My Photo

NF Inc., Northeast


Our beautiful 15 year old daughter Leah, was diagnosed in July, 2007 with Neurofibromitosis-Type 2 (NF2). NF2 is a rare disorder where the child is lacking a protein that suppresses tumor growth. Without this protein, children like Leah develop benign tumors that grow in the brain, spinal cord and throughout the body. These tumors can grow large enough to cause neurological problems like deafness, blindness and seizure activity depending on their size and location. Sometimes the tumors can be surgically removed, but at great risk to the child.

She was 7 years old and had a skin lesion or "bump" removed from her chest and scalp for purely aesthetic reasons, at the recommendation of her pediatrician. A routine pathological screen raised some concerns and the tissue was sent to Boston Children's Hospital for confirmation. Leah had a spontaneous occurrence of NF2, a random mutation during cellular division resulting in the lack of a protein that suppresses tumor growth in nerve cells. Surgery is the only current treatment option for Leah and others suffering from NF2.

In December of 2008, Leah underwent her first operation to remove a spinal tumor at C2-3 and lost sensation in her left shoulder. In August 2009, Leah returned to the operating room to undergo an anterior and posterior cervical spinal fusion, as the first surgery compromised the stability of her neck. In March 2011, moderate hearing loss in her right ear lead to exploratory surgery of Leah's middle ear during which a large NF2 tumor is discovered and subsequently removed. Complications and tumor location impacted the facial nerve and resulted in facial paralysis. Leah lost her ability to smile and fully close her right eye. She was 11 years old.

Just two months later in May 2011, Leah headed to Boston where Dr. Hadlock took the first step in bringing back Leah’s smile. A nerve was transplanted from Leah’s left calf to her face to replace the previously damaged facial nerve. A year of healing then needed to take place before step two, facial re-animation, could even be considered.

In October 2011, a spinal tumor at C3-5 is removed from Leah’s neck, which was in very close proximity to the location of her first spinal surgery in 2008.

Most recently, Leah underwent the next step in regaining her smile. She traveled back to Boston where Dr. Hadlock transplanted muscle tissue from Leah’s thigh to her right cheek. The muscle was then connected to the previously transplanted nerve in hopes of providing the required “energy” and “lift” to bring back Leah’s smile. It’s just after the New Year’s now 2013 and Leah was recently back in Boston for a post-op check up with Dr. Hadlock. Although the surgery was not without complications, Leah’s face is healing well. It will be mid summer before the swelling is completely gone and the new nerve and muscle combination have a chance to “get to know each other”, but all signs are pointing to an improvement in facial symmetry and possible re-animation.

NF2 brings many unknowns; where is the next tumor growing? Are the current tumors holding steady or are they growing? Are they in a dangerous place or a “good place”? Yes…when in tumor fighting mode, there is such a thing as a “good place”…

Thank you for visiting our blog and learning more about NF2 and Leah's story.

John and Linda Manth