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.

Summer of June

June Corley Jessee was born on April 29, 2012 at 6:28pm.  She was 8 pounds 9 ounces and 21.25 inches of pure love.  As with any mother, June’s first few moments in this world will be forever imprinted in my brain.  I remember how June’s skin felt against mine when the doctor placed her on my stomach after delivery.  I remember the sound of her first cry, and I remember how proud and happy my husband was as he cut the cord, took June’s first pictures, and told me her weight and height.  I also remember how blessed we felt as we looked into her beautiful blue, almond shaped eyes.  It was by far the best day of my life. 

Today, I have even more love for June than I did on that rainy day in April, but it is a love that also comes with worry, fear, and desperation.  This summer, Matt and I have taken June from doctor to doctor, hospital to hospital, specialist to specialist.  At first she was diagnosed with "Auditory Neuropathy," a type of hearing loss associated with how the brain processes sound.  Then, when June was a little over two months, she started having Infantile Spasms "IS," a catastrophic form of epilepsy found in babies.  One of the biggest challenges with IS – and there are a lot – is that there are many, many possible underlying causes. This means there are many, many possible outcomes.  I'm told you will see families with children who are struggling greatly, as well as children who have made tremendous progress. Another mother warned me not to compare June to other children with IS and try not to get too hopeful OR too fearful from the stories I’ll read.  

Now, we are focused on figuring out why June is having Infantile Spasms.  She has already been through so much including an MRI, a spinal tap, many EEGs, eye exams, a VEP and several blood and urine tests.  So far all the tests for underlying causes have come back negative, but we are still waiting on the genetic test results (there are about 30 genetic disorders that can cause IS).  At the same time, we are treating the spasms aggressively. So far, we have tried six different medications and while June has stopped exhibiting outward signs of the spasms, the EEG shows that June is still having abnormal brain activity indicating spasms.  June’s neurologist told us that it can be difficult to tell when a baby is having spasms and often the only way to tell is through an EEG.  He also said that unfortunately it is an all or nothing proposition.  June could be having 1 spasm or 100 spasms a day and the concern would be the same. Unlike other forms of epilepsy where the goal is to control seizures through medication, the objective with infantile spasms is to stop them completely so that brain activity can return to normal.  Only when the EEG is normal, can a baby begin to develop normally.    

Next, we are taking June to Dr. Chugani at Children’s Hospital of Michigan who is the foremost authority on IS so that he can evaluate her for surgery.  In the meantime, we are trying different medicines and hoping we will find our miracle drug.

Please keep June, Matt and me in your thoughts and prayers.  I've been amazed at how brave June has been over these last five months through all the tests, doctors, and medicine.  Through all of this, she still smiles, laughs, and squeals.  She is a happy baby even after all that she’s been through, and we can all learn from her strength.  I know we will get through this, but we need your continued prayers and support to help us.