Dear Friends of Leah:
It is the time of year when we give thanks, visit with family and friends, reflect back on the year that has been and look forward to what the future will bring.
What a year it has been!
We had another successful NF2 research fundraising campaign in 2016. Between our 9th year working with the Buffalo Marathon, all of your continued monetary support, donations from the NF2 Michigan group, and a donation from NF Northeast, we were able to fund a $60,000 research study on November 28, 2016 titled, "Examining mTORC1/mTORC2 and Ephrin Receptor Pathways in Vestibular Schwannomas." While the science is very technical, it extends the work from previous studies we have funded with this same NF2 dedicated researcher, Dr. Vijaya Ramesh and the lab located at the Center for Neurofibromatosis Research & Allied Disorders at Harvard Medical Center. This study takes another step toward trying to understand what makes the tumors on the hearing and balance nerves or Vestibular Schwannomas (VS) grow in all NF2 patients. If we can understand how they grow, we can use medications to block the pathways that allow or stimulate their growth. This in turn would stop the tumor’s growth and their devastating effects, which in NF2 patients, almost always leads to total or partial deafness. It is exciting to see all of our hard work over the past nine years start to produce actual results that can impact NF2 treatment options. We set out to find treatment options beyond life threatening surgeries and ultimately a cure.
Together you ARE helping us make a difference!
As most of you may know, Leah had one of her Vestibular Schwannoma (VS) type tumors removed in the late August in Los Angeles. (See previous blog posts in August for details) We all have vestibular nerves in each of our ears, right next to our cochlear nerve, or the nerve needed for hearing. The vestibular nerve also aids in hearing and is essential for balance as well as spacial and depth perception. The VS’ are tumors that arise from these nerves in NF2 patients. Leah's VS' were both growing to the point that her brainstem was developing pressure, so surgery became inevitable. To relieve the pressure on the brain, the VS on the right side was removed. While the pressure on her brainstem was relieved, it unfortunately resulted in total deafness in her right ear. During the surgery she had an Auditory Brainstem Implant (ABI) placed. The ABI transmits sound through a processor into Leah's brainstem, where the sound is interpreted by the brain. Leah does not hear actual words through the ABI, but instead sounds that help her brain, together with the hearing she still has in her left ear and lip reading, allow her to understand what is being said.
(see ABI graphic below)
Leah wears the external head piece (transmitter & processor) every day, "teaching" her brain to hear in a new way. Leah also continues to study American Sign language and Lip Reading to prepare her for the day when removal of the VS in her left ear, will likely leave her totally deaf. In an effort to continue to preserve Leah's hearing in her left ear, Leah also continues with her chemotherapy treatments (Avastin), every other Tuesday at Buffalo's Women's & Children's Hospital as her hearing in her left ear continues to be 100%, believed to be because of the tumor shrinking efficacy of the Avastin over the last 3 years.
An update on the rest of our growing family --
This year Erin, Leah's older sister, will spend her Spring semester in Morocco, immersing herself in the Arabic language to continue towards her degree and passion, Intelligence and National Security, at Mercyhurst University. Christopher, Leah's older brother, completed a semester at Niagara County Community College while he awaited his dates for training in the Air National Guard. He will leave at the end of January for basic training in Texas, then immediately move on to the fire training academy, also in Texas. When Christopher returns in July, he will work for 6 months active duty at the Niagara Falls Air Base for on-the-job training as a fireman.
Leah will be home alone with John and I, where she is captain of the indoor track team and getting ready for outdoor track and the next hunting season. As a high school Junior, Leah is enjoying her first year in the Culinary Program at the Niagara Orleans BOCES, but still thinks a career in medicine awaits her as she starts to decide what to do after HS graduation.
We wanted to thank you all for your continued thoughts, prayers and support -- we truly are blessed!
Happy New Year and may good health, happiness and peace be with you in 2017!!
Linda, Leah, John, Erin & Christopher Manth
The Manth Family