Helen's 6th Birthday

We celebrated Helen's sixth birthday this past week.  Six years ago we were ill prepared for the day she was born.  My husband and I were in the middle of a raging stomach bug that had left me incapacitated the previous 72 hours.  My husband had succumbed to it the night before her arrival, announcing at 6:00 a.m. that he was not leaving the bed. With full empathy for what he was experiencing, I started the day with the intent of allowing him the grace and support he had offered me the previous three days.  As I exited the shower sometime around 6:30 am my water broke.  Poor hubby dragged himself out of the bed as we quickly rushed to get two year old Little Philip to day care for the day and make the necessary phone calls to both sets of grandparents to begin their drive from out of town to meet us. Helen arrived a little after 2:00 p.m.. March 18, 2005, a healthy 7 pounds, 12 ounces.  She was supposed to arrive some time around April 6, 2005.  I still clutch my belly thinking about what another three weeks would have shown on the newborn scale.

Early.  If there is one word to describe Helen, that would be it.  She was our surprise gift from God, as we learned in 2004 that our silly earthly plans were out the door, and heavenly plans were in progress for us to become a family of four.  She was an early arrival on March 18, 2008.  And as any friends or family know, Helen is an early riser.  The only time she has ever slept until 8:00 am was due to a fever.  She is a planner, providing early instructions for her seventh birthday within minutes of her sixth birthday party ending. Writing letters to Santa on December 26th each year for the next year.   While the early rising is clearly inherited from her daddy, the early planning and execution of complex event coordination is most definitely inherited from her Type A mother.  

Happy birthday, Helen.  You are such a joy and light to us and everyone that knows you! 

Helen also saw Dr. Korf this week for her annual NF check up. While she has a few new harmless cafe-au-lait spots, common in children with neurofibromatosis, there were no concerns. Now that she is six, we are about a year away from being mostly out of the woods for any potential optic glioma tumors and plexiform tumors that usually present before age seven. Hooray for some good news and the ability to breathe a little easier as spring arrives!  But, as an expert planner and providing early communication for the Birmingham NF Walk, Helen reminds everyone that has not yet registered for the May 10th Birmingham NF Walk to do so today! Visit www.nfwalk.org/al to register or donate.  

Oncology Consult Review

We completed our final consultation today with Dr. Alyssa Reddy, pediatric oncologist at Children's Hospital here in Birmingham.   We checked Philip out of school around 10:00 a.m. and headed to the Egg and I for brunch so that he could use his reward coupon he earned from Mrs. Krusinski for being a diligent reader.  Since it is due to expire March 21st, he has been rather insistent that he be able to use it soon.   This seemed like the perfect day.

All smiles!

From there we headed to Clinic 8 at Children's Hospital for our noon appointment.  Let me just say that it is a place of great hope, anxiety, and fear and probably everything in between.   This is the hematology and oncology clinic.  Many children were wearing masks and some were clearly undergoing intense treatment.  Parents looked tired, encouraged, worried, hopeful, exhausted, and I'm probably missing 90% of what was really going on in the hearts in that room which makes me completely inadequate to communicate.   We observed nurses and other staff calling patients by name, passing through the waiting room greeting children and families with warm smiles, kneeling down to get on the level of each child.  There is a strong sense of dedication and care in Clinic 8.   If this is where we may be spending some time in the future, it was an encouraging observation to file somewhere in the back of my mind...just in case.

Dr. Reddy was very informative and made us feel very confident that she had fully reviewed Philip's medical situation.  I don't think we expected to be given a silver bullet to cure Philip today but I think we had talked ourselves into expecting to hear about an encouraging chemotherapy option for our son.  

Dr. Reddy talked about two drugs that we knew about and gave us the name of a new one (Tamoxifen) to consider as she and the rest of our medical team talk about what direction we take.  None of them offer a guaranteed positive outcome but have shown some improvement for some patients.  I guess we knew this but hearing it again just stinks.  For now, we will remain in the wait and see mode.  We will wait until after the next MRI in late June to have another check on tumor growth rate.  We will wait until summer before a decision to start on a chemo regiment that will be less invasive for a school-age child.  We will wait until July in the hope that a miracle cure will be discovered in the next four months.  We will wait....

That sounds pretty dismal but this is just the reality of living with NF.  "Time is on our side" is the quote I remember most from Dr. Reddy today.  These NF tumors are very slow growing and that is one reason why they do not respond to typical chemotherapy drugs.  Slow growing is good in that Philip is still not experiencing any pain or risky symptoms from his tumor.  Slow growing is bad in that we just do not know if and when a symptom may show up that warrants action.  One month, one year, two years, 10 years?  If the tumor continues to encroach on his airway, what can we do?   Is that action chemo?  What if that fails?  Surgery?  We just don't know.   As of  March 11, 2014, there is no effective intervention to confidently shrink Philip's tumor.   We would love for someone to tell us we are wrong.

So ending on some positives:

1.  Philip is NOT IN PAIN

2.  Philip is not showing any serious concerns other than the obvious cosmetic issue of a large tumor

3.  Fellow students and friends are not bullying or treating Philip differently because of his tumor.  

4.  Philip seems ok with talking to peers who ask him  "what's wrong with your neck."

5.  Dr. Korf is a rock star in the NF world.  We are in the best hands possible.  

6.  God has our precious son in the palm of His hand.  He's got this.  

7.  God has placed such a strong community of support around us through our church, community, and work place.  The outpouring of prayers and thoughts has been incredible.  

No one could have prepared us for how much we would need #7.  #7 reminds us that #6 is true when we find ourselves doubting and then we remind ourselves of #1-5 to return us to the peace of trusting #6 again.  As someone who is a complete control freak, it takes a lot to admit I need something that I can't do for myself or my family.  This is a hard lesson for me in allowing the body of Christ to minister to our family and for me to be ok with admitting that we need it and accept it.  We need it and we are so grateful for all the love and support we have received.

So, if you haven't had a chance to sign up for the Birmingham NF Walk scheduled for May 10th, perhaps this update will motivate you to join with us, Team Moss, to raise funds and awareness that moves the Children's Tumor Foundation towards making our hope a reality in finding a cure for NF in our children's life time.  Sign up to "Join My Team" or "Donate" on the right hand column of Team Moss's fundraising page. 

Image from Philip's January 2014 MRI.  The white mass on the left is the tumor.   His jaw line is on top, spinal column in the center bottom. The tumor is NOT in his brain but rather, in the neck.    The solid black area in the middle is his airway.   This shows why airway is our greatest concern for tumor growth.

ENT Consult Review

Today we met with Dr. Audie Woolley, Philip's ENT.  Dr. Woolley did the original surgery on Philip back in 2011 that resulted in his NF diagnosis.  That little 2 cm tumor he removed back then has grown significantly, and is now the subject of many, many medical voices as everyone seeks to provide the best medical recommendation moving forward.

So our consult today was not good news.  Dr. Woolley was professional, kind, and incredibly informative.  We truly appreciated it when he asked the nurses to take Little Philip out to the lobby so he could speak candidly with us as parents.  When he returned, he shared that Philip's situation is very bad.  Basically his neck is full of golfball sized tumors.  Dr. Woolley differed in opinion from Dr. Korf's opinion in that Dr. Korf believes the neck to be one large lobulated nodular tumor.  Dr. Woolley's opinion is that there are several lobular tumors, all involving different nerves.  Neither opinion changes where we stand right now.   The main tumor of concern does involve the vagus nerve, as Dr. Korf had previously indicated.   This is the most concerning of the tumors due to its encroachment on the windpipe and esophagus.  To operate at this point would mean loss of significant function.  The only reason we would operate is if Philip develops issues with breathing, swallowing, or if he develops debilitating pain from his tumor.  There is a mild obstruction in his throat due to the tumor but it is not currently affecting his breathing or swallowing.  If we do have to operate this is what we are facing:

Tumor on the 12th cranial nerve - partial loss of tongue movement
Tumor on the 11th cranial nerve - significant loss of shoulder movement and nerve function
Tumor on the vagus nerve (10th cranial nerve) - loss of voice box function, permanent hoarse voice
Tumor on the 9th cranial nerve - partial loss of sensation at back of throat

Dr. Woolley estimates there are about 6-8 tumors in Philip's neck, all involving major cranial nerve function, and again, the one of greatest concern is the vagus nerve, also the hardest to get to should we need to operate.   People can function without a vagus nerve, but aside from the loss of a voice, many people have significant abdominal issues with reflux or emptying of the abdominal cavity.  It's not pretty but it is doable.  We pray we don't have to find out.

He strongly encouraged us to consider chemotherapy to try to shrink or stabilize these tumors.  He said he would consult with Dr. Alyssa Reddy, pediatric oncologist, after our March 11th consultation with her, and as with other specialist we have met with, all would report back to Dr. Korf who continues to serve as our "quarterback" to call the best shot Little Philip has.

Dr. Woolley shared that he has had about three significant NF patients in his 20 years of ENT practice.  He has consulted with the "big guys" as he calls them down at UAB, including Dr. Bill Carroll and Dr. Eben Rosenthal about Philip's case and they all agree that surgery means significant morbidity, meaning,  sacrifice of significant bodily functions.  We like what we see on all three online vitaes for these three men and are appreciative of Dr. Woolley's professional approach to seeking multiple perspectives prior to meeting with us.  Dr. Woolley is highly regarded in his medical field.  We are in good hands.

While we were viewing the MRI with Dr. Woolley in the clinic's computer kiosk area, we sensed the concern on the faces of nurses and other medical personnel as they watched over our shoulders the computer screen showing the massive bright white tumors that take up so much of the space of our son's neck.  As we exited the clinic, the silence was deafening,  We passed through a sea of smiling faces whose eyes turned quickly to Little Philip, revealing great concern for him.   It was almost too much for us to hold our emotions together and not fall apart in front of our son.

The patience required of us until our March 11th oncology appointment is torture.  But as anyone that has followed our story knows, we believe in a greater God than this broken, messed up world offers. We continue to hold firm to our faith that God has great plans for our son.  Little Philip's sweet spirit and incredible passion for being a good friend to everyone is a sweet balm to our weary parental souls. His smile, his giggles, his silly personality, it makes our hearts burst with love for him.   God intends him to be a light in this dark world.  We believe that with every fiber of our being.

A sweet friend was such an encouragement to me today.  She reminded me of a beautiful verse that has spoken volumes to my heart today as I tried to keep it together and carry on with the day as a mother, wife, employee, and friend.

And I will give you treasures hidden in the darkness - secret riches,  I will do this so you may know that I am the Lord, the God of Israel, the one who calls you by name.  

-Isaiah 45:3

That verse is going up all over our house as we bind it to our hearts and minds in the coming weeks. We pray that it may be an encouragement to you as well, if you are facing darkness and fears in your own life.  

We continue to covet your prayers and good thoughts, specifically for Little Philip but also for us as parents as we seek to be loving, and encouraging to our son.

Neurosurgery Consult Review

Philip enjoying the morning snow on February 13, 2014

Today we met with Dr. James Johnston, neurosurgeon at Children's Hospital of Alabama.  This is one of the three appointments Dr. Korf wants us to have in order to better understand the direction we will take if Philip's neck tumor continues to grow the way it has so far.

Given Philip's tumor's growth, about 20% this past year, is outpacing his typical growth each year. Eventually, serious concern may appear.  Those specific concerns cannot be fully predicted at this time.

• We know that as of today, both carotid arteries and jugular are displaced by the tumor and other important blood vessels are heavily involved by the tumor. 
• The tumor is growing around the windpipe but as slow growing as the tumor has been, it has not constricted his breathing or ability to swallow, even with the small area behind his left tonsil that shows slight pressure on larynx. 
• Then there are the nerves involved.  Facial nerves are certainly a concern, as is the vagus nerve that runs from the abdomen through the neck and into the brain.  The thought is that this tumor is growing on the vagus nerve, a very important nerve not to damage. 
• Vocal chords are not affected at this time, but could be.  
• There is some very minor deformity to some bone structure as well.  

In other words, there is a lot going on in that section of the neck so for this to be the place for a tumor, makes it difficult to predict.    When you see the size and shape of the tumor, it is hard to believe that Philip has no pain and no symptoms at this time.

It sounds like we are at the end of the watch and wait approach.  After our next MRI in June 2014, we will likely be looking at some kind of chemo option and if that fails, ultimately knowing what kind of surgical approach is going to be best should we have to make that difficult decision.

Dr. Johnston says the entire team listed below, along with him, will form a full review board of Philip's case  to make the best medical recommendations to us.

Dr. Audie Woolley, ENT Monday 2/25
Dr. Alyssa Reddy, Oncology, 3/11
MRI (pray for no growth) - 6/20
Dr. Bruce Korf - end of June to evaluate where we stand

As parents, this is emotionally exhausting. We are scared, but God knows that and is bigger than our fears.  We pray for peace and an increase in wisdom as we take these next steps as parents of a child with significant NF health concerns.  And most of all, we pray that we can make Philip's life as joyful and care-free as possible.  At age eight, he is wrestling with questions most children his age would never think to ask.  We have always said we do not want NF to define him, but with so many doctors appointments, it is hard for him to not ask questions and worry.  Our hearts feel like they want to burst from how much we love him, and want to see what God has in store for him.   He's a pretty special kid who just lights up the room with his special sense of humor and sweet spirit.  Thank you for your continued thoughts and prayers!

Team Moss has set a $5000 goal supporting efforts to find a cure for NF.  Join us May 10th at Railroad Park in Birmingham for the first annual NF Walk, providing support for the outstanding work of the Children's Tumor Foundation.
Team Moss's Team Page for NF Walk 2014 Birmingham, AL

MRI January 2014-Optimistic

Brave Philip just before his MRI

While the radiologist report shows that Philip's tumor has not shown significant growth, volumetric measure shows that it has grown about 20% since January of 2013.  So, we will repeat another MRI in six months and at that point, if the tumor shows growth, Dr. Bruce Korf suggested that we discuss chemotherapy as an option.  The good news is that the two chemotherapy drugs he talked about are very low intensity.  One, Gleevec, is a pill and the other is Interferon,  a once a month IV infusion.  The side effects are flu-like symptoms for the day or so after treatment.  The bad news is that these two drugs among are among very few drugs known to show any affect on NF tumors (about 20% success).  There is no cure for NF, so being in that 20% is the goal.  Surgery is the last resort since it would be such a high risk surgery due to the tumor's involvement of such critical functions (nerves, blood vessels, etc).  The only reason surgery would be deemed necessary is if the tumor became life threatening or malignant.  The tumor is displacing the mucosa (membrane) of his oropharynx (area of the throat just behind the tonsils) but it is not narrowing his airway.  The tumor is also displacing his internal and external carotid arteries and the internal jugular vein, but they do not show any signs of constriction and are therefore, no cause for concern.   As a mother, hearing these observations makes you feel like you are standing on a thinly frozen pond filled with sharks with someone giving you the thumbs up from the shore, all the while hoping the sun won't start to shine.  

So our next steps are to meet with Dr. Alyssa Reddy, pediatric oncologist, Dr. Audie Woolley, our ENT who originally did Philip's surgery resulting in his NF1 diagnosis, and we will also meet with a neurosurgeon, just in case surgery becomes necessary in the future.  Dr. Korf is an incredible doctor who is very detailed and patient communicating with us.  He believes that having these three medical opinions is the most thorough way to review Philip's situation in order to make the best decision for his future care, whatever that may or may not involve.  Also, we are meeting with Dr. Peter Ray a pediatric plastic surgeon to decide whether or not to remove another tumor that is on Philip's left temple.  It is a little sensitive to him due to the location, but it is also growing larger and down the road, may be a cosmetic concern that we don't want to impact his confidence or make him self conscious anymore than he already is.  

As I have said over and over, "Big" Philip and I have deepened in our faith in the last two years , and without our faith, we don't know how we would handle all of this.  But, we do have our faith, and we know our son is a child of God, with angels watching over him every moment.  Added to that faith are hundreds of family and friends that love us through times like this.  We are forever grateful.  We are thankful to be here in Birmingham, with Dr. Korf and his team at UAB NF Clinic who is world renown for being an expert on NF1.

We are now the state leaders for the Alabama Children's Tumor Foundation chapter and encourage you to like our facebook page here.  We will be launching the 2014 Alabama NF Walk in Birmingham to raise important funds for the work of the Children's Tumor Foundation.  More information, including the date and specific location, will be announced very soon.  We look forward to bringing together families and friends from around the state and surrounding states to raise awareness and funds to find a cure and successful treatments for children and adults like Philip.  We know other families are facing NF challenges much worse than ours and as long as we have the ability to raise awareness and support research for NF, we will.  

So long story made short, we will have a few more school absences this spring to cover these important medical appointments  but our prayer request is that the tumor will somehow stop growing on its own.  Thank you so much for your continued thoughts and prayers!  

2014 New Year's Resolution - $15,000

I am confident that 2011 and 2012 will always be the most painful and difficult years of my life.  I pray nothing worse comes my way in future years than what I faced during those two years.  But, I am also confident that  I will look back on 2013 as the year I began to rebuild my faith, my hope, my trust in God's plan for my life and the life of my precious family members.  Saying it is one thing, living it is another.  As each year passes and 2011 and 2012 become more distant memories, I will continue to discipline my heart and mind to daily put my faith into practice not for my own's sake but so that my faith will bear fruit in my relationships with everyone I encounter.

What is on my heart now is that it is January 2014.  That means it is time for Little Philip's upcoming MRI to see if his tumor has grown since July 2013.  The tumor was stable from January 2013 to July 2013.  So if there is no new growth this month, that would mean we have gone an entire year without growth.  That is my fervent prayer and I ask my family and friends to pray with me that the results will show no new growth.

As a means of stepping out in faith, the Moss family is committed to raising $15,000 for Children's Tumor Foundation in 2014.  Helen will turn six in March and Little Philip will turn nine in July.  9 + 6 = 15.  $15,000 for the fifteen years our children have been blessings to us this side of heaven.  We are working on plans to host an NF Walk in Birmingham and will announce details as soon.  We encourage all our friends and family to participate with us here in Birmingham.  If you can't make it, please contact us and we will help you participate in a nearby NF Walk or Run in your own neighborhood.

As always, we cannot thank you enough for your continued thoughts and prayers for our family.  We thank you for allowing us into your own lives to pray for you as well and we look forward to celebrating joy that comes in 2014!

-Renie

Little Philip update and Dr. Korf's Award

At our July 2013 check up with Dr. Korf, we inquired about a new tumor growing on Little Philip's right temple.  At the time, it was pretty small but since then has become more noticeable and somewhat sensitive.  The question we asked in July was whether or not we should attempt to manage any tumor growth that appears on the face.  Dr. Korf was very receptive to our concerns, but also rightly pointed out that future tumor growth is hard to predict.  He understands our desire to remove facial tumors but at some point, we may lose the battle to completely avoid cosmetic concerns.

Fast forward to today when we met with Dr. Peter Ray, a pediatric plastic surgeon here at Children's Hospital of Alabama.  We were very pleased with how intentional Dr. Ray wants to be in his approach to potential removal.  As a quick review, NF tumors grown along nerves, so any removal poses potential nerve damage and when it involves the face, there are lots of important nerves that control facial expression.  Dr. Ray pointed out that the location of Philip's tumor suggests that he needs to take into consideration the function of the eye (blinking, eyebrows, etc.).  He asked to further review Philip's latest MRI before making any decisions about surgery so we will revisit with him in about two months.  That will be after another January 2014 MRI and our follow up appointment with Dr. Korf.

Speaking of Dr. Korf, we were honored to have our story included in a recent video shown at the Children's Tumor Foundation annual Benefit Gala, where Dr. Korf received the 2013 CTF Humanitarian Award.  We could not agree more with the accolades for his thirty plus years of dedication to research to diagnose, find a cure, treat, and care for patients with neurofibromatosis.  We are truly blessed to live where we are to receive his care and for the leadership he provides to the UAB NF Clinic.