On Nov. 29th we started giving Joshua Carnitor which is a supplement for his Carnitine deficiency that was prescribed by our keto team at Swedish. On Monday, Dec. 3rd Joshua started not eating all of his meals and started acting a bit "off". This continued through that week and by the end of that week he was not eating at all and barely drinking. Due to him not eating and drinking we were not getting his vitamin supplements in him and he was feeling worse and worse every day. We spent the weekend talking to on-call doctors who didn't know much and so I talked to some of my fellow keto moms and tried to do what they told me, but nothing was really working as we couldn't get him to take anything. I went out of town on business Monday, Dec. 10th for 3 days and left him in Chip's capable hands. Chip was in contact with our Swedish team all day Monday and Tuesday and by Tuesday afternoon they told Chip he had to take Joshua to the ER for IV fluids and a suppository. So, off Chip went to the ER with both kids and my Mom was nice enough to meet them there since I couldn't be there. I tried to get an earlier flight home, but could not and I had a flight out early the next morning so stayed with my original flight. Joshua was in the ER for about 5 hours and got a couple rounds of IV fluids and was sent home. It turns out that Carnitor actually boosts ketones and so his ketones shot up too high and put Joshua into ketosis which made him lethargic, nauseated and not want to eat. Then when he wasn't eating, his ketones continued to rise and it just continued to get worse and it is hard to break the cycle until you can get their ketones lowered. His ketones in the hospital were 6.88 which is very high for him.
The Swedish team worked with us for the next couple days to get his ketones lowered - we reduced the amount of Carnitor he was getting from 3 MLs to 1 ML twice a day and we reduced his ketogenic diet ratio from 3:1 to 1:1. The 1:1 ratio on the diet only lasted one day (last Thursday, Dec. 13th) and then we took him back up to 2:1 the following day and that is where we have stayed (for now). It worked as he is now eating, drinking, taking all his vitamin supplements and having more energy. We will probably slowly work our way back up to 3:1 or somewhere between 2:1 and 3:1 until we find the "sweet" spot. I thought for sure as soon as we lowered his ratio he would start having seizures again and we would lose all the control we had gained and we did, but just the 1st night. He had 1 seizure the night we dropped his ratio to 1:1 and he was seizure free the 10 days prior to that (our longest streak). However, after that 1 night he has not had any further seizures that we can see. This is great news considering his ratio is lower and he is still not having seizures. As an aside, during this week Chip's car broke down, our computer broke and I got sick with a cold - it was just a lovely week for us.
Yesterday (Dec. 19th) we took him into the hospital for a 24 hour EEG to see how much activity he is having, especially the sub-clinical seizures (ones you don't physically see). I was very anxious as I really wanted this to go well so we could talk about weaning him off more medications. This is the 1st EEG we have had since starting any of the diets (our last EEG was in April). We went in yesterday at 12:00 and he was hooked up by about 3:00 and they started recording. We, of course, can't see the EEG charts while they are recording so we have no idea what they look like while we are there. He did super getting hooked up and was a real champ - for those of you who don't know it takes about 45 minutes to an hour to get hooked up which is a LONG time for a 4-year old. Once he is hooked up he is confined to the room as he is connected to all sorts of wires to monitor him. We did not see any seizures while we were there, not even while he was sleeping which is of course, what I wanted. He did well over night and him and I hung out most of the morning. They brought in a Wii to play so he was having fun playing Mario Kart most of the time we were in the hospital. They came and un-hooked him today about 2:30 and then we headed over to his neurologist's office to get the results of the EEG.
HERE COMES THE GOOD NEWS!!!!
Joshua only had 2 (count them - 1 and 2) sub-clinical seizures in a 24 hour period!!!!! This is AMAZING news for him! He had 0 clinical seizures and only 2 sub-clinical. For those that don't know or don't remember, Joshua's very 1st EEG in January 2012 showed he was having seizures approx. every 10 seconds! Every 10 seconds to 2 in 24 hours is nothing short of a miracle! I couldn't believe it. I really didn't know what to hope for going in to today's results - I just knew I wanted less sub-clinical activity than before and boy, did I get it. He had 1 seizure 10 minutes after he fell asleep and then another one at about 4:00 in the morning and that WAS IT!!! I still can't believe it - not even as I type it. The ketogenic diet (not any drug) has worked a miracle in my son and I am so lucky to have found it. I started crying happy tears right there in the lobby as we were talking as it was just so exciting. So, what does that mean going forward???? It means we are now going to wean him off of another drug - Zarontin! We start weaning tomorrow morning and I'm so excited about it. After we get Zarontin weaned then we will talk about his last drug (Zonegran) and see if we will wean straight out or get another EEG and then decide what to do. And, the awesome thing in all of this is that his ratio is still only at 2:1 so I can't help but wonder that if we went back up to 3:1 then perhaps his sub-clinical activity would be 0? I REALLY wanted Joshua to go seizure free for Christmas and start getting off some of his drugs and I got my wish! I'm just so thrilled - I don't need anything else. Also, Joshua's previous EEGs showed some background slowing and this EEG showed none so we got rid of that too - AWESOME!
I'm feeling like all of our hard work has paid off and we have PROOF that we are doing the right thing by keeping Joshua on this diet. It is working when none of the drugs did and it is unbelievable the progress he has made in 7 months since we started diet therapy. I feel like we are getting back on the right track - he is eating, drinking, smiling again, has more energy and is having hardly any seizures! Whoo Hoooo!!!! We have not "seen" a seizure since Dec. 13th so today will be 7 days of clinical seizure freedom if he has none tonight. It is not the longest streak yet, but hoping this one sticks and we have no more breaks. It would be awesome to have no seizures in 2013 at all!
I am so thankful for all the Doose parents who pushed me in the direction of MAD and Keto and for the awesome support of our family and friends through all of this madness. It feels amazing to be supported as I have talked to many parents whose families are not supportive and that just makes the whole process that much harder. Chip and I are very lucky to have such amazing families and friends that have helped us through this past year.
Can't stop crying happy tears tonight and I can't wait to celebrate a wonderful Christmas with my kids.
All My Love,
Michelle
A few pics of him during his EEG - what a trooper! He truly is my hero - love my little guy so much!
Measuring his spots on his head for the leads
Sorta smiling as they got the first few on
Not so sure he is still liking it, but hanging in there
All hooked up - lots of wires hanging out!
A grumpy moment! :-)
Getting un-hooked
It's so exciting to read about it, even after you told me last night. Tears from Auntie Dawn, too! Love you!! YEAH!!!!!!
ReplyDeleteThat is so great. It's the best christmas present you can get!!!
ReplyDeleteUncle Jimmy