End of the Year Update

It has been sometime since we updated everyone on our family's activities, especially related to Little Philip's progress since enrolling in the MEK trial through the NIH.  For those of you who follow us on facebook, you know it has been a challenging semester for our son, transitioning to fourth grade while dealing with a pesky strep infection that would not go away.  This infection exacerbated the side effects of taking his new oral chemo causing him to miss a significant amount of school.

Philip's health has improved, although he continues to suffer from significant fatigue and chronic headaches that make it difficult to keep up with his peers at church and school.  He will often nap in the morning while at school, skipping physical education.  We work to communicate with his school what we think may be helpful, but we are trying to tinker with different changes in his daily and nightly routine, hoping to minimize the fatigue and discomfort.

We have no choice to but make this work.  We cannot think of what would happen if we allow his tumor to continue to grow.  We do know from other patients enrolled in this trial that their tumors have shown significant response to the drug.  In one case, there is a boy who started the trial about a month ahead of Philip and his tumor showed 13% reduction this month.  Philip will return mid-January for his first MRI and a significant number of medical evaluations.  We hope and pray that we have  good news to report back.

We are focusing on Christmas and being with family, resting.  With two full-time working parents, managing a complex medication situation while providing much needed love and attention to our daughter has been very challenging but we have managed as best we can.  We are thankful for the many ways our friends and family have provided support to us.  Selfishly, I do not know how this mama would have survived without the many Starbucks gift cards provided to us to make it through the long days at the NIH and often, just needing a pick me up on the way to work after a long night of issues or making up for house work, home work, or general life responsibilities that get backlogged.

We are so incredibly blessed to have Vestavia Pediatrics as our general pediatrician, helping us to manage the November strep infection and ongoing monthly blood draws that are required for Philip's enrollment on the MEK trial.  Those blood draws are no fun, but Nurse Sarah and Nurse Brenda do everything they can to make it light hearted.  We would be remiss without acknowledging the incredible heart of Dr. Simpson, who regularly prays with us in clinic.  It means the world to us to know he shares our faith and hope that bolsters our spirits for our son.  Meanwhile, Dr. Bruce Korf is our "coach" overseeing all aspects of Philip's neurofibromatosis case, always on the cutting edge of the latest research initiatives and willing to spend the time needed for us to learn and educate ourselves about what may be on the horizon to help our son.

Here, at year's end, we believe our 2015 total funds raised for Children's Tumor Foundation exceeds $32,000.  We are also aware of several benevolent gifts made on our behalf to the UAB Neurofibromatosis Clinic where we receive our genetic care through Dr. Bruce Korf.  We cannot thank our supporters enough for providing funding towards research efforts that we know will lead to effective treatments in our children's lifetime.   And finally, several friends and families provided personal funds that we have set aside to assist us with requires flights to and from the NIH in Bethesda, allowing both Big Philip and I the ability to be together with our son for these important trips throughout the time he will be enrolled in this chemotherapy trial.    After taking Little Philip by myself in November, I will never do it again. It was an emotionally difficult trip to be there alone with our son, especially since Little Philip became very ill from the effects of the chemo, combined with an allergic reaction to cephalexin.  I need my husband by my side, and he needs me too.  So thank you, friends and family, for providing us with the ability to make that happen.

We have much to be thankful for here at year's end but we will not publicly share all the ways we rejoice as we pray for the ability to scream from the mountaintops after January's NIH trip when we hope to share good news.  Please pray for the medical team responsible for Philip's care, including everyone at Vestavia Pediatrics, Dr. Bruce Korf at UAB, Dr. Brigette Widemann at the NIH, Trish Whitcomb, NIH clinical care managing nurse, Andrew Baldwin and Marielle Holmblatt, his NIH clinical nurse practioners, Doctor John, the NIH post-doc fellow that helps with Philip's exams, Kara Heisey, NIH patient care coordinator, Mrs. Pam, his NIH neuropsychologist, and the staff at the Children's Inn that always welcomes us with a smile and encouragement when we stay at the NIH.

2015 Alabama NF Walk - Donate or Register Today!

9 days until the 2015 Alabama NF Superhero Walk. Neurofibromatosis has caused a lot of changes in our family in the last year. In November of 2014, we were humbled to receive the 2014 CTF Strength and Honor Award at a beautiful Benefit in Detroit that raised over $3 million for clinical research through the Children's Tumor Foundation. This November we will be taking our son back to the NIH for a follow up exam to his recent enrollment in a chemo drug trial we hope and pray will shrink the inoperable tumor in his neck. 

After receiving that award last year, we were back in our hotel room late that night. Little Philip was holding the award and said "The sphere symbolizes NF and the figures below are treating current patients by holding the sphere and open-handed as they search for a cure." He looked up at us with the biggest smile. I kid you not. 

If you've not donated or registered to walk with us yet, please make the commitment with us to push that NF sphere off the cliff in Philip's lifetime. Register or donate today at www.nfwalk.org/alabama

AZD6244 Clinical Trial - We are In!

We have been so incredibly busy over the last month that we have failed to share our exciting news.  At the end of August, we received the call that Philip had been selected to enroll in the AZD6244 clinical trial for inoperable plexiform tumors.  We were overjoyed to have this answer to our prayers!  

In mid-September we took Philip to Bethesda, MD for an intense week of medical evaluation and testing to qualify to begin taking this promising new oral chemotherapy drug.  He began taking it on September 24th.  He will return to the NIH in Bethesda in January for an MRI to see if the drug is working.  Please pray that it is working this very minute to shrink the tumor away from Philip's airway and vital blood vessels.  

The trip was intense but also a lot of fun! We stayed at the Children's Inn at the NIH which is an incredibly wonderful Inn where children and their families stay at no cost.  There were activities and field trips throughout out time there.  Philip especially enjoyed the game room and he met several other children staying there with their families.  We also attended a Washington Nationals game where Philip went onto the field and into the dugout before the game.  He even got Steve Strasbourg's autograph!  

This is not been the most eloquent or well thought out blog post but in an effort to remain honest and real with our family and friends, I will simply say that we are emotionally and physically exhausted as a family.  This has been an emotionally draining month for us but we are not without hope and encouragement from friends and family that continue to lift us up in prayer and support.  We look forward to gathering together as an Alabama NF community in a little over a week at the Alabama NF Walk where we will see new and familiar faces of NF and remember that our leadership and advocacy efforts are vital not only to our son but countless other children and adults living with NF.

2014 Children's Tumor Foundation Annual Report

I am pleased to share with you the 2014 Children's Tumor Foundation​ Annual Report.  Within these pages are messages of hope through reported advances in research made possible through your ongoing support.  Within these pages, I see the names and faces of family and friends who have supported us through the full range of emotions we have as parents to a child significantly affected by neurofibromatosis.


We are less than $1000 from meeting our 2015 Team Moss goal of raising $20,000 for Children's Tumor Foundation.  Whether you can join us on October 18, 2015 at the Alabama NF Walk or not, we encourage you to partner with us by registering or donating today to help us meet our goal.

Next week we leave for Bethesda, MD, for Little Philip's grueling medical evaluation prior to beginning the AZD6244 drug trial.  We are thankful that this trial is made possible by the combination of our family and friends, the committed talent, expertise, and passion of our medical team led by Dr. Bruce R. Korf​, the outstanding Children's Tumor Foundation staff that has become like family to us, and the countless scientists working at this very minute, literally in places all around the world,  fueling our hope that a cure will be found in our children's lifetime.  Thank you, friends and family, for loving us so well.  Your benefit not only the Moss family but over 2 million people living with NF worldwide.  A few images from the full 2014 Children's Tumor Foundation Annual Report are below that show some familiar faces that appear within the report.  The full report is listed below as well.

Conversation in a Canoe

I intended to share this story months ago but in retrospect, I am thankful for God's timing that delayed me from sharing it then.  I sat down several times over the last few months, intending to share this story, but various life circumstances prevented me from doing so, mixed with a little self-discipline on my part to resist the temptation of over sharing.  The story now means more to me, and I hope to someone else that may be reading this now.

Let's go back to Mothers Day weekend, when we traveled to Mississippi to be with family at a beautiful little patch of nature, complete with all manner of wildlife, including close encounters with snakes and fish that seemed to jump onto the hooks of rookie fishermen (including me).  I admit that I was less than enthusiastic about the trip.  We had been going at a feverish pace in our family, due to extra demands at work, after school activities, maintaining volunteer commitments at schools, our church, and in the community.  To top it off, that was the week that we stopped chemotherapy for Philip, having learned that his tumor was not responding to treatment.  Mothers Day was the farthest thing from my mind.  I was a wreck, and felt spent but had a to-do list a mile long that was not going away.

As we drove to Mississippi that Friday night, my patient husband reached over and grabbed my hand and thanked me for making the trip, acknowledging that it was one more activity in an endless sea of chaos that month.  I listened to our two children in the backseat chattering with one another.  I closed my eyes, longing for the ability to be more like these children, carefree, innocent, oblivious of responsibility and a smart phone color-coded schedule.  As soon as we arrived, I was relieved to be there and felt my spirit refreshed by the joy surrounding me in the form of catching up with family members we had not seen in a while, the sound of children running and laughing together, as well as an abundance of good food and fellowship in store for the next two days.

The next day, we heard an update about a close family friend that is battling advanced cancer.  He had been reading a devotion for those facing cancer that encouraged him to spend intentional time with significant people in his life.  Later that afternoon, Big Philip and I stole away in the canoe together and paddled around the lake.

"You know Renie, that is what I am trying to do with Philip," my husband said.  The canoe stopped moving as I realized this was going to be a serious conversation.  Paddles down.  He continued.

"We don't know what the future holds for our son, so if that means we aren't able to devote as much time for CTF fundraising this year in favor of spending more time with my son, that is what I'm going to do.  That is why being a Cub scout den leader means so much to me.   I want to spend intentional time with him.   I want to be significant in his life as his father."  

My husband is right.  There is no rebuttal.  We are fighting, doing everything we can to ensure that a cure for NF is found before NF acutely affects our son's life.  If NF has its way with my son, I need him to know I did everything possible, that I held nothing back, making a pest of myself in every way possible to garner one more sponsor for the NF Walk, gather one more family like us, that needs to know they are supported and not alone in this fight.   I close my eyes and picture the faces of the many NF parents and children, young and old, that I have met over the past three years.  Some are no longer with us.

However, the memories we want our son to have of his childhood are no different than the dreams of other parents. We want Helen to be as unaffected by NF as possible too.  Intentional time spent with significant people.  Making great childhood friends and growing up together, family vacations and trips, camping, swimming, learning to ride a bike, taking healthy risks, laughing so hard that you can't catch your breath.  We want these memories to outnumber the countless MRIs, chemo pills, doctor's appointments, blood draws and more.

This is why I have hesitated to write this story because I do not know how to end it.  There is no happy ending, not yet, but many happy chapters that continue to unfold.  We fight for the ultimate happy ending in the midst of battling for more moments of joy and laughter.  There are plenty of these moments, more moments spending intentional time with significant people.  It should not take a life altering circumstance like ours to pause and think of ways everyone should be spending more time with significant people in intentional ways.

We have spent this summer working on being more intentional in reflecting on each of our prayer warriors and supporters that never seem to tire of sharing an encouraging word, a helpful hand, a willing spirit to continue to the fight to cure NF alongside us.  Many of these same friends and family fight by providing a normal childhood experience for our son, helping to push the shadow of NF away, allowing him to just be a child, full of wonder and excitement, without fear or anxiety for a little while longer.

Thank you to my husband, Big Philip, who is willing to fight my fighting spirit in moments like we had in that canoe that day.  As we celebrate our 14th anniversary this week, I am amazed at your patience with me and courage to hold me through the stormy emotions that I sometimes cannot escape.  You are my rock and quiet source of wisdom just when I need to hear it the most...like in a canoe in the middle of a lake from which  that I cannot escape.  God is funny like that sometimes.

And so, our story continues but not without a deeper understanding of what is significant in life to be intentional about and the countless friends and family that are very significant to our family.

Wait Listed

We wanted to update you on Little Philip's health and I will try to be as brief as possible.  Before I do, I want to start by thanking each and every one of you for your continued support through your prayers and encouragement.  

Third Grade End of the Year Pool Party

A reminder that there is no cure to neurofibromatosis and no proven treatment for plexiform NF tumors like Philip's.   Everything we do is to manage the situation as best we can until a cure can be found.   Here is a quick recap of Philip's most recent NF journey.

In early 2014, Philip's medical team felt that the tumor in his neck was growing too fast.  Despite having surgically removed it 2011, it had grown back, starting to surround his airway, displacing his carotid arteries, and involving major nerves in the face, digestive system, and upper body movement.   His physicians continue to be concerned that if they can't stop the tumor's growth, high risk and life threatening concerns could occur.  To date, he has experienced no pain and no concerns.  Our goal is to keep it that way.

Since surgery is no longer an option, Philip began taking oral chemotherapy in August 2014 and stopped taking it at the end of April 2015 when an MRI showed that the tumor had grown an estimated 18% in 9 months.  There is no other treatment that his medical team recommends other than a promising clinical trial through the National Institutes of Health/NCI.  The drug is called AZD6244 (Selumetinib) and there are 50 pediatric slots. 

Philip is currently on the waitlist and as of this past week, his position is a "high teen" number due to his case being considered high risk.  We should know by August at the earliest, or November at the latest if he is selected to participate in this two year clinical trial.  If he is selected, then we will travel to Washington, DC several times a year to complete testing and MRI imaging while on trial.  

Other than having neurofibromatosis type 1, Philip is almost 10 years old, came in third place in his school-wide spelling bee, advanced to Webelos cub scouts, reads roughly 500 pages in books each week, and he is going to be a junior counselor in a chess day camp this summer before heading off to his third year of sleep away camp.  He's super smart, super silly, and has the biggest heart for family and friends.  When children ask him "what's wrong with your neck?"  he says it doesn't hurt his feelings and that he is happy to tell them about NF because it's the only way we will find a cure if more people know about it.  

I should mention that Helen does not show any concerns with her NF1.  Big Philip also does not show any concerns, although he suspects he may have always had ADD from NF1, but he is not using (or allowed to use) that as an excuse in finally finishing his doctoral dissertation in higher education administration.  

We will raise $20,000 in 2015 for clinical research through the Children's Tumor Foundation and, along with several other Birmingham area NF families  we will host the 2nd annual Alabama NF Walk on Sunday, October 18, 2015.  We know that over 70% of all current NF research has been informed by data from Children's Tumor Foundation, including the trial for which we are wait listed.  We also know that over 80% of each dollar raised goes straight to clinical research and patient education and only 6% is spent on management and administrative cost. (Link to CTF financial reports).  We are also thankful for family and friends that participated in the rather scandalous but fun Cupid's Undie Run as "Team Moss"  in several cities across the country in February, raising the first $2900 towards our $20,000 goal.  

If you would like to join us or support us as a virtual walker or donor this year, please register here.  Knowing the walk is only one way to raise awareness,  we are eager to speak with any one interested in learning more about our commitment to support the Children's Tumor Foundation and would welcome the opportunity to share ways others can join with us as well.  

2015 Mother's Day at the Hunt Camp, #ikNowaFighter

Third Grade Bible Presentation at church

Chemotherapy Stopped

Today's oncology appointment was important because we knew the disappointing news we were going to receive. Philip's situation is not without hope but the hope is now in getting selected for a clinical trial.

The latest NCI volumetric analysis of Philip's tumor showed approximately 12% growth over 4 months. So, over the course of his 9 months of Gleevec oral chemotherapy treatment the tumor grew about 18%. Gleevec did not work. It was a long shot at about 30% efficacy rate for other patients but it was our only option.

Dr. Reddy, Philip's oncologist, is sending medical records to the National Cancer Institute (NCI) in the hopes he may be selected to participate in a clinical drug trial for Selumetinib Sulfate, or AZD 6244 which is much easier to pronounce. Dr. Korf's office at UAB is also sending all of their records.  We know several families that are current participants in this study and they have shared that their children's tumors have often not only stopped growing, but are SHRINKING while on treatment. At least three of these children have neck tumors very similar to Philip.

We have emailed the principal investigator (recipient of the 2013 Children's Tumor Foundation's Friedrich von Recklinghausen Award) as well as the study administrator. We hope to hear back very soon about how viable this trial is for our son since his physicians consider his prognosis very strong for inclusion. He meets all trial criteria. So now, we wait.

It was very disappointing to leave Dr. Reddy's office today with no return appointment scheduled. But, we are very thankful for everyone that came into our exam room to offer encouragement. That includes Jeff Barrett, Children's Social Worker who always provides us with ways to keep our spirits up; Sasha Ramini, Dr. Reddy's awesome nurse practitioner who knows all my coolest girlfriends; as well as Dr. Reddy herself, who we cannot not say enough good things about. Her commitment to research, collaborative approach with colleagues, strong understanding of clinical trials, and encouraging personality continues to be a blessing to our family.

So, we are not completely without hope, but remind ourselves and everyone that has been praying for Philip that despite the tumor's continued growth, he continues to experience no pain, no discomfort, and is closely monitored by the best of the best in medicine right here in Birmingham, AL. We are so proud of Philip's strength throughout his medical challenges, and his ability to always stay goofy and see the humor in life. Thank you for your persistence in prayer for our son.

Chemotherapy Update

We haven't officially shared how Philip's Gleevec (oral chemotherapy) therapy has been going since he began taking it in August of 2014.  Thankfully, all of the main side effects we were concerned about have not happened.  He has had no abdominal discomfort, no headaches, and while he seems to be a little more lethargic than usual, it has not affected his quality of life or academic performance.  He continues to be the goofiest, silliest nine year old boy you have ever met and brings us joy each and everyday through his big hugs and warm smile.

If you've watched the video from the Detroit NF Benefit, you saw that we had one bad night where we were instructed to go to the emergency room after Philip spiked a fever and was sick to his stomach in October.  It was the start of a strep infection that was quickly put down with the usual antibiotics and a day in bed, surrounded by a pile of beloved books to read.  Philip's favorite activity is reading and we estimate that he reads anywhere from 300-400 pages each week.  We are surprised his Kindle doesn't smoke from how often he reaps the full benefit of his Amazon Prime membership. Since that one bad night, Philip has only been sick to his stomach 3-4 more times. That placed him in the minority among his friends who got the stomach bug or worse this winter.  

November was his first MRI after starting oral chemotherapy, his sixth scan (technically seventh since he also had a PET scan in 2013).  The initial radiology report ruled out the scary stuff like malignancy or compressed blood vessels and nerves.  Dr. Reddy reviewed the MRI with us and to the naked eye, it did not appear any larger than in his previous June 2014 MRI.  It is important to know that the way in which Philip's tumor is officially measured is by volumetric analysis, something that is much more accurate than the traditional 2D MRI measurements.  The image below shows why the shape of an NF plexiform tumor makes it difficult to accurately measure growth between different MRIs.   Please note that the image below is not Philip's tumor.  

From http://www.nfmidwest.org/volumetric-measurement-of-tumors-in-neurofibromatosis/

The volumetric analysis showed that the Philip's tumor still grew between June 2014 and the November 2014 MRI, but it was the slowest recorded growth since 2012.  Clearly, something has changed and our medical team believes it is because the Gleevec.  In January 2015, his dosage was doubled from 200mg per day to 400 mg per day.  Again, other than some slight increases in crankiness and lethargy, you would never know Philip is taking chemotherapy.  He is unbelievable. So that is all good news!

We are noticing an increase in the appearance of sub-cutaneous NF tumors on Philip's body.  These are a different kind of NF tumor than the large nodular plexiform in his neck.  They do not pose a risk of turning malignant although some may become painful during his lifetime in a way that surgery becomes necessary.  This is why we had one tumor on his forehead surgically removed in March 2014.  He has a few on the back of his scalp that make brushing or combing his hair painful if you are not careful.   There was a time when these tumors were my worst nightmare due to where they may appear over time and what mean spirited peers might say or do.  Now, these are the least of our concerns, although we are keenly aware of how they may make him more self-conscious over time.  It's just another aspect of NF that we hate because we can't stop them from happening.  

We are gearing up for an April 1st MRI that we hope will show an even greater impact of Gleevec therapy.  We understand that the odds are stacked against us based on the experience of other NF families we know and love that have been here before us.  But, we are remembering the relief we felt from the November MRI that showed slowed tumor growth and hoping for the same outcome or better this time.  

Thank you for your continued prayers and support of Children's Tumor Foundation!  The 2015 Birmingham NF Walk will take place on October 18, 2015 at Veteran's Park.  If you would like to join or donate to Team Moss, select the blue "Join Team" button or the grey "Donate Now" button on our Team Moss webpage.  Our goal is $20,000 and we are confident we will get there by October 18th through the tremendous support of family and friends who have gone above and beyond to love on our family with prayers and support since Philip was diagnosed with NF in 2011.