Haley Is My Hero

Wednesday morning was a very busy one...a seizure at 3:30 AM, another at 4:45 AM, went to the YMCA at 5:30 AM, ran over six miles, and then the phone call from my husband, Bobby at 6:25AM.  Haley had fallen and might need a stitch in her lip.  I went directly home and looked at Haley's lip.  In lifting up her swollen, bloody lip, I discovered that there was no left front permanent tooth any more.  It had either been broken or pushed back up.  When I examined her closer, I could see the rough edge of the permanent tooth peeking out about 1mm from the gum.  So then it was a "splash" shower, get dressed again and off to the ER.  The ER felt she did not need a stitch, but it was nice visiting all the folks who have worked on Haley over the years.  We had not been in the ER for at least two years.  Then it was off to the dentist...and this was all before 9AM.  

So the verdict is that Haley is I must now complete my rotation in dentistry and orthodonics and maybe endodontics.  After all for me to be a full fledged "Mommy Doctor", I must investigate these fields, LOL!!  The dentist said if Haley was younger, no problem, the tooth will come down on its own.  If she was older, no problem, root canal.  But no, Haley is in the "gray area".  It all depends if the root is closed or not.  Since there is not a lot of studies on this kind of thing.  As her dentist said, "there aren't any volunteers who want their tooth push back up in to their head".  Crazy, I know.  So we are all learning here.  What I believe is in her future is the pulling of the tooth back into place by an orthodontist and bracing it and a root canal procedure.  

Haley through all of this has been completely unphased.  She has not missed a beat and remains her happy upbeat self.  She looked pitiful with her swollen lip and missing tooth, but she did not care one bit.  We hope she continues on this theme.

To add insult to injury on Friday it was her brothers' birthday.  She hovered a bit too close to the birthday candles and singed her eye lashes.  The girl can't get a break!!  Keep her in your prayers!  The coming week has dental and orthodontic appointments, can't wait!!!

January 29th is a day that will forever live on in our lives as the day that things changed.  At the time we had no idea.  Eleven years ago today my husband and I along with Haley had spent the greater part of the day shopping at Home Depot.  We were looking for lighting fixtures for the house we were remodeling and would soon to move into.  We got home late in the day and I was preparing dinner and Bobby had Haley lying on his chest wearing her pink Osh Kosh overall with yellow duckies.  The ones with the yellow bow on the back and she had on a yellow turtleneck and socks.  Funny we can remember it all so clearly.

 When I looked at Haley she appeared to be flushed, so got out the ear thermometer and one ear read 96.3 and the other ear 100.3.  I thought that was weird.  So to the changing table we went to get the rectal thermometer.  As I was taking her temperature, her eyes rolled back and she began convulsing.  I don't know how i knew, but I knew...she was having a seizure.  I immediately gathered up things and we headed off to the ER.  Living in a rural area, waiting for an ambulance means waiting for the volunteers to go to the ambulance and then try to locate you.  It was much, much quicker to take her on our own.  The rest of the evening was tests, blood draws, x-rays, etc.  We were fortunate to have our pediatrician already in the ER when we arrived to give us words of wisdom from a doctors point of view and even more important from a mom's point of view.  We left that night with a diagnosis of "febrile seizures" due to an ear infection.  I slept that night with Haley on my chest listening to lullabies by "Twin Sisters" recordings.

So here we are eleven years later.  It took six and half years to get the diagnosis of Dravet syndrome.  I look back over the years and think in some ways we were blessed to not know what Haley's condition was during the younger years.  We held out hope that one day, if we could stop the illnesses and reduce the seizures and get of the medications, then she would be "normal".  Back in those days, I did not want a label.  I wanted you to see my daughter.  I thought that "those" children in the Special Ed class were NOT her peers.  I tried in vain to help her learn and to move forward.  So then again, maybe it would have been easier had we known her true condition at an early age.  I know that we would have not tried some medications and then tried them again.  She would not have been so over medicated when she was five.  We would have persued therapies earlier.   Now I find myself wanting the labels in every color and size.  I adore "those" children in her special education class who give me hugs every morning and greet me as "mom".  

What does it do to think what I should have done or could have done??  I love the phrase that Dr. Wayne Dyer uses in his book, "The Power of Intention", "don't 'should of 'all over yourself"!  After all, if you should of then you would of, right?  We will move forward from this day and continue on our Dravet journey along side of many other brave moms and dads.  This definitely was not the life I imagined for myself and I would not sign up for it.  However, I am so richly blessed by experiencing it.

One of my New Year’s resolutions was to keep Haley’s website more current. It was probably one of my resolutions from last year as well. I was sitting down to recap the year and then the thought was to show it in pictures. I don’t know about you, but 2011 flew by. I am not sure why or if it is true what they say, the older you get the faster time flies. Last year was a blur in some places and dragged along in others.

As you can see in the video we had some major events happen in Haley’s life. The addition of Stiripentol to her medications has made a huge difference in seizure control. It is not a complete “fix”. I am not sure Dravet has a complete fix at this point. What we have noticed with Stiripentol is that Haley has less seizures, fewer seizures and more “spontaneous” language. My best example of the increase in language has been Haley actually saying where she wants to go. For her eleven years she pretty much has been loaded in the car with little information and off we go. It is not unusual for Haley not to question where she is going. She is extremely laid back in that way. Once and awhile she will ask where she is going. But this summer she actually requested, or demanded, to go someplace. We were driving to IKEA to meet up with other Dravet families while visiting my parents. From the back seat Haley proclaims, “I waaaaant too goooo too the stooorrrree”. I replied, “Haley, we are going to the store”. To which she responded, “Noooooo, I waaaant to goo to the store with the ‘red dot’”. Of course we all know that as Target! I was so excited, if I could I would have turned the car around and rewarded her, but we had people to see! But we DID stop by the “store with the red dot” later that night.

Another funny example happened right before we went on this venture. I was trying to comb out Haley’s very curly hair. Apparently I was bothering Haley by doing this and I might have hit a knot or two or four in the process. Haley looked up at me with her huge brown eyes and said in perfect “tween-speak”, “Mommy, you drive me nuts!!!” I nearly fell over in laughter! Fast forward a couple of months to Hurricane Irene. We had gone back to my parents for “safety”. The first night there Haley had the hiccups. You know the ones that are so deep seated they hurt. She had hiccupped for a good hour before bed. As I was putting her to bed I told her, “Haley you are driving me crazy”. To which she responded, “No Mommy, I don’t drive you crazy, I drive you nuts…..and you drive me nuts too!” She has a great sense of humor.

The other major event was the surgery on her feet. Haley had cadaver bones inserted in her ankles in an attempt to bring her foot into a more neutral position. Prior to the surgery her feet were so pronated (collapsed inward). The rolling of the foot inward was causing her knees to come together and her hips to be shifted back. This all together caused a “crouched” gait when walking. One of her feet was worse than the other and that is the way she came out of surgery. One foot looks “nicer” than the other. When the casts first came off, I don’t know why I thought she was going to be able to walk perfectly. Initially I was disheartened when she could barely walk at all. But then rationale kicked in. She had been non-ambulatory for the first three weeks and then in walking casts for the second three weeks. And even though she had the walking casts, she was pretty much sedentary for the entire six weeks.. So it made perfect sense she needed to relearn to walk. Fortunately a local physical therapy group had hired a pediatric PT and Haley did not have to venture far for services. We are so pleased with her new PT site and the results we are seeing in Haley.

A funny story that happened during the non-ambulatory phase…I was attending a Zumba class with some regularity last Spring and Summer. One day another mom came up to me after class to ask had I changed my fitness program, she thought I looked more muscular in my upper body. I thought and said, “No, I still come to the Y to lift weights and do cardio, but no, I have not changed anything.” It was not until I was walking to my car that it dawned on me, that all the lifting and carrying of Haley’s 65 lbs. plus casts had an impact on me!

 I think one thing I can say our family has learned this year is to laugh. In years past things have been tense to say the least in our house. The stress of a disabled child coupled with twins and our own business made things just a little crazy. For reasons, that I can’t exactly explain there is a lightness in our household. It could be that the twins are older and less needy or that Haley’s seizures are better controlled. Perhaps it is because we have a wonderful caregiver for Haley right now. We would be lost without Caroline. We love having someone work with Haley that can assimilate into our crazy family. Or we may attribute to the fact that after 10 years of Dravet we have settled into the diagnosis and the acceptance that Haley will always be with us. Whatever the reason, we do see the blessings that this horrible condition has brought to us. I think we are able to do this because of our faith in God and the belief that there is a higher purpose for Haley’s condition.

Haley's school physical therapist has given Haley this bike "on loan" to try for awhile.  Yesterday was her first time out.  She did so good, with the exception of heading for drainage ditches on occasion.  But her face shows her happiness beaming, ear to ear!  I apologize for the poor quality of this video!!

Gracie and her "friend" Susie in the back ground!!

 After years of talking about the elusive drug, Stiripentol (Diacomit), we finally have it in our possession!!  It has been a long and involved process to secure the drug and have it fully funded for Haley.  We pray that it will give Haley some relief from the almost daily generalized tonic clonics that she has been experiencing lately.

We first heard of Stiripentol shortly after Haley received her diagnosis of Dravet syndrome four years ago.  We saw it on the IDEA League Family Forums.  It was a drug that produced in France and available in Europe, but not the USA.  At the time it had just had a huge price increase in the cost of the drug.  There were many families that were able to import this non-FDA approved drug.  Some had miraculous effects and others did not have great success.  We were shown the what, how, why and where to get the drug IF our doctor would write the prescription. 

Back in 2008, we did not think it was going to be possible to get the drug for Haley and we thought it also was more beneficial to the kids who were suffering from "Status Epilepticus", a seizure lasting 30 minutes or longer or clustering of seizures with no consciousness in between.  Since Haley had not had an episode like that since 2005, we did not push the issue to get the drug.  However, time went by and we tried and failed new drugs and the prescriptions she was on were not giving her much of a break seizure-wise.  Lately the longest we go without a seizure is 2 or 3 days. 

In 2010 we decided we wanted to try the "magic" combination that has been known to work for children with Dravet syndrome.  We heard it at the IDEA League 2008 Family and Medical Conference and then again in the 2010 conference, Stiripentol, Frisium (Clobazam) and either Depakote or Topamax was the combination that works for some Dravet kids.  So we began our quest to get these non-FDA approved drugs.

Just an aside, both drugs are available in the USA because they have orphan status.  An orphan drug is a drug that has shown to help a rare disesase or condition.  Since these drugs serve a small population, there is not a huge return on investment for a pharmaceutical firm to put out all of the money for clinical trials.  Basically it all comes down to the almighty dollar.  So sad....  However, Frisium is about to be approved in the fourth quarter of this year.  It will be rebranded as "Onfi" and available in the USA.  The clinical trials for the drug were focused on individuals with Lennox Gastaut syndrome a much larger population than Dravet syndrome.  We are so happy that the drug will be approved by the end of the year.  Stiripentol is another story.  So far the ONLY group Stiripentol has been beneficial for is Dravet syndrome.  Haley's doctor was helping to advise the IDEA League with getting the drug started with the FDA approval process said that the data supplied by the European studies would be "laughable" to our FDA's strict procedures.  Unless we can find another group that would benefit from the use of this drug, it will not be approved any time soon.  It is just not cost effective.

In 2010 we were able to finally get our doctor to write a prescription for Frisium.  We were going to pay for it initially out of pocket, an expense of nearly $300 a month.  Fortunately with the help of the IDEA League and other parents who have traveled down this road, we were able to get the drug approved by our primary insurance company.  Surprisingly, it required very little effort.  I guess when you send the insurance company a 20-page packet with the documentation on how the drug has helped other Dravet children and various approval letters from their own counterparts in other states, they have little to deny!  In April of 2010 we started weaning Haley from Klonopin, a drug that is similar to Frisium, but has more adverse side effects, to Frisium.  The wean from Klonopin was supposed to be very difficult and we did it painfully slow.  I am happy to say, we had very little adverse effects and Haley came through the drug "exchange" a more vibrant little lady.  With the addition of Frisium, we saw more spontaneous language and more personality.  We were so happy and ready now to tackle Stiripentol.

In the summer of 2010, I contacted Haley's old neurologist who is now a state senator too!  Lucky us!!  He agreed to help us with our endeavor to get the drug approved through Virginia Medicaid.  Stiripentol out of pocket is very costly.  A month's supply could cost well over $1000.  We just do not have the money to put out in advance.  Her old doctor is a true Southern gentleman and a wonderful advocate.  He sent the letter in the the agency in July and rumors were heard that Stiripentol was covered in Virginia by August.  We even heard from the pharmacy in NYC that they had become an "approved provider" for Virginia.  It all sounded so promising!!  Sooooo we waited and waited to hear an official confirmation....and it never came.  Summer slipped into Fall and right before Thanksgiving the pharmacy called and said, "all we are waiting for is a coding form to know how to bill".  I was seriously doing a happy dance, jumping up and down...so excited!!  Then right before Christmas, the pharmacy called to say, "I don't know what happened, Virginia is now saying 'NO'".  Immediate deflation...  Shortly after the holidays we learned that the reason for the "No" was because it WAS NO A FDA APPROVED DRUG!  Well, duh!!  That is why we wrote them in the first place!!!

In 2011 we started again.  I contacted her old neurologist, and he made some calls.  We hear once again from the pharmacy in NYC and they say, "We spoke to someone from VA and they are very apologetic, the just realized it was an orphan drug".  So we were back in the holding pattern waiting once again for the process and coding for billing.  Long story short, on Thursday, I called the pharmacy around 2:30 PM again to ask IF they had received what they needed from Virginia.  The answer was no.  So I called Virginia and actually spoke to a person and not an answering machine.  The ball started rolling and it quickly picked up speed, by 3:30-4:00 PM, we had new prescriptions, payment was guaranteed and next-day air was promised!!  Woo hoo!!! 

So on Friday afternoon, I walked into my house to find a small box about 8x8x8 was sitting on my table.  I literally picked it up and hugged it and then ripped into it!!  We are so excited to finally receive this medication and we pray that it will provide relief for Haley and our family.  This has been a long, tiring journey.  But every day we are encouraged by new friendships, gestures of compassion and the others that have traveled this road before us.

The Lancaster School SCA presented Haley with a check  for $800.00 in her honor.  The SCA hosted a  "Social" for their classmates with the Haley Smith Fund as the charity of choice for this year.  Mrs. Tara Booth stated that in her nineteen years of holding a fund raiser of this sort, this is the largest amount they have ever raised". 

They presented the check to Haley during their "Morning announcements/TV news".  Of course, I cried when they presented the check.  I am continually humbled and honored to be living in such a compassionate community.  During the week before the social, I would be taking Haley to her classroom and I could hear the kids whispering, "that's the girl who we are raising money for".  Along with the whispers came more acknowledgement of Haley with smiles and waves.  After she was presented with the check on "TV", one young man saw her later and said, "Congratualtions".  The following day a young lady who we do not know waved to her and said, "Hi, Haley!"  More tears from me! 

A few weeks ago in our local paper there was a letter to the editor that said they had not seen more ruder kids than this particular middle school.  In some regards I did agree with the writer of the editorial, some kids can be rude.  But I think we have to be careful to typecast and label these children.  What they did for Haley was far from rude.  Child learn what they live.  I appreciate those who made this very generous donation to Haley's fund.  One mom told me how she had given her nine-year old daughter $10 for the social and her daughter took $12 of her own money as well.  When her daughter got home she said, "Mom,...I'm broke!...but do you know what?,....it was for a good cause..."  Oh so touching...and more tears.

Everyone's journey is different with this syndrome.  We traveled a long road full of hope that Haley would out grow her seizures and be a "normal" child.  "If only we could get the right mix of medicines then the seizures would be less,...then for sure she would be 'normal'", is what we thought.  That is what I can remember from the good days when Haley was young and we had no official diagnosis. 

I know some of my family members were upset that the diagnosis for Haley took so long, but recently I heard of someone's daughter being diagnosed who was 43 years old....wow, she is as old as my younger sister.  I can't imagine how that mom has made it so long without knowing, yet knowing their child was different.  I know for us the time not knowing made me stronger, wiser, and helped me grow in my faith.  For at times all I had was my faith.  Not knowing made it easier to decide to have ONE more child, yet God in his infinite wisdom, gave us twins instead.  I am not sure if we would have had more children, if we had known what life was going to be like with Dravet syndrome.  Ignorance was bliss in this department.

I know a mom whose child was diagnosed really young, under a year.  The doctor that diagnosed her child, then told the mom that her baby would not live to see five.  Can you imagine living those years with that prognosis?   I am happy to say that the child defied the doctors prognosis and is almost eight.   But having that diagnosis early has its blessings too.  You know what drugs to avoid.  You know in advance that you need to get your child involved in multiple therapies to improve their quality of life.  It is a bitter pill to swallow so young, but you at least have a path to follow.

So which is better?  Is there really an answer?  I know God gives us what we can handle when we can handle it. Like I said before our family was much better knowing later or we might not have siblings for Haley.  I am who I am because of what we went through.  I learned doctors do not know everything, that they are "just practicing medicine".  I learned a lot about myself and others.  Some people can be very uncaring and cold...and others can be surprisly compassionate.   

I feel very blessed at this point in my life.  It is hard to convey that to someone who is newly diagnosed.  When I hear someone has been diagnosed after a time of searching, there is mixed emotion.  I am very happy that they got their diagnosis, but at the same time I am bummed that it is THIS diagnosis.  However, we are all in this together and we can band together to help one another.  We all become part of an extended family that understand each other's "normal".

I saw a quote at one of my favorite restaurants, Food for Thought in Williamsburg, VA.  I wish I would have written it down.  It went something like this, "Life is not fair, it just is...it is how we react to it that makes all the difference".  I really like that.

I am constantly humbled by the members of our community that want to reach out and help Haley.  Today a friend shared a website that they created to help us reach our goal of $30,000 for Haley's research goal.   The website is called "Miracles Happen" and the link is www.cafepress.com/jesusfulmiracles  

Please check out the link and see all the goodies that you can purchase to Help Haley's cause!

Thank you Tom and Alison for your dedication to Haley and our family.   We love you guys!!!

When you were born, Haley, I had dreams of my life with my little girl. However, those dreams were soon shattered and replaced with new visions and dreams that were never on my radar screen. I mourn the loss of those dreams, hopes and wishes, but through the mourning I have found strength. In the past decade I have complained to the “powers-that-be” on occasion that THIS was NOT part of my “Mommy contract”, but my cries have fallen on deaf ears. But now reflecting back on my decade with you I have been so blessed by you and have learned some great life lessons. I have learned it is OK (and necessary) to ask for help, “things” are not as important as people, patience is learned and trials help you learn them, that you cannot know true joy unless you experience some low times, that people are truly good when given an opportunity to help, that doctors are JUST “practicing” medicine, that moms can be a force to reckon with when we band together for a cause we believe in…I have learned to slow down, to be ready for a change in plans at ANY moment, or several changes in plans….and I have learned not to get disappointed when things don’t work out. Most of all I have learned to rely on God and prayer to get me through the tough times. Through all of this I am so thankful for the little girl I have. The experiences that have happened over the last 10 years have served to make my life richer! We love you Haley!! Thank you for your laughter! Happy Birthday you are a very special little girl!!

We are in the process...or should I say, we have always in the process of remodeling here.  Such is the life of being married to a builder.  Like the cobblers children, we are the last on the list to get our work done.  Anyways, we are getting ready to do a major rooms change.  Haley's brothers, Peyton and Parker, are going to move into Haley's room and Haley is getting a new room.  (Hopefully we can add some theraputic element to it) 

Today I have been cleaning out her closet and her dresser.  It is bittersweet.  There are so many keepsakes and momentos that I saved thinking as Haley grows up she would love to see these things.  Like the ribbons that announced "It's a Girl" on the maternity ward.  Or the cute "collectibles" that were given in her infancy.  Since she was born in 2000 there were so many "Millenium" momentos as well. 

I am heavy hearted as I once again must bury the dreams I had for my precious daughter.  The goals and desires we had for her are now replaced with the reality of raising a child with a profound disability.  Yes, there are so many blessing that come with Haley's life.  However, it is hard to live in "Holland" (reference to a poem "Welcome to Holland~it can be read on my "Things Pondered" page) and keep dreaming of the exotic places you thought you were meant to go.

Last weekend the reality hit me so hard.  I broke down in tears and could not function.  I wished I could "resign" from being Mom all together.  Fortunately there are so many other Dravet moms and dads out there that can relate.  A phone call from one of them really lifted my spirits.  With my companions on this Dravet journey and my faith in God I was able to see life from another point of view.  My spirits are now lifted.  It is still is hard.  Especially the mourning of "what could have been"....