Haley Is My Hero

Haley was born on August 20th.  It was an easy, healthy pregnancy and uneventful  delivery.  Haley developed normally and was a happy little girl.  On January 29 around 5 PM, the lives we knew forever changed. At the time we did not know how much they would change.  At five months old (in the picture to the right, she is five months old)  Haley experienced her first grand mal seizure on her changing table.  I was taking her temperature rectally because she looked flushed and the ear thermometer read 96.3.  While I was taking her temperature she started to convulse and her eyes rolled back in her head.  My husband and I rushed her to the ER which was about eight minutes from our home. She did not stop seizing for over 25 minutes.  The ER staff finally got the convulsions under control and ran numerous tests on our little baby.  Fortunately the pediatrician on call, Dr. Barbara Kahler, was already at the ER and was there to answer any and all questions.  By the time we left the ER it was close to midnight.  Haley was diagnosed with an ear infection.  We were told that the seizures were probably febrile (seizures brought on by a temperature).  We were told that febrile seizures were common, but not at this young age.   

 So life went on….Haley continued developing normally.  Then on my first Mother’s day weekend we were rushing back to the ER.  This time she started to seize while she was in her highchair at a friend’s marina.  The seizure started out to be generalized (involving the entire body) then focalized on her left side.  This seizure lasted over 45 minutes.  After stabilizing Haley, Dr. Kahler and the ER staff decided to have her transported to Richmond, VA to the Medical College of Virginia (MCV) hospital.   We had to wait for a pediatric ICU team ambulance to drive from Richmond (an hour and twenty minutes) to come and get us.  I never in my wildest dreams thought that motherhood would bring these events.  We arrived at MCV at 2 AM and were admitted to a room.  They ran tests on Haley all night including a spinal tap.  The next day there were more tests.  We ended up staying all weekend at the hospital and coming home not knowing any more than it was another febrile seizure.  After this second seizure our lives would never be the same.  For me, going anywhere with Haley was stressful.  

 I was constantly worried about if she had a seizure in this setting or that setting, how would I handle it?  Any noise that sounded like a seizure made me jump and my heart was in my throat.  I was always on pins and needles.  For the rest of 2001 Haley would have three more tonic-clonic seizures.  None of these thankfully went into status epilepticus (a seizure lasting over five minutes or repeated seizures without time to recover to consciousness).  All of the seizures in that first 12-month period were traced to a fever caused by an ear infection (all but one, one was a Urinary tract infection.) 

 After our hospitalization we saw the attending neurologist at MCV.  I was not pleased with him for many reasons and asked for a second opinion before we started Haley on Phenobarbital.  Our second opinion took us to Children’s Hospital of the Kings Daughters in Norfolk, VA when Haley was 15 months old.  We saw Dr. Ralph Northam (now Virginia State Senator Ralph Northam, M.D.).  He was wonderful.  He had great bedside manner and thought we should wait for medication.  To him at that time Haley was developing normally and was a bright little girl with curls.  She could speak in sentences and almost say her ABC’s.  She also knew most of her letters, numbers and shapes.  Dr. Northam was the first to prescribe Diastat Acudial rectal syringes.  This is a rectal valium delivery system that could stop the seizures if they lasted over 5 minutes.  What a blessing that was for us, but it came at a great expense!  When we started using them in 2001 we paid about $86.00 for two syringes with 5mg of medication.  Now in 2008 we pay $115.00 for two syringes with 10mg of diazepam.  That is our co-pay with insurance.  Last year, 2007, we hit our drug cap in July due to the number of syringes we used!  This year if we hit our prescription cap in June and had to pay $175 for ONE syringe!   We have administered the syringes at least 6 times a month on average.)  Even though the medication in these syringes cost pennies the pharmaceutical company behind Diastat Acudial feels justified in charging the price they do because “they are saving you a trip to the ER”.  

 From 2001 to Fall of 2003 we were with Dr. Northam.  He followed her carefully screening her with EEG’s and MRI’s regularly.  He even got us in a study for Trileptal to get a video EEG when she was about 2 ½ years old.  The longest Haley has gone without a seizure since she was born was during our time with Dr. Northam.  She went four months in 2002 without one convulsion.  This happened after she had tubes put in her ears.  We thought we had finally solved the problem of the febrile seizures…get rid of the ear infections.  During this time with no seizures our guard came down.  Bobby and I decided we would like to have ONE more child.

They say that if you want to make God laugh, make plans.  So we were planning on one more child and Haley not to have any more seizures.  Right about the time I got pregnant, Haley’s seizures were back.  The four months seizure free was too good to be true.  I will never forget the week I term the medical week from “heck”!  That week we went to confirm the pregnancy on a Monday.  The sonographer was not there so we could not have an ultrasound and I was asked to return later in the week.  Since Haley had a “well child” visit on Wednesday with her pediatrician we scheduled the ultrasound the same day.  Bobby would not go with me because he had to work.  (All of the appointments were in Richmond, an hour an twenty minute drive)  That night Haley had a seizure and for the first time in over a year and a half we did not take her to the ER.  We stayed in touch with her doctor by phone overnight and went back to Richmond (Mechanicsville) the next day to make sure Haley was “well” for her “well-child” appointment the next day, August 20, 2002, was Haley’s second birthday.  It was determined by the rash on her back she had a viral illness and we could come back tomorrow for her scheduled appointment.  On Wednesday, Haley and I jumped back in the car and drove back to Richmond to do my ultrasound, her doctor’s appointment, and shopping for her weekend’s birthday bash at our house.  We went to my appointment first.  I was very stressed because they were running late and when it was my turn the tech was taking soooo long!  When she turned the monitor to me and asked me if I “would like to see your baaaabies???…wait…let me look for a third….”  It was then, I realized why.  I was in shock and said she better NOT find a third!  Twins do not run in either of our families.  I was so stunned by the news, I was halfway across Richmond when I realized I did not stop and check out after my appointment.   

 Another part of the making plans makes God laugh is I never wanted three children because I did not want a middle child.  Well, I got three and there is not really a middle child, LOL!  I decided not to tell Bobby over the phone about our expanding family.  My mom asked me to video tape his response because she wanted to see it firsthand.  (I did and surprisingly enough, that video made it onto America’s Funniest Home Videos and placed third.  It was an awful trip!  Haley ended up in Children’s Hospital in Los Angeles and never made it to the taping.)  On Friday, Haley had another seizure due to an ear infection.  This was the first time she had two seizures in week.  Looking back that was nothing; in the years to come she would have over 20 grand mals in one day!  She did go to the ER with that seizure.  Then on Saturday was her big birthday bash and she broke her arm, back to the ER we went.  As one of my clients said to me after that week, “God gives you two more children and you go and ‘break’ the one you have!”  We felt like the world’s worst parents!  During this week we also called Dr. Northam and requested Haley be put on medication…we could not stand the uncontrolled seizures any longer!

 At two years old, Haley was still developing normally and was ahead of her peers in many ways.  She could write her name and knew all her letters.  She knew most of her shapes and numbers.  The one number she knew the most was “24”…which to Haley is, “Jeff Gordon, my favorite racer!”  They way she wrote her name back when she was two is still about the same way she writes it today, each letter is all over the paper, sometimes backward, sometimes not.  It was not very noticeable that her learning slowed down.  Dr. Kahler, her pediatrician, assumed it was due to the medications she had been on and she had been on a bunch.  In August 2002 she started Tegretol, that did not work and we went to Phenobarbital, Depakote, Tileptal, Topamax (which made her lose her speech) Lamictal (which made things worse)!  Up until we did Lamictal we were on a mono-therapy drug program, but while on Lamictal we added Keppra.  Since then we have always had more than one drug.  Haley has been on almost everything available on the market!  The other drugs that have been tried and have been unsuccessful are Tranxene, Zonegran (another one that made her lose her speech), Dilantin, Ativan (as a daily drug), Felbatol (this one has a side effect of Aplastic Anemia, scary!)  Felbaltol was the first medication that the doctor actually asked us to think about prior to prescribing.  This is due to the fact the medication was pulled from the market in the 1990’s.   Keppra made our very mild mannered, affectionate child was sent home from school one day early due the affects of this medication.  She hit children in her class with scissors, sprayed the plant spraying water bottle around the class and then scrubbed that area using the nail brush, and she was “trying to put pencils (pointy end first) into her belly”.  All of this was truly out of character for Haley.  Once the drug was removed our loving, affectionate little girl is back! We have constantly juggled medications.  Presently we are back to Depakote, Felbatol (which causes Haley not to eat) and Klonopin also called Clorazapam.   Still we have little to no control.

 In November 2003, we left Dr. Northam.  He referred us to Dr. Eric Kossoff at Johns Hopkins.  We were bound and determined to get Haley on the Ketogenic diet which is a high fat , low protein, low carb diet that is very, very strict.  Since you have to be hospitalized for a week to start the diet there was a waiting list.  However, Dr. Kossoff offered us to be in his Modified Atkins Diet study.  It made sense for us to try this stair step approach to changing Haley’s diet.  So we tried the Atkins diet, but to no avail.  Haley was the only child who left the Atkins study to go to the Ketogenic diet.  Hindsight, tells us that even if the diet was working we would have not known because we had been put back onto Lamictal by Dr. Kossoff.  He insisted we try it again, even when I said that the last time we were on that drug the seizures increased as the dosage increased.  All he said to me was…that is not a side effect of this drug!  In September 2004 after returning from our trip to America’s Funniest Home Videos, we went to Johns Hopkins and started the Ketogenic diet.  At first it looked like it might be doing the trick, but unknown to Dr. Kossoff and us we had stacked the deck against us.  Again hindsight is 20/20!  Haley was on Traxene, Dilantin, and Lamictal at the time.  The Dilantin and Lamictal are known to make kids with Haley’s condition worse.  The Tranxene is valium-like drug and prevented rescue drugs from working to stop the seizures.  With this in mind you will understand why from January 2005 to April 2005 we spent one day week in the ER with uncontrolled seizures (up to 30 one day), status epilepticus.  We had many hospitalizations and even hit our ambulance cap that year.  The routine was the same…we would go into the ER, they would call Dr. Kossoff, Haley would be loaded with Phenobarbital, and send us home or we would be sent by ambulance to Norfolk, VA.  Then one week later we would be back in the ER again.  We figured out that she might be going through Phenobarbital withdrawal and so we started trying Dilantin, if we had only known the damage we were doing….

 By April 2005 Haley’s delays were very apparent.  She could not walk with a normal gait; she drooled, and was extremely lethargic.  Basically our bubbly, beautiful, happy child was a zombie.  Bobby and I were a mess too!  With all of this going on we had twin boys that had been born 7 ½ weeks early and were getting little sleep.  In retrospect, I wonder how we survived! When we finally left Dr. Kossoff in July 2005, we discovered Haley’s drug levels were 2-4 times the therapeutic dose.  Our time at Hopkins was the worst experience during this whole journey.  My biggest complaint is that this doctor would never listen to the other “M.D.” in the room, the “mommy doctor” who knew the patient better than he could ever imagine.  If he had listened, we may have gotten a diagnosis earlier. 

In June 2005 we were already getting our “fourth” opinion.  We were back where we started, MCV in Richmond.  We saw Dr. Jack Pellock, the head of the pediatric neurology department, who decided, “Let’s start all over.”  We were off to do more testing!  So within three months time we were had a MRI and a 72-hour video EEG.  After our video-EEG, a very non-compassionate doctor read off our options as if he was reading the “day’s special” at a restaurant.  The first option is to revisit drugs that she has been on before, the second option is Vagus Nerve Stimulation surgery (implant a device like a pace maker to the vagus nerve), and the third option is a corpus colostomy (where they would sever the hemispheres of her brain up to 2/3.  This would not stop the seizures; it would just prevent them from spreading to both sides of the brain.)  Needless to say, option number three did not sound like a good option to me.  So we went forward with option number one.  We revisited Depakote and then we tried was the questionable medication, Felbatol.  

 Haley continued to have seizures.  From the time she was two years old, one of the triggers for her seizures seemed to be any change in temperature either internal or external.  Fevers were still the biggest culprit for sending Haley into convulsions.  However, if she ran around and overheated she would have a seizure.  This prevented her from being in most activities.  She would also seize if she had a sudden chill, like when the wind came across the water on a cool day, down she would go into a grand mal.  Since we live on the water near the Chesapeake Bay this was a daily issue.  Even getting out of the bath tub would send her into a seizure.  In the summer of 2007 Dr.  Pellock was scratching his head, he said it has been two years, let’s go back and retest her and dot all our “I’s” and cross all our “T’s.  This time he added a genetic test to the repertoire.  He asked me to go the internet and look up SCN1A mutation and SMEI (Severe Myoclonic Epilepsy of Infancy).   When I got home I immediately went to the computer and looked it all up and for the first time it was like reading Haley‘s autobiography.  We had the genetic testing done in August and in September 2007, after six and a half years of uncontrolled seizures we finally had a positive diagnosis.  Haley has Dravet syndrome (Pronounced “dravay“).  

With the diagnosis of Dravet came peace.   We knew "why" she had seizures, but we still do not know "how" to stop the seizures".   With Dravet syndrome there is not known cure.  There are no drugs known to help these children.  Many of the drugs can make the seizures worse.  We experienced that over and over with Lamictal and Dilantin.  We are now waiting for the next drug to gain FDA approval.  When the IDEA League (The International Dravet syndrome and Epilepsy Action League) contacted the National Organization of Rare Diseases (NORD) to see if they would pick up a drug from France as an orphan drug for Dravet syndrome, NORD told them they had never hear of Dravet syndrome.  Guess we must be really rare!  Just last month, with the determination of many IDEA League moms and the assistance of KM Pharmaceuticals, NORD approved Orphan status of Stiripentol for Dravet syndrome.  Now the IDEA League is headed to the FDA for approval.  It is amazing what a group of dedicated and passionate parents can do together.  We are called "Tiger Parents".  (A father from Greece wrote a poem with that title you can read it at "I wish I said that.." link at the top of the page.)  The IDEA League has been our lifeline.  Through their family forum we have gained so much knowledge and support.  Life would be much more difficult without them. 

Until a new drug is approved by the FDA (our neurologist estimates that wont be for another year), Haley will continue on a multi-drug therapy.  Presently she is on a combination of three different drugs.  Despite the medications, Haley continues to have almost daily seizures.  Her seizures have evolved into primarily nocturnal seizures.  The majority of them happen in the early morning between 4-6 PM.  Needless to say, sleeping soundly is rare around our house.    Despite the convulsions and delays, Haley continues to be a loving, affectionate and very compassionate child. 

On January 16, 2008, Stephen Hollister of Tustin, CA. posted on the IDEA League website’s family forum an idea for a fundraiser.  He posted about a contest, Parade Magazine’s America’s Giving Challenge”, co-sponsored by the Case Foundation.  The challenge was for charities to create a “badge” and have the most individual unique donors.  The four charities would win a $50,000 grant.  Stephen thought it would be doing well if we had at least 700 people donate to our cause.  Never in his wildest dreams did he think the IDEA League would come in the top four, after all we were entering the challenge with only 16 days left in a 49-day competition.  I was intrigued by the challenge and after a couple of days of watching the numbers increase in very small increments, I decided to do something more.  I started with an email with no pictures to close friends and family.  With help from my dad, I refined my original email and included pictures of Haley, and WOW!, did that email travel to others outside my circle of friends.  I was surprised by the email response I received from as far away as Iraq.  I had three different soldiers contact me and wish us well and let me know they would be praying for us.  I was urged by an aunt of a friend to go to our local paper with the story.   On January 24, 2008 with one week left in the competition our little paper ran the story on the front page with a picture of Haley “above the fold”.  The response from the article was truly heartwarming.  By the weekend, Haley had a webpage that is run by an individual employed by my local internet provider, a woman pledged to provide t-shirts for all the Dravet families that were going to walk in the National Walk for Epilepsy in Washington, DC, and an individual we never met (who has property in our small town, but lives outside of D.C.)  offered to give us air miles if we ever needed to travel for medical needs or just needed a vacation!  There were many other blessings both tangible and intangible that came from the challenge.  As one friend wrote to me about the whole giving campaign, “it restored her faith in people again”.  So many people wherever we went in town were watching the IDEA League’s numbers on the challenge board.  There were also so many people watching that we did not know…the emails had made it to a soccer website posting board and a sports fisherman posting board.  During the competition the world did get a bit smaller.  The final day was January 31st and the contest ended at 3:00 PM.  It was so incredible, by 3:00 PM the IDEA League, the small charity group with fewer than 300 members came in first place with over 2900 individual donations (unofficially ~ they needed to rule out any duplicate donations.  On February 21st the contest was finalized and although we did not maintain first place we still were in the top four and secured one of the grants.  After the competition, the president of the IDEA League did report that the IDEA League raised over $75,000 from over 3400 individual donations.  The numbers of donations was higher by her standards because some individuals did not feel comfortable making donations online and mailed them in. There were others that made donations directly to our website and not through the appropriate venue.  The whole competition helped at least this parent feel a part of the IDEA League and empowered to do something for my child’s cause.

Perhaps the biggest surprise and the most heartwarming result of the Giving Challenge came from a friend and neighbor of ours.  On a Wednesday morning I was going to the gym before the sun came up and I passed by our neighbor, David Nova’s house.  David is a part-time professional sport fisherman.  I had heard he was getting a new boat and there it was in front of his house as I passed by.  In the predawn light, I was trying to admire the new paint scheme, when something familiar caught my eye….it was the IDEA League logo!  What??  Could I be seeing things???  I knew David was up to something on Haley’s behalf because his cousin’s wife contacted me right after the giving challenge ended asking if Haley’s picture could be used for  national  and maybe international fundraising, but she could not tell me “who” or “what”.  After my workout, I drove back by the boat and the sun was now up and I slowed to look at the logo again and I was blown away when I noticed that next to the logo was my precious little girl’s picture and her name!  David was inspired to help because of a personal connection to Epilepsy.  David’s cousin’s son, Connor, has a form of epilepsy that is treatable.  Connor’s first seizure had happened on a plane about a year earlier.  The seizure scared his family because Connor came close to death when he stopped breathing.  David decided to raise awareness for Dravet syndrome so that children like Haley may one day have a medication or treatment to help control their seizures.  David had contacted the IDEA League director, Joan Skluzacek, to get the logo.  Then he received permission from his sponsor, Reggie Fountain of Fountain Powerboats.  It was truly amazing that someone would do this for us.  Words cannot describe the emotions we felt.  Then David took it a couple of steps further.  He has made the logo available for other Fountain boats to put on their boats.  He said there was at least one other captain that was going to put it on their boat.  Then recently he told us that “team Fountain” has decided to give 10% of their prize winnings to the IDEA League.  Wow, this all came from our little article in our little small town paper…the power of sharing your story…. 

For I know the plans I have for you," declares the LORD, "plans to prosper you and not to harm you, plans to give you hope and a future.  ~Jeremiah 29:11

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~Haley Is My Hero~