PRINTER'S NO. 1693
THE GENERAL ASSEMBLY OF PENNSYLVANIA
SENATE RESOLUTION
No.
335
Session of
2018
INTRODUCED BY BOSCOLA, BROWNE, SCAVELLO, FONTANA, DINNIMAN,
SABATINA, GREENLEAF, MARTIN, RAFFERTY AND COSTA,
APRIL 23, 2018
INTRODUCED AND ADOPTED, APRIL 23, 2018
A RESOLUTION
Designating April 23, 2018, as "Fibrodysplasia Ossificans
Progessiva Awareness Day" in Pennsylvania.
WHEREAS, Fibrodysplasia ossificans progressiva (FOP) is an
extremely rare connective tissue disease in which a mutation of
the body's repair mechanism causes soft connective tissue,
including muscle, tendon and ligament to be ossified
spontaneously or when damaged, causing joints to become
permanently frozen and producing a "second skeleton"; and
WHEREAS, FOP causes loss of mobility to the affected areas of
the body, including the inability to fully open the mouth,
limiting speech and eating; and
WHEREAS, Breathing complications can also occur when extra
bone formations around the rib cage restrict the expansion of a
person's lungs and diaphragm; and
WHEREAS, FOP is inherited in an autosomal dominant pattern,
which means one copy of the altered gene in each cell is
sufficient to cause the disorder, and most cases of FOP result
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from new mutations in the gene; and
WHEREAS, The symptoms of FOP usually appear in the first or
second decade of life, with the majority of patients diagnosed
by 10 years of age, depriving children of normal development;
and
WHEREAS, Most children born with FOP tend to have malformed
toes, which helps distinguish this disorder from other skeletal
problems, and a child with FOP typically develops extra-skeletal
bones at the neck and on the shoulders, arms, chest area, legs
and feet; and
WHEREAS, FOP is so rare, it is considered an orphan disease;
and
WHEREAS, Since the disease affects so few people, its
symptoms are often misdiagnosed as cancer or fibrosis; and
WHEREAS, Currently there are 285 confirmed cases of FOP in
the United States; and
WHEREAS, In 1991, the FOP Laboratory at the University of
Pennsylvania was founded by Eileen Shore, Ph.D., and Frederick
Kaplan, M.D., and, in 1997, the Center for Research in FOP and
Related Disorders was established by Dr. Kaplan and Dr. Shore;
and
WHEREAS, There is no cure or approved treatment for FOP; and
WHEREAS, Activities that increase the risk of falling or soft
tissue or joint injury should be avoided as even minor trauma or
surgical removal of extra bone growths may provoke additional
bone formation; and
WHEREAS, As a result of limited treatment options, the median
age of survival is 40 with proper management, but a delayed
diagnosis and surgical interventions can worsen FOP; and
WHEREAS, A number of pharmaceutical companies focused on rare
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disease are currently in varying stages of investigation into
different therapeutic approaches for FOP; and
WHEREAS, Raising public awareness about FOP on the
anniversary of the day the FOP gene was discovered in 2006 and
about the current research being conducted into its causes could
encourage the medical community's continued search for a cure
and development of treatment and prevention strategies for this
rare genetic disease and other musculoskeletal disorders
involving extra-skeletal bone formation; therefore be it
RESOLVED, That the Senate designate April 23, 2018, as
"Fibrodysplasia Ossificans Progessiva Awareness Day" in
Pennsylvania.
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