Mom's Journal, JUNE 2009

- Well, the Ketogenic diet is not over yet! We had one final chance to test it’s efficacy. Our neurologist suggested that we increase the ketone level and then we would evaluate whether there is any change in seizure frequency or intensity. Increasing Hannah’s ketones is easy. She is very ketotic even on a 2:1 ratio, however the challenge is keeping the ketones high through the night. There is no easy way to do this unless you enter into experimentation. Our Ketogenic dietician is wonderful and we worked together to figure out how to maintain high ketones during sleep. One would think that ketones would naturally climb during sleep when Hannah is fasting, but the opposite is true. One of two scenarios may be at play here. Either she is getting too many calories and through the night is able to convert stored fat to carbohydrate, thus dropping her ketones. The other scenario is that she is not getting enough calories at night and is burning her protein for energy when she runs out of fat to burn. Either one will drop the ketones, now the question became, “what is Hannah’s scenario”. So we literally picked a scenario and went with it. We decided that since her weight was healthy and she was not putting on excess pounds, that it was more likely that she was running out of fat to burn and was therefore burning protein. We increased her ratio to 2.5 grams of fat for every 1 gram of carb/protein combined, for the first four meals of the day. Then at night, we increased her ratio to 3:1 with 50 more calories, to allow for more fat to burn overnight. Within a few days, her ketones climbed from her normal blood ketones of 3.8mmol/L to 5.5 mmol/L. We kept a log of seizures versus ketones for three weeks and noticed a definite trend. If her ketones are lower than 2.4 mmol/L she has a seizure during sleep. If only it was this easy. While we definitely see seizures with low ketones, we also see the odd tonic when her ketones are in the 5-6 range, so we are on the right track, but it is not always the answer behind seizures.

- The first week in June, she had about 4 tonic seizures and 2 head drops. The second week she had only 2 tonic seizures. The third week she had 4 tonic seizures. The last week, she had none. Climb aboard the wildest rollercoaster ride in the west! Both Steve and I are convinced that the Ketogenic diet still controls some of Hannah’s seizures and we are not going to take her off of the diet when she is unstable. I think that would be a very bad idea. The Ketogenic diet has never been the complete answer for Hannah but it has certainly always helped somewhat. In Lennox Gastaut syndrome, you have to take the positives and embrace them, and if the diet controls a small percentage of seizures, than it is worth the time and effort. Hannah really doesn’t complain about her food, as long as you let her chose the keto-meal plan that she wishes. She tends to stick to 4-5 basic meals that are her favourite. She loves ground beef, olive oil and brown rice. But her all-time favourite is brown rice, olive oil, and hemp hearts. This is the meal that we use for bedtime snack because she likes it to be “extra-buttery”. She insists that we call the olive oil “butter”. We are able to add the high level of olive oil to the rice and hemp heart mixture, to make almost an olive oil soup, and she loves it (Lord knows why?????? But she does). Yuck!

- We have had some promising news in relation to our quest to bring a new medication to Canada for Lennox Gastaut Syndrome. The medication is called Rufinamide (I think I told you about it in the May Journal entry). Rufinamide is a new anticonvulsant, and the first ever anticonvulsant designed specifically for LGS. It has a different mode of action than any other available anti-seizure medication on the market because it does not depress the central nervous system. Instead it works on sodium channels at the neuron axon level, decreasing neuron excitability, without the negative cognitive side effects. This medication is approved in the USA as an add-on therapy but as yet has not been prescribed in Canada. We were told that rufinamide was not being made available in Canada by Easai Pharmaceutical, the company that manufactures it. Well, once again, this was not the case. After endless phone calls and emails to Easai, they are definitely willing to make this medication available in Canada. I could go on and on with my intense frustration that we were tasked with discovering the truth and the local health system was incorrect in their information, but I will just get cranky.....so I will leave it alone. Long story short.....we wait and hope that this will soon be an option for Hannah. If our quest is successful, Hannah will be the first child in Canada to be prescribed rufinamide. Keep your fingers crossed!

- We are waiting for an MRI to be scheduled at the hospital, but have heard nothing yet. There seems to be a very long waiting list, even when the requisition is for an urgent MRI. Hannah’s metabolic/genetic consult is not until October. Mommy is far too impatient to wait, but unfortunately, there is no way around it. She was scheduled for a renal ultrasound and a cardiology consult (routine tests for kids on the Ketogenic diet) on June 30 but I don’t have the results yet.

- Good news!!!! Hannah’s new AFO’s (ankle foot orthoses) are working spectacularly well and her gait has improved significantly. She now wears the AFO’s predominately during sleep to stretch her heel cords. This is perfect because it allows her the freedom to run and play and work her muscles during the day. The physiotherapist is closely monitoring her progress, and we while we might need to consider the AFO use during the day, so far night time use is working well. We are very excited to see such a drastic and nearly immediate improvement. We love our physiotherapist!!!!

- I had two weeks of scheduled holidays from work, so we decided to make the best of it. We planned a camping trip to Calaway Park just west of Calgary for June 5, 6,and 7th. Due to the boys’ hectic baseball schedules, we do not have a lot of time for family camping, so we were determined to make time. We prepared all of our gear, and headed out on Friday night for a fun-filled weekend of camping and theme park rides. I am not sure if we have bad luck or we just choose poorly, but....you guessed it.....we woke up Saturday morning to 3 inches of snow!!! This was the first reported June snowfall in Calgary in over 30 years! Sheessshh!!!! Oh well, a little snow and cold wasn’t going to stop us. Hannah was having a great weekend and was very stable. We have learned to embrace the good times and make the most of them! We put on our winter gear, with rain coats over top, touques, mittens, etc and headed out to the park. We had a blast, and no surprise, there were no line-ups for the rides because no one was stupid enough to go to Calaway park in the snow! (except us of course!). We went on every ride in the park at least once over the next two days. Even Grandma and Grandpa braved the weather and met us on Sunday for a day of fun! It was great...cold, but great! Hannah made it through the entire weekend with only one head drop! Yippee! Snow in June???? No match for the Ellefsons!!!!

- Hannah had swimming lessons in school at the beginning of June and she loved every minute of it. She was quite smitten with her young handsome swimming instructor!!! What a flirt Miss Hannah can be!!!

- Kindergarten was a great success! Hannah loved it! She did very well, and made some very good friends. Hannah graduated from kindergarten on June 21!!! Next year, GRADE ONE!!!! I can’t believe it! She is growing up so fast. Hannah’s kindergarten teacher was the best of the best. The day was filled with mixed emotions. All three of our children had this wonderful teacher for Kindergarten. She is moving to a new school in September, and things will not be the same around our school without her there.

- Kalen finished Grade 4 with a very good report card and Connor has successfully completed Grade 1. Connor had some tears on the last day of school. He absolutely adored his Grade 1 teacher and he was completely devastated when he walked out of her classroom for the last time. He wanted to be in Grade 1 forever! It was so sweet. We are hoping that Hannah will be lucky enough to get Connor’s teacher in September.

- The last week of June was a bit rocky. We had a nasty flu bug run through the house like wildfire. First Connor got sick, then mommy (it isn’t very common for mommy to be down for the count), and then Kalen got sick. Unbelievably, Daddy and Hannah did not get sick!!!! The flu is a complete disaster for Hannah because she can’t eat her Ketogenic diet, she gets dehydrated almost immediately, and she can’t hold down her meds, so for this small miracle....we are so thankful!

- We are looking forward to a great summer together. We spend our time enjoying life and squeezing as much fun and excitement as possible into every day.

- We are only 150 days away from getting Hannah’s new service dog! We don’t know which dog she will be matched with yet, but here is a sample of some of the dogs currently in training:

Buckeye the service dog

Elmo the service dog

Lucky the service dog

<< MAY / JULY >>