choose joy and know that this will pass.  

Perspective

I have heard it said, “It is all a matter of perspective.” As we walk this journey, I am                                     realizing how true that statement is.  

The day after Christmas we were blessed with the opportunity to return to Disney World, specifically Magic Kingdom, thanks to a Christmas gift from Joe’s parents.  Thanks to the DAS (a disability pass that Peter qualified for) we were able to do all that Peter wanted to do plus more.  Peter rode Big Thunder Mountain four times which is his absolute favorite ride.  The next day we went back to Give Kids the World Village where we stayed during Peter’s Wish Trip. 

 

Our day at Disney was a fun-filled, jump-for-joy, action-packed kind of day with a lot of walking in between (around 22,000 steps) with over 100,000 other people.  Our day at Give Kids the World was slower-paced and a lot less crowded. 

 

At Magic Kingdom, nick named the Happiest Place on Earth, we saw lots of families, and people who were mostly abled-bodied and in pursuit of the next ride, show, meal, or souvenir.    At Give Kids the World, we saw Peter’s star, which he decorated and placed on his wish trip 2 years ago.  We connected with several other Alumni Families while we were there.   We connected with Luke, his aunt, and grandma.  Luke’s cousin passed away in May from brain cancer at just 16 years old.   They chose to honor his memory by volunteering at Give Kids the World Village and going back to some of the places that he loved from his wish trip.  We saw other families some, like us who were back with their wish kid, others, like Luke’s family, were missing their wish kid. 

 

Having the two days back to back was an interesting dose of reality and perspective.  For some, the greatest worries at Magic Kingdom were figuring out the lightning lane and getting the best spot for the fireworks.     At Give Kids the World Village your very presence at the Village meant your child had a life-altering, life-limiting, or terminal diagnosis.   The joy and happiness provided at the Village or by the granted Wish comes at a cost and it is a high one.

 

Childhood is supposed to be a launch into the future full of possibilities.  For Peter and others with life-limiting or terminal diseases childhood may be all that there is.  The reality is that I will probably have to plan my oldest son's funeral while his current 4th-grade classmates are planning for college or weddings. 

 

Putting things in perspective allows you to look up and around at others on the journey.   The journey we are walking is not an easy one, but it could be harder.  It could also be easier and in some ways, things are easier right now.   Yes, things could be better, we could have a fully sighted son without Batten disease, but that is not our story.  Things also could be worse, we currently are able to make memories with Peter, we can communicate with him, and he is with us, he does not currently have to have lots of medical interventions and treatments. Looking around the village, I was reminded once again to Choose Joy for the moments we have.  To be intentional about making the memories we can make.   I was reminded to live in the moment and trying to make the most of each one.  Today Choose Joy!  

A little off

 The Christmas Tree is up well before Christmas. Peter LOVES Christmas.  We told Peter that we wouldn’t start decorating for Christmas until after Halloween.  On Halloween day Peter was asking when can we take down the Fall decorations.  At first, I had no clue why.  Then we remembered that Halloween was when we told him that we could start to put up Christmas decorations.  

The Christmas Tree in the living room is not where it should be.  We have a beautiful bay window that everyone driving by can see into.  The Christmas Tree should be in the center of that window.  But the toddler and the dog like standing in the middle of that window to watch the traffic go by or bark at the mailman.  It is easier for Peter if the tree is tucked away and not in main area or high traffic area.  So, the tree is up early and is also not positioned where it would look the best.   

 

Sometimes life happens early, sometimes things aren’t quite in the right spot.  My 9-year-old is losing his memory.  He is experiencing some of the symptoms of childhood dementia that come with his Battens diagnosis.  He also struggles with sensory and processing issues.  On some things he is getting too much input and in other areas not enough. 

 

Memory is a tricky thing when you love someone with dementia.  Peter’s childhood dementia and sensory issues have been the things we have struggled with the most over the last few months.  The struggles don’t always look the same, the behaviors aren’t always easily traced back to this one thing, but the struggle is real and often results in tears, frustration and even anger.   

 

Sometimes it looks like being upset that we can’t do the thing he wants or finish something right now and we are able to get at the heart of the issue; a lot of time it is because he is afraid he is going to forget what he was doing or wants to do.  Sometime it is tears or anger because he can’t remember a concept he knew or can’t finish a task because he doesn’t remember how or what his plan was.  Other times it is digging in his heels and refusing to do anything other than what he wants at that moment.  Other times he struggles to control his body or to deal with sensations or the way things feel or taste.  He is on the move almost non-stop.  Sometimes it is normal 9-year-old boy energy and other times it is clear that he is searching or needing something that we don’t know what it is or how to help. 

 

After trying to handle things on our own for a bit we reached out to his Battens team at Nationwide to see about possible help and advice.  From those conversations and our own research, we are going to be trying a few things to see if they will help.  One is a new therapy type that other Battens families have found success with, ABA therapy. The other is working with Occupational Therapy to understand what his sensory needs are and how we can help to meet those in both the home and school setting.  We are hopeful these new tools and therapies will help Peter to be more regulated.  That will (hopefully) in turn, make for a happier and less emotional boy who can focus on playing with his brother or whatever project he wants to do instead trying to deal with big emotions.   The hope is also that this will help the sleep issues that are ever present.   

 

Thanksgiving is next week, and Advent starts right after.  In this season of thankfulness and of waiting and preparing for the birth of the Christ child, we continue to hope and to be thankful.   Even though the frustrations are many and at time overwhelming, there are still many moments of laughter, joy, happiness, and family memories.  We are thankful for TEAM PETER and for everyone who joins us in helping Peter live his best life for as long as possible.  We are thankful for our family and jobs/ministries.  We are hopeful that these new therapies and interventions will be helpful.  We continue to hope for a scientific breakthrough to help Peter and all those who have Battens.  Ultimately, we have our hope in Christ, who allows us to walk this journey with grace, hope and even joy.  

 

It you are still reading - Choose Joy no matter the circumstances.   

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.