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NF Northeast

  • NF Northeast
    The Mission of NF Northeast is to find a treatment and the cure for neurofibromatosis by promoting scientific research, creating awareness, and supporting those who are affected by NF.

In the News


Our beautiful 17 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.

December 2012, Leah underwent the second surgical step in regaining her smile, facial-reanimation surgery. She traveled back to Boston where Dr. Hadlock transplanted a muscle, nerve, vein and artery complex 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 some of Leah’s smile. While not a full smile, her new smile is Leah’s smile.

September 2013, Leah began Avastin treatments in hopes of halting the growing Vestibular Schwannoma (VS) tumors that put her in jeopardy of losing her hearing.

In February 2015, Leah had a lower eyelid tuck on her right eye to improve the ability of the lower and upper lids to fully close, retain more moisture and protect her eye more fully.

July 2015, Leah had the tumor in her throat reduced in size (de-bulked) by Dr. Rahbar at Boston Children’s Hospital to open up her airway and preserve her ability to swallow. A similar procedure is likely in summer 2017 to further de-bulk this tumor.

Early August 2016, Leah had a Cardiac Ablation at Golisano Children’s Hospital in Rochester, NY to remedy a re-occurring rapid heart rate that could not be corrected with medication alone.

Late August 2016, Leah traveled to the House Clinic at St. Vincent Hospital in Los Angeles for removal of her right-sided VS (hearing tumor).

On April 26, 2017 Leah underwent her 13th major surgery in 9 years as a result of Neurofibromatosis-Type 2 (NF2). She had her airway tumor de-bulked again as it continued to obstruct her airway. She travelled to Boston Children's Hospital where Dr. Rahbar was able to sufficiently de-bulk the tumor while staying clear of Leah's vocal cords. Another desired outcome of the surgery was also achieved; Leah received the fantastic news that she no longer needed to sleep with a CPAP machine!
You may recall in August 2016, Leah’s Auditory Brainstem Implant (ABI) was surgically placed while in Los Angeles. She returned to LA in Nov 2016 for the ABI to be turned on for the first time. After another trip to LA for fine-tuning, she switched her follow up care to the ABI clinic at Massachusetts Eye and Ear Institute in Boston, as it is closer to Buffalo and in the same time zone! The Manths have been back to Boston three times and can now move to an annual ABI tuning schedule. While not yet dependent on the ABI, the more Leah trains her brain to use the ABI while she can still hear, the better prepared her brain will be should she need another ABI placed on her left side in the future.

Leah continues to receive Avastin infusions. With the move of Buffalo’s Children’s Hospital to the medical corridor downtown, she now goes to Roswell Park for her infusion. It’s also been moved out to every 3 weeks, allowing Leah’s kidneys to recover in between treatments. Her doctors are looking at adding another drug to her regimen, as with a tumor condition like NF2 there are numerous types of tumors to combat, therefore a “cocktail” of medications is likely in the near future to keep the various tumors from growing.

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
[email protected]