Hi friends! Checking in to update you all on Elijah. He is over his respiratory infection, but still trying to get back to his baseline. Because of his breathing issues he hasn't been able to go back to school yet, but has been getting plenty of stimulation at home with all his sisters friends and neighbors coming to hang out. 😜 We are inviting you all to join team "Elijah's Victory" to celebrate Batten families, raise funds for research, and spread awareness of Batten Disease. This is a virtual 5K, you can complete it whenever you can, take a nap, or get together with us on June 9, International Batten Awareness Day, and we can walk or run it together. (When you follow the link scroll to the bottom to find the registration section) https://bdsra.redpodium.com/2019-batten-5k?fbclid=IwAR35SsjjK6VhXFsw6Xpfbnw_8_H5o7h2OGkg8iDnLbVMKGtd10j-Xx_TOTY
Although rare and genetic diseases, and many times the symptoms, are uncommon to most doctors, rare diseases as a whole represent a large medical challenge. Combine this with the lack of financial or market incentives to treat or cure rare diseases, and you have a serious public health problem.
Here are a few statistics and facts to illustrate the breadth of the rare disease problem worldwide.
Elijah has Late Infantile Batten Disease, a rare inherited genetic disease.
WHAT IS BATTEN DISEASE? * Children and adults with Batten disease have inherited genetic material from their parents that may take some years to come to bear in their everyday lives. And when it does, families are often in shock when they learn how a normal child or adult (Kufs form) could change quickly, and without seeming cause. Regardless of where your loved one is in the progression of Batten disease, BDSRA wants you to know there is a community of people like you learning about and coping with this life limiting illness.
Batten disease is one of approximately 50 diseases called lysosomal storage disorders (LSD), meaning that genetic mutations disrupt the cells ability to dispose of wastes. With Batten disease, cells are thrown out of balance with the build-up of proteins and lipids (fats).
Because of these damaged cells, patients with Batten disease suffer progressive neurological impairment, which includes:
Although Batten disease can vary widely in its progression, Elijah has followed a "typical" progression for CLN2-Late Infantile Batten Disease:
How does the disease progress?
Children are healthy and develop normally for the first few years of life. Towards the end of the second year, developmental progress may start to slow down. Some children are slow to talk. The first definite sign of the disease is usually epilepsy. Seizures may be drops, vacant spells or motor seizures with violent jerking of the limbs and loss of consciousness. Seizures may be controlled by medicines for several months but always recur, becoming difficult to control. Children tend to become unsteady on their feet with frequent falls and gradually skills such as walking, playing and speech are lost. Children become less able, and increasingly dependent. By 4-5 years the children usually have myoclonic jerks of their limbs and head nods. They may have difficulties sleeping and become distressed around this time, often for no obvious reason. Vision is gradually lost. By the age of 6 years, most will be completely dependent on families and careers for all of their daily needs. They may need a feeding tube and their arms and legs may become stiff. Some children get frequent chest infections. Death usually occurs between the ages of 6 and 12 years (but occasionally later). (from bdsra.org)
Elijah is going to turn 11 in 19 days. He's constantly battling seizures, chest infections, and incredibly tight muscles in his arms and legs. We love him so much, and are amazed that he continues to fight so hard. Although there is still no cure for Batten disease yet, there is a lot of research and trials going on, and we know one will be found very soon.
This day, 5 years ago, in 2011 we received Elijah's diagnosis of Batten Disease. The geneticist called that day to tell us that everything was consistent with Batten Disease and we had one more test to confirm the type, which turned out to be Late Infantile. We were lost and confused, scared and worried. With the diagnosis we thought our time with Elijah would be extremely short, less than a year because he was deteriorating so quickly. Five years later almost everything has been taken from him, his sight, his personality, his ability to eat and move, but he's still with us. Every day is a struggle for him to keep breathing, but he does it, he's so strong and full of fight and life. We have been blessed with these years, all the people we have met and friends we've made through Elijah.
I wanted for a moment to give up on trusting The Lord. Wondering what would life be like if I let go of faith, so many have long ago. At least it seems that way. Jesus is a single conversation, a quiet prayer with no heart in it. A prayer with no thought of walking it through. Words for many to see Jesus.
I sit next to a boy who cannot express love in any human way, at least I cannot see it all the time. I often wonder if I am imagining that he knows me. Rarely a smile or sigh, just that far off look and his eyes rolling back in his head. Perhaps that is the way of faith. Look hard at God until you see Him clearly. Like looking long into my son's face until I see that He knows me, he loves me, and desires me to speak to him even if he cannot respond.
I do not pretend God is visible to me. I still cannot see him or audibly hear his voice, but in an inhuman way, a supernatural way I know He is here. Directing my thoughts now, possibly your thoughts. I am not in control. I try to be, but it does not work. But I see he who talks to God regularly and as become more aware of his presence, more acquainted with His character and can trust him in a greater way. He has not programmed his brain to believe fairy tales and believe the invented dreams of an ancient man.
He does not ignore reason and science. Somewhere along the way the many have cleverly crafted a plan to make God and faith in Him appear foolish. There was a Great Wall erected between science and faith in the unseen world. Programs, books movies and every device known informed the mind and heart that God was invented. A pleasure tool to bring happiness to weak people. As I look upon a struggling child these thoughts paraded through my mind. It is over they say to, "God has forgotten you. He never was here to begin with. Look about you. So many pencil in Jesus on a card never fulling intending to give EVERYTHING to Him., No! Just the bare minimum to cross the gates of heaven. They believe that is all it takes. God only wanted card carrying Believers but not life altered, radical men and women fully given to Christ." So give up and just parade as a believer in Jesus, no need to sell the farm." But I let go of these lies. If anything this child in bed has been used to drive me closer to God, closer to His holy and purposeful intent for my life. I do not have all I want but I see better that I truly have all I need. Just out taking a walk and I get tired. I grow weary and afraid. I lose my way at times and make mistakes and even willfully turn from Gods perfection for me but I just keep walking toward Jesus. Keep praying I will follow His lead. Keep relying on Him to direct me from the inside through His spirit.
How can God pull back from what He has paid for at such a high price ?
How could I turn away from a God who has held no good gift from me, giving me His own life in exchange for my wretched sinful heart? What the world sees as a problem, a hindrance to my personal happiness I see more now as joy and eternal life. A victory crown I plan to give to God. I can do nothing good, nothing without His grace. It is sufficient for me. I ask that I will be able to keep on walking, keep on trusting Jesus for all.
It is okay to doubt but how long will it dictate your life and pull from you any hope at peace and freedom? Give them to Jesus.
Life. The race of a single day run together as a marathon of months and years.
The defining moment. The first day. Life was there, it was only hidden behind muscle, skin, bones and blood.
That first piercing cry after a long pause of silence and anticipation. Will this little life survive his first seconds? Will the world hear his voice and see glory through him? The long and anticipating pause.
Everyone, nurses and doctor breathed relief when the little boy screamed out.
Besides being a little orange, and an extra day in the hospital, Elijah was a normal healthy boy. All eight pounds and 22 inches of him. His parents wrestled the next few days and weeks. Tired but not frustrated. They were tired, but emerged in joy. This was their beautiful son. He took much, but he gave much more.
His mother and I carried him through the doorway of our home. It seems like a little shack in my mind, but it was one of the finest, happiest days of our life.
It plays like magic in my memory now.
We could only see every day getting more incredible, as we watched him cry and eat. When I brushed his first teeth with foot cream thinking it was toothpaste. When he peed on my face as I gave him a bath.
He crawled across the room, he sat in the tall grass and hid in the leaves. We took him for walks down the street and said hello to the neighbors.
His first steps at the beach.
Never a hint that these would be our memories, they would be all we had left of our son as his life would slip away.
I can see these moments in our history in pictures, and then my brains pulls up images and sounds, smells and aromas. The place in my mind where all these precious moments are held. They are pulled up like old movies, faded and choppy . I close my eyes and watch them over and over again. I rewind them and fast forward hoping to see some detail I missed. I can see him at Church in his grandpa Tim's arms. He is wearing a white outfit. He is getting dedicated. Everyone is smiling but Elijah is sleeping. I can see His first birthday. He was born March 19, 2006 two years after the passing of His grandma Duska. His mom and I saw him as a kind of gift. A March child meant green Saint Patrick cake. And the cake was all over the place. Elijah even wanted to share his mess by extending his cake covered hand to us. He pushed himself around on his little motorcycle his grandpa Keith got him. All the exciting toys a one year old boy can get. Brings joy to a fathers heart. When I walk around a toy store these days it is different for me.
This is a very sad road for me to walk down. I miss that happiness, those days of surprise when my dreams for Elijah were unopened.
I imagined all joy. The kind of joy only a man, a father can see. I never saw this hospital bed in his room. Who dreams that for their son?
Elijah was born full term, the result of a pregnancy with absolutely no problems. He was a hefty weight and length and thrived from the beginning. He was never a good sleeper as a baby, but he grew and met all his milestones. He learned to walk, to run, to talk, to draw, to sing, to kick a ball, to make friends, to swim, to question. When he was 2 years old, he had his first seizure. We were told it was normal, that many children, when sick, will have a seizure as a result of a high fever. Then he got sick again, and he seized repeatedly all night, we couldn't keep his fever down enough to stop them. But when he got well they all stopped. We went on and were just always on alert to cool him off immediately should he get a fever, we were told he would grow out of it by age 5.
A few months later he started falling down. Just out of nowhere he would fall. We were sure he was being a normal sibling, trying to get attention away from his new baby sister. But within just a few weeks he started falling so frequently we couldn't let him walk anywhere on his own, we couldn't let him go outside, in the kitchen, near coffee tables, anywhere with a hard or sharp surface. We were sent immediately to a neurologist.
He was started right away on anti-seizure meds, and they worked. He also went through the normal testing, MRI, EEG, blood draws. Diagnosis was epilepsy, we were told many children grow out of it.
He then began to have behavior problems, worse sleeping problems, and he would get sick often. We just thought we had a "sickly" kid and thought nothing more of it.
We began to notice that he had a hard time learning things like colors or motor skills like riding a tricycle, so we had him evaluated by the school system. We were told he had ADHD and he was behind but once he got in preschool he would catch up.
He started preschool and he DID start to pick up on his skills, he loved going to school so much. But right before Christmas during that year he got the flu, and overnight, literally overnight, he stopped walking and talking.
The neurologist told us he had a virus in his brain and he would get better and get back to normal. The neurologist told us that month after month as he adjusted Elijah's meds, telling us that he just needed the right combination of medication and he would get back to normal. He did regain some of his skills, but it was temporary, and overnight lost them again. The last video we have of him walking on his own is from January 2011, when we were at the beach celebrating Aaron's birthday.
When he started preschool he was "normal", when he graduated preschool he couldn't talk, he couldn't walk unassisted, he had uncontrollable seizures, and if he wanted to play outside he had to wear a hard helmet with a face mask to protect his teeth.
Over that summer we switched neurologists. The new neurologist knew immediately what Elijah had, but of course we had to go through some extensive testing to confirm it. In the meantime Elijah started Kindergarten. He could no longer walk and had to start his school year out in a wheelchair. But he was still very interested in school, he could eat on his own, crawl around and play on the floor. By November that year we finally had our diagnosis and we knew he would never "outgrow" this.
When Elijah finished kindergarten he had a feeding tube, he had lost all his neck control, he couldn't sit up on the floor and play.
When Elijah started First grade, just a few months after graduating kindergarten, he no longer had hand control, he couldn't even hold onto a toy. And somewhere along the line he lost his sight.
Now he has almost no control of his body, besides moving his head side-to-side, his muscles contract into a curled up position constantly, he can't handle the secretions his body produces, he has seizures every day, sometimes he stops breathing. Every single time he leaves the house he needs: a wheelchair, a suction machine and various attachments, oxygen tank, an extra oxygen tank, oximeter, diapers, extra diapers, wipes, emergency seizure meds, comfort meds, formula, syringes, tubing for his feeding tube, nebulizer machine, extra clothes, leg braces, and more. He weighs over 50 lbs, his wheelchair weighs over 50 lbs, there's a lot of heaving lifting around our house.
One thing he does still have control over is his emotions, and he can show them to us through his eyes, through his lopsided smile, a grimace, or a moan.
Batten Disease is a fatal disease, it is always fatal, there is no cure. For more information about Batten Disease you can visit www.bdsra.org
I saw him today, Elijah.He came walking around the corner of where I was sitting and studying. He sat down across from me in a chair that was placed there for him. The open chair to come any time. He was tall and skinny like I was at his age. But always he walked taller and with confidence, not like I remember myself. I measured my appearance and abilities to those around me. Not Elijah. He had a confident humility and a spirit of victory glowing off him. He brought a presence of grace to the rooms he entered. The people around him could not tell him who he was, for he knew God had already declared it. Elijah embraced it fully, with no hesitation, with no doubt. Not as I remember myself. This day Elijah sat there with his head down low. I knew right away he was feeling bad about something. I said, "What's up sport?" That is how I called him before he got sick. Then I sort of set that name aside because I was not sure I wanted to call him that and more. He has lost his ability to grip a ball but not his desire. No power in his body but such a force kills in his eyes. He would run a marathon and back if his body could keep up with his soul. It seems a waste almost to put such love in a body that cannot express it in ways all people could see. Then most are not able to comprehend true love. Good enough to settle for desire and emotions that parade as love. Not God’s love. It is a ride of the wind and flight across stars and then that is only skimming the night glow of the ocean. There is a whole depth we would dare to see.
Elijah came into this world on a Sunday morning. One of the first calls I made was to our Pastor at Church. He announced Elijah's arrival to the congregation that Sunday morning. We always hear people say things like, “that man or woman touched a lot of people's lives.” I think after Elijah’s Birthday party I have a better understanding of what that means. How one little boy can touch so many lives. He can inspire people to live, he can point people to trust Jesus Christ.
I looked at Nicki a few days before Elijah’s party and said, “you are going all out with this party.” I saw Nicki spend several weeks making robot decorations and buying party foods. Her sister Alanna made a cool party invitation that we handed out to friends, family, and neighbors. We knew people would ask, “what should we get for Elijah.” He can’t play with toys like other seven year old boys. We knew he would enjoy a swing, so we asked for one. Elijah has so many friends, many of whom he has never met. Many of them we have never met. The day of the party it was awesome to see many of them in one place. His school teachers showed up while he was outside in his chair. He heard their voices and we could tell in his face, and in his eyes, that a familiar person was there. Like a boy running around playing with his friends making fighting sounds and hitting things with sticks, Elijah in the back and forth movements of his eyes and his head was overjoyed by the excitement of that day and all the sound from the people. I can see him laying on the floor as his sister Grace opens her Birthday presents. Their Birthday's are only a week apart. I guess it is a blessing to have birthdays so close to each other. Grace can help Elijah blow out the birthday candles and open his presents. Elijah did not care for cake and presents, he knows birthdays are not about presents. The presents would not be that special without the people who celebrate with you. Elijah had so many people celebrate with him that day.
The amazing people at Elijah's school saw his invitation and our request for a swing and many individuals took it upon themselves to gather all the funds needed to purchase the swing. On behalf of his friends at school Elijah’s two teachers presented the gift. It was almost enough to cover the entire cost of the swing. The rest of the gifts we were able to put in his account to put toward a wheelchair van. It was a moment of joy. I was overwhelmed. I wanted to hug every person who gave their money for Elijah but more than that they all showed real love. What a treasure to be Elijah’s Father.
At the end of the day we put Elijah down to sleep. No sleeping medication was needed that day. The excitement was enough. When I asked Nicki about the effort she was putting into this party she said, “We don’t know how many birthday parties we will have to give him.”
It may sound sad, and I guess it is, but does anyone know how many birthdays they have? I guess it makes you think about how you look at each day of your life. A birthday is not celebrating just another year, but another year God has given you to live.
I know every day cannot be a party with balloons and cake and cool Robot music but we can cherish our son, like he is a priceless diamond we traveled thousands of miles to find. Look at every little child in that way. Life is a precious gift not to be wasted.
October 14th at Grantland Baptist Church a dream came true. It brought me and many others great joy and inspiration. Along with my brother Ben, we baptized my son Elijah. Several months ago I had a dream that I baptized Elijah, it was so vivid and clear. Though Elijah cannot verbally express his love for Jesus, it is evident through his expressions. He needed my help to preach his sermon, and it was an honor to do so. Baptism is an outward expression of an inward heart transformation, and only God knows what is in Elijah’s heart and mind. God wanted me to understand His heart. I have many videos and memories of Elijah talking about Jesus. Elijah would often sit in his room looking at his picture Bible and say, “Jesus die. Jesus is in the Bible. He is in heaven.” God says, “Truly I say to you, whoever does not receive the kingdom of God like a child will not enter it at all.” NASB