IT HAPPENED IN FRONT OF MY EYES

Dear Jacob,

One of my favorite hours of the week is five to six pm on Sunday evenings. Joakim and I are changing into our sweat pants and we go to yin yoga. We lay on our yoga mats and stretch for an hour. It's often very painful. Sometimes I just want to give out a little scream from a too tight hip flexor or glute muscle. Sitting in an uncomfortable position for several minutes forces relaxation of my mind, and focus on my breathing. You, Jacob, often come and visit me during this hour. I sometimes leave class with tears in my eyes.

This past Sunday, you came and visit with me. I had flashbacks from the last month with you. And in the middle of a deep glute stretch, I had a realization. You were dying in front of my eyes during your last months of life. I refused to see it. I had an explanation for every time you were more tired, and needed that extra one or two naps. You who never spent time sleeping your entire life.

I had an explanation for your edema that got so very bad in the end. I massaged your legs, I massaged your arms, I massaged your hands and feet, but there was just so much extra fluids your body couldn't get rid of. I can still feel your little hand in mine. In the end, there was this worry about how much you were able to pee each day. We measured every diaper. How happy I was when your fluid intake matched your fluid output. There was a sigh of relief when the day ended on plus minus.

I had an explanation for your increased seizures. They were subtle. You were sometimes in and out of them. Maybe there was yet an infection? Maybe your body was under more stress? Maybe, maybe, maybe. There were more questions than answers.

Your second to last hospital stay, I wasn't sure we were ever going to take you home. You had been sleeping for days, and there was no true explanation why. With some medication changes on board, you decided that this was not going to be the end, and we took you home one final time. I will always remember the happiness of rolling you through the atrium of the hospital and out in the summer sun.

We made it at home for six whole days. It was six very stressful days. We were constantly checking labs and talking to nephrology for your kidneys. We struggled to keep your fluid balance in check. We struggled with seizures. We struggled. We really struggled. But we pushed on as we always had done. I do remember your last weekend at home. I remember taking you to the pool for one last time in the water, something you loved so very much. We had a good time in the pool, and then you fell asleep in the shade holding your dad's hand. It was a perfect moment, and I will cherish it forever. Your friend Brittany came to visit. She read to you, and in the middle of the story, you did decide it was time to take a little nap. Nevertheless, you did enjoy story time in the backyard on your last Sunday. Probably my strongest memory from that weekend is Sunday evening. Due to you taking several naps throughout the day, you had no interest in going to bed at night. Sarah was off from school, and the two of you decided to watch Kardashians on your iPad in bed. As I was getting your room ready for the evening, you were suddenly so alert and normal again, and vocalized with your sister as she was holding you tight, laying behind you in bed. It was one of those moments you want to stop time.

Tuesday evening, we were back in the ER. If someone would have said you had another five days to live, I am not sure what I would have thought. It was not at the forefront of my mind that Tuesday evening when I packed our bags to take you in once again. What I did know is that we couldn't take care of you at home with all your edema, kidney problems, and seizures. We watched you like a hawk, on the phone with doctors all the time, and still not feeling we could help you.

We spent the last five days of your life in the PICU. It started out with everyone thinking you had been hit hard by an infection. Scary diagnoses were thrown around. In the middle of possible diagnoses, we thought there might be a reason for why you have acted so very sick lately. It gave us hope, something to hang on to. I also remember at some point that Joakim and I looked at each other in the PICU and we were both thinking how can we safely care for you in the home? Your care had gotten extremely difficult, and we were responsible for monitoring and medicating your very sick body at a level that felt scary at times. We were of course both up for anything as we had always been, but we definitely knew you were very sick. We talked about end of life. We talked about what would happen to you. I felt I was always close to tears at the same time as I was trying to stay strong for you and everyone else.

That last Friday is probably the day when it truly sank in. Your time on earth was nearing an end. You had been sleeping, only waking up for short periods of times, for three days. Some of your labs were still through the roof, but with no explanation. Our favorite attending sat down with us, and said that she was concerned your brain had gotten worse. That could explain the lab values. She wanted to give you the weekend to show her she was wrong, and on Monday we would do another brain MRI. I knew what this all meant. I knew we would do a brain MRI on Monday. I knew the MRI would be much worse. I knew we would be faced with questions of end of life. I knew, I knew, I knew. This is the one thing I had dreaded your whole life, having to make the decision no one should have to make.

But you decided differently. You decided it was your time. No more brain MRIs, no more labs, no more interventions. Your time had come. And once again your showed us who was the boss. You decided, and for that, I am forever grateful.

Sixteen months later, I can start to see how the puzzle pieces fit together at the end. I don't think I could take it fully in as it happened, but your last month we knew. We knew even as we continued to fight.

Sixteen months later, it's easy to get caught up in the busyness of work and life. I don't like when that happens because I don't want to take life for granted. I don't because I know not all of us can take every breathe for granted. This weekend, Colorado has been absolutely stunning. Wherever you look, you see fall beauty. We would have painted pumpkins and gone for a walk in the sun. So, I made it that perfect weekend with family and friends coming in and out, picking pumpkins, working in the yard, and going for long walks. I stayed true to you.

Sweet Jacob, fall is especially hard without you. It was your season. Thanks for sending me butterflies and colorful leaves lately.

Jacob, I love you to the moon and back,

mom.

CALLING YOUR NAME

Sweet Jacob,

Everything got so silent after you passed away. Our house was full of sounds when you were in it. You surrounded yourself with people, the noise of the oxygen tank, the beeping of your feeding pump, the sound of the suction machine. It was never truly silent in our home until the day you were not with us any longer.

When you and your sister were cuddling and hanging out together, there was really no need or room for anyone else. You had your special bond, and you loved when she was with you. You were so happy when Sarah visited you in the hospital. You immediately felt better. You even put up with your sister wanting to watch the Kardashians as long as she did it with you.

Sarah has had to figure out a lot this past year and a half. She has had to figure out how to live without you, her one and only brother. She has had to figure out how to be an only child. She has had to figure out how to live in this world with more knowledge of life than most fifteen years old.

Sarah has done it with grace. I am not saying it was easy. In fact, it was everything but easy. It hurt so much Sarah couldn't always be thinking of you or even mention your name. It was too close to her heart. Her grief had to come little by little.

It hurt me to see her pain. As a mom, I wanted to fix it, fix her, but I knew I had to be patient. I knew I had to simply be there for her. Do it on her terms.

The one thing I have been doing is to keep talking about you. I tell stories about you. I make sure to mention your name often. I make sure our good memories are remembered. I talk about the times we spent in the hospital. I take her and your dad to your bench, and sit and reflect on you, on life. Sarah sometimes smiles at the memories, sometimes she is simply not in the mood to talk. I tell Sarah when I miss you.

A few weeks ago, Sarah started saying your name again. It was like the most natural thing in the world for her. She was telling her friend a story about you. I am not sure what the story was about any longer, but I do remember how my heart filled with this overwhelming feeling of love. Later the same weekend, Sarah started asking some questions about your medical conditions. We talked about it. It was a short conversation, but it was our first mutual conversation about you.

It might not sound like a lot, but there is so much power in hearing and saying your name. I am so happy I kept talking about you. I sometimes felt like a broken record, but I know now, it was the right thing to do.

And Jacob, do know that you're always in our hearts, calling your name or not.

Sweet Jacob,

I love you to the moon and back,

mom.

MITO DOES NOT DEFINE ME

Sweet Jacob,

Your long standing friend and nurse Gemma wrote this in light of Mitochondrial Awareness Week. I was thinking you would enjoy it as much as I did.

                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

 

CHARLIE GARD'S ANGELS

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 hands, and the hands 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.

SEIZURES DOWN MEMORY LANE

Dear Jacob,

Sometimes I let myself feel a seizure. I let myself truly feel the pain of your seizures. It is something that is ingrained in me. We endured so many together, way too many for such a little body. Way too many in ten years of life. The longest seizures lasting eleven long days and nights. That was a very challenging time for you, for us. I still to this day can't imagine a stronger person than you. Everyone told us that was it. You had completely different plans, and went on living for another six and half year. You showed the doctors wrong. You showed the world wrong. How lucky was I they were all wrong.

When I think back on your seizures, it always involved a clock, preferably where we could track time by the second. The beginning of a seizure was often subtle with you. Did you just stare out in the open or was it the beginning of a nasty seizure we didn't know if we could stop or not? The times when your eyes were starting to look up and to the right, to the left. Were you going to get "stuck" with your eyes or would they come down again? You were always trying so hard to break the cluster. Seconds turning into minutes. Knowing that for each minute, we were getting closer to execute your seizure action plan and give you your rescue medications. Medicines to be drawn up. That nasty sticky red medicine that I still know the taste of. My eyes never leaving your pulse ox, adjusting your oxygen level as needed. Many times calling your neurologist at the same time as I was watching you like a hawk. I never broke down in the middle of our emergencies. I pushed through, kept my head clear, all to advocate for you.

And then there were the times when the seizures took over. Whatever we did, we couldn't control them. The seizure monster took over our lives, our souls, our happiness. If you are someone who has never called 911, you have not experienced a true medical emergency. It's scary. You're not in control. You're needing immediate help - now.

I can't even count the times I had to call 911. It was familiar territory to us. I knew what the dispatcher would ask me. I knew what the ambulance crew would ask and would do. I learned to open the front door, so they simply could walk in. I made sure your medication sheet and seizure action plan were available for them. On a good day, my bag was packed and ready to go with you. Other times, the cell phone didn't even accompany me. But I never, never knew how you would do. I was a hawk in the ambulance. I often had to negotiate with the medical crew. I never wanted them to start a line in the ambulance. No one ever succeeded, so why put you through that pain. I had to give in at times when they wanted to medicate you in the ambulance. I knew where on the highway they would call Children's to let them know you were coming. I was always happy when we were greeted by our favorite ER doctors and nurses.

We had many, many hospital stays due to seizures. They were often frustrating hospital stays. Neurology is not a clear science. One thing that might work for one patient might have the opposite effect on the next patient. Neurologists who didn't know you made me scared. They approached you with their typical cards, which we already knew would not work. Negotiations, patience, and trial and error. I got to witness the hierarchical structure in the hospital where I just needed to get to your doctor, no matter what. Advocacy and patience all bundled up in one.

Jacob just home from the hospital after having uncontrolled seizures.

In your ten years of life, I never, never got used to your seizures. Each one of them ate at my soul. Each of them was a threat to your life. You went in and out of periods of epilepsy, and they took over our lives. They took over your brain, your body, your mind. They made me feel helpless. They made me wish for a miracle, a miracles to make you seizure free. One wish that never happened.

Today, I don't need to worry about your seizures. Your brain is not seizing any longer. In my world today, there are plenty of opportunities to sweat the small stuff. Plenty. So to feel your seizures from time to time make me stay real. Make me not forget about what truly matters. And it makes me remember you because even on one of your hardest day, you were my beautiful boy. I could hug you. I could kiss you. I could be with you.

Sweet beautiful Jacob,

I love you forever to the moon and back,

mom.

ANOTHER SCHOOL START

Dear Jacob,

It was a little relief when your first anniversary was over. We had made it through all "the first ones" without you. I of course knew this wasn't it, but maybe it would be a little easier to relive holidays and other special days with one year under the belt?

It worked with a busy travel schedule around the globe and little vacations throughout the summer, and then bang! Right there was school start once again. Right there was my birthday, and the grief came to say hello once again.

I did better this year than last when I hardly could make it in to your school without being a complete mess (Dear Jacob). I was able to take Sarah to her school without crying every five minutes. But I had the urge to find you. I took Friday off to make it a long birthday weekend, and first on my list was to climb Flagstaff. I found you in the butterflies and in the stillness of Walker Ranch where I often feel like the only human soul in universe. The sky was clear blue against the black mountains and green grass. I sat at the amphitheater where your ashes are spread, and talked to you. It was our own conversation, and one to only be shared between mother and son. Between you and I.

Jacob, I am getting faster on the downhill! I felt so good going downhill and feeling the wind against my skin and my face. I felt like flying! I hope you get to experience that as well.

And then I went to your school. I went to sit on your bench. I went to sit under the big tree. I went to hear the sound of your classmates on the playground. I went to look at the door that was your entrance to this school. I cried. Sad tears since there will be no more school starts for you. Happy tears because you had the most amazing teachers and friends at this school. In fact, three of your favorite teachers made your memory bench happen. They wanted you to have a permanent place at your school, and how lucky am I, that they made it happen. As I was sitting on the bench, I texted two of your teachers to let them know where I was, and I was thinking how incredibly lucky you were. You made a forever imprint on some of those teachers who I today call my friends.

I thought about you and I running in the annual school run. You loved it so much that we went around and around despite that stroller of yours being a tad heavy. I thought about your last teacher, and how incredibly positive she was about all your strengths. Not once did she see anything you needed to change or improve. What a beautiful refreshing gift she was to both of us in the endless IEP meetings we endured together.

After I had my moment on your bench, I was ready to go home. I felt ok. School start means I have to visit at your elementary school, probably for years to come. I am also hoping to meet with some of your teachers throughout the year, bring a coffee, and simply sit and chat about you on your very special bench. One of my absolute favorite things to do.

This past week reminded me that grief will come and sit with me when it wants to. It's like a force I can't avoid or fight. I can keep it under bay for a little bit, but it will always win. It will always find me. It's something I will have to face head on. Grief and love go hand in hand. It goes back to one my favorite Winnie the Pooh's quote:

Sweet Jacob, I love you to the moon and back,

mom.

RANDOM SUMMER REFLECTIONS

Dear Jacob,

Life has been a whirlwind over here. I have been traveling a lot, and been to new places in the world I never knew if I would see for sure. It’s a little bittersweet, since I wouldn’t be able to travel this way if you were still in my life. Know that I always carry you in my heart wherever I wake up in the morning. You are never far from my mind. I see you in the sun rise and in the sun set. I see the little signs you give me of butterflies and rainbows wherever I happen to be.

A little message to the Buddhist Gods in Tokyo.

Your sister is enjoying her summer break. She is not interested in it coming to an end. We got to spend a few days together all of us in the mountains recently. We brought her dog, and it’s amazing to see the special bond the two of them have. You would have loved her little dog, and he sure has a little of your strong minded personality we so need in our family.

I got to go to the pool a week ago, which is something I haven’t done much this summer. I really missed you there. That was the one place we always took you in the summer. It was your happy place, and something we could do as a family. I missed the weight of carrying you in to the pool, and taking a little break on the steps so I could rest my arms and you could get used to the water temperature against your legs. I missed seeing your happy face and you vocalizing on your floatie. I missed getting you ready in the shade after your swim, and holding your hand when you were resting on the sun chair. I do miss you so much.

And in the middle of missing you as only a parent can do, I try to find a new normal. I search for moments of happiness without you in it. It’s different, and it isn’t always easy, but that we both already knew about life. There is emptiness where you should be. But by now I have learned to wake up each morning, and my first thought is not to check on you to make sure you’re ok. I have learned that our home doesn’t have the same sounds and smells as when you were in it. I have learned that the rhythm of my day is quite different now. I have learned to not live by your daily schedule. I have learned that the sound of an ambulance doesn’t mean another emergency for you, even if sirens still bother me. I have learned firsthand that change is the only constant in my life. I try to hang on to those special moments in life that often are so simple, the moments that make up a happy life. That is the one gift of life I will always associate with you.

Some happy moments from this summer:

Sarah on the Mexican beach.

Celebrating your 1st anniversary with friends and balloons.

Run along the river Spree in Berlin.

Sarah off to Kentucky for her first out of State summer camp.

Lisbon with a very dear friend.

Completing another Courage Classic supporting the Mitochondrial Clinic at Children's.

Singapore

Greetings from Tokyo!

Sweet Jacob,

I love you to the moon and back,

Mom.