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Haley's video story and other thoughts

Posted by Haley's mom on November 21, 2009 at 10:01 PM Comments comments (0)

As I see so often posted in blogs...wow, it has been awhile since I last wrote...  Time flies when you are having "fun".  Well, not really that much fun, but life is happening and there is little time to write.  So much has happened since my last post, let me catch you all up to date.

 

Haley's Dravet Story Video:

I had my first try at making a video of Haley's Dravet story.  It was a bittersweet experience.  I think it was the first time I dug through files of photos and looked at old videos.  I would look at the dates on the media items and think to myself, "just two more days and your world will be rocked by Haley's first seizure."  Then it was, "Wow, that photo shoot was just after Haley got out of the hospital, I can see where the I.V. lines were run in her arm".  It was amazing how difficult we thought life was back then when she only had five seizures in the first year.  Now five seizures in a day is nothing.  It was also difficult, but reaffirming to see that she could run and do so many things with coordination when she was younger.  Now she has lost those abilities.  She was such a delightful, happy child with a great sense of humor!  I am very pleased with how the video turned out.  I hope it will go on to raise awareness for Dravet and dollars for research.

 

 

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Medication update:

We went to see Dr. Pellock in October and we thought we would be coming off the Klonopin. Instead we have decided to wean Haley off of the Banzel by lowering her dosage a half a pill every two weeks. It will take us two months total to remove the drug completely. She is on the last half-pill dosage for the next two weeks. We have seen a decrease in the complex partials and daytime seizures. Her "normal" now is to go 3-5 days and then have a seizure in the morning before she wakes up and one again in the evening before bed. It is sad when you start to predict them. Our plan next is to look at another new medication, Vimpat. It is designed for complex partials and is approved for people 17 and older. Dr. Pellock was willing to campaign for our insurance to cover it; he thought they would deny it because it was not approve for her. Now that she is not having many we will probably look to another avenue.  We go back to Dr. Pellock on December 15th.

 

Haley's New School:

Haley has also started a new school this year.  I believe when I posted last we were in IEP (Individualized Education Plan) discussions.  She is enrolled in the Northern Neck Regional Special Education Program that is housed in Lancaster Primary School.  She is able to attend four days a week for two hours a day.  She absolutely loves her teacher, Mrs. Woolard, and going to her "new school", as she says.  I think she is happy to do something without her brothers and she LOVES all the one on one attention.  We are very pleased with everything so far!!!

 

More Medical Issues:

Haley's feet have continued to collapse and pronate (roll inwards).  This is a co-morbid condition with Dravet syndrome.  I was contacted by another mom/mum of a Dravet child whose daughter had surgery on her feet this past summer.  It was a success and her daughter is able to walk better.  Haley right now can not walk far distances.  She zig-zags as she walks and throws her feet around.  She almost tripped me today as I was walking too close to her.  We met with her orthopedist in September to discuss surgical options.  He is a wonderful doctor with great bedside manner.  We discussed the situation for "over 25-minutes" (he put that in his notes) and ended with a possibility for surgery and a follow up appointment in January.  His reservation is that he may have to do the surgery again down the road for her.  On Monday, November 23rd, we are heading to UVA to get a second opinion.  I don't think they have ever heard of Dravet syndrome there...we will be the ambassadors once again.

 

Marine Corps Marathon:

One positive thing about having a rare syndrome is that you instantly connect with other parents living in the same world.  I had the honor and pleasure of meeting a dad of another child with Dravet at the Marine Corp Marathon in October.  Derek Amson, Riley's dad, traveled from Crewe, England to run in the 26.2 mile race.  He did it as a fund raiser for the IDEA League.  It was an entertaining visit with him and his friend, Andrew Cotton.  We met the night before the race, had dinner, and he bestowed upon me lovely gifts of chocolate!!!  YUM!  Then I brought Haley and my parents to meet him at the finish along with Andrew. 

 

 

It was so inspiring to see all the runners at the event, it really made me wish I could run one.  SOOOooooo, I wrote that on the IDEA League's family forum and have been challenged by another mom to do that next year.  We will see.  I am going to sign up for my first half-marathon next year, Nicole....!!!!

 

Another Hero for Haley:

Jamilah Sawyer, the guidance couselor at Haley's school, also ran the Marine Corp Marathon.  I know her from the YMCA and see her nearly every morning at 5:30 AM.  She totally surprised me and touched my heart with her generous, selfless spirit...she presented Haley with a gift bag a couple of days after the marathon.  I never in my wildest dreams could have known what was inside.  When I came back to school to pick up Haley, tears rolled down my face when I learned Jamilah gave Haley her Marine Corp Medal.  She has such a good Christian heart and wrote the most lovely letter to Haley. 

 

 

Haley is probably the only nine year old that has worn two Marine Corp Marathon Medals from two different participants that live on two different continents.  But how amazing a little bit of ribbon and metal can tie together so many people.  These are the moments I cherish in the difficult times. 

 

Awhile back I posted on my Facebook status, "I wish I could have a day that I did not have to think about seizures"....later in the day I realized that if that day came, there either would have been a cure, or I would not have my Haley here on this earth.  So in light of Thanksgiving looming ever so close,...I am thankful for the seizures and having Haley in my life, I am thankful for the moms/mums and dads that I have met on this journey, and I am thankful for so many who have joined us in our fight to raise awareness and funds for Dravet syndrome.

Just an update on drugs....

Posted by Haley's mom on September 24, 2009 at 10:20 PM Comments comments (1)

Where does time go? I have meant to write to let you all know how things are going with Haley and our family. Thanks to all of you that have inquired. I will try to update more regularly in the future.

The last time I wrote Haley had just started Banzel. Initially it was wonderful for her. We saw noticeable decreases in the generalized tonic-clonic seizures (grand mal), but we also saw an appearance of some complex partials, myoclonics and drops. Every medication has it tradeoffs. We made increases in this medication very gradually and she did have better days. The biggest thing we did notice with the addition of Banzel was her increased speech. At times it got somewhat annoying to me, but then I quickly reminded myself there are many Dravet kids that have no language left and their parents would give their right arm to hear their precious child utter “mom” or “dad”.

March went by and there was nothing too remarkable about that month. We made some adjustments to her Depakote because the Banzel does interact with that drug. In April things got worse all of the sudden. It wasn’t until I was making sure we had all her drugs for her Make-A-Wish trip that I realized what could be the culprit. At the beginning of April we had been given the generic form of Depakote, and according to her seizure calendar there was a direct correlation to that new form and seizures. We went off the generic and back to the name brand and amazing the seizures decreased.

The highlight of our year had to be our Make-A-Wish trip in April. We went to Give Kids the World in Kississimee, FL. They enabled us to go to Walt Disney World, Universal Studios, Sea World and enjoy a ton of activities at their location. It was incredible. Haley really enjoyed meeting Cinderella. They whole time she was in her princess dress she was in character. You can check out the photos on the photo page.

May was an OK month of seizure control, but June was amazing. We had only eight seizures total that month…then things turned ugly! The summers are always harder due to the heat. However, Haley did not do much outside this summer and it was not a bad “heat” summer. The seizures picked up big time. They were no longer isolated to her sleeping hours. Now they were coming in the middle of the day, in mid-sentence, “boom”…down she would go into convulsions. Those are so difficult to get used to.

In August we went back to the neurologist and had the plan to increase the Banzel again. Then on the car ride home from Williamsburg (about an hour from our house), Haley went into a terrible seizure. It is an awful feeling when you cannot stop or pull over safely. You try to keep control of your vehicle while trying to reach your child. As I glanced in my rear view mirror, I could see she was so blue…blue-gray. It is terrifying, yet you try to maintain composure to help her. We finally managed to pull over and she came to. Those are the most frightening seizures. Since that day, we have had very few “seizure-free” days.

We went back to the neurologist last week. We decided that the Banzel had some positive effects at a lower dose, so we cut her dosage in half. We also believe that the Klonopin (Clorazepam) that she takes has lost it’s efficacy. It is a valium based drug and is excellent in controlling seizures, but over time you need more and more to get the same effect. Increasing the dosage too high renders any “rescue drugs” ineffective. What a tightrope we walk. So we are going to wean her off that drug starting in October. From what I understand it is an awful wean. Many moms and the doctor have told me, things will get worse before they get better. What fun we have to look forward to as we wind down 2009!!

Another new drug...

Posted by haleyismyhero on February 27, 2009 at 10:37 PM Comments comments (2)

Haley started a new medication last week.  The new addition brings her pharmaceutical “cocktail” up to a four drug mixture.  Haley has been on over a dozen different medications and combinations of medications since she was two years old.  None of which have controlled her seizures for any length of time.  Some of the prescribed medications made her seizures worse, even though that was not supposed to be a side effect of that particular drug.  For Dravet patients, Lamictal and Dilantin are two drugs that can really make things worse.  Haley has been fortunate in that side effects have been minimal with the drugs that she has taken.  Here is a brief overview of what she has been on and what my small brain can remember about each one.  This is not the exact order of how they were prescribed.  I wish I had kept better records, but during all of this the twins were born prematurely…I consider that time and the months that followed, my “black hole”…

 

Tegretol- We started on this drug and did not see any results right away (within a week) so we stopped and went onto the next drug.          

Phenobarbital- We had poor seizure control with this one.  Haley was lethargic. We went off of it and then back on it and back off it a second time.  When Haley had the multitude of ER visits in 2005 due to clusters of seizures (status epilepticus), the ER would give her a loading dose of Phenobarbital.  I would bring home a “rag-doll” of a child.  The first 24-hours she was a zombie; the next 24-hours she was stumbling all over the house.  She might recover about and be “normal” about 4 days out.  By the seventh day we were back in the ER with another loading dose.  Her treating neurologist at that time said that “one of the side effects of withdrawal from Phenobarbital is seizures…”  I do not like this drug.

Depakote- Some control.  She stayed on this the longest, but eventually went off it, then on it, then off it, and now she has been on this the longest of all the drugs.

Trileptal- She was in a study for this drug.  She was fortunate to get her first video EEG as part of the study.  Unfortunately she did not have any seizures for the 72-hours she was in the hospital.  However, once we got home she had a severe tonic clonic within the first hour, figures!

Topamax- When Haley was on this drug, she lost some of her language or ability to speak.  It was heart breaking to hear her say, “ma..ma….ma….ma…..(then finally) mom.”  Her regular speech came back within 24-hours of going off the drug.

Zonegran- This was another drug that took away Haley’s speech.

Tranxene- This is a valium-like drug that worked well at first.  Then is started blocking the receptor sites for her “rescue drugs” which landed us in the ER and hospital repeatedly in 2005.  We finally went off of it because of the resistance she had against the “rescue meds”.

Lamictal- In hindsight this was the worst drug!  We were put on it by our first neurologist and as he raised the dosage, her seizures seemed to increase to me.  When I told this doctor my thoughts, he immediately ( and safely) stopped the drug.  Our next neurologist wanted to try it again.  I told him what happened the last time we were on it, but he was not concerned.  As he increased the dosage, we saw the same increase in seizures.  This doctor, however, did not take her off the drug…he said, “that is not a side effect of Lamictal”.  Once we were diagnosed with Dravet syndrome we did learn this is the ONE drug that these kids should stay away from!  If the second prescribing doc would have “thought outside the box” we might have had a diagnosis earlier.

Dilantin- Another drug that is not good with Dravet kids.  We used this as well as a “loading dose” when the Phenobarbital was in question.  Haley was on Dilantin while on the Ketogenic diet.  When she was on the diet she had to take the IV solution orally, YUCK!  I truly think her tastebuds were ruined by that.  It was nasty stuff!

Keppra- She first went on Keppra in conjunction with Lamictal.  Since Lamictal causes seizures with Dravet kids we thought we would revisit it.  The second time she was on it she was mean!  It made her nasty, which was kind of amusing for us.  Haley is so sweet normally, but on keppra we saw a different child.  She liked to tell her physical therapist, “you can leave now”.   She was also very disruptive in the classroom.  Once we went off the drug she was back to her sweet self. 

Klonopin- Haley is on this drug now and it seems to have some positive effects, maybe.  The down side is that the longer you are on it, the more of it you need.  We are waiting for another drug to get FDA approval that is similar without some of the lethargy.

Felbatol- This was the first drug that the neurologist said, “I would like to put Haley on Felbatol/Felbamate.  I would like you to go home and read about it and take about two weeks to think about this drug before you answer”.  Felbatol was pulled off the market in the 1990’s because there was a link to aplastic anemia.  So it was very scary putting her on this one.  She spent a year on it with regular blood tests and no negative side effects.  Haley went off it because we did not think it was doing anything for her.  Then seizures picked back up and we went back on it.  She is still on this drug today.

Lorazepam-  This is another valium-like drug.  Haley spent a short time with this one as her daily medication. The problem with this is that the more you take, the more you need.  We only use it now as a “rescue drug”.  We will administer this orally if she is alert after having a series of seizures.

Diastat- This is our life saver…and a very, very expensive one at that!  This is a rectal syringe of valium (diazepam).  When we first purchased this it cost us an $86 co-pay for two syringes.  That was in 2001.  Now in 2009 it would cost us almost double that with a co-pay.  The manufacturer says “we are saving you a trip to the ER”.  In 2007 and 2008 our use of this drug caused us to hit Haley’s drug cap of $5000 by July and June respectively.  These syringes without insurance are about $175 a piece!  In 2007 we used 10 in one month!  That is no fun!

Banzel- This is our most recent addition to Haley’s drug regimen.  So far we don’t know.  She started it on a Thursday night and her seizures picked up.  We upped her dosage the following Tuesday and so far (this is Friday) we have been seizure free since then (3 days…).  Only time will tell.

 

One thing Haley has learned from all these medications is a talent for swallowing some big pills, even two or three at a time.  She unfortunately also knows where a tourniquet goes on her arms for all the blood draws.  She doesn’t even flinch now.  She just watches the whole process.  When it is all over, she pronounces, “I am very, very brave….God made me strong”.  Yes, sweetheart, God did make you very strong and very brave! 

Haley's "New Feet"

Posted by haleyismyhero on January 6, 2009 at 5:09 PM Comments comments (3)

One of the co-morbid conditions that come along with Dravet syndrome seems to be flat feet with ankle pronation (ankles turning inward).   Up until last year Haley did not have too much trouble with her walking, but in the spring of 2008 we noticed that her ankles were getting worse and causing her knees to bend and come together.  The beginning of a "crouching" walk was appearing.  We went to one orthopedist and he basically dismissed us and rolled his eyes at me.  He did not know he was speaking with another M.D., "Mommy Doctor" that is...  So after the IDEA League conference in August we made another appointment at Children's Hospital in Norfolk.  Dr. St. Remy was wonderful!  He actually was interested in hearing what I had to say about Dravet syndrome and Haley's prognosis.  Soon after our appointment Haley was fitted for SMO orthotics.  We thought everything would be great! 

 

We picked up the orthotics in early November.  They were beautiful light blue with pink and purple hearts, what could be better???  Since we had waited to buy any new shoes until we got the orthotics, Haley and I immediately went shopping after picking up her new devices.  That is when it hit me.  These braces were going to make it apparent to the world that my child had a disability.  How humbling all of this has been to both Bobby (Haley's dad) and me!

 

When we went to the shoe store, we sized her foot with the orthotics on.  She immediately went from a 13 to a size 2 WIDE.  It took us several tries to find a shoe wide enough for the orthotics.   Fortunately we found a pair of K-Swiss shoes that fit nicely over the orthotics and went on with just a little bit of a struggle.  As I stood back to watch Haley walk in her new shoes, all I could think of was she looks like the little girl from Frosty the Snowman.  There she was with her toothpick-thin legs with these huge shoes.   As for Haley, she could care less what she looked like.  All she wanted to do was ride the dinosaur ride in the kids section. 

When I walked in the door at home I waited for Bobby's reaction.  I had told him on the phone that he probably would not like them because they protruded from the shoe.   I also let him know that she had to go up two sizes in shoes.  He echoed my sentiments that a disability was now apparent, but with pants on it was not too bad.

 

Being who I am, once I put the kids to bed that night, I immediately went to the computer to find the shoes that were "made for orthotics".  In my naiveté I thought somehow these shoes would be smaller and fit more like a "glove" over Haley's braces.  I ordered two pairs of "Hatchback" shoes, one shiny black patent leather for church and a new pair of tennis shoes.  They arrived and to my dismay they were larger than the K-Swiss!   Of course Haley loved the new black shoes, calling them her "tap shoes". 

 

Thanksgiving arrived and it was time to visit the family and show them the "new feet".  Haley decided to wear her black and white leopard print skirt and black tights and black shoes.  Bobby immediately did not like the idea.  But what could we do? We just shrugged our shoulders and went on our way.  Once at my parents? house you could see the relative?s glances from Haley's face, down to her huge, disproportionate feet and back up to Haley?s smiling face!  Nothing different than both Bobby and I did the first time we saw the orthotics.  Bobby removed the shoes at the first opportunity.

 

We did find that Crocs worked well with the orthotics.  Since they are already so big and bulky, they make the orthotic less apparent.  Not to mention how much easier it is to get them on over the devices.  It certainly helps out Mommy when she is dressing Haley.

 

When it was time to visit Santa, Haley again chose her most favorite clothing item to wear?the leopard print skirt.  I quickly talked her into pants; after all it was too cold for a skirt!  Bobby hollered from the next room (privy to our conversation due to the baby monitor that is always on in Haley?s room) that he did not want Haley wearing the black shoes.  His reason?they were too uncomfortable.  When I was alone with him I asked, ?too uncomfortable for who?...you or Haley?? 

 

We both agreed that we needed to learn another lesson in life from Haley.  It is not how you look, it is who you are, it is not how you are dressed, it is whether or not you enjoy the place where you are, and Haley does that better than most.  Thank you Haley for another life lesson!

 

Thank you David Nova, Edie Jett & Team Marlin Maniac

Posted by haleyismyhero on December 16, 2008 at 9:31 PM Comments comments (0)

We had an awesome weekend in Virginia Beach!  We were invited by David Nova to attend the American Striper Association's (ASA) final tournament and 2008 Awards Ceremony.  We in turn invited representatives from the IDEA League since David said he would like to present a check to them.  Thank you Laura Cossolotto, IDEA League president, and Kim Skriba, IDEA league Board Member, for joining us!!  Saturday was a crazy day getting everyone picked up from the airports and to the hotel and marina.  Fortunately it all went pretty smooth...even with Bobby going to Richmond International Airport originally instead of Newport News to pick up Kim and her son, Ryan Smith...and even with Lisa being delayed by a train stopped across Route 17.  We all made it in time to see David Nova win "Angler of the Year" and Edie Jett win "Lady Angler of the Year".   We were so touched as a family (Smith family and IDEA League family) that the ASA would make special arrangements  in their ceremony to highlight our cause and present us with a "large" check (both figuratively and literally).  Words can not express our gratitude to David, Edie and all of Team Marlin Maniac for your support to the IDEA League and our family.  Because of people like David and Edie, the IDEA League will be able to move forward and fund research into Dravet syndrome.  Hopefully they will one day find a combination of pharmceuticals that will stop the uncontrolled seizures and ultimately a cure!

December 2008

Posted by haleyismyhero on December 8, 2008 at 11:53 PM Comments comments (2)

"How are your kids doing?"  Is a question I hear almost daily.  I know some hesitate to ask because they do not want to hear bad news about Haley.  The question is a difficult one to answer on a daily basis.  One day can be terrific for Haley.  She can do well at school, want to eat, be full of laughter and very agreeable.  The next day could be a different story, three seizures before waking up.  Valium administered....then we have a walking zombie...sometimes.  Bobby came to me one day telling me he liked how a friend inquired about Haley.  He would say, "How is your daughter, today?"  That one word makes all the difference.  Dravet syndrome is a day by day experience.  There are not many adults known to have Dravet.  I am sure they are out there, they have just been misdiagnosed.  We thought it was bad to wait close to seven years for a proper diagnosis, but then a mom joined the IDEA League family forum whose daughter had just been diagnosed at 22 years old.  My heart just broke for the pain this mom must have endured through the years.

Haley did well today and is vastly improved from her catatonic state that she was in since before Thanksgiving.  We saw her neurologist last week and he 'tweaked' her meds and presto we have personality again!  Fortunately, Haley is a very pleasant and loving child.  That makes it easier to cope with the syndrome.

Some exciting news for Haley is that we are awaiting word to see if her "Make a Wish" trip will become a reality.  Our family met with the Make a Wish team on Sunday after Thanksgiving.  Haley had three seizures that morning and a dose of valium.  Therefore, she was not real communicative, but she did not have to be.  Who needs to be when you have two excited brothers saying everything they would like to do?!?  Haley's wish is to meet Cinderella and her "friends"...her friends are not the other princesses ...they are the birds and mice (you know, just like the story reads...Haley does...she has memorized every book).  They are going to try to arrange a meal with Cinderella.  It should be exciting.  For me it will be back to my old employer, I worked for "THE Mouse" in 1988.  It was fun!

This year has been an incredible year.  The America's Giving Challenge started the year off with a bang.  (You can read about this on "Haley's Story" page)  The we participated in the National Walk for Epilepsy in March.  This was the first time we were surrounded with families that truly understood what our lives were like.  One mom said it best when she said, "it was so nice to just be able to talk and not have to explain my child".  Then in August we met families from all over the globe at the IDEA League Second Bi-Annual Family and Medical Conference.  What an honor it was to meet Dr. Charlotte Dravet herself. Being in Chicago with 140 other families was surreal.  We almost felt normal.  It was strange to see a complete calm room when a nurse would walk in and say a name , "is having a seizure".  There was not a dry eye in the house when doctors would present video clips of their patients having seizures.  What a bond we share.

This weekend ahead David Nova has invited Haley and her family to attend his fishing tournament awards banquet where he will present Haley with a check for the IDEA League.  Two parents are coming in to town to join us.  We are excited to see them again!

Along with the highs of 2008, there were the lows... So far since we have been on the parent forum five children have been lost to Dravet syndrome.  The first two were from overseas.  The third child was from Illinois.  His father, Scott, shared his pain with us on the forum and I am so thankful he did.  Because of his transparency we began to see that we need to treasure every day with our children.  We should not wait until "we get them straight".  I admire this father greatly.  Even though his son is gone from this earth, he is keeping his memory alive with all he is doing for the IDEA League and other parents who have lost their children.  Thank you Scott for your strength and courage, your son would be so proud of you!


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