February 19, 2008

Once again a lot has happened since the last update. In December Sarah had her VNS removed due to an infection that had been there the whole 4 months she had it - she did not show the normal signs of infection other than swelling, but it finally became obvious. After the surgery to remove it, and the infection was identified as MRSA, she was started on an IV antibiotic called Vancomycin. She had this drug once before and had a minor reaction to it. This time she had a severe anaphylactic reaction to it, and had to be rushed to the PICU and intubated. She was on a ventilator for 9 days. We brought her home Dec. 22, on home IV infusions for several more days. Matt and his wife Laura were in town for the holidays, so at least we got to all be together, at home, and did manage to have a nice Christmas.

In January Sarah had pneumonia, which we successfully treated at home. She has been homebound from school since December 10, except for 2 days we sent her back in January, but she was just too tired.

Now we have another huge dilemma. Sarah has a dislocated right hip and according to 2 surgeons, needs surgery to correct it. There is a window of opportunity to fix it to where it will be a functional hip, and we are nearing the end of that. Right now we have the surgery scheduled for March 24. It would involve a lot of pain and 6 weeks in a body cast. I wish there was some other way around this - I am going to look for one just in case. But we have been told there is not. I wish Sarah could have a long period without any medical treatments or illnesses or surgeries so she could have time to feel better and get stronger. I will try to keep you all posted on this.

BUT - THE GOOD NEWS is, and I am going way out on a limb here publishing this on the World Wide Web - Sarah has been SEIZURE FREE since December 22, 2007. This is the longest sz free period in years. It is attributed to an increase in 2 of her medicines in December (she is on 5 sz meds, one is not FDA approved), or it could also be a miracle - in my opinion it is a miracle no matter how it happened. We were so worried about an increase in sz once we lost the VNS, but so far so good!!!

One other thing - we need to do another fundraiser for Sarah. There were 3 fundraisers for her in 2004, and we have not done any since. If anyone has any ideas, please contact me (see the contact page). I have some Party Lite items for a raffle if anyone has a venue where this could take place. I tried to get a booth at the Lindbergh Craft fair, but they don’t allow raffles or items that are not crafts. We need to replenish her fund to cover the things that are not covered by other means - and unfortunately there is still a lot of that.

As always, thank you very much for your interest and prayers.

Sandy Smith



November 8, 2007

WE NOW HAVE A DIAGNOSIS

I was doing my usual late night neurology research one night in August and happened upon a rare epilepsy syndrome that seemed to amazingly fit Sarah’s history. I emailed Sarah’s epileptologist, who emailed me back that, coincidently, just a few days earlier, Sarah’s original neurologist had also emailed her with the same idea – that Sarah may have Dravet’s Syndrome.

We waited several weeks trying to get the pre-authorization from our insurance company to do a DNA test to confirm the diagnosis. October 1st – 3rd Sarah was admitted to SLCH with her second bout of pneumonia for 2007, and while we were there, she finally got the test done. Then we waited about a month for the results. Sarah tested positive for a variation to her SCN1A gene, consistent with Dravet’s Syndrome.

It is very unlikely that this variation was inherited from Dave or I, but we are both being tested anyway, in part to rule out implications for Matt and Graham. It is much more likely that this variation happened spontaneously in Sarah.

SO – what does this mean?

Seizures

We have seen a definite decrease in the amount and length of seizures since I last updated. The VNS is helping - so we are in the lucky 50 – 60% who are helped by it. It is way past time for luck to go Sarah’s way.

The new drug I mentioned in the last update (Diamox) had to be stopped after about 4 days due to adverse side effects. HOWEVER, Sarah has started on yet another new drug from Canada (not FDA approved) called Clobazam. This drug is known to be helpful in treating Dravet’s Syndrome.

Right now we are working on reducing 2 of her other seizure drugs since we have added the Clobazam. Medicine dose changes can take a long time – you have to do them gradually.


School

Sarah missed the first two weeks of school due to lack of seizure control, then she went to school for about a week, and then had homebound status for the next 6 weeks as we worked out the seizure control, and she had the pneumonia. Her first day back was Halloween – and she is only going half days at this point because she is still very tired and lethargic.

We are working on tracking down the reasons for the lethargy. One reason is that she has a urinary tract infection, which we just found out about and started treating. Another reason may be she is still trying to gain strength after having pneumonia, and another reason could be the amount of meds she is on, which as I mentioned before, we are gradually reducing.

Hopefully this will all add up to Sarah feeling much better before too long, and being able to learn and do more.


Vision

Sarah recently got a new pair of eyeglasses provided by the Kirkwood Lions’ Club. The glasses help her to use both of her eyes together. This is very important, as in another 2 months her eye doctor will determine whether she needs another surgery to further correct the alignment of her eyes. It is our hope that with the new glasses she will be able to do the rest of the correction herself by learning to control her eye muscles better. Sarah’s vision has improved a lot in the past 3 years, and may continue to do so for several more years. Thanks very much to the Kirkwood Lions.


Bicycle

St. Louis Variety once again came through for Sarah by providing her with her very own bike! There are pictures of her on it on the photos page. She has enjoyed riding it on our deck and around the neighborhood. It is very good for her muscles, joints, and circulation to ride the bike, and the best part is she enjoys it. Like all equipment for special needs kids, these bikes are very expensive. We cannot thank Variety enough for all they have done for Sarah.


In Conclusion

I try to keep these updates as short as I can but I don’t do it often enough to be able to keep them very short. Thanks for sticking with me this long. And thanks very much for your interest and support.

Love,
Sandy



August 7, 2007

Medical News


Since we last updated a lot has happened. Sarah finished her first school year at Southview, and missed the last week of school due to being in the hospital (SLCH) with pneumonia. She was sick for about 3 three weeks total, then started summer school, which was over in mid-July.

We had been trying since December to help Sarah with her gagging/choking spells that she was having three or more times a day. It was frightening for Sarah and for us because without intervention, Sarah could not stop choking. It was one of these spells that caused the pneumonia, as she aspirated fluid into her lungs. Nothing the doctors tried was working. Since March, her school decided it was too risky to feed her orally.

In April we finally hooked up with a wonderful GI doctor, Dr. Lynda Brady, who had a different idea of what might be going on and how to treat it. Sarah is now having only a couple of gagging/choking spells a week – instead of several times a day. This was such a huge change for the better, after 5 months of these episodes, and hopefully she can go back to eating lunch at school this fall.

Sarah had two recent surgeries. The first was eye muscle surgery on July 17th. We had planned to do it earlier, but it got cancelled because of the gagging/choking. This surgery aligned her eyes so that she can see the same scene with both eyes. Before, she would see two different scenes with each eye, and her brain could permanently shut one eye off if this was not corrected. We are excited about the improvement in her vision that this will bring.

However, what was to be same day surgery turned into a 4-day admission with 2 status (prolonged) seizures of about 1 hour each, and clusters of shorter seizures. Both seizures took a lot of IV medicines to stop. These were the first status seizures Sarah has endured since the five-hour seizure.

Which brings me to the incessant topic of seizures. Since the beginning of July Sarah has had a marked increase in seizures in quantity and length. In fact, as of right now, her neurologist does not think we should send Sarah to school until the seizures get better. Dave and I finally made the decision to have the VNS (Vagus Nerve Stimulator) implanted, a decision we had been struggling with since last fall.

So, the week after the eye muscle surgery, Sarah underwent another surgery to implant the VNS. It will be a number of weeks or months until it (hopefully) starts to reduce her seizures. We won’t know what the maximum benefit will be for a year. There is somewhere around a 60% chance that it will help her to some degree, depending on whose statistics you believe.

We also started her on a fifth anticonvulsant medicine (that’s five at the same time, and the 12th medicine we have tried for seizures) as hopefully a stop-gap until the VNS starts working. The new medicine only works for about 3-4 months, then the person develops a tolerance and it stops working.

OK – NOW FOR THE GREAT NEWS:

Sarah finally got her Lite Gait for use at home, thanks to the awesome generosity of St. Louis Variety. It was delivered in June. After 3 insurance appeals and an appeal to the state of Missouri over a year and a half, Variety stepped in and provided this very important equipment for Sarah’s development. You can see her walking in the Lite Gait at Good Shepherd on the movies page. We are so thrilled to be able to do this therapy at home, and look forward to sharing more movies of Sarah’s progress.

If you are not familiar with Variety, please visit their web site. They are so very important to the special kids of St. Louis. They have provided many other pieces of equipment for Sarah, including her wheelchair, feeding chair, car seat, and stander. We recently added an article about Sarah from their newsletter to the Articles page – it is from 2004, but for some reason (!) I was a little disorganized that year and lost track of it until now.

The other great news is that Sarah is going to be on the Sarah Lopez waiver. This is a Medicaid waiver program in the state of Missouri that provides Medicaid as a secondary insurance to 200 children in the whole state. Sarah has been on the waiting list for nearly 3 years. We got the news in June that Sarah was going to be receiving the waiver, but as of right now we are still waiting for all the paperwork to take effect. This will be a huge help to our family financially, which comes at a time when the fund that was started 3 years ago for Sarah’s expenses is getting very low.

SO – we are looking for the new seizure interventions to take hold, so we can get back to helping Sarah move forward, get stronger, and most of all HAVE MORE FUN!

Thank you for your interest.
Love,
Sandy



February 8, 2007


Sarah Jane is currently 5 years old and attending Southview School. She has come a long way since her big seizure nearly 3 years ago - the anniversary will be March 28.

In July 2006 she completed 12 more HBOT treatments, for a grand total of 109. She was very sick in August and September, resulting in 2 different hospital admissions for GI problems. After not having the opportunity to do her weekly Lite Gait therapy at Good Shepherd School for many weeks due to being so sick, when we started back in October, she made a huge leap forward. She began taking many more independent reciprocal (left,right,etc) steps. There are videos on the video page of this site showing her progress. We are continuing Lite Gait at Good Shepherd, and continuing our year long dealings with the insurance company to get one for home use. We will have the final answer on that within 30 days.

Unfortunately Sarah's seizures are continuing despite giving her the max doses of 4 anti-seizure drugs daily. The seizures have progressively gotten worse since May 2006. We are currently taking her to St. Louis Children's Hospital for most of her care, and the experts there are recommending surgery to implant the Vagal Nerve Stimulator, or VNS. We are thinking about this, and in the meantime, we have started another therapy called neurotherapy which has been successful for some people in helping control seizures. We are working with a local doctor, Dr. William Collins, who has also consulted with Dr. Jonathan Walker in Dallas, Texas, in determining the treatment protocol for Sarah. We are actually doing most of the therapy at home in order to progress faster and greatly decrease the cost. So far we have done over 20 treatments. We do this almost daily - the goal is daily, but we have missed a few here and there - for example last Sunday, which was Graham's (Sarah's little brother's) first birthday party! Well, actually, Saturday too, which was the day we cleaned up the house for Graham's birthday party.

Sarah and Graham don't have a lot of chances to do things together at this point. They do interact though, and we are looking forward to more of this as Graham gets a little older. He is a very happy strong baby who brings a lot of joy to all of us.

Sarah's vision has improved to a great degree, and she is on her second pair of prescription glasses. (She outgrew the first pair). We just had extensive testing done at SLCH to determine what she actually can see at this point, and we are planning on having eye surgery done soon to correct the alignment of her eyes. Sarah participated in Therapeutic Horsemanship in the spring and fall of 2006 and we are thinking about whether to sign her up again for this spring. It is a wonderful program and we have seen Sarah benefit from it.

Thank you so much for visiting us here. We are on a mission to improve this website and update it more frequently. God Bless you!


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