Patient Spotlight: Jordan’s Journey with Neurofibromatosis

Jordan’s journey with Neurofibromatosis (NF) started at her 10-month check up.  Our pediatrician noticed that Jordan had over six tan birthmarks, which are referred to as “café-au-lait spots”. As a mother, of course, I noticed these since when she was born, and often wondered why she had so many of them when neither I, nor my husband, Brian, had any. The pediatrician explained that having over six birthmarks was an indication of a condition called “Neurofibromatosis”. My heart sank. As a medical professional, I knew about NF.

We were referred to St. Louis Children’s Hospital for an eye exam, because many children with NF sometimes develop Lisch nodules (tiny bumps on the iris). The eye doctor found nothing upon examination, but suggested we see another doctor who specialized in NF.  We were referred to Dr. Gutmann at the Washington University NF Center and have been under his care ever since.

At our first appointment with Dr. Gutmann, he felt that Jordan very likely had Neurofibromatosis Type 1 (NF1), but without two signs of NF1 present, an official diagnosis could not be made. We did this process annually, with everything seeming to be fine until the summer of 2009 when Jordan was 3 years old.  One day she told us that her head hurt.  My husband and I laid in bed that night wondering why.  At this point, I was pregnant with our second child.  We remembered that I had said my head hurt that day.  After talking, we decided I wouldn’t say my head hurt in front of her as a way to see if she was just mimicking me.  We also decided to see if she voluntarily complained the next day about her head.  The next day she played and acted normal.  I still had a nagging feeling that something just wasn’t right.  In the months that followed, I had a casual opportunity to consult with our pediatrician about this headache incident with Jordan.  Our pediatrician said not to worry about it unless it stopped Jordan from her normal daily activities.

As 2009 progressed, we welcomed our second daughter, Isabella, into the world.  Jordan handled this change as well as any three year old child can.  She has always loved being a big sister and caretaker.  During this time, Jordan continued to complain about her head hurting.  On two separate occasions between October 2009 and February 2010, she stopped playing and went to lie down because of a headache.  The first time this happened was during Halloween trick-or-treating.  Shortly into our fun-filled evening, Jordan said she wanted to go home because her head hurt.  This was alarming to me because no child wants to stop getting treats or playing with other children.  We called to report this to Dr. Gutmann, and he advised us to obtain an MRI of Jordan’s brain.

In March of 2010, at the age of just 3 ½, Jordan had her first of many MRIs.  As parents, we were terrified.  We didn’t know what to expect, much less how to explain the process to our sweet little girl.  We did our best, but cried as they wheeled her away.  Everyone from the nurses, anesthesiologists, and child life psychologists did a wonderful job with Jordan.  They always showed expert care and professionalism toward us.  They guided us where we should wait and how long the testing would take.

The next day, I called for results.  The MRI confirmed that Jordan did indeed have NF1.  It showed an optic nerve glioma (tumor on the optic nerve that sits behind the eye).  The glioma was the second indicator that confirmed the diagnosis.

I asked several questions.  What does this mean for Jordan? What if the glioma grows?  What do we do next?  We were told to wait for three months to repeat the eye exam and MRI.  If the glioma showed growth, or would have any effect on Jordan’s vision, her treatment would be chemotherapy.

I had to tell my husband that our child had a tumor.  Although I wanted to wait to tell him in person, he insisted to know the results over the phone.  I can still recall his emotions in the sound of his voice.  We needed to hold, hug, and support each other at that moment.  I felt helpless.  As parents, we felt devastated and heartbroken.  We grieved after hearing the results.  Our family and friends grieved along with us.  As this point, we knew who our true friends were.  They cried with us, they called, they took the time to listen and support us.

I explained to Jordan that she had a “bump” on her eye.  I explained that this was the reason she was having headaches.  I explained that the doctors had to watch her bump by having more MRIs and eye exams.

The following three months were long and hard.  I tried not to worry.  We prayed a lot.  During this time, we purchased a new home and were trying to remodel it.  My husband re-injured his back and required surgery.  We had a new baby to take care of, and Jordan was an active 3 year old. I worried that our child may have to start chemotherapy. I was overwhelmed.

Thankfully, Jordan’s next MRI showed no change.  We did this regiment every 3 months until June 2011, when we were able to move to every 6 months, as her optic glioma was stable and showed no signs of growth or change.  In June 2012, Jordan had her 8th MRI yielding yet more good news: the glioma was still stable with no growth.  Dr. Gutmann’s advice was to have another eye exam in 6 months and an MRI in one year.  Jordan has since graduated to annual eye exams only. Although I felt nervous ceasing the MRIs, they reassured me that I would know if something was “off” because Jordan was older now.

After Jordan’s diagnosis was confirmed, I started feeling like I wanted to raise awareness about NF.  I researched fundraising and started by creating a team called Team Little Buddies (Brian had always called Jordan his Little Buddy) for a local walk for NF.  We were the top fundraising team that year!  We did well raising $7,473.24 for that organization in two years.

I switched gears after learning that we could direct our fundraising efforts locally to the medical team that has been with us from the start. I wanted to host our own event.  The planning and brainstorming started (with those family and friends who stood by us when Jordan was diagnosed). I wanted to make the event a fun day for the whole family, but mainly focused on children.  After many hours of thought, the 1st Annual fuNFest was dreamed up.  The “N” in fuN and the “F” in Fest represents NF.  We also used Jordan’s name as an anagram for our slogan – Join Our Rage, Donate Against Neurofibromatosis.

FuNFest is truly a fun day filled with something for everyone to do. Some of the many things at fuNFest are bounce houses, Cow Patty Bingo, silent and live auctions, 50/50 and other raffles, a cake walk, bake sale, food, drinks, kids games, gun raffles, merchandise sales, etc.  After 7 years of successful funfests, we have raised and donated over $160,000 to Washington University NF Center to accelerate the scientific discovery of a cure for NF.  I give thanks to our community who supports us and the tireless efforts of each friend and family member that come together to make our event successful every year.  My cup truly runneth over!

Over the years, I have asked myself – what did I do to cause this to happen to Jordan?  I struggled with this for quite some time.  Through the grieving process, I questioned why this happened to our child when I didn’t drink or smoke during pregnancy.  I tried to eat healthy. I don’t understand why, but I believe all things happen for a reason.  I know we are making a difference by raising awareness about NF through our annual fuNFest event.  So many people had never heard of NF, but our community is now aware of this disorder because of Jordan’s journey.

Photo Credit: Diekemper Photography

And how is Jordan doing today?  She’s great.  She makes straight A’s at school.  Although she struggles a little in math and her balance and coordination are not always the best, overall, she is doing wonderfully.  She has the biggest heart of anyone I know.  She has such an encouraging, kind and caring nature about her.  I still worry every time Jordan has a headache, or any ache or pain.  I think this is very normal for a parent with a child with NF.  I pray for and believe a cure for NF will be discovered in Jordan’s lifetime.  Her journey with NF is unpredictable, but we pray that NF will never severely affect her.  I am so grateful to have Dr. Gutmann and his team on our side and so close to our home.  I believe in them and their efforts to find the cure for NF.