Our Hero

So much time has gone by since my last post that I don't even know where to begin.  With the holidays life got hectic, and I put off writing about June.  I have a list of blog topics I planned to write about, but today I will just stick with an update on June. Overall, she is doing pretty well on the ketogenic diet and is tolerating it well. Although she continues to have seizures daily, they are not as frequent as before we started the diet.  A few weeks ago we switched from a breast milk, olive oil, and soy formula mixture to a strictly powdered formula. Since switching to the formula, we have seen a big improvement in June's development and a decrease in seizure activity.  We think the problem with the diet before was that we weren't entirely sure how many carbohydrates were in my breast milk - it can vary. So while we thought June was on a 4:1 ratio, we were not certain.

Some of June’s accomplishments over the last few months are listed below.

•  June mimics us and is making different sounds.  This makes us optimistic because it shows she is taking in information, processing it, and then sending it back out.  Her neurologist was particularly pleased about this.
• Her head control continues to improve.
• She can lift her head and look side to side when she is on her tummy.
•  She can stand in her bouncer for short periods of time. She needs assistance to make sure she does not whip her head from side to side, but she is improving.
•  When I hold her she is starting to push off my chest and look around.
•  When she sits supported on my lap or against the couch she is able to keep her head steady for longer periods of time.

• She is more aware of her surroundings and is easily comforted when Matt or I hold her.

•  She loves her bath whereas before she would cry the entire time.

• She calms to music, especially Ella Fitzgerald & Louis Armstrong.  Her favorite song is "Isn't It a Lovely Day." She is also a fan of Jack Johnson, Nora Jones, and Otis Redding.  Random I know, but their voices calm her.

• She is tracking objects more smoothly and consistently.

• June sleeps for longer periods of time, and we are trying to wean her off her swaddle.

I would like to take a moment to point out how huge it is that June has made so much progress.  I don't always fully appreciate how difficult it must be for June to grow and develop given all the chaos in her brain.  I recently came across a blog post from another mother of a baby with Infantile Spasms where she showed a picture of a baby's EEG with Infantile Spasms and a picture of an EEG from a baby without Infantile Spasms.  I thought it was extremely powerful, so I am going to borrow from her post to show you the difference.

This is a picture of a normal EEG.  Notice how it is pretty and organized.

This is a picture of an EEG pattern consistent with Infantile Spasms.

Given how unorganized the activity is in June's brain, the fact that June can mimic us, lift her head, respond to music, and differentiate between Matt and me is truly amazing.  We are so proud of her.   I know I've said this before but I'll say it again. I am in awe of our June.  Since she came into this world 8 months ago, her personality has been fighting to come out.  And she has managed to do it.  I sometimes wonder that if I was fighting the same illness, could I be as strong as June?  She must get her determination and tenacity from her Daddy because I am quite certain I would want to give up.  All of our babies with IS and other forms of epilepsy deserve a round of applause because they are in all senses of the word heroes.

June in her bouncer.

Striving for Acceptance

It’s been a few weeks since I wrote an update on June.  To be honest, it’s because I’ve been having a tough time.  When I decided to write this blog I wanted it to be a way to update our family and friends about June, but I also wanted to use it as an outlet to process the pain that comes with caring for a baby with special needs.  I didn’t want to use the blog to vent my frustrations. I wanted everything I posted to be written with intention and meaning because after all I’m doing it to help heal.  

All that said, it took a lot of restraint to not use the blog as a place to release my emotions.  I can’t tell you how many times I sat down to write with tears in my eyes about my anger, sadness, and fear.  My plan was to post everything I was feeling so I could get my emotions out for the world to see because I couldn’t handle them inside me anymore.  I wanted them to be someone else’s problem. Thankfully, I’ve moved beyond that stage of grief for the time being. 

Nothing in particular happened to set off a spiral of negative emotions.  In fact, from a big picture perspective, it’s actually been a good few weeks – the diet is going well and June is still making progress developmentally. I think it was a combination of small situations that fed off the other that led me to feel down.  First, we have seen a lot of improvement in June’s development since starting the diet, but as time’s gone on, the progress has slowed.  It hasn’t stopped, but it isn’t as drastic as it was in the beginning.  While I think that is normal and to be expected, I felt discouraged.  I wanted it all to happen as quickly as it did initially. 

Next, June had another EEG which showed she is still having seizures.  I knew it was going to show that because I could still see her having them, but I was hoping for some improvement which the EEG did not show.  Now, it is still too early to make any assumptions, especially since June was not in optimal levels of Ketosis when she had the EEG, but I still felt disappointed and scared. 

On top of that, I was surprised at how difficult Halloween was for me.  When I saw all these kids running around in their costumes all I could think was, ‘Is that going to be June? Is she ever going to run up to our neighbor’s door and say “trick or treat?”  And the truth is, we don’t know.  That is a hard pill to swallow, especially for me.  Uncertainty terrifies me, but unfortunately that is my world…. that is all of our worlds, actually.  I just seem to be more aware of how uncertain life is than I did before all of this happened and sometimes I want to go back to being oblivious to how vulnerable we all are.

Last, as June gets older and the developmental milestones become more apparent in babies her age, it is difficult to see that she is not reaching them.  I’m struggling with accepting that June is following her own timeline.  In yoga we are taught to focus on our mat.  This just means to accept where we are in our practice without judgment or criticism.  So, just because the person next to me has loose hips and can do double pigeon while saying how good it feels doesn’t make her better than I am because I can’t even do half pigeon without wanting to cry.  I am trying so hard to take that lesson in yoga and apply it to my life with June and her development, but it is challenging.  I can’t help but get sad when I see babies June’s age or younger doing things that she can’t do yet.  It just doesn’t seem to be clicking for her yet and I am struggling with just being okay with where she is right now because the fact of the matter is I don’t have much control over that anyway.  I can provide June with optimal situations to thrive through things like occupational therapy, audiology, and speech therapy, but that is all I have control over and that can make a momma feel powerless.  So, all of this combined is why I’ve had a tough few weeks. 

But I want to end on a positive note with some good news to report. June had a behavioral hearing test this week, and it showed she is responding to noise 15-20 decibels softer than she did on her previous test.  At home, we’ve noticed June reacting more to sound since she started the diet, but it is encouraging to see that reflected on a test.  It’s amazing to watch her hear things for the first time.   Last week she was looking at herself in the mirror and she squealed, then she paused, looked at the baby in the mirror, and then started to cry because she scared herself.  It was the most precious thing I’ve ever seen.  I wish I had it on video!  Since then, she’s warmed up to the baby in the mirror and now smiles back at her when she squeals. 

Yesterday in an interview on “Katie” Michael J. Fox said,  “my happiness grows in direct proportion of my acceptance and inverse proportion of my expectations.”  That resonated with me for our situation with June.  We had so many expectations and none of them included what we are dealing with now.  In my wildest dreams I never thought I’d spend most of my days of June’s first year in doctors' offices and hospitals.  I thought I’d be in playgroups.  But the more I resist accepting our situation, I think the unhappier I will be.  Acceptance won’t happen overnight but maybe I can slowly release my grip on my expectations.   

Ketosis

Matt and I are pleased to report some happy news – June is in "ketosis!" I think that sounds so funny to say about a baby, like she is in some far off galaxy, but that is how the doctors refer to it, so that is how I will describe it too.  All it means is that she is now using fat for energy instead of glucose.  After our trip to Detroit to see Dr. Chugani, Matt and I decided to start June on the Ketogenic Diet.  When we followed up with our doctor here, we realized June wasn’t doing as well as we thought on Vigabatrin.  So, we decided it was better to move forward with a new treatment rather than go backwards to something that wasn’t working well.

On Monday, June started the diet in the hospital. We were there for four days, and we spent a majority of the time with the dietician learning about the diet. Each baby or child on the diet has a specific diet “ratio” of fat to carbohydrate and protein grams combined to follow based on weight, height, and ketone levels.  It can take some trial and error to get to the proper ratio.  As of now, June’s ratio is roughly 3.25:1, which means her diet provides 3.25 grams of fat for every 1 gram of carbohydrate and protein.  Sound complicated? Well, it is.  That is why you start the diet in the hospital with help from the dietician.  The doctors also needed to monitor June to make sure she handled the diet okay.  To make the formula, we mix RCF (Ross Carbohydrate Free) soy formula, breast milk, and olive oil.  Since June is so little it is fairly easy to administer, but we still need to measure each ingredient with a gram scale and when the recipe calls for 18 grams of something, it has to be 18 grams. It can’t be 18.1 or 17.9.  I’ve spent a lot of time adding and removing little droplets of liquid because of a measly decigram.  Additionally, not only do we need to make adjustments to June’s food, but also her medicines, lotions, diaper cream, and even tooth paste (once she has teeth to brush).  I never would have thought lotion, for example, would have carbohydrates in it, but a lot do and using it could throw off June’s state of ketosis.  In order to tell if June is in ketosis, we test her urine several times a day with test strips.

All of this sounds like a big to-do, but it is all worth it because June has made so much progress since starting the diet. On Wednesday, she clasped her hands together for the first time and began to bear down weight on her legs.  She can also lift her head higher when she is on her tummy and she is more focused on her environmental surroundings.  Yesterday, she even seemed to respond more to noise.   When she was sleeping she woke up to the sounds of people talking and when the phone rang she began to cry because it woke her up. We never had to worry much about noise levels when June was sleeping, but I am so happy that I need to now.  It is an unbelievably great worry to have! We are hopeful that if June has made this much progress in less than a week, she will continue to improve as time goes on.

I will be the first to admit that I am shocked by June’s improvement.  When I first heard about the diet, I thought it was a bunch of hocus-pocus.  As a society, we are taught to believe medicine only comes in the form of a pill.  I couldn’t believe that changing June’s diet would actually improve her development and possibly stop her spasms.  In a few weeks, June will have another EEG to see if the diet is halting the abnormal activity in her brain, but the doctors said to give it 2-3 months to see if the diet will make her EEG normal. 

Thanks for reading.  We fight on.

June found her hand while cheering on the Cardinals in the hospital

Fight On

We just finished our meeting with Dr Chugani who was helpful in answering our many questions about June.  After reviewing her PET scan and EEG, he said June is not a surgical candidate because her seizure activity is diffused throughout her brain.  In other words, there is no epileptic focus to remove at this time.  The nature of her epileptic activity leads him to believe that June most likely has a genetic disorder.  This could be caused by a spontaneous mutation, something I may carry, something Matt may carry, or something we both carry.  As I've mentioned, Dr. Chugani is a pioneer in research of infantile spasms, and he is leading a study on the role genes play in infantile spasms.  He asked us to participate since he is able to test all three of us.  We are hoping the test will give us more answers in addition to the genetic testing done on June this summer.

Dr. Chugani also talked to us about his recommendations moving forward.  He thinks June should go back on Vigabatrin – one of the frontline medications used to treat IS, for a longer period because of the improvement she showed on it.  If that does not work, he suggests we then try the ketogenic diet.

June has been so brave during the tests.  She keeps smiling and laughing and stole the nurses' and doctors' hearts.  They were smitten with her, but how could they not be?  Thanks for everyone's thoughts and prayers.  Your messages and comments have helped Matt and me to stay strong.  We are so blessed to have such an amazing support system. We will continue to fight on for June.

June on her first plane ride to Detroit

June Goes to Michigan

June is looking forward to her first plane ride today as we head to Children’s Hospital of Michigan to meet with Dr. Chugani who is the foremost authority on IS. We will be there for two days. Tomorrow, June will have a series of tests, including a three-hour EEG and a PET scan to evaluate her for surgery. On Wednesday, we will meet with Dr. Chugani to discuss the results. In our wildest dreams we never thought we would pray for June to be a candidate for brain surgery, but that is what we are hoping for now. Since June has failed most of the medications we are running out of options and the stories we’ve read about the babies who have undergone surgery sound promising.

When we get back from Detroit we will likely start June on the ketogenic diet, which is high in fat and low in carbohydrates. It is used to treat difficult-to-control seizures in children and babies. Our doctor at STL Children’s Hospital tells us that babies who have failed other medications have experienced success in controlling spasms with the ketogenic diet. Studies show that babies and children who try and fail two medications to treat epilepsy have a 1% success rate with other medications. June has tried 8. We are getting into very low numbers here, so when her doctor told us the ketogenic diet has about a 3-5% chance of working on June, we thought it was worth a try.

You may be wondering how the diet works and how you would start the diet on a 5-month-old baby. To put it simply, the diet mimics aspects of starvation by forcing the body to burn fats rather than carbohydrates. Normally, our bodies run on energy from glucose. When there is no glucose in the body, we begin to burn stored fat and convert it into fatty acids and ketone bodies. The ketone bodies pass into the brain and replace glucose as an energy source. An elevated level of ketone bodies in the blood, a state known as ketosis, can lead to a reduction in the frequency of seizures. Doctors do not know why this diet can prevent seizures. They also do not know why it works for some babies and not others. I am now realizing more than ever why research funding is needed for epilepsy because there seems to be limited information.

In order to start the diet we will be admitted at Children’s Hospital for 5-7 days. Since June is so little the diet should be fairly easy to administer. She will get a special formula with the proper fat to carbohydrate ratio. I think I will have the hardest time with this medication. I love nursing June, and while we’ve had a few hiccups (no pun intended) in the process, it feels like the only thing that has gone well for us. She finds it comforting and so do I. It brings tears to my eyes to think I may have to take that away from her…from us.

The doctors tell me that I can still nurse while she is on the diet. I will just have to limit the amount of time she nurses and supplement with the formula. However, I have a feeling this will be more difficult than it sounds, especially since June has been refusing a bottle. I worry she won’t go back and forth that easily and we may have to stop completely, but she may surprise me.

Thank you for your continued prayers and support. We will update you on our trip to Michigan when we get back.