MITO DOES NOT DEFINE ME
by GEMMA DETWILER

                It was in 2010 that I would begin what I would define as the most important part of my nursing career.   I had only graduated nursing school in 2009, and had just begun my new adventure.   As luck would have it, I ended up with an amazing family, with the most amazing boy I had ever met…Jacob. 

                I can absolutely say that I didn’t truly know what defined a person until Jacob taught me.  In the early days of “Gemma and Jacob”, I would take stacks of school books and printed literature on seizures, mitochondrial disease, mitochondrial function, drug interactions, etc…  In my school set brain, I needed to absorb as much of this as possible because, Jacob was a little boy with mitochondrial disease.   I needed to know about mitochondrial disease.  This was the ONLY way I was going to be successful. 

                I wouldn’t say that diving back into the books was a waste of time; I definitely got a better understanding of what Jacob’s fight was all about, but I now realize, I was defining him by his disease.   Just when I thought I was starting to get it, Jacob decided to show me a few things.  By this, I mean he decided to teach me a few lessons.   The one I learned the quickest was:  Jacob does not like following the book.   Not just the book on mitochondrial disease, Jacob didn’t like following the book on ANYTHING!    Jacob had the sweetest eyes, with the chubbiest red cheeks, and the most sincere smile… but this boy was feisty!!!  

                I slowly started taking fewer and fewer books to Jacob’s, because the best resource was Jacob himself.  I started taking cues from Jacob.  The way he moved his head, his eyes, the way he twitched his fingers and toes, even just the rhythm in which he breathed.  Jacob started to teach me, that mito did not define him.   Jacob was not, “a little boy with mito”.   Jacob was: a son, a brother, a grandson, a friend, a nephew.  My job was NOT to learn everything I could about Mito, it was to learn everything I could about Jacob, and how to help Jacob fulfill each of his roles to the fullest.  I would help Jacob, be Jacob.  

                Because he was so fragile, Jacob was homebound for much of our first year together.   We would get out for walks and doctor’s appointments, but never go to do anything incredibly crazy.   I decided we would make the most of our 4 walls.  We crafted, we painted, we sang, we danced, we even tried to annoy Sarah.  We plastered Jacob’s handprints and footprints on absolutely anything, and most importantly, I gave this boy as many kisses as I possibly could. He was in and out of the hospital so much that first year. Despite this, after each discharge, Jacob did not come out “a little boy with mito”; He came out a fighter, a warrior, a superhero. 

                Once Jacob made it very clear that Mito wasn’t going to have its way, we got to go back to pre-school.  This was the coolest, and most incredible part of Jacob’s life that I got to experience.  I would obviously need to stay in school with Jacob, which would mean that classmates may need to see me perform a suction or a transfer.  At the beginning of each year, I would talk to Jacob’s classmates about who I was, why I was with him, and I would show off our fancy equipment.  I would explain that Jacob had something called “Mito”.  I would turn on the suction machine so they could hear how loud it was, and explain that what I was doing would not hurt Jacob.  I would explain that sometimes they might see a little tube on Jacob’s face to help him breathe better, or that sometimes we might have to leave the classroom if Jacob felt overwhelmed.  I answered questions like “Why don’t his legs work? Does he eat ice cream?  How does he eat?  Why doesn’t he talk?”  What I found myself naturally adding was that Jacob was still just a boy.   That he had a sister, he liked to draw, craft, listen to music, and to be with friends. 

                While in pre-school, I could still carry and lift 42lb Jacob with ease.  Friends would ask him to ride bikes, play basketball, play trucks in the sand, play pirates on the slide.  Somehow, we did all of those things.  Jacob hardly missed a beat in that classroom!  I even recall wearing footie pajamas with Jacob for pajama day.   Not once did anyone say, “I know Jacob has Mito, but can he play?”  This was all because this little boy decided Mito would not define him.  

                As Jacob got older, and a bit heavier (62lbs), some of these things became a bit more difficult.  But, we managed.  Enter Brittany and the communication device.  She gave Jacob an independence he never knew, and she gave us the opportunity to get to know this little boy in a new way!  Sure, he occasionally threw out “Jackass, I don’t like it, I want to go somewhere, playboy”, but…he sure did make us laugh!  This boy was just as feisty as he was funny.  And once again, Jacob did not let Mito define him. 

                In his own little way, Jacob taught me something.  We are not defined by our disease or our physical abilities.  We are defined by our strength, our perseverance, our thirst for life.  I carry this lesson with me each and every day, and I take every opportunity I can to pass a little Jacob to my boys.  Jacob was the BEST son, grandson, nephew, brother and friend… all because he decided “Mito does NOT define me.”  

 

---Jacob, I miss you each and every day… my handsome.

 

Love you always,

Gemma

 

MITO DOES NOT DEFINE ME
by GEMMA DETWILER

                It was in 2010 that I would begin what I would define as the most important part of my nursing career.   I had only graduated nursing school in 2009, and had just begun my new adventure.   As luck would have it, I ended up with an amazing family, with the most amazing boy I had ever met…Jacob. 

                I can absolutely say that I didn’t truly know what defined a person until Jacob taught me.  In the early days of “Gemma and Jacob”, I would take stacks of school books and printed literature on seizures, mitochondrial disease, mitochondrial function, drug interactions, etc…  In my school set brain, I needed to absorb as much of this as possible because, Jacob was a little boy with mitochondrial disease.   I needed to know about mitochondrial disease.  This was the ONLY way I was going to be successful. 

                I wouldn’t say that diving back into the books was a waste of time; I definitely got a better understanding of what Jacob’s fight was all about, but I now realize, I was defining him by his disease.   Just when I thought I was starting to get it, Jacob decided to show me a few things.  By this, I mean he decided to teach me a few lessons.   The one I learned the quickest was:  Jacob does not like following the book.   Not just the book on mitochondrial disease, Jacob didn’t like following the book on ANYTHING!    Jacob had the sweetest eyes, with the chubbiest red cheeks, and the most sincere smile… but this boy was feisty!!!  

                I slowly started taking fewer and fewer books to Jacob’s, because the best resource was Jacob himself.  I started taking cues from Jacob.  The way he moved his head, his eyes, the way he twitched his fingers and toes, even just the rhythm in which he breathed.  Jacob started to teach me, that mito did not define him.   Jacob was not, “a little boy with mito”.   Jacob was: a son, a brother, a grandson, a friend, a nephew.  My job was NOT to learn everything I could about Mito, it was to learn everything I could about Jacob, and how to help Jacob fulfill each of his roles to the fullest.  I would help Jacob, be Jacob.  

                Because he was so fragile, Jacob was homebound for much of our first year together.   We would get out for walks and doctor’s appointments, but never go to do anything incredibly crazy.   I decided we would make the most of our 4 walls.  We crafted, we painted, we sang, we danced, we even tried to annoy Sarah.  We plastered Jacob’s handprints and footprints on absolutely anything, and most importantly, I gave this boy as many kisses as I possibly could. He was in and out of the hospital so much that first year. Despite this, after each discharge, Jacob did not come out “a little boy with mito”; He came out a fighter, a warrior, a superhero. 

                Once Jacob made it very clear that Mito wasn’t going to have its way, we got to go back to pre-school.  This was the coolest, and most incredible part of Jacob’s life that I got to experience.  I would obviously need to stay in school with Jacob, which would mean that classmates may need to see me perform a suction or a transfer.  At the beginning of each year, I would talk to Jacob’s classmates about who I was, why I was with him, and I would show off our fancy equipment.  I would explain that Jacob had something called “Mito”.  I would turn on the suction machine so they could hear how loud it was, and explain that what I was doing would not hurt Jacob.  I would explain that sometimes they might see a little tube on Jacob’s face to help him breathe better, or that sometimes we might have to leave the classroom if Jacob felt overwhelmed.  I answered questions like “Why don’t his legs work? Does he eat ice cream?  How does he eat?  Why doesn’t he talk?”  What I found myself naturally adding was that Jacob was still just a boy.   That he had a sister, he liked to draw, craft, listen to music, and to be with friends. 

                While in pre-school, I could still carry and lift 42lb Jacob with ease.  Friends would ask him to ride bikes, play basketball, play trucks in the sand, play pirates on the slide.  Somehow, we did all of those things.  Jacob hardly missed a beat in that classroom!  I even recall wearing footie pajamas with Jacob for pajama day.   Not once did anyone say, “I know Jacob has Mito, but can he play?”  This was all because this little boy decided Mito would not define him.  

                As Jacob got older, and a bit heavier (62lbs), some of these things became a bit more difficult.  But, we managed.  Enter Brittany and the communication device.  She gave Jacob an independence he never knew, and she gave us the opportunity to get to know this little boy in a new way!  Sure, he occasionally threw out “Jackass, I don’t like it, I want to go somewhere, playboy”, but…he sure did make us laugh!  This boy was just as feisty as he was funny.  And once again, Jacob did not let Mito define him. 

                In his own little way, Jacob taught me something.  We are not defined by our disease or our physical abilities.  We are defined by our strength, our perseverance, our thirst for life.  I carry this lesson with me each and every day, and I take every opportunity I can to pass a little Jacob to my boys.  Jacob was the BEST son, grandson, nephew, brother and friend… all because he decided “Mito does NOT define me.”  

 

---Jacob, I miss you each and every day… my handsome.

 

Love you always,

Gemma

 

THE TIGHT ROPE
by JOEL WESTBROOK

Circus's have many exciting acts. Crowds are impressed with elephants walking in formation. They hold their breath when the lion tamer puts his head in it's mouth. It is amazing to see how many clowns come out of that tiny car. The acrobats draw all eyes as they "fly" on the trapeze. The one that causes all to hold their breath is the one walking, doing flips, and must keep their footing on the tight rope.

My life of living with "Mito" has become the "tight rope". Every day or even every hour is a balancing act. At any time my brain, muscles or nerves threaten to "fall off". I am grateful for the days when it seems I am on the ground. There are days when my feet don't want to walk the line, when I have to be glad for my support base. Then there are the days when I feel like I am falling and hope there is a net.

I have scared my wife and some medical providers. Things my body does, shouldn't happen. Heart rate, swallowing, temperature, balance, memory, thinking... stuff my "Mito" folk know all too well. The mind can't stop asking some questions. "Will this get worse?" "How long will it last?" "What else or new will show up?" For now I'm managing to stay on the rope. Some I know have "Fallen Off" and we sorrow their passing.

On a daily basis there are signs of hope. Treatments and techniques are being explored. The level of understanding of Mito is growing. I hope my time on the rope will inspire and offer answers to those that are next in line. Staying on the rope is amazing to some, confusing to others and requires change. The 10 years I have had with Mito is a lot different than the first 48 before.

I hope that my life will be about more than me.

Author Joel Westbrook, living with mitochondrial disease for the last ten years.

 

THE TIGHT ROPE
by JOEL WESTBROOK

Circus's have many exciting acts. Crowds are impressed with elephants walking in formation. They hold their breath when the lion tamer puts his head in it's mouth. It is amazing to see how many clowns come out of that tiny car. The acrobats draw all eyes as they "fly" on the trapeze. The one that causes all to hold their breath is the one walking, doing flips, and must keep their footing on the tight rope.

My life of living with "Mito" has become the "tight rope". Every day or even every hour is a balancing act. At any time my brain, muscles or nerves threaten to "fall off". I am grateful for the days when it seems I am on the ground. There are days when my feet don't want to walk the line, when I have to be glad for my support base. Then there are the days when I feel like I am falling and hope there is a net.

I have scared my wife and some medical providers. Things my body does, shouldn't happen. Heart rate, swallowing, temperature, balance, memory, thinking... stuff my "Mito" folk know all too well. The mind can't stop asking some questions. "Will this get worse?" "How long will it last?" "What else or new will show up?" For now I'm managing to stay on the rope. Some I know have "Fallen Off" and we sorrow their passing.

On a daily basis there are signs of hope. Treatments and techniques are being explored. The level of understanding of Mito is growing. I hope my time on the rope will inspire and offer answers to those that are next in line. Staying on the rope is amazing to some, confusing to others and requires change. The 10 years I have had with Mito is a lot different than the first 48 before.

I hope that my life will be about more than me.

Author Joel Westbrook, living with mitochondrial disease for the last ten years.

 

CHARLIE GARD'S ANGELS
by Maria Hopfgarten

This is not a political post. This is not a post to take sides. This is not a post about right or wrong.

This is a post about compassion and heart for the hardest decision a caregiver has to make. This is a post about knowing what a mom's eternal love for their child feels like. This post is about making decisions for a loved one that will hurt so very bad for you. Decisions that can't be reversed or regretted. This post is for the utmost respect for parents who have had to say farewell to their child, and wake up every day with a void where their child should be laughing, screaming, and living.

None of us know the conversations or decision that took place in Charlie Gard's hospital room except his parents and his doctors. The media might think they know, but they don't. Period.

What I do know is that there is today no cure for mitochondrial disease. There is no miracle treatment in the US or anywhere else in the world. There is hope for drugs that can prevent the disease from progressing, but there is yet a miracle drug to be found that can restore failed mitochondria.

What I also know is that the heart breaking decisions Charlie Gard's parents were faced with, I have been faced with. My friends have been faced with these decisions. We didn't appear in courts or social media. We fought next to our children and loved ones at the bedside of a hospital room in an intensive care unit. I have hold my child's hand, and the hand of other very sick children, who I knew the most difficult decision had been made for. I knew it would be the last time I hold the hand still being warm.

These decisions were never made in a vacuum. They were never made by either the medical team or the parents. They were made together, not always in parallel. Most often not in parallel. The medical team seeing the signs earlier than the family. The medical team carefully bringing up the topic of quality of life. The medical team trying to feel out where the family stands. Trying to bring options to the table. Tears, hugs, and anger all bundled up into one crazy mess. Denial, hope, despair. Achy bodies from too much tears and muscle tension. Blurry eyes from too little sleep and too many tears.

When I understood the magnitude of mitochondrial disease and the devastation of the disease, I feared for years that I would have to make a decision like Charlie Gard's parents. I feared that my child would end up on life support, and that taking out the breathing tube would mean no more breaths for my child. I feared that I would struggle with the right decision, and knowing when the end had come.

In the end, I knew. I knew like Charlie Gard's parents knew. I knew that the only right decision for Jacob was to remove his breathing tube. He had slept for days. He wasn't sick with another pneumonia or virus. His body simply couldn't take another breathe. There was no medicine in the whole wide world that could change that.

I will never forget what unfolded in the PICU room that last weekend of Jacob's life. We were surrounded by Jacob's circle of friends who loved him so very much. There were tears and laughter from all the stories and memories our boy had created in his ten years of life. There were doctors by our side who didn't just care about medicine, but cared about our boy and knew they would miss him dearly. There are memories from that day that will make me start crying any time I think about them. When his long standing best friend and nurse hugged him like she could never let him go. When Sarah refused that this was the time to let go because she needed more cuddle time with his brother. When my dad called me right before we had to make that dreadful decision reality. When I laid next to you in the hospital bed your last night tracing your face with my fingers, so I would never ever forget a single piece of your beautiful face. And I will never forget your last breathe.

There are too many Charlie Gard angels to count them. They are happening every day in hospital rooms around the world. There is no right or wrong. There is no taking sides. There is just tragedy to have to say goodbye to a child where there is no cure in sight and no more medicine to help. What I hope for Charlie Gard's parents is that outside of the media they had time to be with their Charlie, and that they came to peace when the most devastating decision was finally made.

This post is for Charlie Gard's angels and all the parents and caregivers out there who had to make the most dreadful difficult decision in their lives. They did it out of love for someone they loved more than life itself.

Love,

Maria.