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Shortly after her birth on November
1, 2001 at Rainbow Babies and Children's Hospital in Cleveland,
Ohio, Sydney's
neonatologists noticed lesions and nodules on her
skin and found that her liver and spleen were enlarged. Sydney
underwent several diagnostic tests that day, including skin biopsies, blood
work and ultrasounds, These tests resulted in working diagnosis of Langerhans Cell Histiocytosis
(LCH) being reached on November 2. We were told that LCH is a rare histiocyte disorder involving abnormal accumulations of
certain white blood cells, known as histiocytes,
in the form of lesions or tumors throughout the body. However, her doctors
still had not ruled out infection as a potential cause of the skin lesions
and other problems. Given the
uncertainty about her condition, Sydney’s parents, family and friends
were required to wear sterile gloves while holding her.
After several days on the post-partum floor, we were transferred to the
pediatric hematology/oncology floor for additional evaluation and testing
by doctors from numerous specialties, including dermatology, neonatology,
pediatric opthamology, pediatric infectious
disease and pediatric hematology/oncology. During the next several days
between November 5 and 7, Sydney
underwent a bone marrow biopsy, several skin biopsies, a series of blood
tests, and CT scans and MRI scans of her head and chest. Throughout the
invasive tests and procedures that she was forced to endure, Sydney
remained strong and persevered with courage, dignity, and grace well beyond
her years.
The biopsies indicated that Sydney
was not suffering from LCH, but instead was afflicted with a different and
even rarer histiocyte disorder -- Juvenile Xanthogranuloma (JXG). The CT scans and MRI scans
revealed that the lesions were not only on her skin, but had invaded her
liver, lungs, spleen, and potentially her brain. Even with these findings,
we were told that JXG was "a benign, self-limiting disease." Her
doctors hoped that the lesions would slowly regress over the next six to
eight years without need for treatment, unless Sydney
began to experience organ dysfunction or other serious complications. The
treatment plan would involve weekly visits with her oncologist for
examinations and blood work to assess her physical condition, monthly CT
and MRI scans to assess the status of the internal lesions, and semi-annual
eye exams along with regular visits to her pediatrician.
After a week in the hospital, we were discharged on November 7 with our
precious daughter and we remained at home for the next 11 days. During this
time period, we took Sydney to
the pediatric hematology/oncology clinic for two difficult visits that
involved numerous blood tests. After some poor blood test results and an
emergency room visit on the day of her second clinic visit, we were
becoming very concerned about her condition and whether we would need to
begin some form of treatment. We were advised that Sydney
would need to see pediatric liver specialist within the next week to assess
her worsening liver function and to determine if a treatment plan needed to
be implemented. However, during the next several days, we noticed that Sydney's
stomach had become very distended, that her appetite was decreasing, and
that she had become more jaundiced.
When we took Sydney back to the clinic for her next visit on November
19, her blood counts and her liver function had declined so significantly
that she had to be admitted to the hospital for closer observation. Later
that afternoon, Sydney underwent an ultrasound exam which revealed that her
liver had been severely damaged due to lesions that occluded her hepatic
vein and prevented normal blood flow into her liver. Sydney was immediately
admitted into the Neonatal Intensive Care Unit, where she spent the
remaining 11 days of her life. CT scans and an MRI demonstrated that the
number and extent of internal lesions had significantly increased and were
causing her liver and spleen to become enlarged.
Her doctors immediately started Sydney on high-dose steroids in hopes of
reducing the swelling that the lesions had caused in her liver and spleen.
We met with Sydney's doctors on the morning of November 20 and were told
that chemotherapy provided the only chance for reducing the lesions and
preserving some liver function. Sydney received her first course of
chemotherapy that afternoon while resting comfortably in her father's arms.
She underwent a second course of chemotherapy several days later.
Unfortunately, her body did not respond to the chemo. Meanwhile, her
white blood cell and platelet counts were dropping,
her metabolic system was becoming unstable, her liver function worsening
and her fluid retention increasing. Fluid was drained from Sydney's
abdominal cavity on three different occasions in an effort to ease the
pressure on her lungs and to permit her to breathe with less effort.
However, with her liver and kidneys now failing, Sydney's
body was not able to prevent the fluid from reaccumulating
more quickly -- and in increasing quantities -- as each day passed.
After watching our brave warrior struggle to breathe, we decided to
place Sydney on a ventilator on November 27 so that she could rest and
regain her strength. By this time, Sydney was not healthy or stable enough
to undergo another round of chemotherapy. She had very few white blood
cells and required frequent infusions of platelets and packed red blood
cells to replace what chemo had destroyed.
We lost our dear Sydney less than 48 hours after she went on the
ventilator. Sydney put up a tremendous fight. She was a brave warrior. She
taught her parents and her family more in 28 days than any of us have
learned in our whole lifetimes thus far. Although we know how much we love
our little angel, there is so much that we do not know about this disease
or why it took Sydney so quickly. JXG was not "supposed" to act
like it did or to progress as rapidly as it did.
Sydney's autopsy results are coming back slowly. The gross autopsy findings
have shown that the lesions were not only on her lungs, liver and spleen,
but also in her diaphragm, esophagus, and pericardial sac. Her doctors also
suspect that the pinpoint findings on her head MRI's
were intracranial lesions associated with JXG. Based upon the extent of her
disease, we have been told that it was a miracle that Sydney was ever able
to come home or that she survived as long as she did against such
obstacles.
We still have a lot of questions. We will be talking with doctors as
more pathology and autopsy results become available, and hope to gain more
information or at least better insight into this disease. This message
provides just a glimpse of what happened to our Sydney and cannot even
begin to show the impact of her life upon everyone who met or heard about
her.
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