Team Sydney 2012 in Providence, RI
The past two years, I have run a half-marathon each summer for the Dravet Syndrome Foundation, part of their Race Towards a Cure RACRE (Research and Cure Refractory Epilepsy) series. In 2011, I was the solo member for “Team Sydney.” Last year, my husband Paul, and two other friends joined me to complete “Team Sydney” for 2012. Both of these runs were completed in Providence, Rhode Island. This year, we were hoping to form a Team DSF Pittsburgh, but unfortunately, the race we were to run was canceled. So for 2013, “Team Sydney” will be running in Philadelphia, PA on September 15th.
This year, it sounds like “Team Sydney” will be made up of at least 6 runners… myself, Paul, Sydney’s Aunt Anna, a co-worker of mine, Kristy Clements & her husband Jayson, and a new friend and fellow seizure mom, Becky Kmetz. Our team grows each year!!! Each runner pledges to raise $500 for the charity, Dravet Syndrome Foundation. So with six runners this year, Team Sydney will need to raise a collective $3000.
Sydney with Mr. Mike Sugg as "Toby Keith"
Last night, we attempted to raise that total amount with our fundraiser “Concerts 4 A Cure.” A new non-profit organization Ray of Hope did an awesome job organizing and running this benefit in Sydney’s honor. Unfortunately, our turn out was poor, so we did not get to raise any of the funds for the DSF.
Our event consisted of a dinner, DJ, basket raffle, various vendors, and to top it off, four country concerts! Three of our performers were Tribute Artists portraying several of today’s top country stars, including Tim McGraw, Kenny Chesney, and Toby Keith. All three of these artists were fantastic. You wouldn’t have known you weren’t seeing the real deal! If you ever have the opportunity to go see any of these guys live, I can tell you that you will not be disappointed! Our fourth performer happens to be a former student of mine. Miss Kourtney Jean Zufall, who won our local Froggy station’s Idol contest. She was absolutely beautiful and sang like a dream. I can tell you one day soon she will be making it big, and I will be able to say I taught her science when she was in ninth grade!
Mr. Joe Grooms ("Kenny Chesney") singing to Sydney & nurse Debbie
With such an awesome evening, it was too bad we didn’t pull in a bigger crowd. I am continually puzzled as to why our fundraising events don’t draw in people. For the most part, I think it may be as simple as folks not understanding the severity of Dravet Syndrome. Of course, no one has ever heard of it, with it being such a rare disease. Other than a few neurologists, I have not yet come across a doctor who knows what it is. Every time I begin to explain Dravet Syndrome to someone by mentioning seizures, I immediately see people lose interest. I don’t know if it is because seizures are such a frightening/ugly thing to behold and folks would rather not talk/hear about it, or if it’s because everyone knows someone who has seizures and “so & so is fine” or (my favorite) “my dog has seizures.” Oh folks, Dravet is SO MUCH MORE than seizures. Not to mention with Dravet Syndrome, these are seizures on a scale that you can’t even imagine.
Everyone knows that when an adult or child is diagnosed with cancer, it is devastating news. Since we all understand cancer as a diagnosis, allow me to draw a few parallels between cancer and Dravet Syndrome, to try to show how devastating Dravet is as well. One thing about a cancer diagnosis is that often cancer can be cured, be it through operations, chemo, or radiation no doubt though the road is a hard one. With Dravet Syndrome, THERE IS NO CURE. We don’t have that chance. Do our kids face operations? Yes; many throughout their lifetimes. Our kids need ports inserted because their veins are shot; VNS devices implanted in their chests/necks in the hopes of shortening some of the seizures and then future surgeries to change the batteries; multiple corrective surgeries on their lower limbs because as they approach their second decade of life, the low muscle tone they suffer from begins to break down their feet & ankles; brain surgeries to try to shorten or halt certain types of seizures…and the list goes on.
Mr. Allen Scott ("Tim McGraw") and Sydney
Another similarity between cancer & Dravet Syndrome is that, in both cases, the individual faces death. If the cancer is such that death will occur, often there is a time table with it. Kids (or adults) with cancer can plan to do certain things or visit with each other and at least express their love or mentally prepare for what is to come. With Dravet Syndrome, any moment could be our last together. No matter how hard my day with my daughter has been, I always try to remember as I am laying her down in her bed, that this could very well be the last time I look at her alive. I always try to tell her I love her at bedtime, just in case it is my last opportunity to do so.
You see our children have a fairly high rate of passing away from something called SUDEP (Sudden Unexplained Death of Epilepsy). Dravet Syndrome affects channels (or pathways) in the brain. Some of these same channels carry the instructions that keep our lungs breathing and our hearts beating. In a child with Dravet Syndrome, sometimes while they are sleeping those instructions do not get passed correctly and the child dies because their heart simply stops beating. Sad to say, but if we lose Sydney, I’m hoping for SUDEP to be the cause because nothing could be harder to watch than to see your child seize for so long that they must be placed in a coma to try to stop the seizure, only to find that their brain was too severely damaged for them to ever go on normally again.
Our children are developmentally delayed (often labeled as retarded- oh how I hate that word) and have behavioral issues (many end up with a dual diagnosis of Autism, but in our case, the autism is a side effect of the Dravet). Their delays make it difficult to explain to them why they are having seizures or need more blood drawn or need to have another surgery. Sydney has recently learned to say the word “Why?” Oh, how it breaks my heart, when she is cluster seizing while crying & asking me “why?” in between each seizure.
Sydney had a pretty bad night tonight. She began seizing around 6 p.m. with her typical myoclonic jerks. You might have a slight idea what a myoclonic jerk is if you have ever jerked suddenly as you were falling asleep. That motion is similar. In Sydney’s case, her myoclonics tend to cluster, which means she has them back to back to back sometimes lasting for hours. Tonight, she was jerking so hard she was being thrown backwards or knocked to the side EVERY 10 SECONDS and this lasted for OVER THREE HOURS, with her crying and screaming in between each one. A neurologist would advise us to give her her rescue meds to stop the clustering. But WE WOULD HAVE TO USE THEM EVERYDAY, which would render them ineffective after a while for when we truly needed them. So, the seizures go on…
Sydney's meds...oh, we do THIS twice a day!
Most likely, Sydney was so bad tonight, because we are trying to wean her off one of her rescue drugs (Ativan) that she has needed to be on now for over a month due to various illness. The drugs they place our kids on are horrible & have horrible side effects. Sometimes the drugs turn them into drooling zombies. Sometimes the drugs take away their desire to eat. Sometimes the drugs make their hearts race and make them so hyper they can’t calm down or fall asleep. Sometimes they can’t wake up no matter what you do. Sometimes the drugs make them scream and scream and scream until as a parent you think you are going to lose your mind listening to it and knowing no matter how much comfort you offer, you can’t do anything for them. None of the drugs can/should be stopped cold turkey and it is incredibly difficult to wean their little bodies off these drugs. I have been trying for years now to wean various drugs off of Sydney. Take my word for it…drug weans are horrible.
Sydney knocked out from a big seizure
Cancer drugs (chemo) are horrible too. Sure, they kill the bad cancer cells, but they also kill the healthy cells of your body. This is why folks who are battling cancer become ill and immuno-suppressed, meaning even a common cold is very dangerous for the individual. Our kids are immuno-suppressed too. They seem to get every little thing that goes by or can suddenly spike a temperature out of nowhere, which can bring on a highly dangerous form of seizure known as status epilepticus. Sydney has had TWO bouts of unexplained fevers, which led to Status Epilepticus & two lifeflights to Pittsburgh in JUST THE LAST MONTH!!! Her last status seizure lasted 45+ minutes before they could get it stopped. As a parent, you are praying every damn second of that time period…
There is so much more to Dravet Syndrome that I haven’t even touched on in this blog, but I know it is getting long. Let me wrap it up by briefly explaining how severely Dravet affects the normal family life… Because of Sydney’s immunosuppression and her various seizure triggers (light, heat, cold, excitement, etc…), she (and we) rarely leave the house. We go to work while a state-provided nurse stays with our daughter in our home. We are only approved for nursing care for while we are at work, so we must come straight home to begin caring for Sydney. Our house is kept very dark (dark wood blinds in almost all our windows) and cool. I am a teacher so I am off in the summer, but rarely can I go outside and enjoy the summer weather. Even if Sydney takes a nap, I can’t sneak outside for she must be constantly watched in case a seizure should begin. I can’t simply run to the store to pick up bread. The fluorescent lights in stores are brutally hard on Sydney. If I decide to risk it, then I must bring her oxygen and emergency kit full of meds, stopwatch, suction device, etc…in case she should happen to have a seizure while we are out. Often, if we take her out somewhere for any length of time, we have pre-medicated her with Ativan to hopefully keep the big seizures away. My husband Paul & I can’t go out to a concert, hockey game, or even dinner easily. We can’t simply call up a babysitter because dealing with our seizure kid is too terrifying and stressful for people who aren’t used to it. Even my family is terrified to get stuck watching Sydney for more than a few minutes.
Aaron, not happy about being asked to hold Sydney when she was having a bad morning & Mommy needed to get all Sydney's medicines ready. He'd much rather go play Pirates!
Our five-year old son’s life is also greatly affected. He is constantly asking me to play either outside or somewhere in the house that I can’t take or monitor Sydney. I hate telling this little boy “no” all the time! I’m constantly having to come up with reasons (other than Sydney) as to why I can’t do as he asks (because I don’t want him to grow up resenting her anymore than he may already). Her constantly screaming & crying when she is clustering with her seizures is more than he can handle many days. He gets incredibly frustrated & angry anymore when she is crying. I hate that he rarely gets a break from it. He has witnessed many scary events with his little sister. I can’t tell you how many times he has asked me “is Sydney going to die?” This picture was drawn by Aaron about a week after Sydney’s last status seizure. Basically, he drew the scene as he remembered it. I did not ask him to do this; the drawing was totally unprompted.
Five-year old Aaron's drawing of Sydney's seizure
The scene shows the ambulance outside the house, with its lights flashing. The two paramedics (one of which is holding Sydney) and Mommy are running down the steps to the ambulance. And as always, Daddy & Aaron are left behind in the house. When I asked him what was in the sky, he told me they were meteors. It took me a few weeks to realize the significance of meteors in the sky. A few months ago, he told me he no longer wanted to watch a cartoon on PBS called “Word Girl.” Apparently, there was an episode on that dealt with meteors and it scared him. He told me at the time, he kept having nightmares about the meteors and Word Girl. I think the meteors being present in his drawing are showing his fear or how scary he finds this whole situation to be. Sad…
Dravet Syndrome Foundation- Steps Towards a Cure
Ok, so I did not give you this peek into our lives to make you feel sorry for us. Instead, I and my “Team Sydney” teammates are going to be asking for your help in the next two months. Help us fight Dravet Syndrome by raising money for the Dravet Syndrome Foundation to further fund research for this severe condition. Any monetary gift is tax-deductable. You can use this link to donate (Dravet Syndrome Foundation) or visit www.dravetfoundation.org and look at “Annual Events,” then “DSF Steps Towards a Cure- RACRE series,” “Philadelphia September 15th,” & then click on “Team Sydney.” You can either donate to the entire team or if you wish, pick a specific runner on the team. We currently have $845.00 raised and our goal is $3000.00 by September 15th. Please consider donating. Any small amount will help push us closer to our goal. Thank you for your help!