A New / Another Diagnosis

A couple of months ago, Brennen started going through what we thought was a phase of fussiness - irritability, fatigue, and at times just being a bit of a jerk. I know, that sounds harsh, but that's really what it seemed like. He had become a terrible teenager. For several weeks, he was difficult to settle and just wasn't his happy ol' self.

Also around this time, it seemed like he was having more seizures than usual, mostly at night, and they were bigger, lasting longer than normal, and they were scarier for us. Brennen has always had seizures, and we have tried a number of different medications over the years, but they have never really been well controlled.

Around the end of May, we made an appointment to see our pediatrician, just to have him looked at and to rule out anything really serious. Of course, any time Brennen shows signs of discomfort, our first thoughts go to his back or his hips. He has had some major work done inside his little body, and I have a constant fear that something is going to go wrong with the instrumentation one of these days. Andrew and I are always checking for protruding rods and screws, and while we have been assured that everything is where it's supposed to be, I know that things can slip. His x-rays that day looked good, so there was no issue there, but we got in to see the orthopedic surgeon just in case. While chatting with us, she noticed that Brennen was making some slightly unusual eye movements, and she questioned whether or not this could be seizure activity. She ordered an EEG, and we rushed over to have that done right away.

Andrew and I sat with Brennen for over an hour and watched as his brain's electrical activity was recorded on the monitor. Brennen was also under video observation, so that when the EEG and his recorded behavior were displayed simultaneously, correlations could be made between recorded events and any accompanying electrographic changes. Sounds wild, but it was fascinating to watch!

The results showed that Brennen was having more seizures than we realized. Like, a lot more. We can obviously recognize when he has a grand mal (generalized tonic-clonic) seizure, but some of the changes that were picked up on the EEG were so subtle, we would never have recognized them as seizure activity. It was shocking for us to learn that these seizures were happening so frequently, and it breaks my heart to know that Brennen had been suffering through that for so long without us realizing. 

On that day in May, Brennen was given a new diagnosis of Lennox-Gastaut syndrome - a rare and often debilitating form of childhood-onset epilepsy. Lennox-Gastaut is characterized by multiple types of seizures, moderate to severe cognitive impairment, and an abnormal EEG with slow spike-wave complexes. This makes for one of the most difficult forms of epilepsy to treat. Brennen has what is also known as intractable epilepsy - a seizure disorder in which a patient's seizures fail to come under control with treatment.

We have increased Brennen's seizure medication, and that seems to have helped things a little, for now. Giving his brain a rest from so much seizure activity has made him sleep better at night, and as a result, he is happier during the day.  

Seizures have become a regular part of our lives, and I almost hate to say it, but we have sort of gotten used to them. Up until recently, we just accepted that they were part of Brennen's diagnosis - cerebral palsy often comes with a seizure disorder - and we would have to live with that. Now, when I look up Lennox-Gastaut syndrome, I see things like "rare and catastrophic epilepsy" and "diminished quality of life". "Reduced life expectancy" is mentioned there as well. This condition comes with an increased risk of SUDEP - Sudden unexpected death in epilepsy - also referred to as a fatal "complication" of epilepsy.

This shit is real.

While we may have gotten used to the seizures, I won't ever get used to the fact that my child has to suffer and fight for his life on a regular basis. There are still many nights that he wakes up having a seizure, and even when he doesn't, I find myself running to his bedside to make sure he is breathing and that he is still with us.

Brennen has taught me many things over the years, perhaps most of all, to be grateful. I am grateful for every moment shared with him, and for every precious memory made together. There are times when I struggle to find peace, happiness, and the "good" amid the challenges. It isn't easy, but I am grateful for all that I have. Brennen is a blessing. His life is a glorious gift, and I am nothing but grateful. 

Tulip Tales: Hannah

I am thrilled to be back with another addition to my Tulip Tales series! Lucy-Anne contacted me some time ago about her daughter Hannah. She wrote, "Hi Julie. I was told about your blog, and I also remember you from a music therapy class we took about 4 years ago, when my daughter was only 3. I think your blog is fabulous and honest. Life is very challenging, and most families do not "get it". I understand you do a lot for families facing the challenge of CP, and I'm sure you have met other families with various challenges. Even though my daughter is now 8, we have not reached out, as we have always felt very alone because of how rare her condition is - stuck in survival mode. I would be interested in telling Hannah's story if you like, and would love to connect with you more."

I am always so excited when I receive messages like this one. I love to connect with other parents of children with special needs. I realize the importance of having a reliable support system, even if it is just one person to talk to who can understand what you are going through. We all need support and guidance on our journey, and I am honoured to be able to help in any way I can. Lucy-Anne, thank you for reaching out, and thank you for sharing your daughter's story. 

*****

Hannah is the middle child of three girls. My first pregnancy was very normal, including six months of all-day nausea. My daughter, Lily arrived via standard vaginal delivery, no epidurals, surprisingly average. We decided that we would like to have our children close in age, and wasted little time trying to get pregnant again. Our second child was born just two weeks shy of Lily's 2nd birthday. My pregnancy was again very normal, and delivery was easier than the first, with no complications. We were blessed with two beautiful and healthy little girls, and life was moving along as planned.

Hannah was about 3 or 4 months old when I started wondering why she wasn't reaching the normal milestones, but having a toddler in the house meant we were always busy and truly I wasn't worried. Hannah was a very happy baby even though we did have difficulties getting her to sleep and keeping her asleep. She had an older sister who kept her entertained and I tried not to compare their abilities.

At about 5 months, I expressed my mild concern to my GP about Hannah's development, and he referred me to a pediatrician just to ease my mind. Of course I was reassured by my husband and friends that everything was fine. We were living in Twillingate at the time. I was a teacher on maternity leave, while my husband worked away as a driller, and we both owned and operated a seasonal coffee shop in the area. Two weeks prior to our appointment with the pediatrician in St.John's, Hannah exhibited a behaviour that I had never experienced with any child, mine or otherwise. She was sitting in my lap while I was on the computer, and I had one arm under her armpits. She started to repeatedly slump in my arms, and then regain her muscle tone. I had no idea what was going on, but I knew something was very wrong. Like most worried parents, I immediately went to Google and typed in a description of what I was experiencing. I saw the words infantile spasms, seizures, epilepsy, brain development, and many other medical terms that I had never before concerned myself with. I immediately called my husband and began sobbing on the phone in panic mode, saying that we needed to get Hannah to the hospital right away.

The local hospital was more of a clinic than anything else. Most procedures required a trip to Gander, including having babies, but off I went to see the doctor on duty. He told me that I was overreacting and "worrying too much like many mothers do".  I was extremely dissatisfied with the response and treatment. Hannah even had another spasm in my arms while the attending nurse watched. I decided to try to calm down and give it some time. For the next 24 hours I recorded every strange movement Hannah made, and the next day I eagerly called the Janeway emergency room. After having a phone consultation with a nurse, she said, "If you are concerned you need to come here right away."  I then proceeded to call the office of the pediatrician that we were seeing in two weeks time. The very kind nurse assistant listened to my story, consulted with the doctor, called me back within a half hour and said to come in for a ten o'clock appointment the next morning.

It was about noon, in the middle of the day and I started immediately packing up my car with a medium sized black lab, my two year old, and my 6 month old. We had our entire house in boxes because we were supposed to be moving to a new house in a few days and my husband had to stay behind to deal with the chaos.

The next day everything seemed to happen at lightening speed, or at least that's how I remember it. We saw the pediatrician at 10:00 am, he called the neurologist at 10:15, we saw the neurologist at about 11:30, and by 1:00 we were admitted to the Janeway. Hannah was immediately started on anti-seizure meds to stop the infantile spasms, which had been confirmed by the neurologist. We were told that over the next few days we would have several diagnostic procedures done to attempt to determine the cause of these seizures. Meanwhile, a lawyer had to meet us in the Janeway lobby to sign the last documents for our new house, and my husband was scrambling to get a ride into St. John's as fast as he could.

On day two, Hannah had an EEG, an MRI, and a CT scan. My husband was by my side when the neurologist told us her diagnosis and ultimate cause of the seizures. Our little baby girl had an extremely rare brain malformation called Subcortical Band Heterotopia. Basically, the grey matter in her brain is accumulated in a band in the centre of her brain, and is diffuse throughout her brain. I like to describe it as a "cloudy brain". We were told that this condition often results in developmental delays in all areas, that she may not walk, and that she may not even be able to talk. The doctor said that because the condition is so rare there is a wide range of delays and it would be very hard to predict Hannah's rate of development.

It was like the Earth had been pulled out from under us. In that moment I knew our lives would never be what we had planned or predicted. I also knew that I would learn everything there was to learn and I would do everything there was to do to help my daughter grow and thrive. She was immediately put on valproic acid to treat the seizures, and they reduced significantly. In later months there were many days without any spasms. She continued to be a happy baby, but failed to meet many of her milestones. We proceeded with genetic testing to determine that neither one of us were carriers of the genetic spelling error that caused Hannah's condition. She was a rare gift, and strike of lightning.

Fast forward 8 years, and Hannah continues to struggle with seizures and has significant delays in all areas. However, she is walking, talking, and laughing every day. Some days are harder than others. We have to survive the days with repeated outbursts, screaming and temper tantrums, to get to the days were she laughs and hugs her sisters all day. We moved back to St. John's shortly after her diagnosis to be able to provide Hannah with as much therapy as we could in her early years. It definitely has had an impact.

Hannah has been on many different anti-seizure meds, but as she has gotten older the seizures have become more intense and harder to manage. We very recently started the ketogenic diet to treat her now intractable epilepsy. So far, we have seen a dramatic decrease in the number and intensity of the seizures, but it varies daily. We have high hopes that this intensely monitored diet will help Hannah to move through her day without being a safety concern.

Hannah has been on the diet now for 3 weeks, and we hope to get her back to a full day of school very soon. It is challenging to make the food portable because it requires measuring every ounce of food, while restricting the types of food shes able to have. Like most kids, Hannah has particular tastes and textures that she enjoys, adding more limits to the types of food she can consume.

As a family of 5, Hannah now has a 2 year old sister. We are very grateful for what we have and especially for how well Hannah as progressed. Hannah has taught us how to focus on the important things in life. We have learned to take one day at a time, and to accept that life doesn't always turn out as planned.

*****

Thank you again, Lucy-Anne. I am so glad that you felt comfortable sharing Hannah's story. I know first hand that your child's diagnosis can be a difficult thing to come to terms with, but once you begin to accept it, the weight begins to lift. You said to me, "It's funny how after 8 years I finally feel like I'm accepting that I am not like 'regular moms'. I guess I didn't want to make others feel bad for me, or always be the 'downer'."  Listen. We all have days of being the downer, and dammit.. we deserve those days! Our lives are challenging, there is no question about it. I just hope that in reading my blog, and the stories of other parents, you realize that you are not alone. We've got your back. 

I look forward to catching up with you over coffee (or in my case, tea!).


If you would like to be featured on Tulip Tales, please email me at: juliebrocklehurst@hotmail.com  Please include a brief description of your child and their condition, along with any pictures you would like to share. I will contact you with specific questions.

Thanks!
-Julie

Megan: Forever Dauntless

"We waited nine months to see her adorable little face. She had ten beautiful fingers and ten beautiful toes and was perfectly healthy when we brought her home. Then, a few days later our world came crashing down. Our little Megan Lindsey became extremely ill. She was continuously vomiting and getting weaker by the hour. We took her to the doctor seven times and each time they said it was just a virus. But it wasn’t. Megan lost over two pounds and weighed less than five pounds. After a week and a half she was finally admitted to the hospital. They did several tests, from CT scans to spinal taps. Then the seizures started. Watching her little blue eyes flutter uncontrollably and wondering if she was in pain just made our hearts ache. The nurse said to my husband Jay, "Now would be a good time to call your clergy." We were not ready to give up on her, however we took the nurse’s advice and prayed. I called my grandmother and asked her to pray for Megan as well, because her prayers were always answered.

Shortly after, we received Megan’s first diagnosis. The doctors told us that Megan had a bleed in her brain but she also had a blood clot. One problem could not be treated without interfering with the other. There was nothing they could do for our little angel. Her older sister Celia, who was almost four at the time, was inconsolable. She had waited a long time for Megan. On her third birthday she blew out her candles and wished for a baby sister. The next day, I found out that I was going to be a Mom again! Celia even named her, and Megan completed our little family.

Later that day, Megan was transferred to McMaster Hospital in Hamilton, Ontario. After more medical tests, we got her second diagnosis - Megan had suffered a stroke. We could not believe that a little infant could suffer so much in the first two weeks of her life. We were numb! Our tears were unstoppable and I don’t think I ever saw as much fear in my husband’s eyes as I did that day. He usually was the strong one but he was hurting just as much as I was.

Over the next few days, the doctors monitored Megan constantly and we refused to think negatively. Finally, we received our first bit of good news. The bleeding had stopped and the blood clot was getting smaller. We were ecstatic! Then they gave us the bad news. They were not sure if Megan had suffered brain damage and what her quality of life would be as she grew. We didn’t care. Our little girl was still breathing and that was all that mattered. My grandma’s prayers were answered.

After genetic testing, they also told us that Megan, Celia and my husband had a blood clotting disorder called Factor V Leiden, which probably contributed to Megan’s stroke. There was more to worry about but we would face it together. Our family was still strong as long as Megan was in it.

Immediately, Megan began therapy to help correct the increased tone in her left arm. We used to call it her 'little chicken wing' arm because she held it so tightly against her chest. She started taking medication for seizures and eventually outgrew her dosage and the seizures stopped. Everything the doctors said might happen didn’t happen. Megan was growing and developing like any other child! Everyone including her doctors were amazed by her progress, and she had many doctors from around the world visiting Sick Kids Hospital in Toronto, completely in awe of her determination and strong will. We never treated her any differently than our oldest daughter and we were determined to give her the best possible outcome.  

At nine months old, Megan began walking. It was a huge milestone for her that no one had expected to happen so soon. We knew she was a force to be reckoned with when she survived a stroke, but we never expected her to be this stubborn! She was imitating everything her sister did and nothing was slowing her down, but just before she turned two we discovered that her left leg wasn’t quite the same as her right. She couldn’t wiggle her toes on her left side and her left leg and foot looked a lot smaller than her right. She was off-balance and was always up on her toes. There was also increased tone again in her left arm. 

The official diagnosis came and it was one that truly scared us. Megan was diagnosed with cerebral palsy. Days later, Megan started physiotherapy and she never complained once. She was not going to let this get her down. As Megan grew, we knew she was not your ordinary little girl. The brain is a very complicated thing, but luckily for Megan, parts of her brain took on different functions that it normally wouldn’t. It completely remapped itself. The damage she had suffered as in infant didn’t affect her at all. The tests revealed that the scarring was there but she was showing no residual side effects. She learned how to read at age three, she walked early and was even evaluated by the psychology department at Sick Kids Hospital which determined she was gifted in many areas.

When Megan was nine years old, the seizures came back and we were devastated. They caused her so much physical pain and the migraines were unbearable. The seizures caused her leg and arm to get tight again and she had to start physiotherapy from scratch to correct it. This time she needed a brace for her leg, and she was not happy. The teasing at school began and the worst was watching her classmates imitate her having a seizure. It was the first time I ever saw my little girl so sad and she went into a depression. Even today, her biggest fear is that she will have a seizure when she is by herself.

Megan was introduced to the Heart and Stroke Foundation. They asked her to tell her story to different groups, including several local schools so that she could educate them on what it was like to live with her condition. She became an awesome public speaker and realized that for her it was the greatest form of therapy. Her confidence was coming back and the sparkle was in her beautiful blue eyes again. She went on to win the Cambridge Children’s Hero award the following year for her work and we were thrilled. She even gave a speech for the Stroke doctors at Sick Kids Hospital for Family Stroke Day and they were all in tears by the time she finished. They finally could look beyond the medical terminology and connect to her as a person living with a disability. It was truly a remarkable thing to witness.

When Megan was given a second chance at life, we were too. We completely changed the way we lived and decided we wanted to be better people in our community. As a family, we made it a rule that we all had to help out at least one charity each year, but of course our girls did many more. They learned at an early age that there is no greater reward in life than giving back and helping others. 

Four years ago we moved back to Newfoundland and both Megan and Celia have excelled. Celia is now studying Business Management and Marketing through the College of the North Atlantic and Megan is a straight A student at O’Donel High in Mount Pearl. 

Last year, with her social action team at St. Peter’s Junior High, Megan got involved with an organization called Free the Children, and was asked to be a student speaker to kick off their 'We Create Change' Campaign Tour Launch. It was an amazing speech that was streamed live across the world. (You can see it below).

Megan also won the Johnson Horizon Leadership Scholarship and became Easter Seals Ambassador 2014. Through Easter Seals, she learned how to play wheelchair basketball and was invited to the camp tryouts for the National Canadian Under 25 Woman’s wheelchair basketball team. She declined this year because she felt she was not ready emotionally for such a big commitment, although it is a dream she may pursue down the road.

We discovered that Megan had a beautiful singing voice and with the help of her amazing vocal coach Justin Nurse, she has become quite the performer. He believes in her and never lets her doubt herself. He challenges her, and because of that, 'Megan’s Night of Music' was born. Last year, Megan hosted and performed at her own concert and raised over $1200.00 for Free the Children and Easter Seals.

Easter Seals has supported Megan and our family since we moved to Newfoundland. They are our extended family and have helped Megan in too many ways to count. Last year they introduced her to Sean McCann, an amazing person who has become quite the supporter of Easter Seals. She was honoured to meet him.

Megan wrote her first song called Forever Dauntless and our family friend Jon Pike from Everglow helped her put music to her beautiful lyrics. Although the song was originally written about a character in a book, it actually reflects Megan’s life and I am certain she wrote it thinking about herself. Easter Seals sent the song to Sean and he asked Megan to perform it with him at the Easter Seals gala last December. Megan was so humbled to perform on a stage with such a musical inspiration.

And now she is at it again! Her second concert, called Megan’s Night of Music 2: “Forever Dauntless” will be held at the Husky Energy Easter Seals House on May 2, 2015 at 7:00pm. The acts this year include Megan, Justin Nurse, Erin Winsor, Everglow, Rex Goudie, Brianna Goss, Michael Duffy, and some of the music therapy participants. With the help of her big sister Celia, this year’s concert will be even bigger and better. It is so heartwarming to see my girls work together to give back to an organization that has given so much to our family. The one thing that I admire about Megan the most is that she never lets her disabilities get in the way of achieving her goals. She has been seizure-free and off her medication for over five years now and we are optimistic that the seizures won’t return. Today, she still has physical challenges and lives with chronic pain and migraines but she is learning to cope with help from her amazing pain therapist at the Janeway. She has learned to love and accept the person God wanted her to be and she absolutely embraces life and never takes a single day for granted. She is truly the strongest person I have ever met and I am so proud to be her Mom!"

*****

Thank you, Brenda for sharing Megan's story. She is an incredible young woman, and a remarkable talent. I am so happy that our paths have crossed. Megan said in her speech, "I believe that each of us has the power to enrich the lives of others, no matter our differences." Her words are motivating and inspiring, and she makes me want to be better and do more. "Searching to belong, eager to create change, and longing to make a difference." We are more alike than I realized.

Go see Megan’s Night of Music 2: “Forever Dauntless” on May 2nd!

Purple Day for Epilepsy

Every year, on March 26th, people around the world wear purple to show their support for those with epilepsy and to raise awareness about the condition.

Approximately 50% of children with cerebral palsy have seizures - episodes in which abnormal nerve activity disturbs the functioning of the brain. We don't typically say that Brennen has epilepsy, but that he has a seizure disorder along with cerebral palsy. Seizures are so common among children with cerebral palsy because brain injuries provide focus for abnormal nerve impulses to occur. Children with quadriplegia (like Brennen) or hemiplegia are most likely to have seizures.

Brennen has been having seizures for quite a few years, and though we have gotten used to them, it is never easy to watch your child go through something so traumatic. Brennen's seizures are usually short, lasting just a minute or two, and he is very sleepy afterwards. We have tried a number of different medications to try and control his seizures. Many have had side effects that we didn't like, and some just did not work for him. It is a bit of trial and error to get the proper therapeutic dose, but we seem to have found the right prescription for Brennen, and his seizures have been less frequent and less severe. I used to get texts from his teacher at school saying, "Brennen just had a little seizure. He is doing fine now, in someone's arms having a cuddle and a rest." I haven't gotten a message like that in a few months. (That being said, he had a small seizure yesterday at breakfast, and missed school because he slept the whole morning). Brennen is certainly not seizure-free, and probably never will be. We have accepted this as our 'normal', and we try to make him as comfortable and as content as possible. 

Brennen is followed closely by a pediatric neurologist, and has EEGs (Electroencephalograms) regularly to measure his brain wave patterns. EEGs are painless and non-invasive, and typically take about an hour. They also usually videotape his EEGs to try and record a clinical seizure on tape.

Brennen has only ever had one seizure that lasted longer than five minutes. It was not stopping on its own, so I had to administer emergency medication (Ativan), which we keep on hand "just in case", and we took him directly to the hospital. He had come around by the time we got there, so they just checked his vitals, monitored him for a couple of hours, and sent us home.

As scary as it is, anyone can have a seizure at any time. One in every ten people will have a seizure at some point in their life, and over 10,000 people in Newfoundland & Labrador have epilepsy right now.

A seizure may appear as a brief stare, unusual movements of the body, a change of awareness, or full body convulsions.

Seizure First Aid

First Aid for seizures involves responding in ways that can keep the person safe until the seizure stops by itself.

Here are a few things you can do to help someone who is having a generalized tonic-clonic (grand mal) seizure:

• Keep calm and reassure other people who may be nearby.
• Prevent injury by clearing the area around the person of anything hard or sharp.
• Ease the person to the floor and put something soft and flat, like a folded jacket, under his head.
• Remove eyeglasses and loosen ties or anything around the neck that may make breathing difficult.
• Time the seizure with your watch. If the seizure continues for longer than five minutes without signs of slowing down or if a person has trouble breathing afterwards, appears to be injured, in pain, or recovery is unusual in some way, call 911.
• Do not hold the person down or try to stop his movements.
• Contrary to popular belief, it is not true that a person having a seizure can swallow his tongue. Do not put anything in the person’s mouth. Efforts to hold the tongue down can injure the teeth or jaw.
• Turn the person gently onto one side. This will help keep the airway clear.
• Don't attempt artificial respiration except in the unlikely event that a person does not start breathing again after the seizure has stopped.
• Stay with the person until the seizure ends naturally and he is fully awake.
• Do not offer the person water or food until fully alert
• Be friendly and reassuring as consciousness returns.
• Offer to call a taxi, friend or relative to help the person get home if he seems confused or unable to get home without help.

Here are some things you can do to help someone who is having a seizure that appears as blank staring, loss of awareness, and/or involuntary blinking, chewing, or other facial movements.

• Stay calm and speak reassuringly.
• Guide him away from dangers.
• Block access to hazards, but don’t restrain the person.
• If he is agitated, stay a distance away, but close enough to protect him until full awareness has returned.

 

Consider a seizure an emergency and call 911 if any of the following occurs:

• The seizure lasts longer than five minutes.
• The person has another seizure soon after the first one.
• The person cannot be awakened after the seizure activity has stopped.
• The person became injured during the seizure.
• The person becomes aggressive.
• The seizure occurs in water.
• The person has a health condition like diabetes or heart disease, or is pregnant.

On March 26th, wear purple in support of all people who live with epilepsy.

Help eliminate the stigma and fear associated with epilepsy and brain trauma!

The Canadian Epilepsy Alliance has a wonderful new website with lots of information on epilepsy and seizure disorders. 

Epilepsy Newfoundland & Labrador also offers a range of programs and services to meet your needs concerning all aspects of living with epilepsy.