April 27, 2010
It’s been well over a year since the last update. Thank you for coming back and checking in!
Last August, the whole family enjoyed the Wiggles live at the Scottrade Center and got to meet them in person! Graham was truly beside himself, and 2 of the Wiggles came right up to Sarah and Graham and sang to them during the show.
Right after the concert we went to Chicago for the International Conference on Dravet’s Syndrome. We learned a lot, met Dr. Dravet and other families from all over the world. We also managed a side trip to the American Girl Store for fun. This trip was made possible with help from three of Sarah’s caregivers who are from Chicago.
After the trip, Sarah started school. She did very well and missed very few days. Sarah also started aqua therapy at Ranken Jordan and really enjoyed her “swimming” sessions (complete with a Hannah Montana bathing suit!).
In November my dad bought us a 2004 full size Ford van to accommodate a wheelchair lift, which was provided in February by St. Louis Variety. It has made transporting Sarah so much easier and safer. We are very appreciative to both my dad and Variety. As Sarah grows we have to adjust how we do things – we still have some modifications needed to our house. Next on the agenda is adapting the bathroom.
In January, Sarah was admitted to the Children’s Hospital for a respiratory virus. She was only there for 5 days, came home and got back to her regular routine within a couple of weeks.
On March 28 we passed the five-year anniversary of Sarah’s “big seizure,” a day she celebrated by taking 119 steps with her left foot on her Lite Gait (mom helped with most of the right foot steps). This was the most steps she had taken in one session since her hip surgery in April 2008. It was wonderful to see such a great accomplishment on that very day.
But the year was about to take a bad turn. Sarah was admitted to SLCH on April 7, 2009 with a respiratory virus, which worsened very rapidly. She moved to the PICU on April 9, the day before her 8th birthday. She had developed ARDS (acute respiratory distress syndrome), which is very serious and has only a 50% survival rate. She was put on a ventilator, and due to respiratory and cardiac failure on Easter morning progressed to an oscillating ventilator, which is almost the last resort. She had 2 chest tubes, 13 medicine pumps, and her prognosis was not good. We were told that if she survived she would likely need a tracheotomy and be permanently on a ventilator. Sarah spent several weeks in the PICU before moving to a regular room, and her recovery was nothing short of miraculous. No trache, no vent. The doctors were totally amazed and needless to say we were grateful beyond description. She did sustain permanent lung damage, and now sleeps with oxygen through a nasal canula at night. We brought her home May 16, 2009.
All of this time in the hospital was really hard on Graham, who handled it as well as any 3 year old could. He stayed overnight with a variety of people and never knew for sure what was going to happen next. He thrives on routine, and this year has had some periods where the routine did not exist. I am very proud of him for rising to the occasion and being so good and brave, and at the same time am sad that he felt he had to be so good and brave. He saved the bad behavior for me after we all got back home. I totally understood what was going on though, and we got through it.
After coming home from Children’s, Sarah began slowly gaining her strength back. She was back in the hospital 3 times over the summer, once for a UTI and two other times for a respiratory illness. She had GI problems for a couple of months after the ARDS, which caused her a lot of discomfort and distention at times. On the positive side, Sarah did well with her therapies and really enjoyed being home with her family. It was wonderful to see her beautiful smiles and hear her happy voice again.
Sarah’s Baclofen pump was five years old in June of 2009, which is the recommended age to replace it due to the batter life. Sarah was not well enough in June to undergo surgery, and we had to cancel a surgery date in August because Sarah was already in the hospital with pneumonia. On September 14, 2009 she did have the surgery, and did very well, and went home Sept. 15. Unfortunately we returned for two more admissions during the next three weeks, due to more GI problems caused by the anesthesia and then another pneumonia.
During one of these admissions we were told that an x-ray showed “multiple” spine fractures. Further investigation showed that these had started and had been accumulating since 2006. We are now in the process of figuring out why her bones are in this state, and how to improve them. We have done some testing already, and will do inpatient tests in early June.
Sarah has already been hospitalized 4 times this year, but in between is happy and doing well with her homebound teacher and therapists. I am working on getting more therapy for the summer, and we hope that her health improves to the point that she can finally go back to school in the fall.
Sarah had a great 9th birthday this year - she was well and enjoyed being with family and friends.
Thanks again for checking in!
July 21, 2008
It has been 5 months since the last update. The updates would be shorter if I did them more often! Sarah had bilateral hip surgery on 4/21/08, and did very well. She was in the PICU for 4 days and then sent home. She had surgery on both femurs, and also on the right hip socket. She is now back to weight bearing and has started working on her Lite Gait again. We have had 3 follow ups with the surgeon and he is very pleased with how Sarah is healing. It will be several more months before she regains all of the strength she had pre-surgery, but she is on her way.
Sarah had a few other hospitalizations since the last update, one for seizures, one for a respiratory illness and one for a 24 hour eeg.
Sarah is currently doing VERY WELL in many ways! She is happy and alert every day, she is not tired like she was for several months over the winter and spring. She had perfect attendance at summer school, and did well every day at school. Last school year she only attended school 30 days - she was homebound the rest of the school year. She will be going back to school full time August 14 - we are hoping for a much better year.
New things we have noticed - an increase in the use of her hands and generally more responsiveness and interaction. Over the past 3 weeks or so Sarah has begun to consistently put her hands in her mouth - especially the right hand. Her grasp is very good right now, and she is moving her fingers individually in a way we have not seen before. She is very vocal at times, and very expressive with her face. It is so great to be able to connect with her more.
Sarah has had a lot of congestion for a number of months (since March). We saw an allergist who tested her and we found out she is not allergic to all the stuff they tested for. The allergist prescribed a nebulizer treatment which we have added to Sarah's already formidable medicine regimen, but it has really helped. We were having to suction her 4-5 times a day before the nebulizer, and now 3-5 times a week. We are waiting to see a pulminologist about this next, as no one so far knows why she is so congested. We had not used our suction machine very much at all for the past 3 years or so, so this is a big change.
In May, one of Sarah's caregivers who was graduating from SLU, Katie Karpinski, put together a hugely successful Trivia Night fundraiser for Sarah. Candace Bingham, who organized a big fund raiser in 2004 for Sarah, and my long time friend Barb Ellis helped put it together, as well as many volunteers from Katie's service club, Alpha Phi Omega. A lot of work went into this event, all during the end of the school year - we were very touched and grateful for all the efforts on Sarah's behalf. She will greatly benefit from the funds that were raised. Thanks again to the people who created the event, and to everyone who came - it was a fun night.
We have a big week coming up - August 5 we will be going to see the Wiggles Live here in St. Louis - Graham is a HUGE fan, and Sarah is also a big fan, although being 7 years old, she is not quite as fanatical as Graham at this point. We have FRONT ROW SEATS. We also hope to meet them in person, as another of Sarah's caregivers, Emily Heilman, has a cousin who is on the Wiggles crew. Then we are driving to Chicago for a multi-day conference about Dravet's Syndrome - which starts the morning after the Wiggles concert. It looks like it will be a great event - they seem to have thought of everything when they planned it. All four of us are going, and we will have the help of 3 of Sarah's caregivers who happen to live in Chicago. More about all this after we get back.
Thanks for checking in. There is a new movie on the movies page (the piece Variety did on Sarah for their telethon) and some new pictures in the photo area. The Variety piece was filmed during those months when Sarah was so tired and not responsive - unfortunately she does not sparkle in this video like we know she can - but it is still very nice. Variety has been extremely helpful to our family.
See you next time -
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.
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?
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.
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.
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.
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.
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.
August 7, 2007
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.
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!