The first sign that something was threatening Daniel Knodel’s
health appeared when he was 16 years old: doctors discovered that
huge tumors on each side of his brain stem had infiltrated his
auditory nerves. “We just collapsed,” said his mother, Barbara.
Surgeons removed the tumors, but Knodel became completely deaf.
It was the beginning of a journey now nearly 20 years long; in
that time, more than 250 tumors came to grow in Knodel’s body, some
as small as a few millimeters, others as large as 6 centimeters,
all caused by a rare genetic mutation that allows tumors to form
anywhere in the body where there is a nerve. More than once,
Barbara Knodel said, they found that most doctors had no
familiarity with a disorder as rare as NF2. In one instance,
without access to such specialized care, her son came close to
death. “We almost lost him,” she said. “He was way too complex.
There are few doctors who can deal with the complexity of the
disease.”
Steven Chang, MD, proved to be one
of those few. Chang, a neurosurgeon at Stanford Hospital &
Clinics, felt a strong sense of responsibility and empathy for
patients like Knodel. This month, he and nearly a dozen Stanford
physicians and clinicians opened the doors to a clinical
neurogenetics oncology program designed to treat these most
difficult of patients. It is among a select group of programs
worldwide that offer the expertise and experience the Knodels and
others with similar needs have long looked for.
“Imagine how much turmoil and difficulty these patients go
through,” he said. Their disorders attack many organs, causing a
long list of issues whose treatment requires drawing expertise from
a number of fields.
“Patients have to go here for this issue and there for that
problem, trying to get care,” he added.
Nor is the removal of one tumor the end of care, as it is with
many of Chang’s patients “where you can fix a ruptured aneurysm and
the patient goes home,” he said. “These patients have recurring
issues.”
Stanford’s program is a team that includes 12 specialists in
several fields — neurosurgery, epilepsy, neuro-ophthalmology,
neuro-oncology, neuro-otology, neuro-interventional radiology,
urology and general surgery. The goal, Chang said, is to give
cutting-edge care for conditions like Knodel’s, neurofibromatosis 2
(NF2) and others including another form of neurofibromatosis,
schwannomatosis tuberous sclerosis, von Hippel-Lindau disease,
Sturge Weber syndrome and hemorrhagic telangiectasia.
Care is coordinated to ensure that each patient’s visit includes
all the necessary exams and procedures to address the multiple
medical issues characteristic of these disorders, Chang said. There
are so few programs in the United States that many patients must
travel long distances for such care.
These disorders are rare. NF2 appears in just one in 40,000; von
Hippel-Lindau one in 36,000. Neurofibromatosis 1 is more common:
one in 3,000. Without cure, however, living with these systemic
disorders requires the most sophisticated care. Knowing what to
watch and how to watch for it is part of the skill needed over
years of treatment. Knodel’s condition changes, and with each
change comes a new challenge. He has already had more than 30
surgeries. There is no cure for NF2, as are there none for a family
of similarly disabling neurogenetic disorders that repeatedly
assault the brain, spine and central and peripheral nervous system
with tumors. In some of these disorders, the growths are internal;
in others the uncontrolled growths are external, often large and
distorted. The tumors can, in certain of these disorders, be
cancerous. Patients often endure pain, weakness, numbness and loss
of basic body functions. Some may develop a facial paralysis that
prevents them from smiling.
The question in treatment, Chang said, “is what will improve
quality of life and what will not? These patients may have many
surgeries, so it’s important to be as minimalistic as
possible.”
Stanford will offer patients ongoing surveillance and the chance
to build good working relationships with nurses, nurse coordinators
and physicians. The program has built a system to coordinate all
treatments and check-ups. “We’re trying to make it as easy as
possible for our patients,” Chang said. “We want to take the burden
off them.”
That concern includes making sure that at each visit, an
American Sign Language interpreter is present to help patients who
are deaf, like Knodel. He has also noticed that his Stanford
physicians jump quickly to a computer to talk with him by typing,
during an appointment or when he’s at home in Southern California.
“Stanford has surpassed my expectation with providing
accommodations for communicating,” Knodel said. “I’ve been to many
hospitals, and Stanford is one of the few who does this. It’s a
willingness to do things different for the well-being of the person
they’re caring for.”
The program’s multidisciplinary team incorporates collaboration
between specialists, essential for patients with disorders that
have multiple effects on body systems.
“Increasingly in neuroscience we realize we should do things in
a multi-disciplinary way,” said Robert Fisher, MD, a Stanford
epilepsy expert, “so we can address all aspects of care. It is a
great idea for the new Neurogenetic Oncology program to form itself
as a multidisciplinary structure from inception. That’s the best
way to get totality of care.”
When her son was first diagnosed with NF2, Barbara Knodel was
told to make every day count, that he might only live to be 20.
He’s proven them wrong. He’s about to celebrate his 35th birthday.
He has also earned a bachelor’s degree from the University of
Southern California and a master’s in clinical psychology from
CSU-Northridge. As an intern psychotherapist, he started a program
to provide counseling for the deaf.
But NF2 remains a difficult obstacle. “Parents sometimes give up
because it’s so overwhelming,” said Barbara Knodel. The new
Stanford program has given her hope. “It is a rare find,” she said.
“It’s an answer to many prayers.”
About Stanford Hospital & Clinics
Stanford Hospital & Clinics is known worldwide for advanced
treatment of complex disorders in areas such as cardiovascular
care, cancer treatment, neurosciences, surgery, and organ
transplants. It is currently ranked No. 17 on the U.S. News &
World Report’s "America's Best Hospitals" list and No. 1 in the San
Jose Metropolitan area. Stanford Hospital & Clinics is
internationally recognized for translating medical breakthroughs
into the care of patients. The Stanford University Medical Center
is comprised of three world renowned institutions: Stanford
Hospital & Clinics, the Stanford University School of Medicine,
the oldest medical school in the Western United States, and Lucile
Packard Children's Hospital, an adjacent pediatric and obstetric
teaching hospital providing general acute and tertiary care. For
more information, visit http://stanfordhospital.org/.