When it rains, it pours...but the sun will shine again.

April 30th marked 5 months to the day that Ryder had been seizure free.  It was also the same day he relapsed.  Shortly before bedtime that evening, out of nowhere he had 30 spasms over a period of 5 minutes.  After speaking with some knowledgeable parents of children that have the same seizure condition as Ryder, and having them tell me to wait until the morning to contact his neurologist, I took him to the emerg.  We were seen extremely quickly thanks to the handy letter we have from his metabolic doctor that says he cannot, under any circumstances, wait to be seen.  It was our shortest visit to emerg yet (4 hours!), and our first hospital trip that did not involve blood work, yay!  It was exactly what the other Infantile Spasms (IS) parents had said would happen...the emerg docs agreed from my description that it was likely his IS had returned but they did question whether it was simply behavioural.  They sent us home after requesting an "urgent" EEG...for two weeks later.  It's difficult to explain to other people, let alone doctors who think they know everything about epilepsy, how serious this form of epilepsy is.  It's not just a seizure here and there where brain activity is interrupted for a brief period of time.  If Ryder is having clusters of spasms here and there, that means his brain activity is interrupted and chaotic ALL THE TIME, even when he's not outwardly displaying the spasms.  He can't learn.  He can't develop.  He will regress and lose skills for as long as that chaotic brain activity is occurring.  We got ahold of the neurologist the next morning and he was started back on his medication within 12 hours of seeing that first cluster of spasms.  About 1.5 weeks later we stopped seeing the spasms.  On May 21st, Ryder had a clear EEG which we were incredibly surprised by since the medication he is currently on did not work when we had tried it in the fall when the seizures first started.  Ryder will stay on this medication for 6 months at which point we will re-evaluate with his neurologist.  

Ryder is now sitting on his own and is managing to get himself stuck under the couch/chairs/tables because of his crazy combination of rolling, a little bit of bum scooting, and some backwards shuffling on his stomach.  

He is incredibly happy (we all survived the grumpy seizure med phase, thank goodness!), smiley, and loves to laugh.  He has little conversations with us and waits for his turn to "talk".  He has also recently become interested in standing with support.  I am so incredibly proud of the progress he is making.  Having the seizures under control once again has allowed us to focus on all of the positive changes we are seeing, instead of being overly critical of the skills he may have been losing.  With that being said, it has been getting more difficult to spend time with friends whose babies are around the same age as Ryder.  Seeing what other kids his age are doing, it is so difficult not to compare him and see the skills he is lacking.  It would be so much easier to keep him in a bubble and be thrilled with everything he has accomplished and continues to learn, but I adore our friends and their kids and can't imagine out lives without them and the support they have given us.

Something to consider when speaking to parents of children that have medical issues...(and then I will go back to embracing all the positive things in my life, because there really are so many!).  I understand that it is a common saying, but please try and put yourself in the shoes of someone whose child is medically fragile.  I can't even count the number of times of heard (and please know that I do realize I am much more sensitive to these types of things now than I was before): "Blah, blah, blah, my life sucks...but at least my kids are healthy..." or "I don't care if the baby is a girl/boy, as long as it's healthy..."  Umm..so what if you kids weren't healthy?  Or your baby was born with a medical condition?  Then what?  Would you love them less?  Is a sick child less valuable/important than a healthy child?  Of course not.  Yes, your life would likely have additional stress, but it's still your life and that baby is still your baby and you will love them just as much as their "healthy" sibling, or as much as your would have if they didn't have something different about them.  Ok, rant over. :)

Our Crazy (Happy) Life

It seems incredible that is has been exactly 2 months since Ryder was finally discharged from the hospital. Time has flown by at an amazing pace. We have kept busy with lots of appointments, therapy time, and play dates. 

We are ecstatic (to say the least) that Ryder has been seizure free for 4 months and just a few days ago was weaned off of all of his seizure meds.  His neurologist is so confident that he has beaten infantile spasms (IS) that he is not putting Ryder on a "maintenance" med.  He will be med-free when it comes to seizures, and fingers crossed he will remain that way for a long, long time.  The weaning process took 4 weeks to slowly cut down on his dose, but as we continued to reduce the amount he was getting we began to see some very positive changes in Ryder's development and his behaviour.  It was an exciting month around here.  

Sedated and awaiting his ERG (special MRI) of his eyes at Toronto Sick Kids.  This test checks for any peripheral vision loss that may have occurred as a result of the seizure medication.

He is finally sitting up on his own (within the last two weeks) and is able to balance for a little while without using his hands for support.  He also "found" his feet and has improved the flexibility in his hamstrings (both of these factors were contributing to him being unable to sit previously).  

Sitting like a big guy and inspecting his hand!

Another super exciting change in Ryder happened in the last few week as well.  We had stopped offering him bottles for several weeks because it was always such a fight....a fight that ended with Ryder screaming, coughing, choking, aspirating, and/or throwing up.  It just wasn't worth the stress (for any of us) when we knew he could get  everything he needed from the G-tube.  A few weeks ago I decided to try a bottle again, and to my complete amazement he drank from the bottle the same way he would have several months ago...no screaming, no aspirating, and only a little bit of coughing.  As much as I don't want to admit it, having the feeding pump around was really easy to get used to.  We could feed Ryder absolutely anywhere (including while driving in the car) and just go on with our day without having to stop and feed him a bottle.  Having him eat from a bottle again has taken some getting used to since I have to actually stop what I'm doing and feed him.  However, I have realized that I was missing out on snuggling and holding him, and I now look forward to feeding him his bottle.

As Ryder's 1st birthday (and Ethan's 3rd birthday) is just over a week away, I've been thinking a lot about how much our lives have changed over this past year.  We happily became a family of four and welcomed the sweetest little baby in to our lives.  Although the challenges and obstacles we have faced were certainly not what we had imagined, I am blessed to have chosen such an amazing partner in life.  He is supportive, patient, and tolerates my very specific ways of doing things (and the meltdowns that ensue when things don't go my way).  He forces me (I mean this in the nicest way possible) to keep things in perspective.  I am incredibly proud of how strong we have been as partners and parents in what has turned out to be a very unpredictable situation.  Despite the unknownness of Ryder's condition, we continue to live the life I have always dreamed about: we spend quality time together as a family, and are raising two of the sweetest, kindest, and cutest boys I could have ever imagined in a happy and loving home.  What more could I ask for?

Love those cheeks!

Our only limitation is our belief that it is so.

~Moshe Feldenkrais

It's all about perspective!

On January 5th, we finished off Ryder's course of steroids to treat his seizures.  On January 9th, we went for another EEG to check for the chaotic brain activity.  I'm certainly no expert on EEGs, but I was sure that the squiggly lines I saw throughout the 45 minute test were the same squiggly lines we had seen twice before.  I had gone in to the appointment with my scientific journal articles thoroughly highlighted, and with notes made on the next treatments I wanted to try, and in which order we were going to pursue them. Our amazing neurologist came out to get us from the waiting room with the same sympathetic face and body language, and soft-spoken greeting that we had gotten used to when we were about to get more bad news.  When we sat down, his first words were "Well, let's get right to it.  The seizures and hypsarrythmia are gone.  The steroids worked."  To say that those were not the words I was expecting might be the understatement of the year.  Ben and I looked at each other in shock and said nothing.  The appointment was quick.  We took a look at the images from the MRI and saw the areas of Ryder's brain that are swollen (related to the metabolic condition…not the seizures).  And that was it…follow-up in six months.  (Six months seemed like an awfully long time, so I pushed to have another EEG and appointment in three months).  Ryder will stay on an anti-seizure medication until then, and from there we will wean him off and start on a maintenance medication.  I don't think I will ever forget that day.  Things have obviously been difficult the last few months, and just when we get into a routine or wrap our heads around something, another problem pops up and disrupts everything.  This was the best news we could have asked for!

Coincidentally (or maybe not…) on the day Ryder finished with the steroids we started noticing a decrease in his appetite.  Not a huge deal, he was still eating.  Two days later (Tuesday) at a regular metabolic check-up, they checked his weight, length, and head circumference.  His length was shorter and his weight was less than they had been 2.5 months earlier, (both were checked multiple times that morning) and he had dropped 50 percentiles on the growth charts .  I immediately felt guilty (darn mommy-guilt!) for not noticing, but the dietician explained that since both his weight and his length had decreased he still looked very proportional so it was impossible to notice from looking at him.  We left the appointment with instructions to get more calories in to him.  That night he was admitted to the hospital (after an episode of projectile vomiting) for IV and metabolic bloodwork and we were discharged the next afternoon.  On Thursday, he took in only 10 oz of formula/breastmilk.  He began completely refusing to eat.  I spent an hour getting him to eat an avocado/banana mixture that he had devoured in minutes a week earlier.  He then proceeded to throw up the entire bowl.  We fought with him until Friday and decided it had been going on long enough and was only getting worse.  

When we got to the hospital on Friday evening, our metabolic doctor and dietician came to check on Ryder.  As they were leaving, the dietician said, "I will see you on Monday when I get in."  My response was something along the lines of "Uh, well I really hope we're not still here on Monday, but OK"….3.5 weeks later we finally went home!

They put in an NG tube (nasogastric tube...goes through his nose, down his esophagus, and in to his stomach) to feed him and an IV for hydration.  He continued to projectile vomit multiple times a day for the first 2 weeks.  He also continued to refuse to eat by mouth and was seen daily by an amazing OT.  While Ryder was admitted to the hospital he underwent several tests (echocardiogram, holter monitor, EKG, and swallow study), all of which showed normal results, yay!  

NG tube

We finally made the difficult decision for Ryder to have surgery to put in a G-tube (a tube that goes directly from the outside of his abdomen into his stomach).  As we learned through experience, it is *very* easy to pull an NG tube out (Ben and I both did it once, and Ryder pulled it out on his own twice), and putting it back in requires a trip to emerg and an x-ray to check for placement.  The more we thought about the G-tube, the more sense it made: it stays in place, there is no risk of "feeding his lungs" if the tube gets placed wrong, he can take his meds through it even if he starts eating on his own again, and when/if he doesn't need it anymore it can be removed.

Two days after his G-tube surgery.  The lovely hat
is covering the IV they had to put into his head.

We finally came home at the beginning of this week and are still trying to figure out our new "normal" around here.  Ryder is being fed every 3 hours, and each feed takes 1 hour to complete…which doesn't leave a lot of time for getting other things done, but we are getting the hang of it pretty quickly.  Yes, a G-tube has always been one of my bigger fears, but I have realized it's all about your perspective:  Ryder has gained 4 pounds in the last couple weeks and is working his way back to where he should be on the growth charts…he is happy and there is less stress for us because we don't have to force feed him anymore…he is at home where he belongs…he took a few bites of some purees over the last two days…his homocysteine level is the lowest it's ever been...he's almost sitting up on his own...and he is SEIZURE-FREE…So, all in all there is a lot of stuff to be excited and happy about around our house these days!

Another bump in the road

Those weird movements we had been noticing more and more frequently were suspected seizures.  It happens so quickly…each seizure lasts about 1 second.  All of a sudden his head will drop really quickly and his arms go up at the same time.  At the beginning of October is was happening maybe 1-2 times each day.  By the end of October they were happening in clusters of 10-20 seizures a few times each day.

I was able to get a cluster of the seizures (about 10 seizures in 2 minutes) on video and e-mailed it to the paediatrician.  She was in contact with the neurologist and sent him the videos as well.  The following Tuesday, the neurologist called to ask us to come in right away.  After a long and drawn out assessment of Ryder's symptoms and the videos, they told me that he was having something called "infantile spasms" (IS) which are incredibly serious (much more serious than head drop seizures).  He was started immediately on a seizure medication and an EEG was scheduled for the next day.  

Ryder during his EEG

Infantile spasms are caused by a specific pattern of abnormal brain activity.  The way that the neurologist described infantile spasms is like this: There is (abnormal) static going on constantly in Ryder's brain.  For as long as that static is occurring, he cannot learn anything, and his existing skills (smiling, babbling, rolling, sitting, etc) will regress.  In many cases, the babies that get these seizures have "bad" brains (the neurologist's wording) and would not likely develop normally whether or not they were having the seizures.  He is confident that Ryder doesn't have a "bad" brain because of his "normal" development up until this point.

The medication has one major side effect and that is permanent peripheral vision loss that cannot be regained once the medication is stopped.  So off to Toronto Sick Kids we went to have a special test done on Ryder's eyes as a "baseline" to track any potential vision loss in the future (basically an MRI of his eyes).  Good news was that they said his eyes looked normal!

The medication turned him in to a little zombie baby.  He slept about 20 hours per day for the first week he was on the medication.  The seizures stopped as soon as he started on the medication.  It honestly seemed like a miracle and like it was too good to be true.  Unfortunately, it was too good to be true and after 12 days seizure free, he started having small clusters again.  His follow-up EEG was cancelled because if the seizure movements are still present, then that means the chaotic brain activity is still present too.  The neurologist saw us within a few hours of the seizures returning and Ryder started on another medication called Prednisolone (the active form of prednisone).

This was the medication the neurologist had warned me about the first time we saw him.  One of the side effects is extreme irritability.  The doctor stressed again and again how I could not even begin to imagine how irritable and miserable Ryder would be…essentially he said Ryder would be screaming 24 hours a day for as long as he was on the medication…they gave us a prescription sedative for Ryder for the times when we couldn't stand the screaming anymore.  There are also other side effects like excessive weight gain in the face and trunk, increased blood pressure, heart problems, and a decreased immune system (the nurse said I should probably just not leave the house for the next 8 weeks…).

As soon as Ryder started on the medication we noticed a huge increase in his appetite.  He didn't get grumpy right away which was a nice surprise!  The seizures continued for a few days and the doctor increased his dose to the maximum for his weight.  The increased dose seemed to work and we have not seen any seizures since November 30.  His follow-up EEG showed a very slight improvement in the hypsarrythmia (chaotic brain waves), but unless the abnormal brain waves are completely gone, the treatment is considered a failure.

Throughout his prednisolone treatment Ryder was definitely grumpy.  He grunted and groaned ALL the time.  He stopped sleeping…he was sleeping a maximum of 6 hrs each day.  The sedative we had been given, which I had thought would mean a full nights sleep, knocked him out for 2 hours and then he would be up the rest of the night groaning.  It was exhausting.  He stopped smiling and laughing.  He wouldn't try to sit up.  

After about 3 weeks on prednisolone, we were about to get a few smiles and laughs out of him.  It was wonderful!  Other than being miserable, we haven't seen too much regression with his skills, and we are thankful for that.  He loves being held and snuggled, and despite being grumpy, he is still such a calm and laid back little guy.  He is definitely a fighter and we couldn't be more proud of all the things he is learning and trying to accomplish.  

I have heard other IS parents refer to their children's accomplishments as "inch stones" as opposed to milestones and I can certainly see why they do.  When someone tells you that your baby is going to lose all of the skills they currently have and essentially go back to being a newborn, you have to change your perspective.  We are thankful for all of the "little" things and are learning not to take anything for granted.  He can hold his head up, smile, laugh, and roll.  Considering everything he has been through and is going through right now, he is doing well!

Happy Holidays!