LEGISLATIVE RESOLUTION memorializing Governor Andrew M. Cuomo to
proclaim October 2011 as Niemann-Pick Disease Awareness Month in the
State of New York

WHEREAS, It is the custom of this Legislative Body to recognize official
days that are set aside to increase awareness of serious issues that
affect the lives of citizens of New York State; and
WHEREAS, Attendant to such concern, and in full accord with its long-
standing traditions, it is the sense of this Legislative Body to memori-
alize Governor Andrew M. Cuomo to proclaim October 2011 as Niemann-Pick
Disease Awareness Month in the State of New York, in conjunction with
National Niemann-Pick Disease Awareness Month; and
WHEREAS, Niemann-Pick Disease is one of a group of lysosome storage
diseases that affect metabolism and that are caused by genetic muta-
tions; the three most commonly recognized forms are Niemann-Pick Types
A, B and C; and
WHEREAS, Niemann-Pick Types A and B (NPA and NPB), also called Acid
Sphingomyelinase Deficiency (ASMD), are caused by the deficiency of a
specific enzyme, acid sphingomyelinase (ASM); and
WHEREAS, ASM is found in special compartments within cells called
lysosomes and is required to metabolize a lipid called sphingomyelin; if
ASM is absent or not functioning properly, sphingomyelin cannot be meta-
bolized properly and is accumulated within the cell, eventually causing
cell death and the malfunction of major organ systems; and
WHEREAS, NPA and NPB are both caused by the same enzymatic deficiency
and there is a growing evidence that the two forms represent opposite
ends of a continuum; people with NPA generally have little or no ASM
production (less than 1% of normal) while those with NPB have approxi-
mately 10% of the normal level of ASM; and
WHEREAS, The clinical prognosis for NPA and NPB patients is very
different; NPA is a severe neurologic disease that leads to death by 2
to 4 years of age; in contrast, patients with NPB generally have little
or no neurologic involvement and may survive into late childhood or
adulthood; and
WHEREAS, NPB patients usually have enlarged livers and spleens, and
respiratory problems are common; the enlargement of organs and the
respiratory problems can cause cardiovascular stress and can lead to
heart disease later in life; and
WHEREAS, There are approximately 1,200 cases of NPA and NPB worldwide
with the majority being Type B or an intermediate form; and
WHEREAS, Niemann-Pick Type C (NPC) is very different than Type A or B
(ASMD); and
WHEREAS, NPC patients are not able to metabolize cholesterol and other
lipids properly within the cell; consequently, excessive amounts of
cholesterol accumulate within the liver and spleen and excessive amounts
of other lipids accumulate in the brain; NPC causes a secondary
reduction of ASM activity, which led all three types to be considered
forms of the same disease; and
WHEREAS, Type C Niemann-Pick Disease has about 500 cases diagnosed
worldwide; it is believed, however, that the number of people affected
by NPC is higher; and
WHEREAS, NPC is always fatal; the vast majority of children die before
age 20 and many die before the age of 10; late onset of symptoms can
lead to longer life spans but it is extremely rare for any person with
NPC to reach age 40; and

WHEREAS, Niemann-Pick Disease affects all segments of the population
with cases reported from North America, South America, Europe, Africa,
Asia, and Australia; and
WHEREAS, It is imperative that there be greater public awareness of
this serious health issue, and more must be done to increase activity at
the local, State and national levels to support the patients as well as
their families; now, therefore, be it
RESOLVED, That this Legislative Body pause in its deliberations to
memorialize Governor Andrew M. Cuomo to proclaim October 2011 as Niem-
ann-Pick Disease Awareness Month in the State of New York; and be it
further
RESOLVED, That a copy of this Resolution, suitably engrossed, be tran-
smitted to The Honorable Andrew M. Cuomo, Governor of the State of New
York.