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.

Missing Me

Just when I thought we couldn’t get more bad news, we received the results from June’s Flash VEP (Visual Evoked Potential), which measures how the brain responds to flash stimulation or, in other words, light. The results showed that June has a recordable signal from her eyes to her brain, but the signal is reduced and it is slower than normal. If you are like me, you might be thinking, ‘okay, what does that mean?’ I asked that same question to the woman reading the results to me over the phone, but she kept repeating herself. She finally admitted that she didn’t know what that meant, and she would have to ask the doctor and get back to me. Why the doctor did not call me to discuss the results is beyond me. It is really unfortunate that they have someone call a mother with negative test results who can't answer any questions about them. But my aggravations with patient care and inefficiencies are for another post at another time.

While I still have not heard back from June’s ophthalmologist, the little I’ve researched about June’s VEP results sounds very similar to June’s ABR hearing test (auditory brainstem response) results. The ABR tests the brain’s response to sound. For those of you who do not know, June was initially diagnosed with Auditory Neuropathy, a hearing disorder in which sound enters the inner ear normally but the transmission of signals from the inner ear to the brain is impaired. This all leads us to believe that June may not have a hearing problem or even a vision problem, but a brain processing problem. In fact, June’s amazing audiologist at Moog sent an email about June and a brief summary of her health history to audiologists all over the world asking them to weigh in on her diagnosis. A world-renowned audiologist, Dr. Charles Berlin, has asked to review June’s health records to confirm if she even has auditory neuropathy.

So, we are still in the investigating stages and it seems that even previous diagnoses are now being questioned as we learn more about June. Every now and again, I have days where I feel sorry for my family and myself. I wonder why this is happening to June, and I fixate on how this happened. These days usually happen after we get bad news from one of the doctors, which seem to be occurring more and more. Yesterday was one of those days for me. I was desperately trying to make sense of it all and replaying days in my pregnancy wondering if it was something I did to cause all of this. I was so careful and yet this is all still happening and all I could focus on was "why June?, why us?"

Thankfully this morning, I was brought back to my senses. When I woke up I found Matt and June playing downstairs. June was smiling and laughing. That made me so happy. A little later, I turned on music and started to rock June to sleep. As she was looking into my eyes and smiling I heard Jack Johnson sing, “If you would only listen, you would realize what you’re missing, you’re missing me.” Never have words from a song spoke so well to me. It felt like June was saying that to me. Yes, I am sad about all of this, but I still have a beautiful baby girl, and I don’t want to miss a second of enjoying her. So, today I am going to step away from the doctors, the tests, and the worrying and focus on just loving Junebug.

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.

About This Blog

Matt and I decided to create this blog so we could update our family and friends on June’s progress.  June has Infantile Spasms, a catastrophic form of epilepsy found in babies. Feel free to subscribe via email and check back often for updates and pictures.  Thank you all for your support and prayers for June.