About Me
My Birthday
September 2nd, 2003
My Family
Dad: Pete
Mom: Mireille
Brothers: Peter (13) and Alex (9)
Diagnosis: Embryonal Rhabdomyosarcoma Stage Two/ Group Three
Located in Skull Base Treating Hospital: Memorial Sloan Kettering, New York, New York.
We created this website to allow our friends and family to follow Emily’s progress in her
fight against Embryonal Rhabdomyosarcoma (ERMS). On August 29, 2007, Emily was
diagnosed with ERMS, a rare pediatric cancer of skeletal muscle tissue. Although its
cause is unknown, ERMS occurs when an embryonic muscle cell does not properly
develop into an adult muscle cell. All children are born with embryonic muscle cells that
develop into adult muscle cells as the child matures. In this random occurrence, one of
Emily’s embryonic muscle cells did not properly convert itself into an adult muscle cell
and instead became an abnormal cell which grew into a tumor. ERMS is most common
in children between the ages of one and five years old because this is the timeframe in
which embryonal muscle cells convert. Emily’s symptoms arose on August 14 th , two
weeks before her fourth birthday and just one week after our family returned from an
amazing two week vacation in Southern California.
Emily had a truly fantastic time in California. She seemed to be her energetic, high
spirited self, buzzing from one activity to the next. She enjoyed a fantasy lunch with all
of the Disney princesses, spent days splashing in the ocean and playing in the sand, and
even went hiking at Big Bear Mountain. However, just one week later, on the evening of
August 14 th , Emily’s right eye began to appear crossed. More specifically, her right eye
no longer would track to the right of center, a condition known as a 6 th nerve palsy. After
seeing Emily in his office on August 15 th , her pediatrician immediately sent us to the
hospital for an MRI. We responded very quickly to this because Emily had experienced
severe right ear and head pain last February. A March 1, 2007 CT scan was normal, but
we never received a good explanation for those earlier painful episodes. Still, Emily did
have a sinus infection at the time and after receiving antibiotics had not complained
further for over six months. The August 15 th MRI revealed a 2 cm mass on the right
temporal bone (right petrous apex) of her skull base.
Emily underwent surgery to resect and biopsy this tumor on August 20, 2007 at
Beaumont Hospital in Royal Oak, Michigan. Unfortunately, the tumor’s location on the
right petrous bone, deep in her head, made for an incredibly difficult surgery. The tumor
is actually located deep inside Emily’s skull base, near the brain stem, on the boney shelf
that the brain sits upon. Her neurosurgeon,
Dr. Zakalik, actually had to gently move the
right temporal lobe of her brain out of the way, so he could look down into a partially
empty skull to visualize the tumor on the skull base. After seven hours, Dr. Zakalik
emerged to report that while Emily had made it through the surgery well, he had only
been able to remove about 1/3 of the tumor. The remainder of it was impossible to
remove as it was very vascular and was wrapped around her carotid artery. He then told
us the unthinkable that
the tumor was a sarcoma. And, our world stood still. Numb, we
went up to the intensive care unit to see our little girl for the first time. Our fears were
put to rest, however, when we found her alert, wanting a drink and demanding that we get
out of the way of the television so she could watch The Little Mermaid. That’s our
spunky, sassy girl! That’s the spirit that will get her through this.
Emily recovered from surgery with amazing speed and was running around at home in
just four days. In fact, we had to stop her from doing gymnastics several times. Emily
remained an incredibly strong child, both physically and in spirit. In the meantime, nine
long, anxious days passed slowly as we waited for the final pathology report. The call
finally came on August 29 th from the oncologist at Beaumont. The diagnosis of
embryonal rhabdomyosarcoma was definitive and had been confirmed by two outside
institutions. The Beaumont oncologists proposed standard treatment for Emily which
consisted of 42 weeks of VAC chemotherapy plus radiation. VAC stands for three
chemo drugs: Vincristine, Actinomycin and Cytoxan.
Then, on August 31 st , Emily underwent a bone marrow biopsy and spinal tap to make
sure that the tumor had not spread to these areas. A complete bone scan on the 16 th had
revealed no additional hot spots in other bones. Both the marrow and spinal fluid were
negative – thank God. So, this allowed the doctors to arrive at an opinion on staging.
Children with embryonal rhabdomyosarcoma are place into one of three risk categories:
low risk, intermediate risk and high risk. The risk category basically determines a child’s
chance for a cure. Emily is presently in the intermediate risk category. A child’s risk
threshold is a function of two things: (1) the stage of the cancer; and (2) the group of the
cancer. The clinical group is based on the extent of disease and completeness of initial
surgical resection. Emily fell into group three. Children in group three have tumors that
cannot be completely removed, leaving some tumor behind that can be seen with the
naked eye. They have no evidence of spread. This accounts for about half of patients with
rhabdomyosarcoma. This clinical group is then complemented by the stage, which does
not depend on the results of surgery, but on the type and size of the tumor, its invasion of
the lymph nodes and distant organs, and where the tumor originates. Emily fell into
stage two which is defined as follows: tumor arising in parameningeal area and smaller
than 5 cm (about 2 inches) with no spread to nearby lymph nodes or distant sites. Given
the staging, Emily’s chance for a cure is 50%70%.
After extensive research, we decided to consult the pediatric sarcoma team at Memorial
Sloan Kettering Hospital (MSKH) in New York City. Dr. Wexler at MSKH is a national
expert on rhabdomyosarcoma and his team treats more rhabdo than any other institution
in the world. Two days before our September 10 th appointment, the tumor began to
impact Emily’s seventh facial nerve, paralyzing the right side of her face. When we
arrived at Dr. Wexler’s office on Monday, September 10 th , Emily had clearly
deteriorated. Not only was her face drooping, but she had also stopped eating and
drinking. Yet, even as this little angel slowed down, she remained strong and never
complained.
We were tremendously impressed by Dr. Wexler and his team. He presented us with an
option to participate in a phase two clinical study which offers Emily her best chance for
a cure. This aggressive chemotherapy treatment plan utilizes VAC, albeit at higher
doses, plus four additional chemotherapy drugs. The four additional drugs (Carboplatin,
Irinoecan, Etoposide and Ifosfamide) have been used in the past to treat sarcomas in
adults and recurrent rhabdo in children. The study also substitutes Adriamycin for the
standard VAC “A” drug. Dr. Wexler explained to us that if Emily were only to receive
standard VAC treatment and then relapse, that her only good chance for survival would
be surgery. And, that given the inoperable location of her tumor, this would not be an
option. Accordingly, we decided that we need to treat this aggressive beast as
aggressively as possible at the outset. In our hearts, we know that Emily is in the best
place in the world for her treatment. She is a lucky girl.
Emily had a baseline MRI on September 12 at Sloan. This scan showed that the tumor
had grown back and increased in size. This explains the facial paralysis. However, the
good news is that after three days of chemo, Emily woke up in the morning with her
facial movements partially restored. A full week of chemo and her face was completely
better! Emily’s first round of chemo lasted ten straight days – from September 12 th to the
21 st . All in all, she tolerated the chemo well. She was scheduled to begin cycle two of
the chemo plan on October 1 st , but this has been delayed for one week due to low blood
counts and fever. She remains strong and happy.
On days that she feels well, we take advantage of all that New York City has to offer.
We are enjoying the museums, zoos and Central Park, as well as, many activities that are
organized by the Ronald McDonald House and their sponsors. We have met lots of new
friends here who are fighting rhabdomyosarcoma and various other childhood cancers.
The resilience of the children, the strength of their parents and the spirit of comradery
here is magnificent. We are also fortunate to have the tremendous support of our
wonderful New York and New Jersey cousins. We are in a very good, peaceful place to
heal. Please keep praying for Emily and all children and families affected by cancer.
The Phillips Family – Pete, Mireille, Peter, Alex and Emily
October 3, 2007 |
