Drug Therapy Decision and Exciting News

A rare blog post by Big Philip, whose usual writing these days involves his dissertation towards completion of an EdD in Higher Education through the University of Alabama. 

Son, in 35 years of religious study, I have only come up with two hard inconvertible facts: there is a God, and I’m not him. 

Father Cavanaugh, in the film Rudy

The quote by “Father Cavanaugh” in Rudy seems to sum up our situation with Philip.  Making decisions about your child’s health should be easy.  Take this pill and they will get better.  Sure, no problem.  Give them this shot and they will be healed.  Ok, let me have it. Unfortunately, that is not our situation. 

 Neurofibromatosis tumors are unlike cancer or other really bad health situations that you have heard about. NF tumors may or may not respond to chemo treatment. 
Chemo drugs are often used because of the chemicals within the tumor that they target or inhibit. Tumors may or may not respond to current treatment options. And, there are always the potential new drugs, still in the clinical trial phases, that have not been approved by the FDA for children to take. So our next step is to return to Dr. Reddy, our oncologist, and begin the process of getting Gleevec prescribed. We were hopeful to begin Gleevec before Philip started the third grade in order for him to adjust to the side effects but that is no longer a possibility with school starting in two weeks. We were cautious and thorough over the last few months before making the decision to begin drug therapy and that was the right thing to do. There are emotional decisions and rational decisions. Somewhere between the two is where we needed to land. From what we understand about Gleevec, the potential side effects are minimal, mainly abdominal discomfort and fatigue. We expect that we will begin Gleevec sometime in late August or early September.  Please pray that the side effects are minimal so that Philip can enjoy as normal a third grade year as possible. 

 Now for some good news!  We have hinted that we had some exciting news to share in the last few weeks.   If you are an  NBA fan  you may be familiar with Dan Gilbert, the Cleveland Cavaliers owner, and may also know that his son, Nick, has  NF1.  Dan and his wife, Jennifer, have been significant supporters of neurofibromatosis research.

Nick and his dad, Dan, winning the 2013 NBA Lottery

  Last year the Gilberts hosted a fundraising gala "The BeNeFit - A Celebration to Beat NF", that raised over $2 million for NF research.  At that event, the Strength and Honor Award was given to a young man named Victor Chukwueke, whose story is nothing short of incredible. We encourage you to see his story here.  It is truly inspirational and touches our hearts as parents of children with NF1.  

Recently,  Dr. Korf  called to let us know that our family has been selected as this year's recipient of the Strength and Honor Award.    To say that we are blown away would be an understatement.  It is hard to compare our situation to that of Victor.  Our work to establish an Alabama state chapter of the Children's Tumor Foundation is because we cannot just sit and wait for a cure for NF.   Our efforts help boost our faith that in our children's lifetime, a cure will be found so that Little Philip and Helen's hearts are not burdened the way ours are for the health and well-being of their children.  And so, our family will fly to Detroit in November to receive this honor and look forward to adding this moment of hope to our family's journey to further awareness for the importance of improved funding for neurofibromatosis research.  Helen and Little Philip are beyond excited to fly for the first time, and Helen is already planning her fancy attire.  

We don’t know what the future holds with our medical conditions but we do know that we will continue to talk about our experiences and will offer encouragement and guidance to support others diagnosed with neurofibromatosis.  As always, thank you for your love, your prayers, and all manners of support and encouragement you provide to our family.  

When God Doesn't Answer

My son was first diagnosed with neurofibromatosis in 2011.  There was such unimaginable heartbreak in my life that year that I had to fight very hard to stay strong and be the very best mother I could be to both of my children, hiding the dark fears and anxiety that plagued me during a time of great grief and sorrow.  I look back at that time and realize I was desperately holding onto my faith, but also holding my breath and waiting for an ending that I prayed I might share in countless testimonies.   Given my story, it would no doubt pack houses of God with wives and mothers eager to hear how God made good out of utter despair.   I could win the race to make it to God's glorious victory lap He had in store for me.  The race turned into a marathon that is still in progress in 2014.

I am a woman of faith, a faith that has grown deeper due to a journey these past few years that I would not wish on anyone.  I have tried to come up with a more eloquent way to express this, seeking a more optimistic and encouraging story that may be of some use or encouragement to others.   I read the Bible, sometimes intently, sometimes half-hearted, sometimes dismissing it for weeks or months at a time. I seek and read stories of endurance through great trials and even greater heartbreak than my own.  I compare my pain to the misfortunes of others who have the courage to be vulnerable in sharing their stories.   I feel brief solace in the recognition that things could be worse, but I quickly seek forgiveness for such a feeling, overcome by strong feelings of guilt for having the audacity to compare my difficulties to those of others for my own selfish reasons.  I wake up asking for wisdom, and I go to bed asking for wisdom.  I seek wisdom to know God's plan for me, for my son, and for my family.   I believe God has a plan to prosper and not harm me (Jeremiah 29:11) and I repeat this verse to myself often.  I  memorize and hide countless verses in my heart in an effort to bolster my spirit so as not to succumb to the fears and anxiety and let darkness take hold of my heart. Romans 12:12, 1 Thessalonians 5: 16-18, and my latest favorite, Isaiah 45:3.  

A friend recently wrote to me and said "I pray that should my family ever face struggles like yours, that I would be able to face them with the same strength as you do."  I am so appreciative of encouragement like this.  I am forever indebted for the thousands of prayers that have been said on my behalf, and on behalf of each member of our family.  Something deep inside of me shudders to think where I would be without the power of these prayers in my life.  But in the last few months, the marathon has worn me out.  I am battling with God about what his will is for my life and the life of my child.  My latest attempt to make sense of this side of heaven is in the book When God Doesn't Answer Your Prayer, by Jerry Sittser.  I try hard not to place my hope in this book because I learned the hard way that my hope cannot be rooted in anything earthly.  I do hope that this book provides additional wisdom to me.   Sittser clearly shows a biblical support for why God can handle my anger and my complaints, literally my assault on his power.  He gives examples of Job and Jeremiah, who called God to account. In these stories, these men finally snapped and let God have it.    My fear is that the point of this book, like so many I have read before, is to share that I am to use my pain for the betterment of God's kingdom.  And if that is what I am called to do, I will do it.  I've already accepted that role as a Christian.   But doing it with joy and steadfast hope is the challenge. 

With a clenched and angry jaw I cry out to God...

 What about my son's illness could possibly bring you glory?  

Why would you use a child as your pawn?

Why a mother's desperate love for her child to seek your own glory? 

Where is your grace in all of this?

Why would I share this terribly depressing part of my heart in such a public way?  Perhaps sharing where my faith is right now is more transparent and honest than simply saying "God is good, all the time." I feel myself the greatest hypocrite when people praise me for my incredible faith and spiritual strength.   I desperately needed to confess where I am right now because I know I live in a broken world with other believers who have had their heart torn to pieces by circumstances different than my own.  I want them to know they are not alone and that at the very least, I know God can handle the anger and resentment, the questions, the cries of feeling betrayed, the agony of seeking His grace and redemption when he seems (I stress seems) to turn His back on our fervent prayers.  I don't know anything else but to continue to pray that God will come quickly and reveal to me the light I so desperately seek that only He can provide.  The wait seems insult to a barrage of injury but I am confident He will answer.

The best prayers often have more groans than words.

- John Bunyan

July Oncology Consult

Clinic 8 waiting room at Children's Hospital

Today we made the short trip to Clinic 8 at Children's Hospital to meet with Dr. Reddy, pediatric oncologist. We met with her in March, and the recommendation then was not to make any decisions about chemo and to wait until the June 2014 MRI. As a reminder, Philip does not have cancer. He does have a large nodular plexiform tumor in his neck that is displacing his carotid arteries and has begun to push on his airway. It fully involves many important nerves so the idea of surgically removing it is not up for discussion, unless it becomes a life threatening situation.  There is always the 10-15% chance that his tumor could turn malignant.  Surgery is our last resort due to the significant risks and permanent life altering nerve damage that would result. Because of the shape of the tumor, its growth has been measured in volume and has been slow and steady.

There was a growth in volume from January 2014 to June 2014. Over all, the tumor appears to have gone from 80ml to almost 160 ml over 18 months of MRI imaging. Philip has no pain and no difficulty breathing or swallowing, although mom (Renie) notices more heavy breathing lately (possibly her paranoia or what she calls intense concern). Our concern is that we will watch this slow tumor growth take over everything in his neck and be left with no choice but to operate.

 The main problem with chemo options is that there is a low success rate of any available drugs working due to the slow growth of neurofibroma tumors. They just behave differently than cancer and other malignant tumors. The same drugs will not work on NF tumors. We understand that the success rate of the drugs in use today for NF tumors is around 20%. The reason we would take such a low percentage chance is that this is our only option.  Philip does not qualify to participate in one of the latest clinical trials that offers the latest chance of hope until he is sixteen. He turns nine next week. And, as with any clinical trial, there is no evidence that this latest trial is the miracle drug over 2 million people worldwide affected by NF desperately need.

The drug we are considering is called Gleevec. It is a twice a day oral chemo drug with relatively low side effects. Dr. Korf will consult with Dr. Reddy before our appointment with him on July 24th to make sure that our medical team agrees on this step in Philip's NF journey. We want to make sure this decision does not remove us from any future clinical trials in progress that may offer more hope. There are no easy answers.

 There is no clear path. We do not feel confident in this decision. However, we feel even less confident doing nothing.