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.