Nathan's Story: April 2016

Happy Spring Everyone!

As I am writing this update, the back door is open and I hear the chimes and wind blowing. It feels like spring and a time of new beginnings. For Nathan, the past three months have given him many opportunities for new beginnings. He has truly grown into a little person with independent thoughts, a wonderful imagination, and is full of love and compassion. He is a people person like his parents and Samuel is following in his footsteps.

Nathan’s last MRI was on March 7th, almost a month ago. I usually write these updates shortly after the MRI, but this email is late given the variety of decisions we have been faced with over the past few weeks. We were very pleased to hear that Nathan’s MRI looked stable from his scan in December. Unfortunately, Nathan is telling us that something is going on that we are not able to see on the scan. Due to the tumor being deep within the brain stem and the effect of his current chemotherapy on imaging, we are unable to trust the scan to give us an accurate picture. This is extremely frustrating. We want to have concrete information to guide our decisions, but our current circumstance does not give us that opportunity. We have to trust our instincts as parents to know Nathan so well that we can pick up any subtle changes we see. Every day I have to chart 14 different categories from sleep and voice quality to the number of hiccups and times he coughs while drinking in order to try to identify trends that would give us information of progression or decline. That is the only thing the doctors have to make some very serious decisions about how to treat him. The hardest part is that Nathan is a child who has good days and bad days just like every other four year old. We are grateful to the wonderful therapists and teachers that work with him to help us sort through a very intricate process. Nathan has also developed some complications with his kidney’s due to the toxicity of Avastin. Even if Nathan’s symptoms were stable and Avastin was working, we would still have to stop due to his kidney levels. This was the first time we had to consider long term effects on his body when choosing to stay on a treatment. We are starting to learn that after the body has been given so many heavy doses of medication, there is a true art to finding the right balance of push and pull. We are confident that our oncologist is a master of that art.

After Nathan’s MRI, we went to Boston to see his neuro oncology team. We were hopeful all of his doctors would let Nathan stay on Avastin for a few more treatments to see how his symptoms would develop over the next few months. They have a very aggressive approach to treating tumors like Nathan’s because they know it does not take much for long term function to be lost. They always follow the philosophy that the goal is for Nathan to be symptom free. Any time he has symptoms, his body is giving them the information they need to treat the tumor. In that appointment, we were reminded how serious the placement of Nathan’s tumor is. I found myself looking at the doctor, but unable to hear what he was saying at times because I didn’t want to believe it. I have been blessed to know many great teachers and he is by far one of the greatest educators I have ever known. I know the tone that good teachers use when they want you to understand what has to happen to be successful. Dr. Kieran had that tone when he for the first time said, “We cannot afford to wait and watch. The functions that Nathan’s tumor effects cannot afford to be compromised.” My eyes filled with tears and my heart dropped, but I heard his words and trusted him. Those words have stuck with me and helped me to accept that it is time we have to change to yet another therapy.

The tendencies of a majority of low grade gliomas tumors like Nathan’s are to go through different waves of treatment and stable periods until they eventually stabilize on their own anywhere from age 11-22. What makes Nathan’s case so risky is that he was diagnosed at 8 months of age and his current tumor is inoperable. We have a lot of time to buy and there is nothing that can be done to remove what is remaining. A majority of children find a time of stability off therapy with their first or second treatment. Nathan is four and will now be on his sixth treatment. We are now moving into drugs that are not standard treatments for low grade glioma tumors. We have had to consider very young clinical trials where toxicity profiles are unknown and traveling for treatments would be necessary, off label uses of FDA approved drugs, and chemotherapies that have long term risks that we would not like to take.

We have had to learn a significant amount about genetics and how it effects the treatment of Nathan’s tumor. We have now entered the world of targeted genetic therapies. The fact that my brother is a leader in the field of genetics and runs a lab for Human Genetics Research in Boston is absolutely amazing. He has been able to educate us on complicated science that the average music teacher and CPA parent do not understand. After a great deal of research and many conversations with his doctors, we have decided to go with a drug called Everolimus. It is one of the first drugs approved for children that is targeting a specific genetic mutation. It was developed as an anti rejection medication and for children with tuberous sclerosis complex. We have had Nathan’s tumor genetically mapped and know that he has the exact mutation the drug is targeting. The BRAF pathway has been a focus of cancer treatment for many years. The mutation associated with melanoma is the same mutation found in many low grade glioma tumors. Therefore, the drugs designed to inhibit this mutation in melanoma may be active against Nathan’s tumor. Unlike other younger targeted therapies that are available right now, we know this drug has over 20 years of toxicity results and data in children. In patients with Nathan’s mutation, it has a high success rate for either shrinkage or stability. We believe in the science of these therapies and know they have potential to make significant changes in a tumor like Nathan’s. Like any therapy, it does not come without risk and side effects, but it is the safest option we have. We feel very confident that this is the path for Nathan right now. The decision did not come without many sleepless nights and deep prayer. My brother said to me a few weeks ago, “You have already made the decision, now you just have to understand why you made it.” He was right.

The last thing a parent wants to hear is that a treatment for your child is no longer working and you have to explore other options. Although we wish we could stay on course, we feel truly blessed that Avastin worked so well for Nathan for almost two years. That is a significant amount of time on one drug. He has had time to learn how to drink regular liquids for the first time in his life, he has learned how to ride a bike, he has learned how to read and write, he has learned how to jump, climb, and swing. Nathan is able to play soccer with his peers, keep up with all the other kids in Tae Kwon Do, and will start T-Ball this week. These are all things that a parent who receives the diagnosis of a brain tumor in their 8 month old baby do not take for granted. These are things Nathan has worked hard to learn and he fights hard every day to be just like everyone else. We will not stop helping Nathan until we feel he has every tool he needs to be as strong as his body allows him to be.

Nathan continues to go to therapy three times a week to gain strength and endurance for his weaknesses. He is extremely aware of what goes on around him and I know he senses something is different right now. I know that all of the anxiety, worries, and fears have to go into a little box that we cannot open because it is our job to make Nathan feel safe. He wants to watch everything that goes on from placing the IV’s to having his feeding tube changed last week. He does not take his eyes off of the people working with him. He has learned to breathe through difficult situations and be brave through procedure after procedure. He is asking more and more questions that I often don’t know how to answer. I have met some amazing parents who are going through similar situations. They have helped me to find the right words at the right time so Nathan understands his situation without being fearful of what goes on. Nathan and Samuel are best buddies. They find great comfort in each other. God knew what he was doing when he gave us these two little angels. They just play and hug all day long. They make this path one that I would never trade because it makes us who we are. The four of us depend on each other for comfort and strength. We are all in our happy place when we are together. We know that life is precious and time as a family will always be a priority and something we cherish most in our lives.

The past month has probably been one of the hardest months for us since Nathan was diagnosed. There were many realities of his prognosis and long term treatment path that we have had to face all over again. There have been memories and realities I wish I didn't have to revisit. After I spoke to the doctor tonight, I felt great peace that we are on the right path. Spring is a perfect time to accept this new beginning and give every ounce of energy we have to making it as positive as we can. It is a chance to start the healing process again. It is a chance for Nathan to be one step closer to stability and a cure. It is a chance for us to enjoy our precious Nathan during every beautiful day that God gives us.

Thank you all for being part of our lives. Your encouraging words are what make the hard days easier and the good days worth celebrating. We love you and thank you for the commitment you have made to helping our family through this challenging path. God knows we cannot do it alone, so he has given us all of you.

Much love,

Mike, Beth, Nathan, and Samuel