I decided to start putting all my updates about Luella on a blog. We've had so many people ask about how Luella is doing and I wanted a way for people to keep updated without me having to email all the time or post on facebook. So if you want to keep up to date on Luella you can follow Little Luella's blog and read about it here.
I will probably go back and add some older blog entries about things that have happened in the past. So if you are interested to read Luella's history you can check that out as I have time to add more.
And please continue to pray for Luella and our family!
Tuesday, August 30, 2011
Appointment at Gillette Children's
We just returned from a trip with Luella to Gillette Children's Specialty Healthcare in St. Paul, MN. We have been seeing a doctor at the Mayo Clinic and she's been great. She's the one who initially suspected that Luella had Dravet Syndrome and ordered the genetic testing. We were happy with the Mayo Clinic but Jim has been doing a LOT of reading online and found out about a Dravet specialist in Chicago. We were debating whether or not to see Dr. Laux there when Jim found out about another Dravet specialist right here in Minnesota - in St. Paul. We were anxious to see if we could get an appointment with Dr. Wical. We were excited to find out that we could get in to see Dr. Wical AND in only 1 1/2 weeks!
Yesterday we got up super early and headed to St. Paul for Luella's appointment. Gillette Children's is a great place and we really loved Dr. Wical! We visited with her for about two hours and she had a lot more insight to Luella's Dravet diagnosis. She was pleased to see how well Luella is doing developmentally. There still is no way to know if or when Luella may start to regress or have developmental problems, but she did assure us that having Dravet did not mean that she would FOR SURE have those kinds of problems. In the past, the only way doctors were able to diagnose Dravet was just by seeing them so most of the early cases of Dravet were only the kids with severe developmental delays. With this relatively new genetic test, more and more children are being diagnosed and they have found some kids that do not have any developmental delays. That was reassuring and we continue to pray that Luella will continue to grow and develop normally.
I had already contacted the school here and a Early Childhood Special Needs teacher will be coming to our house monthly to meet with Luella and do an assessment of her development. Currently Luella is advanced if anything but this will be a way to catch things quickly if something should happen in the future. Dr. Wical also wants Luella to do a developmental psychology evaluation every six months.
Dr. Wical was over-all pretty happy with the current medications that Luella is on. We are going to stop the Keppra, which Jim and I couldn't be happier about. Keppra has caused Luella some behavioral problems and we feel it wasn't helping seizure control anyway so we are glad to be able to wean her off of that! We will be adding Carnitine - a supplement to help protect Luella's liver. Most seizure drugs are routed through the liver and several can cause liver damage so we are glad to add carnitine. We had gotten a new rescue med for seizures that won't stop since the Diastat hasn't helped the past few times we used it. The new medication is Midazolam and we give it with intra-nasal syringe. Dr. Wical told us to think about getting an actual needle and giving it in her muscle. There are times when shooting it up her nose might not work - if she'd have a bad cold or a nose bleed. Also the Midazolam really burns the inside of your nose. A needle would hurt for a second but it wouldn't have the lasting affect of the nasal route. I think I would feel comfortable giving Luella a shot so we will probably be doing that.
One thing we've read about, something that we don't really want to think about is SUDEP - Sudden Unexplained Death in Epilepsy. Dr. Wical asked us if we wanted to talk about that. She said some parents would rather not know. We wanted to hear what she had to say. SUDEP is similar to SIDS in that it usually happens while they are sleeping and they really don't know the cause. Kids with Dravet have about a 10% chance of SUDEP which was better than the 20% we had read about. One thing they do know is that kids with a certain heart condition are more likely to experience SUDEP so Dr. Wical has ordered Luella to wear a Holter monitor for 24 to 48 hours. A Holter monitor is a portable device that will record Luella's heart activity which will then be studied by a cardiologist. We pray that SUDEP is nothing we have to worry about!
We were so happy with our visit with Dr. Wical that we did decide to change doctors. Driving to St. Paul will be a little further than Mayo but we feel it is worth it.
A quick update on Luella herself - she has been taking Stiripentol since about August 10th. Stiripentol is not FDA approved and we had to have it imported from Europe. So far our insurance company has not agreed to pay for it but we are working on that! Dr. Wical even offered to call them on our behalf. Luella hasn't had a big seizure since August 1st. We think she may have had a couple very short ones (under a minute long) on the 10th and 14th but we aren't sure. So we feel like the Depakote and Stiripentol are really working well for her. One thing that is obvious is that the Stiripentol is making Luella much more tired. We've had to start adding a short morning nap again on some days. And she is VERY crabby! We are hoping that getting rid of the Keppra will help but the Stiripentol might be causing some of that too. We were told that the crabbiness from Stiripentol usually improves after being on the medicine awhile too so we are hoping for that!!
Again, we appreciate all your prayers! Thanks so much for praying for Luella and our family as we deal with all of this!
Yesterday we got up super early and headed to St. Paul for Luella's appointment. Gillette Children's is a great place and we really loved Dr. Wical! We visited with her for about two hours and she had a lot more insight to Luella's Dravet diagnosis. She was pleased to see how well Luella is doing developmentally. There still is no way to know if or when Luella may start to regress or have developmental problems, but she did assure us that having Dravet did not mean that she would FOR SURE have those kinds of problems. In the past, the only way doctors were able to diagnose Dravet was just by seeing them so most of the early cases of Dravet were only the kids with severe developmental delays. With this relatively new genetic test, more and more children are being diagnosed and they have found some kids that do not have any developmental delays. That was reassuring and we continue to pray that Luella will continue to grow and develop normally.
I had already contacted the school here and a Early Childhood Special Needs teacher will be coming to our house monthly to meet with Luella and do an assessment of her development. Currently Luella is advanced if anything but this will be a way to catch things quickly if something should happen in the future. Dr. Wical also wants Luella to do a developmental psychology evaluation every six months.
Dr. Wical was over-all pretty happy with the current medications that Luella is on. We are going to stop the Keppra, which Jim and I couldn't be happier about. Keppra has caused Luella some behavioral problems and we feel it wasn't helping seizure control anyway so we are glad to be able to wean her off of that! We will be adding Carnitine - a supplement to help protect Luella's liver. Most seizure drugs are routed through the liver and several can cause liver damage so we are glad to add carnitine. We had gotten a new rescue med for seizures that won't stop since the Diastat hasn't helped the past few times we used it. The new medication is Midazolam and we give it with intra-nasal syringe. Dr. Wical told us to think about getting an actual needle and giving it in her muscle. There are times when shooting it up her nose might not work - if she'd have a bad cold or a nose bleed. Also the Midazolam really burns the inside of your nose. A needle would hurt for a second but it wouldn't have the lasting affect of the nasal route. I think I would feel comfortable giving Luella a shot so we will probably be doing that.
One thing we've read about, something that we don't really want to think about is SUDEP - Sudden Unexplained Death in Epilepsy. Dr. Wical asked us if we wanted to talk about that. She said some parents would rather not know. We wanted to hear what she had to say. SUDEP is similar to SIDS in that it usually happens while they are sleeping and they really don't know the cause. Kids with Dravet have about a 10% chance of SUDEP which was better than the 20% we had read about. One thing they do know is that kids with a certain heart condition are more likely to experience SUDEP so Dr. Wical has ordered Luella to wear a Holter monitor for 24 to 48 hours. A Holter monitor is a portable device that will record Luella's heart activity which will then be studied by a cardiologist. We pray that SUDEP is nothing we have to worry about!
We were so happy with our visit with Dr. Wical that we did decide to change doctors. Driving to St. Paul will be a little further than Mayo but we feel it is worth it.
A quick update on Luella herself - she has been taking Stiripentol since about August 10th. Stiripentol is not FDA approved and we had to have it imported from Europe. So far our insurance company has not agreed to pay for it but we are working on that! Dr. Wical even offered to call them on our behalf. Luella hasn't had a big seizure since August 1st. We think she may have had a couple very short ones (under a minute long) on the 10th and 14th but we aren't sure. So we feel like the Depakote and Stiripentol are really working well for her. One thing that is obvious is that the Stiripentol is making Luella much more tired. We've had to start adding a short morning nap again on some days. And she is VERY crabby! We are hoping that getting rid of the Keppra will help but the Stiripentol might be causing some of that too. We were told that the crabbiness from Stiripentol usually improves after being on the medicine awhile too so we are hoping for that!!
Again, we appreciate all your prayers! Thanks so much for praying for Luella and our family as we deal with all of this!
Wednesday, August 10, 2011
Mayo Trip
Things went well at Mayo. We just got home yesterday around 4:30pm. We were able to meet with a Dietitian and get some more details about the Ketogenic diet. We don't currently have plans to start the diet but it was good to talk to the Dietitian and find out more about how they do the diet at Mayo. Our current plan is to focus more on finding the right drug for Luella. She just started Stiripentol this morning. Stiripentol is one of the drugs that is supposed to work well for Dravet kids. Unfortunately it isn't currently FDA approved so it's not as easy to get. We had to order it from a pharmacy in New York and we are currently working with Insurance to see if they will cover it. There is also one other drug that we could add - Clobazam. Depakote, Stiripentol and Clobazam together are supposed to be great. We are just starting with adding the Stiripentol to her Depakote. She is also still taking Keppra! Our house looks like a drug store!!
At Mayo on Monday, Luella didn't particularly enjoy getting the leads for the EEG placed - they actually glued them to her head! She cried so hard!! Once they were placed and her head was wrapped up she was fine. They gave us a lot of slack in the wiring and we were able to move around pretty well in our room. The EEG was mostly to determine if Luella's night time twitches were small seizures and to see if she was having absence seizures during the day. Fortunately she is NOT having night time seizures or absence seizures. We still aren't sure why she is twitching so much at night. Her iron levels were lower so in addition to all her other meds she is taking an iron supplement. Low iron can cause restlessness. It can take up to 3 months to build up iron and we've only been on it about a month so we'll see if things start to improve. We were just relieved she wasn't having seizures!
Taking the leads for the EEG off wasn't exactly fun either. Since they were glued on they had to use acetone to dissolve the glue. Stinky stuff!! (Think finger nail polish remover.) We washed her hair there and I scrubbed it again at home. She still has some glue on her scalp but she is tired of me picking on her so we'll work on it later. She has red marks all over her forehead still from the glue/leads.
All in all it was a good visit. We got to talk more with Dr. Wong-Kisiel. It's great how she really takes time to answer all our questions.
Thanks again for all your prayers and support. We really appreciate everything!
At Mayo on Monday, Luella didn't particularly enjoy getting the leads for the EEG placed - they actually glued them to her head! She cried so hard!! Once they were placed and her head was wrapped up she was fine. They gave us a lot of slack in the wiring and we were able to move around pretty well in our room. The EEG was mostly to determine if Luella's night time twitches were small seizures and to see if she was having absence seizures during the day. Fortunately she is NOT having night time seizures or absence seizures. We still aren't sure why she is twitching so much at night. Her iron levels were lower so in addition to all her other meds she is taking an iron supplement. Low iron can cause restlessness. It can take up to 3 months to build up iron and we've only been on it about a month so we'll see if things start to improve. We were just relieved she wasn't having seizures!
Taking the leads for the EEG off wasn't exactly fun either. Since they were glued on they had to use acetone to dissolve the glue. Stinky stuff!! (Think finger nail polish remover.) We washed her hair there and I scrubbed it again at home. She still has some glue on her scalp but she is tired of me picking on her so we'll work on it later. She has red marks all over her forehead still from the glue/leads.
All in all it was a good visit. We got to talk more with Dr. Wong-Kisiel. It's great how she really takes time to answer all our questions.
Thanks again for all your prayers and support. We really appreciate everything!
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| Getting leads placed. |
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| Playing with mommy. |
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| Still happy! |
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| Wrapping a doll's head like her "hat". |
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| Playing with daddy. |
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| Bad hair day!! |
Wednesday, August 3, 2011
Another Big Seizure and Hospital Stay
On Monday morning I posted on facebook that Luella made it the whole month of July with only the ONE seizure on the way home from the Black Hills. About an hour after that post (on Monday), Luella had another seizure. It stopped on its own and she slept for about 30 minutes. When she woke up she started seizing again (around 2pm on Monday). It did not stop with Diastat so we rushed to the ER again. It took awhile in Luverne to get her seizure under control. Then we took the helicopter (4th time) to Sanford Children's in Sioux Falls. Luella was doing well there, mostly sleeping due to all the drugs. Around 8pm as I was holding her she stopped breathing. A team of doctors and nurses were there quickly and got her stimulated enough that she started breathing again. They kept the oxygen on her and noticed her breathing increasing and heart rate going up. Her eyes were closed and she was not responding. She was seizing again. They gave her more meds and got it to stop. It was pretty scary. I think there were about 8 people in her room and several more standing out in the hall. We probably would have been able to come home on Tuesday but because of the seizure Monday night they wanted us to stay longer so we were there 2 nights and came home this morning (Wednesday) right before lunch.
Luella's seizure was due to a high fever this time. Ryker came down with a fever on Friday a even vomited a few times Saturday and Sunday. Luella woke up with a fever on Monday morning. I had been giving her Tylenol and Motrin but it wasn't enough to keep the fever down. When Luella stopped breathing in the hospital she had a fever of 103. She hadn't had a fever before that in the hospital so they weren't very aggressive with the Tylenol and Motrin. I was mad at myself for not being more persistent about getting her some medicine. The doctor there said they "dropped the ball" too. :(
I was completely exhausted and took a nap after lunch today. Monday night I only got a couple hours of sleep, last night was better. Because of Luella's breathing scare, both our Mayo doctor and the doctor at Sanford thought it would be good for us to have a home pulse oximeter to have on Luella when she sleeps - it tells us her oxygen level and heart rate. I put it on her at bedtime tonight and she only mildly complained. I think she probably thought, "Well, this is better than all of the stuff I was hooked up to in the hospital!" The Sanford Home Medical people were great in getting it all set up for us today!! Hopefully that will give me a little more peace of mind at night time.
We are fighting hard for Luella and trying to get her the best possible care. Jim has been able to do some more reading about Dravet and we are figuring out our next steps. We'll be heading to Mayo on Monday for Luella's 24 hour study and hope to find out more from that. We want to thank you all again for your continued prayers!
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