Living with NF
Ken Janik from Cheektowaga, NY and Erica Givens from Nashville, TN both live with NF2 and met through a Facebook support group. After emailing for a few months, they decided to attend the 2013 NF Forum and Nashville NF Walk and meet in person. Ken was one of the top fundraisers for the Nashville NF Walk and Erica was the Captain of Team Givens, one of the top teams. They enjoyed getting to know one another and are now a couple. "We are very happy," says Erica. "Our relationship formed partially because of the NF Forum and Nashville Walk. So thank you very much, CTF, for helping bring us together!"
"When I was 15, I began having bad headaches and a loud, high-pitched, nonstop ringing in my ears. Because my mom, grandfather, and two of my uncles have neurofibromatosis type 2, my mom knew that her worst fear might be coming true. She contacted her neurologist who eventually diagnosed me with NF2.
"My neurosurgeon recommended surgery but my Mom was concerned about the side effects that might occur. She looked online and found a new radiation treatment called Fractionated Stereotactic Radiotherapy (FSR) that seemed to be a good option. Unfortunately the radiation made my right acoustic neuroma swell and necessitated surgery. A few weeks after the procedure, I became very sick due to fluid on my brain, so I had another surgery to have a shunt implanted. About a month later, the shunt malfunctioned and I had another surgery to fix it. All of this happened before I was 17. Then, in May of 2008 I was having a lot of pain in my neck and developed drop foot. An MRI determined that I needed another surgery to remove a tumor from my spine.
"Because of NF2 and all of these surgeries, I became deaf and had to re-learn how to stand and walk. But I refused to let NF2 stop me! I was determined to get my GED; I studied, took the test, and passed with flying colors! Shortly thereafter, I earned my driver's license and got a job which I have worked for over 11 years.
"I have met a lot of great people with neurofibromatosis from online support groups and CTF events. I was proud to be able to walk and raise money for the Nashville NF Walk and help find a cure for neurofibromatosis!"
"When I was 12 years old, I developed a lazy eye. After talking to my pediatrician, I was sent to a specialist to run some tests after which I was diagnosed with NF2. I am the first in my family to have neurofibromatosis.
"I lost hearing in my left ear when I was 14. At the age of 18 I had surgery to remove a tumor that was pressing against my brain stem. Three years later, in the beginning of 2011, the tumor grew back requiring another surgery and radiation.
"After surgery, I graduated from high school, earned a Certified Nurse Assistant degree at a community college, and got a job at the Mayo Clinic.
"In August 2011, I had a tumor removal surgery that took my hearing. Also, my lazy eye got worse; the eyelid shut completely leaving me half blind. That year I went from feeling like I had the best luck to having the worst.
"Facebook support groups were a big help to me. It is through them that I connected to CTF. I am so thankful that I am able to fight this disorder through fundraising. NF2 may have taken away my hearing and half my sight, but it can never take away my strength, courage, and determination to fight for what I want in life! "
"Our son was diagnosed with NF1 at age two. Literally on his second birthday, a plexiform tumor showed up on his buttocks. When we went for his wellness visit, the pediatrician suspected neurofibromatosis and recommended an appointment with a geneticist. The resulting tests confirmed the diagnosis. Due to NF, in addition to the plexiform tumor, Kohta has mild scoliosis, a fibroma, numerous tumors throughout his body, learning difficulties, and a resolved optic glioma.
"Kohta is now eight and thrives! He is in second grade, participates in Cub Scouts, and enjoys swimming and riding his four-wheeler. The school he attends has arranged an Individualized Education Plan (IEP) for him, and he has occupational and speech therapy both at school and privately.
"Kohta was given a special gift of a black lab puppy from the Rileys, an NF family in Wisconsin whose son's experience with neurofibromatosis is similar to Kohta's. He also has an NF pen pal, a boy named Koda, who shares a lot of similarities with him. We truly value giving and receiving support through the Children's Tumor Foundation and within the NF community.
"Kohta often says that he is proud to be an NF Hero, and we are proud of him every day."
• Christine Weese, Kohta's mother
"Bennett is four years old and was born with neurofibromatosis type 1 (NF1). He has an optic glioma tumor that extends into the chiasm, the part of the brain where the optic nerves partially cross. He is currently being treated with chemotherapy. He will undergo 58 weeks of chemo and MRIs every three months. Bennett also has two plexiform neurofibromas, one in his neck and one at the base of his spine.
"You would never know that he is going through so much, though, because of his outgoing personality and loving nature. Bennett always has a smile on his face, is constantly making others laugh, and is an inspiration to everyone around him.
"Any donations made to the Children's Tumor Foundation in honor of Bennett would be much appreciated. We must find a cure for this devastating disorder."
- Jesica Jones, Bennett's mother
One of the questions many people ask when they or a loved is newly diagnosed is “what now?” The answer that they often receive from their physician is “watch and wait.” While that is usually the wise approach for treatment there is more you can do and the Children’s Tumor Foundation has developed programs and resources that provide information and support for you and your loved ones.
Taking the First Steps:
- Get the facts – visit the Foundation’s “What is NF” page to learn about the three disorders that make up NF, then visit the NF1, NF2, or schwannomatosis page to learn about the distinct condition that affects you or your loved one.
- Enroll in the NF Registry – this critical tool in the fight against NF will help make you aware of clinical trials or other research studies for which you or your loved one may be a candidate. Adults can enroll themselves and parents can enroll their children.
- Find a doctor that knows about NF – visit our Find a Doctor page to learn about NF Clinics in your area.
- Get support – the Foundation has chapters or affiliates in nearly all 50 states and a volunteer relations staff that can connect you with local families and local events. Visit the support page here and get involved in your community.
- Stay in touch – register for the CTF website on the top right corner of this page, ‘Like’ our Facebook page and follow us on Twitter for all the latest news and info about NF and CTF.
- Join our advocacy outreach – visit www.ctf.org/advocacy for information and updates on federal funding for NF research and how you can support the effort.
- Get involved – national programs like the NF Walk, NF Endurance, Racing4Research, and Cupid’s Undie Run, are a great way to empower yourself and your community in the fight for treatments and a cure to NF.
- Ask – always feel free to contact the Foundation at email@example.com or 212.344.6633 to learn more about NF, management options, information resources, and support and fundraising opportunities. We’re here to support you and your loved ones.
Sarah Jordan, a family physician, and Suresh Nagappan, a pediatrician, live in Greensboro, N.C. with their two children, Sally and William. In 2007, their daughter was diagnosed with NF1 when she was one month old.
In 2010, impressed by the Children's Tumor Foundation's nationwide work to rally awareness and support for neurofibromatosis, they organized an NF Walk in Greensboro with a lot of help from families in the area. More than 300 people participated and the event raised over $30,000 for NF research.
Another Greensboro, NC NF Walk is planned for May 11, 2013. This time, with the addition of a 5K Run as well.
It has been incredibly rewarding for Sarah and Suresh to see the outpouring of community support in both dollars and time over the past three years. And Sally, now 5, can’t wait for Walk day!
I'd like to nominate my son, Tyler Clendenen, as an NF Hero. When Tyler was born, we knew something wasn't quite right. He went straight to the Neonatal Intensive Care Unit (NICU) after birth, initially for respiratory distress only to find out later that the situation was much more serious. During a CT scan they found a tumor behind his left eye. He spent nine days in the NICU while doctors tried to discern what type of tumor Tyler had. We left the hospital without answers but knowing his life was not in immediate danger.
When he was around three months old, Tyler had a biopsy. Shortly thereafter, a doctor from Stanford University looked at Tyler's scans and identified the tumor as a plexiform neurofibroma. Our young man has a condition called neurofibromatosis type 1 or NF1. This was a new condition to our family as neither my husband nor I have NF.
Soon we began seeing many different types of specialists such asophthalmologists, ocular plastic surgeons, craniofacial surgeons, oncologists, and geneticists. Tyler had a debulking surgery when he was around nine months old as our first attempt at decreasing the size of this mass.
The tumor grew back quickly and it was decided that he should not have surgery again until he was four or five. In the meantime, the tumor was growing at an aggressive rate and there was nothing we could do until he turned three, at which point we could try a drug called Gleevac. It is typically used for leukemia patients but has had approximately a 40% success rate in stopping the growth of plexiform tumors. Tyler stayed on Gleevac for around 14 months, to no avail. The tumor was still growing at an alarming rate.
Our choices were to try another drug, PEG-Intron, and/or explore surgical options. We chose the surgical route and visited Dr. McKay McKinnon in Chicago, with the possibility of trying the PEG-Intron after Tyler recovered from surgery.
Dr. McKinnon said Tyler's MRI showed that what we originally believed to be a single tumor on the fifth cranial nerve is in fact three separate tumors growing off each of the three sensory branches of the fifth cranial nerve. The location of the tumors on each of those nerves is affecting the following areas for Tyler: lower jaw, upper jaw, cheek, head/temple, eye (entire orbit), nose, carotid artery, optic nerve, and parotid gland.
Some of the possible risks of this surgery are severe blood loss, loss of sensation in the lips and cheeks, and damage to the eye sphincter muscle. Some of the risks for Tyler if we do not remove what tumor can possibly be removed: bone deterioration, lengthening and/or destruction of the maxilla and mandible, further deformation of the orbit, further lengthening of the optic nerve as well as loss of vision and damage to the eye sphincter muscle, and loss of hearing.
We decided to go ahead with the surgery and Tyler was operated on in December 2012 at Lurie Children's Hospital of Chicago. Dr. McKinnon's first priority was to follow each tumor to its original origin, cauterize it, and remove it. Dr. McKinnon said that the tumor is “intimately” close to the carotid artery, so he did not go near any of the tumor in that area or the optic nerve. Since this was such an extensive procedure, described as pulling cotton balls out of a briar bush, the surgery lasted about six hours. Dr. McKinnon’s second priority was to correct the bone structure in Tyler's left eye socket.
>The doctor was able to remove the tumor in Tyler’s cheek, maxillary, and parotid gland, and did a 360 degree removal of the mass around his eye. Dr. McKinnon did not have to do any bone grafting but instead moved Tyler’s left cheekbone and jaw up and placed a permanent plate below his eye. This narrowed his left orbit which was too large. Also, Tyler’s levator tendon on his left eye was partially deteriorated and stretched, so Dr. McKinnon cut and reattached the tendon.
The doctor feels that he fell short on how much tumor volume could have been removed from the orbit. The tumor is inside the critical cone of the eye and is involved with the swelling of his eye muscles, which is not typically seen. He described this by telling us to envision the marbling of fat in a steak. This will be an ongoing hurdle as Dr. McKinnon believes the masses around this eye orbit are not isolated tumors. He plans to confer with the Chief of Ophthalmology at Lurie Children’s Hospital, Dr. Marilyn Mets, to see if she feels she can shave off some of the volume.
Over the next two months Tyler will be visiting our local ophthalmologist as well as having an MRI. Then, near the end of March, we will go back to see Dr. McKinnon, and hopefully Dr. Mets, to determine the next step
We still have a long road ahead of us, but through everything Tyler remains a normal, happy, full-of-life child. Everyone he meets becomes his best friend and immediately gets invited to his house to play with his toys. He is truly an inspiration to his family, friends, and community.
You can follow Tyler at either https://www.facebook.com/KeepingUpWithTylerC, or www.everybodyshero.com.
-- Michelle Clendenen, Tyler’s mother