After a very rough few winter months, in January 2015 he got to visit his grandparents and great-grandparents in Canmore, then went to Mexico with both grandmas and his parents to see the ocean and beach for the first time. Although he was still fighting lots of symptoms like throwing up that made him not enjoy Mexico as much as we had wished, overall it was a great experience and I’m so grateful we had the opportunity to take him. Braxton got to play in the sand, experience the ocean, hear some resort music shows, sleep by the pool, and feel the warm tropical breeze. Right before Braxton’s second birthday he was admitted to hospital with seizures. He still struggles daily with different types of seizures, throwing up, not being able to move his body in the ways he likes, and having trouble sleeping. However we were elated to have him celebrate his second birthday with lots of family, especially because some doctors didn’t predict he would live to 2 years. He has a great team of people working with him to make sure he would make it to 2 years and beyond, nurses, doctors and therapists. His favourite is music therapy, which we enrolled him in 8 months of weekly therapy with JB music, on top of his monthly music therapy at CNIB. Braxton also attends physio therapy and speech therapy. Braxton is working on his gross motor skills, trying to learn to sit up, lift his head when laying on his stomach, and bearing weight on his legs with equipment called a standing frame. He has been having a fantastic time in his pool, and transferring the skills he was learning in the water to the floor, giving him the ability to “run” in circles, lying on his side pivoting around his head. He was running and wiggling around our living room so much he rubbed the hair right off a spot on his head and got a bald spot! He is still stuck at the abilities of a 3-6 month old, although he is getting bigger and bigger, he is 3 feet tall and over 25 pounds. He is on the waiting list for a wheelchair, and should be getting it any day now. His fine motor skills are also slowly improving, he can hold light objects in his hands, like a music shaker, rattle, or soft ball. He holds your hand and feels the texture of fabric on the couch or his bed. In April we welcomed our baby, Zander, to the family. Braxton loves being a big brother, and Zander has been a great playmate. Zander and Braxton babble to each other. Zander brings toys to Braxton and will move his arms and legs to make Braxton ‘play’ with the toy. Zander gets excited to see Braxton each morning, and tries to help us with his equipment like his feeding machine and suction machine. The summer and fall were pretty uneventful, just lots of family time in between the many hospital appointments and therapies. We took the boys camping and Braxton got to try riding a horse at Our Lady Queen of Peace Ranch. Braxton really enjoyed going for walks in his stroller and laying on his swing in the backyard. For Halloween Braxton was a wise owl, and Zander was a busy bee. Luckily we had a pretty mild winter and we were able to be out and about lots. The boys enjoyed Christmas celebrations with family.
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Braxton has been diagnosed with Early Infantile Epileptic Encephalopathy. Epileptic Encephalopathies: An Overview (Sonia Khan and Raidah Al Baradie, 2012) states that: Epileptic encephalopathies are an epileptic condition characterized by brain abnormalities associated with progressive cerebral dysfunction. Eight epileptic encephalopathy syndromes are recognized, depending on the child's age. These syndromes include: early myoclonic encephalopathy and Ohtahara syndrome in the neonatal period, West syndrome and Dravet syndrome in infancy, myoclonic status in nonprogressive encephalopathies, and Lennox-Gastaut syndrome, Landau-Kleffner syndrome, and epilepsy with continuous spike waves during slow wave sleep in childhood and adolescences. Epileptic encephalopathies are severe brain disorders of early age that manifest with (1) EEG activity that is often aggressive, (2) seizures that are usually multiform and intractable, (3) cognitive, behavioral and neurological deficits that may be relentless, and (4) sometimes early death. EEG demonstrates primarily burst-suppression patterns in the neonatal period, hypsarrhythmia in infancy, and slow generalized spike-wave discharges in early childhood. Braxton's History with Epilepsy: Braxton had his first seizure in August 2013, at 3 months old. Most of Braxton's seizures are tonic, they make his whole body go stiff. They usually last about three minutes, although we have had a couple longer than ten minutes. Seizures lasting longer than five minutes become dangerous. We have tried Keppra, Tripetal, Clobazam, Nitrazepam, Topiramate, phenobarbital, Sabril....nothing is working to stop the seizures. We were originally given ativan as a rescue medication to administer when his seizures last longer than five minutes and it failed to stop his seizures. We now have midazolam as a rescue medication. In December, at 7 months old, Braxton was diagnosed with infantile spasms. Like his seizures we are still struggling to control the spasms with medication. The spasms look like a startle, he has a tiny jump, just a second long. The problem is with infantile spasms is that his brain can be having seizures that we can't see on the outside though movement or stiffening, and so there is no rescue from too many or too long seizures. This type is also associated with regression in babies development, but luckily typically only last a few years. Between the spasms and sedating medications, Braxton is once again like a new born. He has lost the ability to eat, often struggles to breath, and has little motor control. In January Braxton had his forth EEG, a test that monitors his brain waves. This time it was a 24 hour EEG. His EEGs show he has abnormal brain waves, most likely a symptom of dandy-walker malformation and other brain abnormalities. Since so many medications have not stopped his seizures there is little chance additional medications will stop them. Unfortunately we have few options, steroids or ketogenic diet were the only two paths presented to us by Neurology. We decided to go the route of the ketogenic diet (http://fortheloveofbubbe.weebly.com/1/post/2014/02/ketogenic-diet.html). Braxton has now been on the ketogenic diet for many months. It has been difficult to find the right fat ratio and amount of calories to out Braxton's body into a state of ketosis to decrease his seizures. Although the diet is not yet effect in reducing his seizures, decreasing the other sedating anti-convusant medications has had a positive effect on Braxton's development. He is more aware of his surroundings and able to respond to us. In time we also tried steroids for periods of time, which did help, but was not a long term solution. In May 2014 Braxton started using medical marijuana and got to his target dose of CBD in August. In September we started monthly IVIG treatment. In November we began theraputic doses of Vitamin B6. So far Braxton's seizures have been intractable (not controlled). We have had to give midazolam, his rescue medication, many times now, and it often does stop the seizures, but also turns our baby into a unresponsive ragdoll. The more times we use the rescue med the more his body gets used to it and it is less effective for future use. We sometimes are able to go months without needing rescue medications, but now when we need them one dose of midaz isn't enough. We often need two or more doses, or sometimes a loading dose of phenobarb or fosphenytoin as well. These meds have to be administered via intermuscular injection or IV and require him to be monitored in hospital as they really drop Braxton's breathing effort. Epilepsy Facts (from http://www.epilepsy.ca/en-CA/Facts/Epilepsy-Facts.html) Epilepsy is a physical condition characterized by sudden, brief changes in how the brain works. It is a symptom of a neurological disorder - a disorder that affects the brain and shows itself in the form of seizures. Epilepsy is a disorder, not a disease; it is not contagious. Approximately 0.6% of the Canadian population has epilepsy. This includes those who take anticonvulsant drugs or who had a seizure within the past 5 years. Each year an average of 15,500 people learn they have epilepsy; 44% are diagnosed before the age of 5. In approximately 50% of cases of childhood epilepsy, seizures disappear completely. The major form of treatment is long-term drug therapy. Drugs are not a cure and can have numerous, sometimes severe, side effects. Epilepsy still can be a very serious condition and individuals do die of it. Experts estimate that prolonged seizures (status epilepticus) are the cause of many deaths in Canada each year. Events that may trigger seizures include:
Our attempts to end Braxton's seizures:
Medications: -keprra -clobazam -trileptal -sabril -topiramate -nitrazepam -phenobarbital ketogenic Diet Medical Marijuana IVIG Vitamin B6 I am so frustrated at being told by doctors that there is nothing they can do to help my son. And at the same time I am so tired of friends/family/strangers inquiring if Braxton is better. “No” I wasn’t to scream to those questioning, “he will never be fixed, never be whole, never be a normal kid”, and at the same time I am beat down every time doctors tell me the same thing.
It is all the things that you think should have solutions: he vomits every single day, he drools excessively when he sits up, he seizes in big fitful clusters every couple hours and small subtle spasms every few minutes, and his oxygen levels drop dangerously low while he sleeps peacefully. But no, instead we are told this is just part of who Braxton is, as if we were talking about how a child has a happy disposition and a talent at music. Braxton is a complex child made unique by a gene mutation that altered the way his brain formed. This slight change to his genetics made his life difficult. His eyes fail to see, his hands fail to hold, his legs and feet fail to stand, his throat fails to swallow. He is so different from his peers I am often floored by the “normal”. I watch little boys legs run fast, pedal bikes, kick balls, and am amazed. Someone offered my child cake and I didn’t even know what to say, because eating cake is so far away from our realm of possibility, since Braxton eats through a tube in his stomach and is on a diet that doesn’t allow sugar. Sometimes other parents say things that hurt without knowing it, like saying “it doesn’t matter if it’s a boy or girl as long as it’s healthy” or “with toddlers we are luckily to just make it through the day with everyone alive”. I tell my husband that today was a good day, and he reminds me that our good days are an average person’s worst nightmare. The altered gene didn’t just change Braxton, it changed my place in the universe. Living in the tear, waiting for the drop. Dear little baby, I have carried you inside me for 254 days now. Although we have been one, we will soon be two. It would be a great gift if you chose to be born tomorrow on my birthday, but I understand if you want to wait the twelve more days til your due. The world out here is scary and you're safe in me now. Plus you've chosen a crazy family to be a part of! But we know you will be a blessing to us, just as we know your big brother was meant to be part of our family. Braxton has taught us so much about life, love, and God, and we know that you too will have your own unique needs, strengths, and lessons to share. There is lots of love out here waiting for you. I can't wait to meet you little baby. Love your mama Zander was born April 13 at 5:59am! He is a great little brother and Braxton loves him.
I am exhausted! Braxton has only slept through the night a handful of times in his 22 months. He is developmentally still at the level of a 3-6 month old, and so has not learned self-soothing skills, or mastered the ability to roll over. He is also visually impaired and has a debilitating form of epilepsy. When he wakes in the night he requires help to reposition his body and a diaper change, often he needs to be soothed through cuddles and touch, and frequently he is up for a period of hours. Braxton only sleeps up to six consecutive hours, so I have always needed to get up sometime in the night. In the past I have accepted this situation as part of the life of a parent of a disabled son, but over the past few months Braxton’s night time needs have increased and my own abilities are limited due to being in the last month of pregnancy. Currently I am up each night while Braxton is awake, usually for two hours between 3am-6am, while Braxton seizes and shakes, then after his morning feed at 6am he throws up and often needs suctioning to clear his airway. Most days we attend appointments, so there are limited opportunities for me to catch up on sleep, even during the days I have someone helping. The lack of sleep is affecting my abilities to be a good mother, and my own health.
Braxton’s needs grew drastically as of December 2014. In December, Braxton started to have an extreme amount of spasming when he woke up. He would wake up around 3am each morning and have a spasm approximately each minute for about an hour. This would be very irritating to him, he would usually cry during this time. The spasms would slow, but not stop, and so he would have to get so exhausted that he would sleep while his body jerked around from the epileptic spasms. This occurred in the night, each morning, and often after afternoon naps, up to hundreds of spasms in an hour. I could not simply go back to bed, but instead spent each night up when he was awake. Braxton’s other types of seizures, such as generalized clonic-tonic, increased in this time, and many days we had to completely sedate him with rescue medication. I took him to emergency on December 18, and neurology suggested maxing out his medication, but this seemed to just make things worse. Homecare was aware of Braxton’s worsening conditions, and both Braxton and my extreme lack of sleep, and did provide us with some additional nursing care during this time. Thankfully after weaning one of his medications, Sabril, Braxton was able to gain some control over the seizures and returned to baseline of 30-50 spasms a day and 5 clonic-tonic seizures a week for a short while, but his sleep schedule did not improve, and his seizures have since gotten worse again. In his medical assessments at his various clinics since December, it is clear that the seizures during December resulted in regressions in many of Braxton’s skills. Most obvious and dangerous is that he began to have much more reflux and a swallow study demonstrated he has frank silent aspiration. He is once again NPO. Currently Braxton has thrown up his first feed of the day nearly every day since the beginning of February. Braxton often requires suctioning to help clear his airway during this reflux, and also frequently needs suctioning after seizures, which is new. Braxton also had a regression in gross-motor skills, and so cannot move his body to a comfortable position in the night, or move his body to safely throw up without aspirating. This means that whenever I hear Braxton cough during the night I have to get up to make sure he is not aspirating. Braxton’s seizures have also become more frequent, especially in the early morning hours. As of January 18, there has only been three days without a generalized seizure. It is now the new normal for Braxton to have about four generalized seizures a day, which require monitoring, because if they last too long or occur too close together, emergency rescue medication is required. Most days at least two of these seizures occur during the night, which means once again I have to be up and monitoring him to make sure the seizures end and he doesn’t go into status epilepticus. Additionally, Braxton has developed a shake or shiver-like tremor when we wakes up in the night. We are exploring the potential causes of these tremors, and have been able to rule out temperature, glucose and ketone levels, and oxygen needs, but the cause remains unknown. As we don’t know if the shaking is neurological, we must be sure to keep a close eye and comfort Braxton during this time. Finally, as we have been told Braxton’s death will probably be because of him stopping breathing in the night, we frequently check his breathing, reposition him, and making sure his oxygen is blowing properly. These new needs lead to very limited sleep. Braxton finishes his last feed of the day at 10:30 pm and starts his first feed at 6am. When I get opportunities to sleep during that time, I have to keep enough awareness to hear Braxton’s movements over the monitor. Seizures sound like rustling sheets, aspiration can just be a cough, these subtle sounds need to wake me up. Additionally there is a huge level of stress associated with these needs. I know that any night my son could die, and it is up to me to prevent this. If I sleep too long or too deeply I could miss an epileptic or cyanotic event that could result in his death. I have been on anti-depressants and sought counseling to help with this reality. Multiple times I feel that I get out of bed and get to Braxton just in time to suction him to keep him alive. But I am human, and a very pregnant human at that, and sometimes in sleep deprived states I can make mistakes. Recently I was so tired I knocked the side mirror off our vehicle going into a car wash. I have been so tired I fell down the stairs trying to get to Braxton in the night, resulting in having to go to the hospital to check on my pregnancy. My family doctor has written a doctor’s note outlining the need to get more sleep. I am worried that if I do not get a decent night’s sleep I will be too exhausted to labour, and cannot imagine how I would be able to take care of Braxton’s needs if I required bed rest or a caesarian section. Braxton will soon be two years old, and while I may be to handle extreme vigilance and lack of sleep for months, I cannot handle it for years. Although Braxton is complex medically and has many needs, he hopefully will be with us for years to come, and I need night help in place to keep attending to his needs long term. “Stay home. There is nothing that can be done here, that can’t be handled by you, in your own house.” The neurologist on-call told me over the phone. “Unless the symptoms become overwhelming” I am overwhelmed I think. But that’s not what she meant. She means if he becomes too dehydrated or stops breathing. At the moment with our oxygen concentrator, our tube feeds, our rescue medications, we were keeping Braxton alive. We had been in touch with the hospital many times over the past few days. It was December 2014, I was 25 weeks pregnant with Baby Two, Brock was home for the holidays. Braxton had been started on Sabril for his spasms, but he got worse, so after a trip to emergency, maxing out sabril, ever increasing seizures, we weaned the new med. We hoped that he would bounce back after the wean, but so far he had continued to slip from us. Our rural homecare had been advising we push to get our son to the hospital for the past few days, but we didn’t know if it would be helpful or more stressful, and so we were dealing at home. Maybe we suck at giving up control. Maybe we have seen that we know than many nurses and doctors about our son’s rare conditions. Maybe we knew during the holidays the staff we would need to handle end of life decisions were away. Maybe we know even in hospital, we parents do most of the feeding, suctioning, monitoring, and care of our little boy, so why bother. But this night it was too much for me, we had sedated Braxton already that day with our rescue medication, and he was seizing again. The neurologist on the other end of the phone line said we could sedate him again, which is the last thing I wanted to do. Those past few weeks his spasms and then generalized seizures had been increasing in quantity and strength. We basically had one to two hours a day that he was his old alert playful self, the rest of day he was fussy, screaming, throwing up, seizing. Neurology said there wasn't much that could be done for treatment for his seizures, we had pretty much tried everything in the past 16 months since his seizures began. More seizures are just a reflection of the progression of his conditions, they said. Now I did want help. I wanted some medical professional to say, we just have to do A, B, C, then your son will be okay. There is supposed to be safety in our medical system. It’s a myth we are led to believe, that if something goes wrong with our health, we can go to the hospital, we can see a doctor, we can be prescribed medication, we can be fixed. The reality is there are too many rare disorders that doctors don’t know about, that hospitals don’t know how to treat. Dandy-walker Syndrome and Microcephaly Agenis of Cropus Collosum are both listed of the Global Genes rarelist (http://globalgenes.org/rarelist/) but Braxton is even more complicated than that, having other add-on brain abnormalities. In this moment I felt very alone. I am not a doctor, I am not a nurse. I am just a mom who is supposed to take care of this complex child at home just as well as a hospital of professionals. It breaks my heart to see him have to struggle, suffer, and seize each day, and I’m his best hope. Braxton survived that night, and the hard days and nights that followed, and the good and bad days since. I haven’t taken him to emergency or had him admitted to the hospital since. He survives not because of the medical system, although we have found a good team of professionals who are working with us. Many people pray for Braxton, and I know that really does help. I continue to fight, research, get approval, advocate, and try alternative therapies. Braxton is strong, he’s a fighter, and he doesn’t seem to want to leave us just yet. Braxton having a break from seizing to open Christmas presents
Braxton was born with his eyes closed. This didn't worry us, he was such a perfect baby. When we got Braxton home nurses visited us and I asked how long it usually takes for babies to open their eyes. I saw a look on the nurses faces that I would soon see far too often, worry. However when other doctors checked on him they agreed that Braxton wasn't opening his eyes since the vacuum birth bruised his eyes. Looking back now I can see how purple his little face was but at the time he was just my perfect baby. Doctors tried to pry his eyes open, shined their little light in, and said they are fine, we just have to wait for him to open his eyes. The doctors were right, after a while Braxton started to peek and we got glimpses of his baby blues. However he didn't seem to "see". At the end of July, at my insistence, my pediatrician booked us an appointment with an ophthalmologist. We didn't get an appointment until the end of August since not tracking wasn't a big concern to the doctors. While waiting for the ophthalmologist appointment Braxton started having seizures. He was admitted to the hospital in Regina and then Alberta Children's hospital. We spent four days in the hospitals where nurses and doctors 'checked' his eyes on average every four hours. No one noticed anything unusual besides he hated to open his eyes. We met the pediatric ophthalmologist at the end of August and he noticed right away that Braxton had cataracts in both of his eyes. Our suspicions were confirmed, at the most he could see light and dark. He needed surgery as soon as possible. An ultrasound of his eyes showed that there might be a stalk that connected the front of his eye to his retina so a typical cataract surgery would not me enough to correct his eyes. Instead we had to go through a complicated process of getting a surgeon, closing down an operating room at the Children's hospital so that pediatric staff could go to another hospital where the surgery equipment was located when there was an opening in that hospital. It took a month and being pushy parents to get all these things coordinated. It was scary as a mom to let go of my baby so he could a surgery under full anesthetic with a breathing tube. The surgery was a success! Braxton got his first glasses when he was 5 1/2 months old. In the cataract surgery the lenses were removed from his eyes so he needed the highest prescription possible. Currently he is still learning to adjust to his glasses, and is opening his eyes for longer periods of time
I have had trouble writing blog posts lately, because so much of what I am experiencing is near impossible to put into words. I feel like I am most often in a state of emotions and experiencing the world with fear, empathy, sadness, elation, hope, regrets, guilt, praise, and prayer, often in the same day. Some of the emotions I feel I do not have words for, like the deep driving need to keep my son safe at all costs that makes me stay up late into the night watching him sleep, or the gut wrenching feeling of seeing Braxton suffer through a procedure that might help him later but hurts terribly now and wondering if I made the right decision.
Unfortunately Braxton has had a bad spin downwards with his health. He started acting out of the normal mid-October. That Wednesday he cried non-stop all night and day, definitely not typical behavior. I took him into emergency, which I’m sure made everyone think I was an over-reacting crazy mama, but I just felt something wasn’t right. The doctors did bloodwork and urine tests, and nothing came back abnormal, so I took Braxton home. After a couple hours of sleep in his bed, Braxton woke up with a barking cough, and we cuddled him through the night. He had a low grade fever on Thursday and some vomiting, but nothing too alarming. Friday, Braxton continued having a bad cough and resumed his crying, I tried to get him into a doctor’s office, but the pediatrician and family doctor were busy. Saturday Braxton started having seizures more and more often, increasing in frequency and duration. I was really hesitant to give Braxton his rescue medication or take him back to emergency, I didn’t want it to be real that he was seizing so much, knowing we have to go to the hospital, in the night, on a long weekend, when no one there would know Braxton. We administered Braxton’s rescue medication Midazolam, and they stopped the seizures, but only for an hour, then they started again. There isn’t much that can sum up that fear, when the rescue med didn’t rescue your son. So we got packed up, knowing this wouldn’t be a short trip back to emergency. At the hospital Braxton was rushed immediately into trauma. We have been there before, so Brock and I were able to fall into place amongst the nurses and doctors, helping to give history, and let them know where to not bother trying to put in a line and what meds don’t work. Braxton was given another dose of midazolam, and a loading dose of fosphenytoin and phenobarbital. Finally the seizures slowed as Braxton drifted into a drugged up state. A few hours of observation later, Braxton needed a second dose of phenobarbital, as he started subtle seizing again. The doctors came to the conclusion Braxton had caught a cold virus, which was making him seize uncontrollably. We were admitted overnight, but thankfully as the medications wore off the seizures went back to baseline frequency and we were able to go home. The following day, Tuesday, the seizures started again, and I called the hospital. We were told if we went back to the hospital Braxton would need to be put under heavy sedation in the PICU, and were advised to stay home, monitoring and administering rescue meds ourselves, as long as possible. I broke down at this point, after a week of next to no sleep, extreme worry, a hospital stay, and the emotional roller coaster. But as the doctors at the hospital knew, Brock and I are well trained through experience and took good care of Braxton and got him though the day of seizures without needing a trip back to the hospital. The rest of the week the seizures came and went until his cold virus was finally fought off. Since this episode we have kept Braxton in a bubble. We haven’t been to play groups, we sanitize everything, and we have a sign on the door to keep sick people away. But even with all of these precautions Braxton caught another bug at the beginning of November. Although this one resulted in him throwing up for two days and a week of phlegmy coughs and sneezes, his seizures didn’t get beyond what we could manage ourselves. I would love to have a day where Braxton didn’t have a seizure. I cannot see that happening anytime in the near future. Until then I live in the moment. Sometimes those moments are scary and overwhelming. Other times I get the joy of Braxton exploring my face with his hands, singing to the radio, mimicking my words back to me, enjoying eating pear puree with his mouth, or playing with his toys independently. Whatever the day brings, I’m here with Braxton to see him through. I haven’t posted in a bit, as life got into a good routine and the strange life of a parent of a complex child became normal. Summer went by in a blur. Braxton woke up early, liking to see his daddy before he left for work, and then we would read library books and cuddle. Usually he would go back to bed once daddy left, but some days we were up until afternoon nap. Braxton got upgraded to a “big boy bed”, which is much easier for me to lift him in and out of. During the day we went for walks around our town, visited the library, went to music therapy, and swam in his pool as often as we could. In July went on a road trip to attend a family wedding and took a flight to visit family in Saskatchewan. Braxton handled both of these new situations surprisingly well. Unfortunately we learned that Braxton still can’t handle loud confusing environments, but we were happy Braxton got to visit many relatives. In August, we had a great day trip to a waterfall, which Braxton really enjoyed listening to. We also had him feel the cold river and he held a warm rock, which he liked so much he held it the whole car ride home (pretty impressive for a little guy who barely has the fine motor control to hold anything). Braxton also liked the smell of waffle cones at the ice cream shop. Braxton has made progress that has impressed the doctors. He’s eating more orally, needing less oxygen, throwing up less, and the latest good news is he has started tracking horizontally with his eyes! Braxton is far more aware of his surroundings, turning his head towards sounds, listening to conversations, playing with toys, and sitting with less head support. We have an aide for PACEkids coming every weekday and she really helps me by making sure Braxton’s stretches and exercises are done daily. Now that fall has come we are busy again with appointments and therapies, but I'm glad Braxton was able to have so many new positive experiences over the summer.
The exciting news of the summer is that Braxton is going to be a big brother! Newest baby Lockie is due April 12, 2014. |
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