8 months old!

This past week we have seen miracles happen for our sweet little Mason. A month ago Mason was still having spasms and his EEG showed no improvement. Dr. Moosa increased Mason's Sabril dosage to the max amount for his weight and age at this point.  Last week Mason had been on the max dosage for 4 weeks and was still having spasms (although they had reduced a lot).  The max dosage of Sabril knocked Mason out.  For 4 weeks straight he slept about 22 hours a day.  He would wake up when it was time to eat and then fall back asleep right when we started feeding him. He even slept through therapy:

He woke up so I started taking pictures. then within 10 seconds he was back asleep. haha.

I kind of think he looks like Chris Soules in this pic. See any resemblance?

Dr. Moosa didn't like that the Sabril made Mason so sleepy so he decreased his dosage last Wednesday a little bit. After a few days of the dosage change Mason smiled and laughed!!!!!!!!!!! He hasn't smiled in 4 long months!  He also started sucking on his hands again and batting at toys.  Although the dosage is lower he hasn't had any spasms either.  This is HUGE progress! We meet with Dr. Moosa on Thursday and he might want to do another EEG to see if it's improved at all. We are so excited for this progress! Go Mason! What's cool is that Casey's parents have been visiting us and they've gotten to see all of these awesome changes in Mason too! 

Mason and Holland kind of have the same look going on. 

Love these sweet kids.

Mason is a little worried...

Happy 8 months sweet boy, we love you so much and we're so excited to see what next month brings! 

Mason's G-tube

Mason finally got a G-tube! I say finally because he originally was supposed to have the surgery the first of November but then he got pneumonia...so we waited until he was all better and rescheduled for the end of November. 10 days before the scheduled surgery he got diagnosed with Infantile Spasms and got hospitalized.  He got put on the ACTH injections and we had to wait until 2 weeks after he was done with the injections to schedule surgery again! So 3 months later he finally got the surgery! We didn't want him to have surgery until he was healthy enough but boy was it a pain to have to keep rescheduling!
We had to be at Cleveland Clinic at 6 am so we had to leave our house at 5:15. My amazing friend Teri came over to our house at 5 so we wouldn't have to wake the girls up early.  She stayed at our house all morning and got the girls all ready for school and drove them around.  Thank you Teri!
I was pretty impressed with how efficiently everything ran when we got to CC at 6 am.  Mason's surgery was scheduled at 7:30 ( he couldn't have anything to eat after midnight) and I was expecting to be sitting around waiting forever like I have had to in the past when I've gotten surgery.  However, after getting all registered and getting our pager we were in pre-op at 6:30! The nurses checked Mason all out and asked questions, then the anesthesia team came in and went over more stuff. I was pretty anxious because a poor child in the room next to us was extremely upset (probably starving) and I was worried Mason would be so hungry he would start crazily crying too but he just slept in Casey's arms while we waited for everyone to come talk to us. At about 7:15 Dr. Seifarth (pediatric surgeon) arrived and went over what the surgery would entail. He said they would perform the surgery through endoscopy.  He would put a camera down Mason's throat and into his tummy to identify where the G-tube should be placed. Then he would make a small incision and place the G-tube. He said in about 5% of kids he cant place the tube through endoscopy so he has to go laproscopic through the belly button.  I handed Mason to an anesthesia resident and they sent Casey and me out to the waiting room. We got our first page at 8:00 saying that surgery started.  Our next page at about 8:30 said Dr. Seifarth couldn't place the tube endoscopically so he had to go through the belly button (of course). At about 9 Dr. Seifarth came out and talked to us. He said everything went well with the surgery but he had to put a lot of air in Mason's stomach (because its so small and never had much food at one time in it) to get it to reach the wall of the belly to be able to put the tube in. He couldn't get it far enough so he had to go through the belly button to see better.  He told us Mason will probably be in a lot of pain from all the air but he should be able to pass it and be fine.  We got to go back to the PACU to see Mason in recovery.  He was already awake and just looking around.  After about an hour in recovery we got transferred to our room where we would stay overnight.

 

 When we saw Mason after surgery he just wanted to stare at Casey and listen to him.  Its was so sweet.

 Mason in the PACU.  He got a few stiches in his belly button, and a few in his tummy to hold the stomach wall against the belly and to hold the g-tube in place.

 Our room ended up being the same exact room that we stayed in for a week when Mason got his NG tube! It was fun to see a lot of the same nurses who helped us 4 months before.
We could start feeding Mason through his G tube at 3 that afternoon, but he could only have 10ml an hour for 4 hours (he normally gets 100ml every 3 hours).  Then 20ml an hour for 4 hours. He handled the 10ml just fine but when the nurse switched him to 20ml he started throwing up. He kept retching even when he didn't have any food in his tummy.  He started to act like he was in a lot of pain so the nurse started giving him morphine which should have calmed him down and made him fall asleep but he just seemed more uncomfortable. At about midnight he started acting really uncomfortable. He was super stiff, his legs were straight out and his feet pointed and his arms straight out and his hands clenched.  He kept moving his head back and forth while he was crying... he did this for about 2 hours.  The nurse paged the hospital pediatrician to come check him out because she thought he might be having seizures. He had no idea what was going on so he ordered an EEG. The EEG techs came at 3 am and put the leads on Mason's head.  After being awake for about 6 hours Mason finally settled down and slept for about 20 minutes then he started doing the same thing ALL NIGHT LONG.

 

  I ended up sleeping through his cries because I was so exhausted.  At 7 am the surgery resident came and checked Mason out and said he looked extremely bloated and he'd send Dr. Seifarth up to see us when he was out of surgery.  Dr. Moosa came in too and said the EEG didn't show any new seizure activity, just his normal spasms and hypsarrhythmia. That was a relief! I had been a crazy mess at 3 am worried that Mason had new seizures and wondering if I made a horrible mistake subjecting him to surgery, I'm glad my worries weren't justified.
That morning Mason kept throwing up.  He still seemed very uncomfortable and it was so sad! I couldn't soothe him because when I held him it seemed to make him more uncomfortable. Later that morning Dr. Seifarth came and said we needed to stop all feeds and vent (leave his g-tube open to let air out) all day. He also said the morphine probably made him more bloated so he could only have tylenol from then on and he had to have gylcerin suppositories and tylenol suppositories every 4 hours to get his bowel going to push all that air out. He also said we couldn't leave that day since Mason wasn't tolerating feeds.  I was fine with that because I didn't want to go home and have Mason get dehydrated and end up having to go back to the hospital.

 

 Thankfully I learned from past hospital stays that snap jammies are a must in the hospital.  He had his IV coming out of his right foot, pulse ox on his left foot, tube coming out of the center and EKG monitor on his chest. Good thing he didn't really feel like moving around too much because he wouldn't be able to!

 

The next morning Mason was doing a lot better and Dr. Seifarth came in and said Mason could start feeds again but we would slowly have to get back to his normal amount.  He wouldn't get back up to his normal amounts until that night so I just decided to stay another night to make sure everything was good (and so I could have more time to have the nurses take care of Mason!).
After a long stay we ended up leaving on Thursday afternoon, and Mason has been doing really well. He's been tolerating his feeds and he seems so much happier without the NG tube, and less gaggy and raspy. I've been loving the G-tube already.  It's so much easier to give meds through, and I don't wake Mason up every time I hook up his feeds.  He hasn't seemed to be in any pain and the g-tube site is healing well.  Overall everything has been great! Now we get to focus on controlling those nasty seizures.

Dr. Seifarth took out Mason's stitches the last day we were at the hospital, but all those red dots were stitches. When Mason needs to eat we just hook up the extension into the button and feed him. When he's not eating we can unhook the extension and close up the button.  The button is really soft silicone and is really low profile so it doesn't stick up much.

Where we're at now

The past few months have been a roller coaster ride. Mason finished the ACTH steroid injections right before Christmas.  The injections were supposed to 1) stop the spasms and 2) clear up the hypsarrhythmia. ACTH is a first line of treatment for Infantile Spasms (IS) and it's effective in treating many children. The steroid injections had horrible side effects but they got rid of Mason's spasms.  However, an EEG showed that Mason still had hypsarrhythmia (hyps for short), so we started to wean him from the steroids and went to plan B.  As we started weaning him from the ACTH his spasms started coming back. The hyps is the horrible part of infantile spasms. Hyps is what keeps Mason from developing mentally and learning new things. The spasms on their own don't cause harm but they are an indication of hyps.

After the EEG still showed hyps we met with Dr. Moosa and talked about Plan B which is the medication Sabril. Sabril is also a first line treatment for IS and is popular because it doesn't come with the annoying side effects that steroid therapy does and it's still very effective in treating IS. However, Sabril does come with a rare but scary side effect: peripheral vision loss. We have to take Mason to monthly ophthalmology appointment while he's on Sabril and I had to sign a paper stating that I couldn't sue the pharm company if Mason goes blind... Dr. Moosa assured me that he's never seen a child lose their vision while taking Sabril but it still scares me. He told me that chance of vision loss is better than IS.

Mason started Sabril right before Christmas. After 3 weeks he got to the therapeutic dose so he got to go in for another EEG.  The 3 weeks he had been on Sabril he was having spasms so I wasn't hopeful at all that the EEG was clear of hyps.  Right after the EEG I met with Dr. Moosa and he gave me the results that the EEG hadn't improved at all, and we had to go with Plan C. Plan C is increasing Sabril to the max dose.  Of course increasing Sabril to the max dose is scary because it increases the chance of vision loss even more, but it still has a good chance at getting rid of hyps.
Dr. Moosa also delivered the bad news that ACTH and Sabril work for most children, but since ACTH didn't work and Sabril hasn't worked yet the chances of other medications being effective are low. We still have hope though, and we won't ever give up until we find something that works. Mason gets another EEG in 3 weeks to see if increasing Sabril has worked. The good news is that since increasing the Sabril dose Mason hasn't had any spasms! I'm always cautious about not getting too excited though because the spasms can still come back at any time, but we're at least hopeful. If Sabril hasn't cleared up the hyps in 3 weeks we're onto Plan D.

I'm actually pretty excited about Plan D.  Plan D is the Ketogenic diet. The ketogenic diet is a high fat, no sugar, no carb diet.  The ketogenic diet makes the body have to use fat for energy instead of sugar which reduces seizure activity. If you want to read more about how it works read this article: (http://www.hopkinschildrens.org/high-fat-diet-can-reduce-of-end-debilitating-seizures.aspx).  It sounds pretty simple but the diet is very strict because any sugar even in medications and fruits etc. can start seizures again.  To start on the diet Mason would have to be hospitalized for a week.  His blood sugar levels have to be checked regularly and a dietitian has to make sure he's receiving enough fat for his body to reach ketosis. The ketogenic diet is proven to treat seizures, and many children on it are able to stop taking other seizure medication because the diet alone is so effective.  How awesome is that!?! The diet isn't as popular as medication because it's so difficult to monitor everything the child eats, and they don't have many options (butter, cream cheese, whipping cream etc). It makes sense that tube fed children are the easiest to put on the diet because there's a formula that they can get through the tube that's ketogenic.
I don't want to have to think a lot about Plans E, F, G or H yet but I have a little bit. I'll leave that for another post.

We got a fun surprise while we were in the hospital last week, Dr. Moss (geneticist) came and saw us and gave us the results of the epilepsy panel that Mason got drawn just before Christmas. We weren't planning on seeing him until the end of March, but his wife was Mason's hospital pediatrician and she told him to come see us.  In short the results were that a misspelling of a gene came up and 2 other kids with that same misspelling have IS. Casey and I got out blood drawn while we were at the hospital so we can be tested and see if we have the same misspelling.  If we do, the gene isn't important because Casey and I don't have epilepsy. If we don't have that misspelling then Dr. Moss will delve deeper. The hope is that this gene can give us answers on what treatments could be effective. On the other hand, there is still the chance that Mason suffered brain damage at birth from lack of oxygen, but that's another post too.