“That was the day I will never forget because the world stood still. My heart and soul was forever changed and so damaged, and Lydia had no idea what I had just learned. I couldn't face her for hours. I told my family and I watched my strong dad cry and my mother break down. Every day after was the same.”
Lydia’s mom and advocate
So similar are the stories of those who have learned that their child is battling Batten disease. This, even though many of the details of each story may be different. They all however, share in common the experience of caring for a child with a rare and fatal disease. Some families have had more than one child that has battled or who may be currently involved. The experience shared involves a type of heartache that is profound. The only thing that can overcome the heartache of the battle is a type of love that is like none other. Love that is undying and which helps a person to move forward. This in order to do whatever it takes for the sake of the one that battles. These are children that we are talking about, defenseless and in need of the attention that is poured out. The circumstances involved may be different but the stories are so similar in terms of the commitment. I was once told by the mother of a young Batten warrior that the journey that is Batten disease can be a lonely existence. I can only imagine that she was speaking the truth. Somewhere on the planet sits a mother with her child. It doesn’t matter where that place might be although it could be several places, all at one time. In fact, that place exists anywhere a young mom moves through the day with one goal in place. That is to help her child to experience the greatest amount of joy that is possible while battling a disease that neither of them has any control over.
Things like paying the bills and taking care of the basics is a struggle but it is one that has to be faced. This child after all, is more important than anything else in the mom’s existence. This child is the center of her world and her reason for waking each morning. Everything that is done comes from a love that is mixed with heartache. These, in combination with each other, provide a type of emotion that is not felt by many and it creates a bond that cannot be equaled. The daily tasks are many and each one is necessary for the welfare of the young warrior. The days are so long and sleep is often fleeting. Some are sympathetic to what is happening within the walls of the place of dwelling and still, there are those that turn a blind eye. Good as gold are friends and family that are willing to extend a hand of support. Not everyone will take the time to consider the special nature of these children. It often seems that they are sent to teach us things and to change us in ways that no one else can. This type of thinking may evade those that are the closest and in the middle of the battle. From a distance, they are more clearly seen. There is another scene that plays out in various places at any time. In a hospital, having lost track of the number of times they have been there, is a set of parents with their Batten warrior. So often, we are talking about a mom because dad had no choice but to return to work. Complications have set in with their child’s health yet again and they are things that have come along as a result of the battle with Batten.
Relentless is the disease as time continues but it is incapable of diminishing the love that exists. Whatever it takes on any given day. Everything that is done is done so out of love and a bond that is greater than any other. It is more than most any can know about. Why do these stories matter? You have to take the time to pay attention. As a parent to someone who has special needs, I find that the kids that battle a rare disease like Batten are the most amazing. They are so resilient along the way and they are that for as long as they are allowed to be so. What they do to the human heart is something that is more than words can often describe. It is a tragedy anytime a parent receives the news that their child has a fatal disease for which there is no cure. Many of the details of what will follow will create a type of love story that has made me take notice. There are many that I am aware of now but in the beginning, there was this one young mom in particular. She had told me that anyone would have done what she had. While many will do anything necessary for their child’s welfare and well-being, not all will be asked to do things that take real courage. It takes courage, when being a young single mom, you have to pick up and leave the home that you are familiar with. Not knowing what awaits you, you leave because you have no other choice. Your daughter’s needs come first and this causes you to take action. Being an ordinary person, without an immense amount of support, you just pick up and move to a place where help exists.
No Love Like It
I met Alicia Headrick in the same way that I have met other parents of kids that battle Batten. That being through social media. The resemblance between herself and her little girl, who is named Lydia, was striking. I have said a couple of things about the love that exists between a mother and her daughter in the past. Being a dad to three boys, I am simply making an observation or two as I state - There is no love like a mother’s love and, there is no bond like that which exists between a mother and her daughter. There seems to be something kind of dramatic to me about a young mom getting into a car with her tiny daughter and leaving home after receiving the worst news possible. Searching for any help that may be available. Finding it and petitioning those responsible to make sure that her daughter is included. Not knowing what her new surroundings will be like, nor what the future will hold. Just going because there is no other choice. I approached Alicia about the possibility of working with me to which she responded by showing interest. So much has taken place since that day and what I write here will be a snapshot of the entire story. Alicia had asked me if I was good at piecing information together after she agreed to work with me and I said that I was. As a result, she forwarded to me several emails that could be used to construct her story.
What I found as I read through them helped me to form an opinion about her that has stayed with me to this day. My feelings were only strengthened when I met Alicia and Lydia at the Batten conference in Nashville. This was that she was an intelligent and articulate young woman. As I looked at the emails, I found the details concerning a single mom on a search for answers. Frantic over what was taking place with her daughter, she reached out to the medical community, looking for someone that could help her daughter. At this point, little Lydia is one that has battled but in the beginning, there was so much that Alicia had yet to learn. With the following, she talked about all that she was just finding out, “I don’t understand why, when Batten parents reach out to me and tell their child’s story, it always starts out so similar to mine. How their child was so healthy and so active and happy when little. Even a few of the kids have a laugh like Lydia’s that’s so contagious. They were enjoying life and love and family and their child was and is the center of all this goodness. Then major symptoms start to show, and you are told your hopes and dreams will just remain hopes and dreams. There’s all these what-ifs and fears and nightmares, and any new possible sign or symptom of regression sends such a terrible feeling straight into your heart.” Having learned so much, she now understands more than one would want to know exists.
These days, there is greater awareness but there is much more that needs to be done. When a parent finally receives the diagnosis of Batten disease, it often comes after an exhaustive search for answers. Sometimes a person has had to deal with a number of different doctors to get answers and it takes a long time to get through the maze. Along the way, a child can be given a false diagnosis. Sometimes, there is more than one. Once the cause is determined, the news brings devastation. The parent finds out. The child that they have brought into this world has a disease for which there is no cure, and it is fatal. All of a sudden, a grieving process begins and that grief is for someone that is still with you. Soon, and even though the heartache continues, a sense of determination takes over. A love that is profound and a bond that cannot be broken will be revealed through action. This child needs you and you are determined to give your very best to help him or her battle. From a distance, what is revealed to those of us who look on, is a type of love story that is like none other. Honoring the child that battles, as well as, those who are by their side is the reason that I write. This is another opportunity to share a story of one that battles. A very special one at that, so let’s get started. Alicia Headrick had dreams and aspirations for her life as she was growing up and I am certain that being a mom was a big part of what she had dreamed of.
This would become a reality for Alicia as she gave birth to a baby of her very own. The big event would take place in the state of Tennessee and in the city of Chattanooga to be specific. Alicia’s baby would be named Lydia Rose. Baby Lydia would make her appearance into this world in the usual way, and without major complications. To this mom of a baby girl, it would be no ordinary event. Rather, it was one that would change her life forever. I am certain that she was not the only baby that was born in the city of Chattanooga on the day of August the 27th, 2011. She was however, to Alicia, the most beautiful. It would be difficult to argue that she was not. Lydia Rose had all of her fingers and toes and she was covered in brand new baby skin. She was a princess from the very first sight of her. Alicia was a mom! The bond that had begun to develop as Alicia carried her unborn daughter only intensified as Lydia was handed to her. Life’s circumstances can take us to places that we might not expect them to and as it was, Alicia would begin parenting as a single mom. Yes, there were concerns about what the future may hold but any that might exist would be overshadowed by the joy Alicia was feeling. This baby girl was beautiful and she was the object of her mamma’s affection.
There are not many things more treasured in a parent’s memories than those of their children as they are growing up. Most always, a child’s upbringing is filled with laughter and innocent fun as life’s lessons are learned. The purity of a child and their innocent ways is one of the reasons that we so enjoy having them around us. Watching all the adorable things that they do. The questions that they ask, the giggles, and innocent laughter are things that are treasured for all of time. These memories are built upon by the ones that are formed as a child grows into adulthood. All as our children go through the various stages of growth. We have all experienced the joy of having little people in our midst and having Lydia around was so enjoyed by her mom, extended family, and friends. She was loved by all who knew her and none more than her mom, Alicia. To say that she loved her daughter Lydia would be an understatement. The dreams that she held for her young daughter were without limit. There were challenges ahead for sure. Ones that any single mother may face. There was, however, also hope for a bright future together. Lydia would grow and achieve her early milestones as one would expect. Not only this but she was becoming more darling by the day. If you had to describe her in only one word that word would be “cute”. If you were to add to that a little bit, you might say that she was “total cuteness”. When she learned how to talk, the sweetest things came out of her little mouth. One example is how she would pronounce her colors. For Lilly, yellow wasn’t yellow. It was Lellow! As she continued to reach new milestones, her vocabulary increased.
Hope for the Future
My exposure to Alicia had shown me that she was an intelligent and articulate individual. She is someone that could meet the challenges that lay ahead and there is no reason that the dreams that she had for Lydia could not be realized. Whether raising Lydia as a single parent or one day married, Alicia could visualize Lydia achieving all the things that future may hold for her. There would be grammar school through high school and possibly, even college. Perhaps Lydia would be involved in sports, or Cheerleading. There might be high school dances and even senior prom night. What parent who loves their child wouldn’t be thinking ahead, at least just a little? As time would continue, however, events would take place that would be a cause for real concern with Lydia.This would begin to change everything for Alicia. So soon after the advent of Lydia's appearance into Alicia’s life, she began to notice things that concerned her. As early as one and a half years of age, Lydia started to give Alicia cause to be concerned. At as early as one and a half years of age, Alicia began to see things in her daughter that could be a problem. Little Lydia was having sleep issues. She was waking early and staying awake until very late on many occasions. Alicia said that Lydia would often wake up crying and she would be inconsolable. What could possibly be happening with a child when something like this is taking place?
Lydia was the center of her mother’s universe at this point and Alicia would, of course, be a keen observer of anything that was different about her daughter. So concerning would this kind of thing be for a young mother and the problem didn’t end with the sleep issues. She went on to say that it seemed liked Lydia’s whole body would stiffen if she was touched. This made Alicia wonder if her daughter was in pain. It’s so disheartening when trouble begins to strike a little person like Lydia. She was a darling little girl and the object of affection for so many people. What was it that was plaguing her? Alicia was also noticing things in relationship to Lydia’s speech. She could not pronounce several letters or speak in sentences with over three or four words back to back. There is a type of maternal instinct that just makes moms key observers of the circumstances surrounding their children. I am talking about a mother’s intuition. Alicia would know her own child as well as, or better than, anybody. She had some real concerns and would bring them up to the pediatrician. What she was told in response is typical. Alicia was told that this was all normal and that she should not worry about it. After all, Lydia was meeting her other milestones. Gradually her speech improved but this would not be the end of it. What would happen next would reveal that there was something taking place with little Lydia that was not normal. Alicia remembers that it was on May 22, 2015 that Lydia had her first grand mal seizure.
This would take place an hour after she was sent to daycare on the day care bus. Alicia would, of course, take Lydia to the hospital where she would be told to not be overly concerned. Alicia was told that one seizure does not mean that Lydia would have another. There are seizures, for example, that are associated with an increase in body temperature. These may only happen once, or on occasion, and they are called febrile seizures.This however, was not to be the case. Lydia would have her second grand mal seizure on June the 1st of 2015. What would it be like to experience such a thing? To watch helplessly as the seizure took its course? At this point, testing would be performed and Lydia would be given the early diagnosis of epilepsy.The drug Keppra was prescribed for her but it would prove to not be effective. Even with the maximum dosage, Lydia was having one grand mal per month. The problems would not be isolated to a monthly seizure but would include other issues that Alicia was noticing. As of August, Lydia had also developed uncontrolled movements and stuttering in her speech. At times, there was also sudden and complete muscle loss. This was all in addition to the grand mal seizures that continued through December of 2015. As if this all wasn’t bad enough, Lydia would begin to display abnormal movements with her little frame. She would also fall to the ground frequently. Alicia had no idea what was going on and neither did the Neurologists.
Alicia wondered, was this all a side effect to the medication or was she seeing Lydia having a new and different type of seizure? There had to be something more going on than simple epilepsy. You’re a mom and there is nothing more important than this life that has been entrusted to you. The desire to make things better is so strong and yet, Alicia had to feel frustrated with her inability to change Lydia’s situation. There was something going on in her daughter’s body that she did not yet know existed. Alicia would try to deal with what was in front of her. She would research the side effects of the medication that Lydia was currently taking and compared them to what she was seeing. At the time, Alicia felt that she may have the answer. Could this all have simply been the side effects of the medication? The procedure for handling seizure disorders in general is to add more and or different medications until the seizures are under control. There is not one type of medication that works for everybody. Perhaps, the seizures were part of a larger issue that had not been considered at this point. A medication named Topamax was prescribed for Lydia next. The Topamax was prescribed in capsule form and the doctor recommended that Lydia be given the medication by having it sprinkled over her food. Alicia suspected that this would be an issue and she explained the situation to the doctor. You see Lydia was having problems with things like swallowing and even speaking. In general, she was having problems with eating and to expect her to get the medication through this means was not to be expected.
In addition to these concerns was added the fact that the medication would not dissolve on food. As a result, Lydia was receiving next to none of the medication. Nothing was working to relieve her of these awful seizures and all of the other issues that Alicia was observing. According to Alicia, Lydia continued to have movements that were out of control and dangerous. She would have a body part jerking quickly every three to five minutes or she was dropping to the ground. Is there any way that we could possibly imagine what it was like for this young mother at that point in time? What about sweet Lydia? What was all of this like for her? What damage was taking place in her as a result of all the seizures. The totality of what all this was doing to Lydia was so disturbing to this bewildered mama. Alicia shared, “She was bruised, scraped, and cut up by now, but the hit her spirit took from the constant physical limitations ... her own little person minded misunderstandings of her condition and mistreatment she received by less than par local daycares is what I saw hurt her the most.” Most alarming was Lydia’s own misunderstanding as to what was happening to her. How damaging was all of this to her little spirit? Alicia continued as she explained what Lydia’s perception of what was happening to her was, “My happy child thought her seizures were people she cared about pushing her down, and she made it clear she felt this way, and that her feelings were hurt worse than the trauma her body was experiencing.” This would be heartbreaking to say the least!
Circumstances Beyond the Ordinary
I can only imagine the concern that Alicia had for her daughter at that point. This little person had become such an integral part of the family and the center of Alicia’s world. She had brought so much joy to all who knew her. Now, Lydia’s health had declined and things were out of control. No one had answers for what was going on. Alicia’s own anxiety was growing to the point that she reached out for additional help. The trauma that all of this was creating was almost more than one could bear. Alicia explained, “I pleaded for help, because Lydia was beginning to be covered in bruises and the daycare was not comfortable with what she was going through either. She was losing coordination, sleep, and the ability to even walk without dropping flat on the ground.” Again, as things continued, it just seemed that more was taking place with Lydia than could be explained with a simple diagnosis. Lydia would continue to have at least one grand mal seizure per month and had seemed to lose control of her little body. She started having potty accidents whereas she had been completely potty trained prior to the start of the seizures. Alicia and Lydia used public transportation for getting around over a two year period and having a child with these issues was making travel more difficult. This was besides the fact that you could just see the changes in Lydia. Whereas you once had a happy child with unlimited energy, Lydia was now more cautious and withdrawn.
Alicia described some of the changes in her little girl, “... though she used to walk up the bus steps and wave to the driver, and even sing her abc's on the bus, she was now at the point where she couldn't walk without holding my hand. She was too tired to sing, and was weary from falling down all the time.” It would make most any person’s heart ache to think about the impact that all of this was having on this perfectly sweet little person. Daily, the level of concern and anxiety over her daughter’s condition would impact Alicia’s own personal well-being. All during this time there continued to be trips to the doctor and the neurologist in a search for answers. It would soon be determined that what Lydia was experiencing was a combination of Myoclonic, Drop, and Absence seizures. Several different medications would be prescribed as the neurologist attempted to come up with a “cocktail” of prescriptions that would control the seizures. This, while a weary young mom hoped and prayed for some relief as her daughter’s situation grew to be more dire. Many times, as I have written these stories, I see a resolve to get the answers needed start to form. Initial contact is made and decisions are left with the treating physician. Sometimes, this initial contact will bring about a conclusion as to what is going on. However, that is not always the case. Oftentimes it will be the parent, or parents, “stepping up their game” and becoming more assertive in order to get results. Alicia had seen enough and she needed answers.
Obviously, there was more going on than just epilepsy and she had reached her limit. Alicia shared what she was feeling, as well as, the action that she took as time continued, ”I repeatedly and frantically called the neurologist until we were scheduled to go to Vanderbilt on October 22nd, 2015 for a better understanding of Lydia's sudden change in movements.” It was on that day that little Lydia was checked into Monroe’s Children’s Hospital for a two to three day video EEG. Lydia would stay in the hospital long enough for the Neurologists to determine what kind of seizure activity she was experiencing. This would also allow time needed to perform any other testing that would help them determine the root cause of all the seizures. In the course of trying to determine what was taking place with Lydia it was thought that she may have a disorder called Doose Syndrome. Doose Syndrome is also known as Myoclonic Astatic Epilepsy. This syndrome affects people in their early childhood and the disorder is characterized by difficult to control generalized seizures. The types of seizures that Lydia was experiencing, i.e., drop and absence seizures, are included in that category. Alicia described her feelings concerning that possibility, as well as, learning what was causing the abnormal movements seen in her daughter, “...my heart broke again that day when reading about Doose, and learning that her abnormal movements were due to myoclonic, drop, and absence seizures that had begun to be noticeable after her 2nd grand mal in June.”
A high percentage of individual children with Doose Syndrome will eventually become seizure free but there are still challenges along the way. The earlier that the seizures are brought under control the better the outcome will be. Failure to find a way to control the seizures can lead to impairment of learning and development. This may lead to mild to severe developmental delay. Many believe there to be a correlation between Doose Syndrome and the development of ADD or ADHD. For these reasons, there would be challenges ahead for this mom and daughter but they were ones that could be overcome. These concerns would weigh heavily on Alicia as she studied the effects of Doose. This syndrome, however, would eventually be ruled out much to Alicia’s relief, however the results of the EEG were revealing. Lydia's test showed seizure activity all over her brain. It even included nocturnal seizure activity. No wonder Lydia had problems with her sleep patterns. Alicia would describe it in such a way as to say that “her daughter’s brain was like a storm.” The drug Depakote was added and other medication adjustments were made. This helped for almost two months. The seizures were under control and the abnormal movements had stopped. This was a relief however, Alicia was concerned about the possible side effects of all the medications that Lydia was on at this point. This although she had to have been satisfied by the improvements she was seeing. The very sad part was that these improvements were short lived.
Two weeks before Christmas, the seizures would return. This time however, they were twice as bad as they were before. This is what Alicia had to say about the return of the seizures, “…she suffered another grand mal, and I watched all four seizure types return. My little girl was back on the floor after getting knocked down again by another drop seizure.” Whatever this monster was, it was relentless. Can you imagine being in the midst of the holiday season and having this type of thing happening to the princess of the family? A time that is normally reserved for being together and winding down from the events of the year was only bringing pure anxiety to Alicia. On top of everything that was happening with little Lydia, the battle with the insurance company had to be dealt with. Moving forward had been made difficult by the insurance company’s stance on further testing. Alicia said that it was declined by the insurance company for, quote, “lack of importance”. The only option that was made available was to add another medication, one that is named Onfi. Again, Alicia not only had the concern for what the seizures were doing to her daughter’s health. She was also concerned about the effects that the cocktail of pharmaceuticals was having on her daughter’s little thirty-seven pound body. This was not an acceptable situation. More needed to be done and Alicia would not stand still and do nothing. She remained persistent as an answer had to be found. She was frantic over what was taking place with Lydia and agitated over the fact that more was not being done.
Road to the Unthinkable
More testing needed to be performed and Alicia was determined to make sure that happened. Doose Syndrome had been ruled out but again, there had to be something more than simple epilepsy involved. More phone calls were made and eventually, more testing would be agreed upon. This time, genetic testing would be pursued and Lydia would have bloodwork performed on her to support this. It was hoped that a mutation in one of Lydia’s genes could be found that would reveal the cause of the seizures. Alicia would wait to receive word concerning the results of the testing but they would never come. Instead, she would receive a letter from the insurance company stating that payment for the testing had been declined. The letter used words like “experimental” and it included phrases such as “not helpful to assist in her treatment”. Wasn’t Alicia going through enough at that point? To have that come from the insurance company at that time seems so cold and indifferent. Alicia said that she was floored by the wording in the letter. How could she not be? More phone calls were made and it was determined that they would return to Nashville to have genetic testing done that the hospital’s program would pay for. The Gene-Rx test was performed, and for this they would have to wait three months to receive the results. Just as one would expect, the time in waiting would seemingly take forever. Alicia’s mama's heart continued to break for this little girl that was the center of her attention. The entire situation was crushing to her.
While waiting for the results, Lydia was regressing. She began to wear a helmet for her safety and she had to wear pull-ups due to accidents. Alicia kept Lydia close by her side for fear of her getting hurt while having a seizure. Her speech was slurred and she was stuttering. She no longer sang songs and was barely able to use a spoon to feed herself. Lydia was Alicia’s baby girl, her reason, and the purpose for waking each day. The months that it was taking to get a diagnosis had to have been so difficult. To witness the regression was more than what most any person her age would normally encounter. Alicia would spend many nights awake due to Lydia’s broken sleeping patterns. She would observe her daughter, watching her body shake and her eyes flutter. Her words concerning those occurrences are so moving to me as I try to visualize those moments in the hours of the night. Her and Lydia together. She commented on little Lydia as she would awaken, “In the mornings, her body was shaky and unstable, but she continued smiling and trying to communicate, and I kept up hope that the test would give us answers and she would eventually be okay. We went to meetings to develop an IEP, for which she did qualify for, and we tried to adjust to all the changes happening all at once, it seemed.” It’s so heartbreaking to know what the outcome would be once the news was received. However, what this mom would do out of a love that is undying would so impress me. I have been doing these stories for a few years now and I have been moved by each individual one.
Each story conveys the feelings that are experienced by the families as the diagnosis is found out. Almost all of them express what is the depth of despondency, grief, and sometimes rage that is felt by the parents and family. When you love someone as much as Lydia was loved, whatever takes place in terms of a response, must be understood. Alicia’s days, and also her nights, revolved around this little person who was so adored. Hope for an answer that would bring a resolution for what was seen in Lydia would fade. This would all happen on April the 16th of 2016. The hospital at Vanderbilt would call Alicia and explain that she would need to contact the neurologists in order to receive the test results. Was this because they did not want to assume the responsibility of explaining Lydia’s plight to her mother? A mom that would be consumed with grief? Was it that they did not have the time to do an adequate job of explaining things? Whatever the case may have been, Alicia persisted and would be given the answer right then. She would learn that it was both copies of the cln2 gene that were mutated. This meant that the TPP1 enzyme was not being produced. Alicia would take the information that was given to her and would do the research. In her own words, is what she would discover as some of the words leaped from the screen, “I researched and found Batten Disease, CLN2-Late Infantile. I read the words NO CURE, NOT ENOUGH FUNDING FOR RESEARCH, CHILDHOOD DEMENTIA and EARLY DEATH.”
All of a sudden, everything for Alicia just stopped on that day. There had been hope that the testing would bring a diagnosis for a condition that could be treated. That hope was now gone. This sweet and innocent child had a disease that had no cure and it was fatal. Everyone was crushed by this news. Her mom and dad, and all others who would soon learn of Lydia’s condition. Everything that made Alicia who she is as a person was changed forever. Along with her daughter, she had begun a journey down a course that is traveled by very few. One that, up until then, she was unaware of. Some might question the depth of despondency that was felt and the raw emotions exhibited at this time. Most all, however, will understand! She expresses what she did and what she was feeling with the following, “I quit my job. I was cracking and so broken. I isolated as much as possible. I felt forsaken by God. I cursed him while standing in my kitchen many days with my fists raised to him.” I think that God understands the anger, although he is also there to bring comfort! This story doesn’t end here. No not even close. Like the other stories, the parent is overwhelmed with the news that brings devastation and hopelessness. Things then change although the feelings of brokenness do not go away. It is just that added to that comes feelings of resolve towards helping their child battle the monster that has taken control. Alicia would begin to take immediate action. All the while, a bond that had already been stronger than most was becoming one that is like none other.
A love that is undying would be on full display as those who would look on took notice. So sweet are the memories that would be formed as those of us who would follow Lydia’s journey see them from a distance. They would, of course, be mixed with challenges that most know nothing about. It was time to take action because of love and Alicia would do just that. Most of us, as parents, would do whatever it takes for the well-being of our children. A very small percentage of us will be asked to do that which is out of the ordinary. Alicia would get right down to business, getting whatever was needed for Lydia. Taken from an email, Alicia stated what was involved at the very beginning, “Even with such a grim diagnosis we have much to do. She's being referred to have leg braces fitted, Speech, PT, OT therapy, and vision testing. She's already passed her hearing test. I requested a geneticist, and am waiting for them to fit us in.” Alicia got up and would do whatever was needed. She continued to care for Lydia after receiving the diagnoses even as the symptoms continued. The disease would bring to Lydia and her mom constant challenges but there was much more to come as their story continued to be written. Lydia would continue to face daily struggles because of Batten. She would rebound in the way that you would expect a little warrior to but the effects of Batten would be very traumatic. Lydia would go through spells of crying and yelling, sometimes at a level that was deafening and with her hands clenched.
The Will to Fight
She would go through cycles. Three or four good days and then bad days that lasted about the same amount of time. Emotions would run from high to low for both Alicia and her little girl as Lydia would sometimes be inconsolable. Even so, Lydia continued to laugh and display that smile that was just so darling. Alicia described what she was seeing in her daughter near the beginning of the battle and would talk about how it would help them to endure, “Lydia's stubbornness and free spirited nature are beautiful qualities to possess, and most importantly it'll help her to continue to fight. it'll help me continue to fight with and for her.” And fight, they would. They would fight together! It is unimaginable to think about what Lydia was experiencing on the difficult days of the battle. The disappointment of this young mom over what had happened is difficult to overlook. She had to have felt that their entire lives had been ruined by Batten disease. Her early dreams of what Lydia might have accomplished in life had been undermined. Achieving the things that she had dreamed of for her daughter would, in all likelihood, never come about. With the following, Alicia talks about what she was feeling at the time and why she would fight through the feelings, “I grieved her losses before they even happened. I grieved the loss of her before she was even gone. I had no idea what my purpose was in life other than to be a mother, Lydia's mom, and now I had no reason to live if she wasn't with me. Still, she needed me and I had to pull myself out of death's grip, to keep fighting for her until the end.”
Out of the most tragic of circumstances will often come the sweetest of details. A love that is like none will be on display for all of us to see. That is if you are willing to pay attention and take notice. Again, what is there that most people would not do for an innocent child like Lydia? And still, some parents will be asked to give more than is required by the majority of us. I’ve heard it said, “You never know how strong you are until being strong is the only choice that you have.” This is a quote fits so well the journey that Alicia would take with her daughter Lydia. Doing the best that she possibly could was about to involve leaving everything that was familiar to her. Alicia had to do whatever was needed to help this innocent life that had been entrusted to her care. Finding something that would help did not come through the recommendation of a physician but rather through a search of the internet. In her search for help with her daughter, Alicia would find out about an experimental drug trial while on the website named clinicaltrials.gov. She found a trial that involved something called Enzyme Replacement Therapy. Upon reading about it, Alicia felt that it looked to be their “last hope” for helping Lydia. All throughout this time, Lydia was continuing in her decline. Of course, Alicia would apply for the program. She would not only find out about the clinical trial but she would also learn of an organization named the Batten Disease Support and Research Association (BDSRA).
Alicia would learn that both herself and Lydia were part of a community that previously, she knew nothing about. It would be in July of 2016 that Alicia and Lydia would attend their first Batten disease family conference, put on annually by BDSRA. While at the conference, Alicia would have the opportunity to meet many of the families that had the same battle on their hands. So many were the innocent faces that had been afflicted with Batten disease. A person’s first time at these conferences can be overwhelming, however, Alicia did also have an opportunity to meet with the representatives from BioMarin. This is the name of the company that ran the clinical trial that Alicia was hoping to have Lydia placed in. The time at the conference passed in a blur and life continued on. A week after the trip to the conference Lydia had lost her ability to walk. She hadn’t eaten for a week and wasn’t moving around. The concern over Lydia’s decline became more severe with each passing day. This would lead Alicia to write an impassioned email to the representatives at BioMarin. The email was a plea for help. It seemed at the time that she did not have hope of Lydia being accepted for the next trial that was coming up. Among other things in her plea for help, she talked about Lydia and the other children at the conference.
Here is some of what was included in her email, “ I looked into these beautiful children's eyes at the conference in St. Louis and they are the most beautiful children I've ever met. Even the ones who have declined so much are so full of life and smiles. That's my Lydia, too. She was an energizer bunny. Always running, always smiling, and always socializing. She is slowly fading from me. Her adventurous, daring, and free spirit is barely noticeable these days. She's taken to sitting around when she used to run from the time her feet touched the floor in the morning. To think I used to complain about how tiring it was chasing after her. Now I don't have to chase her, and I'm even more tired. I am mentally and physically exhausted from watching her suffer.” That was just a small portion of what I read. This impassioned mom said that she felt out of her mind as she was writing and had written to BioMarin out of anger. In response she received an opportunity to conference over the phone with a couple of the reps from the company. Alicia would find that the response that she received was one of understanding. She would make it clear to me that the response was one that was both compassionate and sweet. How very wonderful that must have been? A week before Lydia’s 5th Birthday, Alicia received a call stating that the extended trial was open to five children who had CLN2 Batten disease. BioMarin requested that Alicia bring Lydia up to Ohio so that she could be seen by staff members.
Soon after, on August the 29th, they were in Ohio getting an evaluation performed to see if Lydia qualified for the trial. Alicia would find out that day that Lydia did qualify. So it was a week later that a shunt would be surgically implanted in Lydia’s head. Two weeks later, in September of 2016, Lydia received what would be the first of many infusions. One is given every two weeks. During these infusions, Lydia would receive a solution through the shunt that would replace the enzyme that her body was missing. The commitment for each of the families in this trial is a heavy one but one that is gladly accepted. While not a cure for CLN2 Batten, it does offer a greater quality of life for the children that take part in it. All of a sudden, things had changed for Alicia and Lydia. Alicia knew what they were facing and there was hope and help for Lydia. Most often, knowing is better than trying to cope with the unknown. This mom could now catch her breath for just a little bit and further evaluate the situation that she now had knowledge about. There would be much to come but for now, Alicia felt that to a certain degree, they had been fortunate. Here is a little of what she had to say concerning that time, “Looking back now, and after hearing other rare disease stories from amazing parents, I learned that we had/have more to be thankful for that I didn't even notice!” Alicia was thankful that Lydia’s diagnoses came relatively soon compared to others. Her princess had been granted access to a clinical trial that is available only to kids that battle CLN2 Batten.
A Bond Like No Other
Along the way Alicia would meet some very wonderful people but would also continue to face some real struggles. It is always a very good thing when a child that battles CLN2 receives Enzyme Replacement. The more time that is spent getting a diagnosis and gaining entrance into the program ... the more time the disease has to progress. Alicia’s assertiveness and her desperation would have a profound and positive impact on Lydia’s health. Lydia had lost some valuable time as her mother searched for answers, but the answer would arrive more quickly than in countless other rare disease cases. This would grant Alicia and Lydia so many more quality days together. A bond that was already very strong would become one that is like none other. A sacrificial type of love would ensue and it would be on full display for those of us that would take notice. It has been some time now since Lydia first began her battle and so much has happened. The following is some of what Alicia had to say right after Lydia began her treatments, “Batten disease is an absolute living nightmare. It is hell, and it doesn't discriminate against anyone from anywhere. The only good thing about Batten is the people we have met in the same situation and have become a long distance family to. This includes the wonderful therapists who actually have passion for their career, and the compassionate Batten's disease specialist and her proactive staff in Ohio. The uplifting support of the Batten Disease Support and Research Association, also in Ohio. They have made their presence known from the very beginning, and it is such a light in our darkness.”
Alicia had also referred to some of the people that they have gone on to meet as lifelines. That is in fact, literally true and all of these people would continue their assistance for sometime to come! All of them would become vital to Alicia as Lydia continued on enzyme replacement. This would require the two of them to relocate from their home in Tennessee to the state of Ohio. Over time, and in writing many stories about families that battle Batten alongside their children …. I have seen that there are not many things that parents will not do for the well-being of their warriors. That includes picking up and leaving the home that they are familiar with. They will cross oceans and move to a different country if there is no other option available. Whatever it takes, there is nothing more important than this little life (their child) that they have been entrusted with. While Alicia would not have to cross an ocean in order to get help for Lydia, there would be many difficulties along the way. Think about it. A single mom with limited means, needing a place for shelter and resources to sustain herself and Lydia. She would move to a place that she was not familiar with. Finding an affordable dwelling place in a safe neighborhood would be a challenge. Maintaining a vehicle that is safe to travel in to all of the appointments and to Lydia’s infusions. Car repairs can be so expensive, let alone basic maintenance cost. At a time when most young women would be shaping their careers or finding true love, Alicia had other things to be concerned with. Her priorities surrounded the health and welfare of her daughter.
While Alicia would say that anybody would have done what she had, I can’t help but to be completely impressed by her actions. Separated by the miles from family and in large part alone with her daughter. Yes, they would make friends along the way. There would be all of those people that would become lifelines but most of the hard work would have to be done by Alicia. This mom would do anything to help improve her daughter’s quality of life. Anything to relieve her of the symptoms and progression of the disease. Being a single mom is difficult enough in any day and age. To try to live on your own and maintain a home environment for your child while providing for their needs is a most difficult thing all by itself. What if on top of that, your child has a rare disease? What if the only way to get that child the treatment that they need requires you to pick up and move away from your family. You have to be driven by love and a sense of urgency. I know that I have a habit of repeating myself to an excessive degree. Here I go again. There is no love like a mother’s love and there is no love like that which exists between a mother and her daughter.
Alicia and Lydia would continue on a journey together and it would take them from the home that they were familiar with. The life that they would now lead would be so unlike what Alicia had expected. Once expecting to see all that she had hoped for Lydia, her circumstances were now unlike what most would experience. She was the mother of a child that had a rare disease and that would bring with it a series of rare circumstances. Enzyme Replacement Therapy would not take place easily for Lydia and as a result, it would also be extremely taxing on Alicia. What was meant to provide an increase in the quality of life for Lydia would also bring challenges to this warrior princess. It has now been a little over four years since Lydia started ERT and much has happened. Alicia described to me the setbacks that were associated with Lydia’s treatments and also how it has affected her as a person. Traumatic is a word that comes to mind and the trauma would affect both mom and daughter. The following quote is purposely included as I begin the remainder of this story. Here is what Alicia stated, “... there are times when I stood in front of the washing machine to watch and hear the water fill up, but my waterfall meditations were short lived because, I believe, the flashbacks are opportunistic. If I tried to escape they would find me, and I'd have to check on her by texting her teacher.” The type of love that we have discussed previously was and still is present in great measure when talking about this mother and daughter. A love that is undying.
There would be special times spent together for sure, however they would be mixed with struggles beyond compare. I cannot get out of the recesses of my mind, the pictures of these two people in their dwelling place. A mom alone with her daughter, a daughter surrounded by the abundance of stuffed Minnie Mouse figurines. Those given to her by people who love and care about this little person. Everything that is done by this young mom is done in support of her only child. Even in caring for herself, she is doing so in order to make it to the next day. This again, in order to care for her daughter. I don’t mean to be overly dramatic. I only want to paint a picture using words. Every two weeks, Alicia and Lydia would return to the hospital for the next treatment. Of course, in between that time would be a mixture of doctor appointments and meetings with those who were essential to Lydia’s cause. As I have stated, the treatments for Lydia would not be easy on her. In fact, they created anxiety within her. Alicia would tell me that poor Lydia would begin to cry as soon as she recognized the hospital. Lydia’s anxiety would become Alicia’s anxiety as well. A mother that so loves her daughter. Even times when there were no needles involved, like check-ups, Lydia would become hysterical. Lydia’s high pitched crying has now been suppressed by her lack of strength but they are still a part of the flashbacks that Alicia experiences. In addition to the sight of needles, a big portion of Lydia’s anxiety would come from the nausea that would take place as a result of the infusions.
The Struggle for Lydia
Lydia would be prescribed one sedative or another to help with the anxiety. Each trip home from the hospital was difficult because Lydia would be prone to losing the contents of her stomach (I don’t know of a better way to state it). How difficult would that be to drive the distance with that going on in the back seat? How do you drive and tend to your daughter at the same time? It was the most difficult at the beginning. Alicia would describe the first couple of years in detail. Here is some of what she shared, “For a couple years straight she would get so sick during treatment. She was very nauseated, and suffered from headaches and sensitivity to light. She also ran high fevers that could last from 2 to 4 hours, even after receiving meds. She would sometimes have leg tremors, and would often need rest until the next day.” Lydia would become flush near the end of each infusion and she would vomit each time. This would happen multiple times with each treatment. The feelings of helplessness for Alicia had to be present at times but the will to move forward was stronger. Alicia would eventually purchase a special head restraint to keep Lydia from experiencing aspiration during the trip home. She talked about the sadness over the effect that this was having on Lydia, “I sometimes called my dad crying on the way home. I was so sad for her, and felt awful that I couldn't sit with her and drive at the same time. We had tried several meds for her adverse reaction, but to no avail.” The doctors would finally come up with an anti-nausea medication that was delivered in a different manner to Lydia and this would help tremendously. The solution to the problem would not arrive right away though.
As we first alluded to, Lydia would begin treatment later for CLN2 than would be desirable. This allowed for the disease to progress before a diagnosis was determined. Some of the difficulty she has experienced is related to this fact. She just wasn’t as strong physically as one that receives a quick diagnosis and a faster start with treatment. This, however, has never kept her from being a beautiful warrior princess and one that is able to melt a person’s heart with a single glance. She has always had that awe-factor going for her little self! Lydia doesn’t get nearly as sick as she once did but she also does not bounce back from the treatments as quickly. Whereas she used to attend school the next day, she now needs more rest after the treatments. Of course, Covid-19 has affected Lydia’s ability to attend school this year, just like with most others. She still sometimes has a fever after the treatments and needs rest and recovery afterwards. She has to wait for a while to eat as well. Lydia began treatments at five years of age and she has been a real warrior. She is now nine years old and as one might expect, her overall health has declined. This has, no doubt, been very difficult on Alicia but she has been a warrior of a mom as well. However it may be that Alicia views things, it has to be felt by all who know her that she has been one strong person. Really, she has had no other choice but to be that. With this, Alicia shares a little more about the journey, “I'm thankful we had a piece of hope, an opportunity, even if it’s temporary. I'm sad it has been such a traumatic journey for her. I will always hope for a cure.” As will we Alicia!
There have been other health issues along the way for Lydia. The type that you might expect for a child that battles Batten disease. As well, there have been some difficult decisions. One was associated with the right timing for when to have a feeding tube inserted. Having it inplaced surgically turned out to be a tremendous help in Lydia’s care. Overall, she has been one strong little girl and with as difficult as things have been, she has always bounced back to the point that she is able to. As September of this year rolled around, Lydia would achieve an important milestone for kids on enzyme replacement. That happened as she reached 100 infusions. This has afforded her and her mom many days together. There will always be a big measure of respect from me for those who have suffered loss and have come out on the other side of things. The rest of life doesn’t stand still for Batten disease and it hasn’t for Alicia. As if she hasn’t been through enough with Lydia. She, along with her family, would suffer the loss of a cousin and her own brother. That in itself, has brought so much heartache but she has pressed on with life. She has done so because Lydia’s needs come before anything else. There have been so many sweet exchanges between these two as they have battled Batten together. A mother and her young daughter, alone much of the time but not unknown about. There are so many of us who have seen the sweet details either up close or from a distance. And yet, there have been many hardships.
In relationship to those sweet details, I knew that they had taken place but I wanted to ask Alicia about them. I think that the two of them have been through so much that it is often difficult to reflect on the intimate exchanges that have taken place. Alicia said as much as she responded, “…. I want to tell you all about sweet moments and fun we've had, but Greg, we've just tried to survive moment by moment.” Think about the types of activities that a young mom and daughter would be involved with. Soccer games or dance practice. Time spent at the park, in the sand or at the mall together. Batten however, has robbed them of most things. Alicia shared that as the symptoms of the disease have progressed, they have kept them home bound most of the time. Alicia is a realist and she knows that this battle will one day be over. Holding Lydia in her arms brings her comfort and a measure of peace but this journey won’t last forever. Just as I had thought, there have been many special moments spent together. Here is more of what Alicia shared with me, “She and I have had many, but never enough, snuggles and laughs. That's how we've coped. Those are sweet times always, but they're fading from her ability now.” Alicia struggles with this too and who could not understand that? The journey that is Batten disease is a most difficult one and making memories that will last can help carry a person through to another day. This experience will always be a part of who Alicia is as a person.
Lydia is the center of Alicia’s world. She said as much in this quote that was taken from Lydia's Facebook page, “She's the sun, the center of my universe, and as long as she still shines I'll know where I belong. Right next to her.” When I talk about a type of love that is profound and undying ... does Alicia and Lydia’s story not exemplify what I have been talking about? In that same post, Alicia shares some of the sentiment that has come out of the turmoil that the two of them have faced together, “I remember discovering how well big sunglasses hide tears. I remember her pain. I miss every word she's lost. I miss her voice. Yes, she's worth it. I'd give up everything just to have her pieces put back together. I hope she feels my love, and I hope it's enough.” Alicia, she feels it and she has experienced it through your actions. And yes, she has been so worth it! Just as one would expect, it was love at first sight for Alicia as she gazed upon her newborn daughter. She was a gift that was without, and beyond, price. The love has gone both ways. It could be seen in Lydia’s eyes even when she could no longer say it with words. This little girl loves her mommy. Alicia has been so brave and a hero to her daughter. She had no choice in this but Alicia has remained willingly because of her love for Lydia. The emotions that run through Alicia are so complex. Here, she explains a little more, “She makes my heart shatter daily, but she's also the reason it's still beating. I would never suffer so much for anyone else. She's my withering Rose, and I'm her protective glass case surrounding her. I only want to nurture her, save her, and watch her grow. But, unfortunately, the curse is within us, and her petals still fall.”
Loving Lydia - Life in the Now
Alicia continues, and keeps things real as she does so, “We are two peas in a pod, she and I. Life has battered us, but we're here. Sometimes I'm not sure why, how, or for what reason. Sometimes smiling is too much, but we are here, for now.” Along the way, there have been some sweet moments that will forever stay with Alicia. Many include the friendships that have been made. Many in the Batten community are like family and time spent at Batten conferences together brings with it memories that will never be forgotten. It was at these conferences that this mom and daughter experienced many firsts. Memorable are the times that the other parents would look upon Lydia with love. They had warm smiles on their faces and had tears in their eyes. They had experienced things very similar and these parents knew what the two of them were facing together. Alicia shared one thing in particular that will always stay with her and that is when a Batten daddy asked if Lydia could dance with him. Alicia speaks about it here in her own words, “A bereaved Batten dad asking me for a chance to dance with Lydia at the Father/Daughter dance. My heart felt complete joy to see such a sight. Lydia was bashful, but allowed him to carry her away from me for just one song. It's a moment I will always remember. Some may have prom to look forward to, but I had the privilege to see Lydia being held within the caring arms of a dad, and even more special because he's a Batten dad.” Having been to two conferences myself, I can testify to the fact that they are very special events.
While I have focused on the relationship between Alicia and Lydia as I write, it needs to be said that they are both loved by many, both friends and family. Loved by her parents, Alicia is especially close to her father who has tried to help as much as is possible. In the beginning, he drove from Tennessee to Ohio for Lydia’s first port placement. While there, he carried Lydia to the room she would be in as she was being readied for surgery. This year, Alicia would have to carry her daughter by herself as she underwent surgery to receive a new port for continuing enzyme replacement. And still, that time with her dad will always be remembered. There are so many other experiences that will be a part of who Alicia Headrick is as a person as she presses on in life. Ally, as she is called by her friends, is someone who always will have left an impression on me and she is someone that I have a great deal of respect for. Some who have fought in battle may take exception to using terms of war as people try to relate them to the battle that is Batten disease. After all, there are not many things more terrifying than serving on the battlefield. I do believe that there are many veterans who will not mind the comparison once they know what these families will go through as they battle alongside their children. Especially, this mom who has been through so much. So many return from combat having been changed by what was experienced. PTSD can impact a person’s life negatively for all time. However, many will grow out of what was faced and will live a life of purpose as they conquer and overcome the challenges that were endured.
War can be so chaotic and a person can only react using the training that they might receive. A lot of the time, a person will experience things that their training doesn’t cover. There is no training for what Ally has been through with darling Lydia! She gives us some added detail as to what life has been like for her with this, “I can't decompress if I'm on caregiving duty 24/7 for 6 years. I've been stuck in panic mode this entire time, and I have no doubt she has felt my stress. Survival mode isn’t sweet or pretty, but Lydia puts up with me.” I know, without a doubt that Lydia has felt and knows of the love that her mom has for her. She has been the recipient of a type of love that is profound and undying. Although the sweet details are sometimes missed in the middle of the chaos, they are there. That is, even if they are only seen by those of us that look on from a distance. They are there! Those details have been there throughout this journey which continues. Ally has struggled along the way but she still stands tall, at least in my estimation of things. Again, there have been so many friends and people that have come alongside and their contributions have been so important. Alicia, again, gives us some added insight, “I've begged for help, but have instead received the wisdom of those who are my friends and also have received that which resulted in physical support in the form of professionals. My mental health case manager has been here through this seemingly hopeless situation, and hasn't given up on me yet. She's a tough lady.” What if one day, Alicia is the one that is counseling others? That taking place because she has come through this experience?
Like every protective mother might, Ally sometimes wishes that she was the one that was experiencing the symptoms of Batten instead of her young daughter. She has done everything possible to help Lydia along the way. So much has been given up for the cause that is her daughter’s life but there are no regrets over the actions that she has taken. As Alicia said concerning her little warrior, “She is everything that's good, and was born into a life, filled with bad.” Lydia is just a precious little girl who has fought so hard and her life has impacted the lives of countless people. She has what is the attractive features of all of the ladies in the Headrick family and her little smile lights up any room that she is in. Her laughter will always be a part of the memories that have been formed. She is the model of the resilient little warrior that we so often talk about. Lydia has come through so much, always bouncing back to the degree that this dreaded disease will allow her to. At her very core, she is a beautiful little girl and one whose journey we have followed for sometime now. I have a son that I love who has special needs. There are certain things that I can understand but as things relate to children with rare and fatal diseases, I can only do my best to understand. I have something that crossed my mind. Something that people may disagree with or which may even cause them to become angry. It is thinking along these lines …. So if these kids have a profound impact on the lives of others. If they change people and their impact lasts long after the time that they leave. Can it be felt that in that way, they stay with us forever? Is that a fair question? It’s a bond that can never be broken, nor would you want it to be.
This is a real story and I can’t help but to keep things real as Lydia has been on her journey for some time now. In the back of every parent’s mind, when they have a rare child, are concerns about what the future may hold. This is another reason why this writer’s level of respect for all of you as parents, is off the chart. There are many stories like this one, even if many of the details are different. One of those is the story of a grandmum in the United Kingdom that was trained in palliative care nursing. She had a grandson that was born to a lovely daughter of hers and the connection between this infant grandson and nurse was instantaneous and so very strong. As it turned out, her grandson had the same variant of Batten disease that Lydia has, only it was at a time in which enzyme replacement was not yet available. How amazing was it that she was trained in the exact type of nursing that this warrior would need as he battled CLN2 Batten disease. The grandmum would leave her career to care for her grandson and she would become his advocate and full-time caregiver. The story is one of the most beautiful that I have ever heard of. The two of them would almost literally, and in a real sense, cling to one another for as long as he battled. That is until this charming little man left for heaven. With a void that could not be filled, this grandmum returned to caring for others as she picked back up her career in nursing. I have always wondered. What was it like to be on the receiving end of her care after she returned to practicing medicine? What was it like for her on that first day back?
So many questions. What will Lydia’s impact be on the life of her mom? What will there be that Alicia can’t handle after going through these challenging times? What would she not be able to accomplish? This, given the measure of strength she has shown in the middle of things experienced? What is there that would be too hard for her? Who are the people whose lives will be impacted by a mom who loved her daughter so fiercely? There is so much potential for Alicia's life as she continues and yes, Lydia is so worth this struggle. It is my hope and prayer that the number of days that Alicia and Lydia experience together are a great many. We so need a cure! Thanks for taking the time to read their story. Sincerely,
Blogger and Advocate
Highlighting a previous blog that might interest you:
Journey Unexpected - The Rich Family
One child in a family with a rare disease is a challenge. What if there is two? Heartbreak multiplied you pick up the pieces and will yourself to do your very best. A love that is profound and a bond that is stronger than any other is formed. Two little beauties in one family is two, too many but you seek to make a difference! A journey unexpected...