“There were no awkward stares or whispers. Everyone went about their business around us. As I was kneeling beside Kristiina and comforting her, one of the mother’s came over and squeezed my shoulder, asking if there was anything she could do or help with. I soon came to know that wonderful, sweet woman as Laura Betts. For the first time in forever, this felt ok! I didn’t feel like the freak at the circus everyone stares at. We were totally overwhelmed by the warmth and acceptance of everyone around us. It changed our perspective of our situation for the better, forever! And finally, we didn’t feel like we had to do this alone anymore.”
While attending her first
I may sound like a broken record to some. This, because I tend to repeat myself many times over from one story to the next. I do this for a reason. It is because I want to place emphasis on the things that I feel are important to mention. Some people that read these stories, do so on a regular basis. Some even wait for the next one to be written. There are others that scan each story and look for the highlights, while others read them in their entirety. Perhaps some look on to check out my work. This, to see if my comments are based on fact. Others, just know about the blog’s existence and are happy to know that I care. I do care greatly! I am happy to have people viewing the blog for whatever the reason they have chosen to do so. Greater awareness was never my primary reason for doing what I do although It has helped with that. My purpose has mainly been to honor those who battle Batten Disease, as well as, to share the struggle that is associated with it.
I am at the point where doing this has become a part of who I am. The work that I do has never been for profit although I have gained much from writing these stories. These kids have taught me that I love to write. (Whether or not I am well written is up for the readers to decide). I started writing about these priceless warriors as the result of a chain of events in my own life. It is so easy to create dramatic occurrences through the written word and I don’t want to do that here. Simply put, I became aware and one thing led to another. The events that followed changed my way of thinking. Anybody that knows me, knows how much I love my son who has special needs. As I write this, I am leaving in a couple of hours to go out on a work trip for several days. I hate leaving my guy behind, as well as his mom, but I do it because I sometimes have to. Just knowing he is in the next room gives me comfort. What would it be like if I no longer had him there? Our time together is always priceless. I have no idea as to which one of us will make it to the finish line first because his outcome in life is unknown. What if I did know though? What if a doctor had sat down in front of me and had given me the news? The news that my child has a terminal disease for which there is no cure? That is what a family faces when they are told that their child has Batten Disease.
The climax of their story brings with it the devastating news that they will one day have to say goodbye. How can it be that there is no cure? Change is taking place but it seems to be happening at a snail's pace. Each of these stories contains the dramatic outcome of a search for answers and I include these details on purpose. The initial shock and grief that is so understandable eventually gives way to determination. This, to do one's very best for their child in helping them to battle Batten Disease. I am always amazed by the details of undying love and sacrifice. These families are made up of ordinary people that are asked to do something that is extraordinary. All of a sudden, these families find themselves being part of a community that was previously unknown to them. While it is unimaginable for most of us to go through such an experience, it is understood by those who make up the Batten community. Once the community is discovered, these families become part of a larger group that understands what each are going through. All of a sudden, they are no longer alone and in a state of confusion. The heartache is still there but they are no longer alone. That is what my friend Monica found out at the time that she attended her first Batten conference. I was allowed to attend this event for the first time this year and it was an experience that I will never forget. These kids are absolutely priceless and I am amazed by the stories that these families create by their caring acts of love and affection.
This next story, like the others, is about a family. It is a story about a community that they would discover after a search for answers. What once seemed like a problem that could be solved would actually become a journey that they would take together. Out of these circumstances, a princess of a rare kind would become the center of their attention and her life would change theirs as could no one else's. Monica Perreault’s life would take a course that she never would have thought about. She would experience events in life that one could never imagine. We all experience things in life that are good and bad. Things that are difficult and that bring challenges that are not expected. There are things in life that test us and that require courage. Events in life change us and they make us who we are. Sometimes, other people benefit from the changes that are made in us. Those changes may be affected by a single person and very often, they are. Things usually begin as two people meet and that would be the case for Monica as she met her future partner Robert. They would meet in 1992 through a mutual friend and they began to date. This would take place in Scarborough, Ontario which is in Canada. This is the place where Rob and Monica grew up and it is the place that this story begins.
They were young and It didn’t take long for Rob and Monica to fall in love. What almost always follows is the desire to have a family together. Such was the case for this young couple and their heart’s desire came in the form of a baby boy. Their first child, which they named Matthew, was born in October of 1993. They had a baby boy and they were so excited as they welcomed him into the world. This young couple’s life together had started to take shape with the addition of little Matthew into their home. There would be challenges ahead but they felt that they were ready to handle all that life would bring their way. Rob and Monica had recently moved a few cities away from family and had tried to make a go of it. They found, though, that they missed being close by. That is when they made the decision to move back home. They went back to Scarborough and moved in with Rob’s grandmother who they lovingly referred to as Nanny Cassels. It would not be long after that when Monica received some exciting news. She was pregnant and the best part was that she was going to have a daughter. How exciting this would be for any young mom. Their little family would be complemented by the addition of a little girl, and they had the perfect name in mind.
The time came for the family to welcome their next little bundle into the world. Baby Matthew had been born by C-section but it was time to give a normal delivery a try. Monica would endure five hours of trial labour when it had become too much for her to bare. With a sense of urgency she would tell the the doctors, “I don’t want to do this any longer! I can’t wait another minute! Get her out!” Monica stated that the delivery was a little bit rough as she had a reaction to the small amount of morphine that was in the epidurals. As is the case when you deliver a baby, it is always a labour of love, and the the birth of Kristiina Lynn Cassels was no exception. She was born on the day of May the 15th in 1996, and she came into this world as pure perfection. Kristiina was born at Ajax-Pickering Hospital in Ajax, Ontario Canada. She was delivered, as her big brother had been, by Cesarean section. That means that she was perfectly shaped, and oh, so beautiful. She had all of her fingers and toes and she was covered in brand new baby skin. She felt small in Monica’s arms at eight pounds, 11 ounces. This, being almost three pounds lighter than brother Matthew. Monica had to have been beaming with Joy as she held her baby girl for the first time. Rob and Monica felt that they had the perfect size family now, and they had a boy and a girl! They must have had thoughts and feelings about their future life together with their two beautiful children.
Kristiina’s name, which uses the letter i three times, was not a misspelling as the spelling had reason behind it. Monica reflected concerning her daughters name, “We had chosen to spell her name differently than most to reflect her Finnish background on my side of the family. Her name was perfect and unique, just like her!” Kristiina was unique from the time of her birth and her life story would remain that. She was perfect in appearance at birth and she is, to this day, uniquely beautiful. Monica had no idea, at the time, as to how this little person would impact her life but that impact is being formed continuously. Monica stated that Kristiina was a much different child than her brother Matthew was. She had, in example, a problem with Colic. Kristiina would cry a lot and she was hard to get settled down. Monica joked that she had learned the fact that the sound of a vacuum cleaner calmed Kristiina. This would quiet her down and she described what she did as a result, “Needless to say, I vacuumed A LOT! LOL!” As Monica’s little princess continued to grow, she graced the household with her presence. She was reaching all of her milestones within the normal timeframe and she was developing just like any other normal child would. Monica told me what she had seen in her daughter and it would be wrong to not let her tell you what that was. This is what she said, “She was bright, delightful, and had an immense amount of energy! She had a giggle that touched my heart each time I heard it. Her eyes were so blue that I used to drown looking into them. She was my princess!”
Life at this house continued to move on in the normal fashion. Rob would continue to work full time in order to provide for the family while Monica looked after the children. Things were working out for them as they continued to grow as a family. They would move into their own semi-detached home in September of 1999, to a place name Brampton, Ontario. The couple would ask Monica’s parents to move in with them. This, in order to make things more affordable and to receive help with raising their children. Life was overall quite good for this young family and Kristiina was on target in her early childhood development. It was, however, in her third to early fourth grade year that she started to develop problems with reading. She was also having trouble pronouncing certain words. Monica stated that one example is the word binder. Kristiina would instead pronounce the word as blinder. They didn’t panic over this as Matthew had similar struggles early on. Monica said that things would straighten out in Matthew and that it would happen as if “someone turned on the switch”. Monica thought that Kristiina had inherited the issues with pronunciation from her. This was because she herself had spent many years in speech therapy. Their little darling would continue her education in regular school through an education plan that would accommodate her needs more fully.
We are all changed by the circumstances that surround us. Things that are good, and also those things that are life’s struggles, can change our situation as they impact our future. The same would be true for Monica and her family. It would happen as they would experience the loss of her father in November of 2003. This would shake up anyone and I am sure that the entire family must have been greatly affected by the loss of Matthew and Kristiina’s grandfather. In fact, Monica’s mother and sister would move from the family’s home by the following July. This, because the memories of what had happened became too much to bare for her mom. This would, of course, require adjustment, and that is something that happens for most of us from time to time. Life would continue for a while but then an event would take place that would be a sign of what was to come for this family. It happened as Monica was returning home from working the night shift. She had since taken on a job of her own in order to help out with the family budget. It was December the 6th of 2007, as Monica returned home, that she did so to the sight of emergency vehicles in the front of her home. Monica describes the scene as she returned home, “I came home to police cars, fire trucks and an ambulance in front of our house. I remember time standing still as I got closer to the house and panic rose in my throat. Our next door neighbor met me getting out of the car. It was then, my worst fears came true. They were all there because of Kristiina!”
The image that this leaves in my mind is so moving as I think about the love relationship that exists between Monica and Kristiina. According to Monica, Kristiina had fallen and had gone into a seizure that morning before she had returned home from work. This, as she looked out the front window, waiting for Monica to return. Testing was run on Kristiina after she was rushed to the hospital for follow-up. All of the results came back normal and she was released to return home with her family. It was, however, only days later that Kristiina would have another seizure. Further testing was done, but again, it came back without any findings. Monica said that as the third seizure occurred, they demanded that Kristiina be seen at the Sick Kids Hospital in Toronto, Ontario, Canada.
Yet again, the testing came back showing everything to be normal. Kristiina had an aunt on her father’s side of the family that had a seizure disorder. The doctors felt that the two conditions must be related but still, this left the family feeling helpless and confused. This was their princess. She was so much the center of their world. How upsetting this must have been! The only positive that came out of this was that Kristiina was referred to a pediatric neurologist. Kristiina would see a neurologist at Trillium Valley Hospital in Mississauga, Ontario, Canada. Dr. Conrad Yim turned out to be extremely qualified in handling Kristiina’s situation as he was able to get the seizures under control quickly. She would, however, continue to have breakthrough seizures even while on medication. Monica described how all of this left them feeling as they witnessed the seizures, “They were hard to watch her endure and left us all exhausted. They were so unpredictable and as a result Kristiina suffered many falls and bruises.”
Life for a family often becomes more complicated in general as the years pass by. Throw into the mix of events a child that is suffering from recurring seizures while not having an answer for why it is happening. Well, that can create some real stress in a relationship. All of a sudden, you have this one person that requires so much attention and as a result, other relationships may suffer. Whether or not that was the complete story in Rob and Monica’s case, it was definitely a huge factor. It was April in 2008 and by that time the relationship between Rob and Monica had broken down. According to her, she and Rob had become much different people. It is sad when this type of thing happens but the reality is that it does. This part of Monica’s story, though, would eventually have a very happy ending. In the course of time Monica would meet someone that would be the perfect match for her. That being her future husband Adam Bearman. The relationship started at work in friendship,, but as Monica learned more about Adam her feelings for him would deepen. Monica was struggling with her situation as far as Kristiina was concerned. Having a child with special needs can be so difficult and it often takes the right type of person to deal with the circumstances. Adam was that type of person and as time went on he would show Monica much about her situation. You see Adam had been around people with disabilities for a good portion of his life. In fact, he had even played wheelchair basketball for almost four years. Monica stated that Adam was almost more comfortable around Kristiina’s seizures and special needs than she was. People always come into our lives for a reason and such was the case with Adam coming into Monica’s life. Instead of complicating the situation, he would make Monica’s life easier. There is more that could be said here but in concluding what Monica had to say about Adam, she stated that his own experience was responsible for helping to “change her way of thinking.” Monica would elaborate concerning Adam’s help as she told me the following, “He (Adam) told me ‘the only limitations Kristiina has, are the ones you place on her’. It changed things for me and in me. I looked at what was going on with her differently. I stopped wrapping her in bubble wrap (figuratively speaking of course!) and I let her do the things she wanted to. He helped me to let go and stop molly-cuddling her and it allowed her to become independent and grow.”
Adam and Monica would continue to face the circumstances surrounding Kristiina’s situation together. The puzzle pieces that were an actual diagnosis for Kristiina were yet to be put into place. Adam showed great leadership, unconditional love, and support during the time that it took to find an answer. He was by the families side, and they would not be alone in the struggle.
Monica would make the decision to move with Kristiina to Burlington, Ontario Canada. This because she felt that the school system there had opportunities that were better suited for Kristiina’s needs. In fact, Kristiina loved going to school in Burlington. She made new friends and she had fit in well in her new surroundings. As 2009 rolled around, Kristiina was also doing well when it came to seizure activity. Monica said that at that time seizures were few and far between. They were checking in with the neurologist every six months and her EEGs were showing that no seizure activity was present. Monica stated that Kristiina was so healthy that she was getting sick less than anyone else. She was helping with the dishes and she was so happy. In fact, the whole family was happy and Adam and Monica were happy together! Things would change as Kristiina turned fourteen years old, and as she started high school. Stress and overstimulation would prove to be triggers for seizures in Kristiina. According to Monica the stress of high school started taking its toll on Kristiina. She started having seizures every other month or so. This number was a lot for her. Monica said that the grand mall seizures were especially hard on her daughter as they left her exhausted for as much as 24-hours. She would go on to tell me that it was at about this time that Kristiina was showing signs of regression. The regression would be followed, after a while, by a slight improvement in the affected area. It was obvious that Kristiina’s situation involved more than just a simple seizure disorder, but what was it? A possible diagnosis was mentioned several times but none of them could be proven to be correct. Hereditary Seizure Disorder and Globally Developmentally Delayed were two terms that were brought up as possibilities. Monica said that the possibility of crushed blood vessels in the left frontal lobe and oxygen deprivation were used many times as reasons for Kristiina’s delays. How frustrating it had to have been to not know the answer for what was happening and yet, to have to watch these thing take place. It is very possible that feelings of isolation may have set in as well. This because of misunderstanding by those that are not affected.
Adam had brought a daughter from a previous marriage to the relationship and her name is Sammantha.Together, Kristiina and Sammantha would become big sisters to another beautiful baby girl. Her parents would name her Khloe Lynn Bearman and she was born in April of 2012. Monica stated that the bond between Kristiina and little Khloe is indescribable. She went on to say that it is beautiful to behold. I get the feeling that this entire blended family was extremely close to one another from the beginning. It appears that the difficulties that have come with Kristiina’s condition have only served to make them closer to one another. That is the way that it should be!
It was early December of 2007 that Kristiina had her first seizure and much had happened since then as 2014 rolled around. This is when the neurologist was prompted to have further testing done on Kristiina. He had blood work and metabolic testing done, but Monica said that all of this revealed nothing out of the ordinary. He would continue to have EEGs performed, and this time, the results came back abnormal. This would cause the doctor to dig deeper. He wanted to have genetic testing performed. The search for an answer as to what was taking place with Kristiina was starting to narrow, and soon, the family would know. The puzzle pieces would soon be put together. This, much to the family’s dismay. Monica stated that a call to Toronto Western Genetics Lab was made, but they were told that there was a years wait to get tested, How could that be? She would go on to say that there was no way that she would wait that long. I wouldn’t have either! Monica did an online search for another lab, and she found one named Hamilton's McMaster Genetics Lab. She asked how long the wait for an appointment was in an urgent situation. The lab contacted Kristiina’s neurologist to verify her information, and still, it took four months before blood samples were taken. This, at least, was better than waiting a year. It was in April of 2015 that samples were submitted at McMaster Hospital in Hamilton, Ontario Canada. It is hard to believe what would follow and the time that this family would have to wait to get an answer. Life always moves on whether or not we want it to and to remain patient is so difficult. This, when you are facing a challenge such as this family was at that time with Kristiina. Adam and Monica were informed that it would take, in all likelihood, until December to get results from the test. This was because it would take four to six months to get government approval for the genetic testing to get paid for. Oh my! Yes, Life would have to move on as they waited.
Something really exciting would take place as they did wait. Adam and Monica would become man and wife! Adam had been there unconditionally and he would continue to be the rock of the family. It was in December of 2015 that the couple would tie the knot officially. They did things all fancy like, of course, and princess Kristiina would have her place in the ceremony. She would be one of the bridesmaids and would be dressed like the princess that she was. Monica said that Kristiina beamed proudly while wearing her red dress. I just bet that she was a sight to see! As this was happening, it was also time to inquire about the results of the genetic testing that was done on Kristiina. Monica felt that it was time, but the genetic counsellor wanted to consult with Monica, as there was a problem with the testing. You see, the samples had become untestable. They had somehow become contaminated. Perhaps by improper storage or by being stored at the wrong temperature. Can you imagine the level of frustration that must have risen as a result of hearing this? This meant more blood would have to be drawn and the family would have to wait again for the results. Fortunately, they had previously gained the approval for funding of the cost. Even so, as April of 2016 rolled around, they would have waited a year to learn the results of the testing. At this time Kristiina was 19 years old, soon to be twenty and Monica stated that the entire experience had been a real rollercoaster of emotions. Adam and Monica attended the appointment with Kristiina also being present as they received the news. Monica stated that she should have known that the news wouldn’t be good by the way it’s delivery started. The geneticist and counsellor sat down in front of the family and here is what was said between them as recounted to me by Monica, “Uhh.. Yeah. The results are back.. and we found the reason.” I remember saying “So you know what’s going on? You have an answer?!” And she said “Yes. It’s a rare, neurodegenerative, lysosomal storage disorder.. (pause).. fatal, incurable.. pause.. disease. Batten’s Disease, specifically CLN2. It means the brain cells don’t dump the waste, and it builds up and the cells die resulting in the regression of skills that Kristiina is experiencing”.
And there it was. The answer for what was plaguing Monica’s princess. I think that most parents believe that they are facing something that is fixable once the cause is determined. At least, they have that hope within them until they hear the news that brings with it pure shock and utter despair. Monica puts their reaction into her own words with the following, “None of us had ever heard of Batten’s Disease. My brain stumbled over those words one by one in order to understand what it all meant. Wait! fatal? That means resulting in death! Incurable? That means there’s no cure! My eyes stung with tears. Adam excused us from the room. My daughter was just issued a death sentence. I was devastated!! Thank you God for Kristiina not understanding a word of that appointment! I had so many questions! How? Who does she get it from? (the answer was) It’s a recessive gene. She inherited a copy of my rare gene and a common copy of the gene from her Dad. They went on to explain more stuff which just sounded like babble to me as I was too preoccupied in my own thoughts. They were both very sympathetic to our situation and offered to do whatever they could to help.”
Kristiina was diagnosed as having CLN2 Battens, or Late Infantile Batten Disease. This, even though she was quickly approaching twenty years of age. In her case, she was defined as being atypical. This, because in most cases, this variant of Batten Disease strikes people that are much younger, and therefore, are young children. At first, the geneticist thought that she had Juvenile Batten Disease because of her age, but the results did not lie. The doctor and counselor proceeded to furnish Adam and Monica with some information on the disease that they had put together and directed them to some websites that they felt would help. They also said that testing could be performed on other family members when they were ready. Can you imagine the rush of emotions as their world was quickly turned upside down? Monica continued to share what she was feeling at the time as she stated the following, “ I had not been prepared for this diagnosis at all. Not just the diagnosis but also what would happen to my princess. She would go blind, she would lose the ability to walk, she would lose the ability to eat and then eventually die!! I hid from the world for a few days as I tried to gather the strength and courage I needed to go on.”
Through everything, Kristiina would continue to be her normal cheerful self. Being a parent to a special needs person, my heart just naturally goes out to Kristiina and her family. Seizures and delays are things that we are familiar with, but the awful diagnosis that is Batten Disease is something beyond what we have experienced. It had to have been so terribly difficult to receive the news that Kristiina was a Batten warrior. I am sure that breaking the news to others while watching their response was a challenge as well. They first broke the news to Monica’s mother and her sister Janet. Then, they did so to Kristiina’s school teacher and the rest of the staff at her school. They were crushed as Kristiina was muched loved by everyone that knew her at the time. She is still much loved by all who know her. Monica said that as the school staff listened, tears streamed down all of their faces. I myself, have shed tears as I have poured over the details of these stories. These kids are priceless beyond comparison. As was mentioned earlier, Kristiina’s twentieth Birthday was approaching quickly as all of this was taking place. Her Birthday would come and go quietly. Monica made comment about this stating that it just seemed, at the time, that they had just taken another step closer to her daughter’s death. They did, however, celebrate the occasion at home, and made good and sure to make their princess feel special. Oh God, how truly special she is! Again Adam would take the lead as the mood in the house became somber. Monica said that she was having a difficult time coming to terms with what life had brought there way. She said that she would find herself falling apart often and with great ease. How very understandable! She would go on to tell me how Adam helped in this situation, “Adam persuaded me there was no time to sit around and wallow in self pity, we needed to act. We needed to learn more so that we could make these years, or however much time we had together as a family, special and memorable for us all!! I began researching Batten’s Disease. Looking for other families I could reach out to. The world felt so big and I felt like we were so alone in this.”
Monica started making phone calls as Adam continued to work to provide for the family. She looked for provincial and municipal programs and organizations that might be a help to them. She also posted a plea in one of her mammas groups on Facebook. As she did so, she was shocked to find out that one of the mom’s had a nephew that was battling Batten Disease. How could this be? Through all of what would follow Monica would learn that they were, in fact, not alone. No, not at all. There was a worldwide community made up of families that were facing the same set of circumstances. Supported by social media and other forms of communication, these families were anchored together by organizations like BDSRA and BDFA in the UK. These families were and still are able to understand and support one another as no one else can because only they can fully understand what each other is experiencing. They know the details of what takes place as the disease progresses and they are able to know what each other is feeling on an emotional level. You have to walk in a person’s shoes to know for yourself! Adam and Monica would find Bev and George Maxim of the Canadian chapter of BDSRA and very quickly, they made plans to attend their first Batten conference in July of 2016. Monica said that it was the best thing that ever could have happened at that point. They were able to connect and learn so much about Batten Disease from the many professionals that were in attendance. My opening quote comes from an event that was experienced as they attended the New Family Mixer at the conference.
The gathering was overwhelming for Kristiina because of the large number of people in attendance and as a result, she experienced a seizure. I’ll repeat what was previously quoted at the beginning and include the remainder of what Monica had told me, “I had never experienced anything like what followed. Adam immediately ran back to our room for her rescue meds. Everyone at our table sprung into action! The two men lifted the table out of the way and gently placed Kristiina on the floor on her side. The one lady calmly stated her seizure had started at such an such a time and it had been just over a minute. There were no awkward stares or whispers. Everyone went about their business around us. As I was kneeling beside Kristiina and comforting her, one of the mother’s came over and squeezed my shoulder, asking if there was anything she could do or help with. I soon came to know that wonderful, sweet woman as Laura Betts. For the first time in forever, this felt ok! I didn’t feel like the freak at the circus everyone stares at. We were totally overwhelmed by the warmth and acceptance of everyone around us. It changed our perspective of our situation for the better, forever! And finally, we didn’t feel like we had to do this alone anymore.”
And with that, Adam and Monica discovered that they were not alone. They had the overwhelming support of a big bunch of wonderful people to whom they could relate. People like Laura and Barrett Betts, as well as, many others. It was, no doubt, an amazing experience for them! Over the course of the last couple of years since the diagnosis, there have (I am sure of this) been many close bonds created between the Bearman family and many of the others. The year 2017 brought with it occasions that were reason to rejoice and also things that brought sadness. It was 2017 that Kristiina would graduate from high school with a Certificate of Accomplishment. Monica said that they were surprised when they learned that the Parliament for Burlington Ontario presented Kristiina with the Citizen of the Year Award! She is so deserving of that! Life after high school, though, would bring many challenges for the family. Kristiina would be denied access into several life skill programs because of her diagnosis. Monica said that they would continue to receive denials from the coordinators and she shared an example of the type of thing she would hear from them, “I’m really sorry but we are not equipped staff wise to deal with her seizures. She is a high risk student and a real liability”. She went on to share with me her feelings and also what a typical response from her would be, “I felt like they were talking about Kristiina as if she was a car or a piece of property!! The kind denial emails were always a slap in the face that angered me. I would fire back a nasty email stating how unfair it was to Kristiina to be denied and that it was them losing out in the long run for not giving her a chance.” Who could blame her for her feelings of frustration?
There was an event that brought much sadness to Monica and Kristiina and it had to do with an old friend of Monica’s. Her friend’s name was Lindsay. Monica had hired her to be Kristiina’s PSW and she performed this function a couple of times a week. Kristiina really enjoyed their time together. They would go shopping and at one point, Lindsay introduced Kristiina to a church based group called The Friendship Club. They would, for example, do things such as going bowling together as a group. Kristiina had been seizure free for almost a year and a half as of December 2017 but that would soon change as tragedy struck both families. Here it is in Monica’s own words, “I had been getting Kristiina ready to go out with Lindsay, as we did every Wednesday evening. When Lindsay didn’t show up or text us to cancel, we didn’t know what to think. The next day, we found out that Lindsay had struggled with her own dark demons for sometime and had lost the battle. I was devastated!! What was I going to tell Kristiina? I took a couple days to come to terms with the news myself before I broke the news to Kristiina. We explained to Kristiina that Lindsay had looked fine on the outside, but was sick on the inside, and she had passed away. We told Kristiina that Lindsay was very sorry and that she loved Kristiina very much.”
Lindsay was much more to Kristiina than just her Personal Support Worker. She was Kristiina’s good friend and she was someone that was loved and trusted, just like family. Monica said that it was obvious that the stress associated with the news was too much for Kristiina. She became angry and upset over it and as a result, she suffered a seizure the next morning. The seizure left her worn out for the next few days. Monica said that Kristiina has never completely gotten over the event. This is certainly understandable.
As April of 2018 rolled around, a really good thing would happen. Monica would learn about a day program that would show great promise for Kristiina and she would learn about it from one of her momma friends on Facebook. Monica went almost straight to the program and talked to the coordinator whose name was Bill Murray. She had a lengthy conversation with Bill and, much to her amazement, she found out that he knew what Batten Disease was in detail. This is because one of the former, long time clients there had the disease. Ironically, it was the family of the mom that had initially provided Monica with all of the information about BDSRA and Batten Disease. Monica said that it felt as if this was all meant to be and, in fact, it was as Kristiina was accepted into the program on a trial basis. In time, it would prove to be a great fit for her. The name of the program is Accent Music Therapy and it resides in Burlington, Ontario. Monica says that Bill Murray and the staff there have handled Kristiina’s seizures like seasoned professionals. Perhaps the other day programs could learn a lesson from them! Monica is so thankful for the opportunity that has been presented to Kristiina by Bill.
Monica says that, in many ways, life for them is just like any family. Adam works hard and does a great job of providing for his family. Kristiina’s big brother Matthew lives outside the family home while Khloe and Sammantha still live with mom and dad. They both attend school. For Monica, there are all of the normal responsibilities. Housework and preparation of meals are a big part of her day but the biggest job is being a mom. Specifically, a mom to three daughters. One is a warrior princess and that of course brings with it special challenges. Monica stated that Kristiina cannot be left alone for more than 30 minutes at a time and so she needs to be vigilant. Their activities as a family has to take Kristiina’s special needs into consideration.
The Bearrmans have to be prepared for anything and they need to refrain from activities and conditions that may trigger a seizure in Kristiina. Their social life is affected because most people either do not understand their situation or they are uncomfortable with being around a person with special needs. Both Adam and Monica each have a few friends that they socialize with and their closest family members give them lots of love and support. This would include Monica’s mom Helen and sister Janet. Adam’s parents and other family members are a part of the Bearman’s support system as well. One very noteworthy individual is Adam’s sister and her name is Krystal (Bearman) Blackborow. Krystal had moved in with the Bearman family about a year ago and their relationship has since blossomed. I will let Monica tell you what she means to Kristiina and the family in her own words, “She’s always there for our family. She’s grown very protective of Kristiina, as she is of all her nieces. She helps me out a lot with the girls! I’m so glad I live with my sister-in-law. she’s my best friend! We love her!” That is, of course, a wonderful thing!
There is nothing that this family would not be willing to give up for Kristiina. Monica says that her love for her daughter is unconditional. She is completely empathetic towards Kristiina as she continues to teach Monica so much about the important things in life. Knowing that her time with Kristiina may be limited makes each day count towards forming priceless memories. Monica stated that each smile, each giggles, each kiss, and each hug are priceless to her. Kristiina is her princess and her presence in Monica’s life is a gift that she doesn’t take for granted. She reflects on what she sees in her daughter and what she has been taught about life through her circumstances, “I admire her (Kristina) for her strong will and determination. She never quits trying to do her best! She loves to help everybody! She has this spark about her that is indescribable!! She’s funny and loves attention! She’s my hero! It’s hard to think to think about the future when you’re not sure about what’s to come. Adam has helped me deal with the anxiety that arises in me when I think about the future. I have learned there is no sense in worrying about tomorrow. We take life one day at a time and live for today with no regrets and no looking back. There will always be challenges in life. We do our best to deal with them and just take it as it comes.”
It would be difficult to not consider the things that Batten Disease has taken from Kristiina and her family but Monica says that they have a lot to be thankful for. Kristiina still has her eyesight and her ability to walk and talk! She is able to eat and drink by mouth and any Batten parent will testify to the fact that this is a big deal. As is always the case, a Batten parent thinks about what it would be like to lose their child to this cruel and relentless disease. Like many of the families that battle Battens, the Bearman's cling to the hope that is provided by current treatment that could be made available. Here is what Monica had to say about the subject, “We are hopeful that one day Kristiina will receive the life changing enzyme therapy. For now, we will advocate for her right to quality of life and we will educate others about this disease in hopes of Health Canada approving the treatment that she so desperately needs and deserves. This whole situation has also opened our eyes to the sad realization that these treatments and cures, not only for Batten’s Disease, are more so about the politics and money involved.”
I was flattered by the fact that Monica approached me at this years Batten conference and introduced herself to me. She had been a regular reader of my blog and she wanted to work together in doing Kristiina’s story. Monica is another incredible mommy! There was so much going on at the moment that she approached me and I had failed, at that time, to walk to their table to meet Kristiina. My huge mistake. Hopefully, next year if I can make it to Denver. Looking back at it now, I had seen Kristiina (or Krissy) in the midst of all of the Batten kids and I could tell that she was a crowd favorite. This, I can tell from what I have seen, and also from What I have been told. This being that Kristiina is entirely special.
I know that it is often difficult for Batten parents to recount the experience that is the Batten Journey. This includes sifting through old photographs that help to paint a picture of what life was like before Batten Disease. As I was close to finishing this story, eight children had gained their wings in the span of about two weeks. Flying away together as a group seems to happen frequently with Batten children and I know that the community was shaken when it happened this time. Batten parents were hugging their kids extra tight when this took place. Monica was also deeply affected by this loss and she had to power through near the end of working with me. She has my profound respect and appreciation for this. Kristiina is doing so well and will continue to battle. Advocating for her treatment is of the greatest importance. These kids (and sometimes young adults) are so special and it is difficult for me to find words that are adequate to describe how I feel. The parents, however, know for themselves how these warriors tug at your heart.
I am a friend of, and an outsider to the Batten community. I still do not know, nor can I fully understand what it is like to be on a journey such as Batten Disease. With every new story, I remain amazed by the care that is displayed and the love that is undying. Another ordinary family that has been asked to do something that is extraordinary. Nobody will impact the lives of this family like Kristiina will. She will forever be a part of what makes up the whole and the impact of her life on others will be without end. Such a princess is she!
Blogger and Advocate
Is it them or is it us? Is it the feelings that they produce in us or is it that there is something truly special about them? I think that the answer has to be all of the above. It is them, but they also do something to us. Once you give a little piece of your heart to these kids and their families, they come close to taking all of it. Let it happen!
I would like to welcome to my blog Sanfilippo families, as well as, families in other MPS communities. I am grateful for every opportunity to write about those who battle an MPS disorder. This, of course, alongside their special children. This is a labor of love that is extended to each one of you! Scroll down to see MPS family titles.
Highlighting a previous blog that might interest you:
Forever Royal - The Final Version
One child in a family with a rare disease is a challenge. What if there is two? Heartbreak multiplied you pick up the cause and do your very best to make a difference. That is how a normal family becomes Forever Royal ...
Take the titles listed below and go to month and year listed in the archives.
January 2017 -
*The Story Between The Lines
February 2017 -
*I Know Just Where You Are
*My Kind Of Royalty
March 2017 -
*The Caring Type
*Face to face: A Fairy-tale
April 2017 -
*No Ordinary Love
*A Rare Friendship
May 2017 -
*Tyrell and The Rare Love Story
June 2017 -
*Seth and The Healing
July 2017 -
*Little John and The Reason
*Sands and The Scottish Princess
August 2017 -
*A Rare Kind of Devotion
*The Giggle Box
September 2017 -
*Sweet Montanna and The Status
October 2017 -
*The Blog About My Blog
November 2017 -
*Lydia, I Love You!
*Kayden And The Change In Plans
December 2017 -
*Those That Are Like Them
January 2018 -
*Nora Skye - I Know Just Where You Are
*Brock and The Priceless Memories
*Jamesy Boy and The Treatment
*The Priceless Princess From Beyond
*Forever Royal - Part One
*Forever Royal - Part Two
*That Kind of Love - Conner's Story
*What I See (She is a Butterfly)
*No Longer Alone - Kristiina's Story
*Things We've Talked About - Oscar's Story
*The Reason Why - Hannah's Story
*Kayla's Perfect Princess - Breanna's Story
*Where Do I Go From Here? - An information only blog
*Warriors Unaware - Mia and Kaleb
*Her Little Story
*Someone Like Raelynn
*Forever Royal - The Final Version
*Awfully Beautiful - The Life of Noah and Laine
*A Lifetime of Love - Tegen's Destiny
*Forever Beautiful - Gabi's Story
*The Miracle Baby - Amelia's Story
*Dylan's Life - A Small Town Story
*Princess Grace and the Challenges
*Anything for Tessa - Her Story
*Haley Bug's Battle - A Family Story
Sanfilippo Syndrome and other MPS family titles -
*The Beautiful Light of Haidyn Grace
*Any Distance Traveled - Reagan's Story
*The Missing Someone - Kiernan's Story
Archives - Use title listed above with the applicable month and year.
Highlights from the beginning of gregster60.com -
Benjamin's Daddy - March and June of 2016 - Revised May 2017. Titled Benjamin's Daddy Revised.
My Fearless Adventure - April and May of 2016
A Different Type of Warrior - January 2017
Larry - February 2017