BILL NUMBER: S7857
SPONSOR: MAYER
 
TITLE OF BILL:
An act to amend the public health law, in relation to the establishment
of a program for familial dysautonomia, Canavan's disease and Tay-Sachs
disease screening and counseling
 
PURPOSE OR GENERAL IDEA OF BILL:
This bill would require the Department of Health Commissioner to estab-
lish a screening and counseling program for familial dysautonomia, Cana-
van disease and Tay-Sachs Disease.
 
SUMMARY OF SPECIFIC PROVISIONS:
Section one of the bill adds § 206-c to the public health law to require
the commissioner of the department of health, with amounts appropriated
therefore, to establish a program for familial dysautonomia, Canavan
disease, and Tay- Sachs disease screening and counseling. Blood rela-
tives of known disease victims and members of high-risk population
groups for these diseases, especially those who are in or entering their
childbearing years, shall be given priority for screening and services
established by this act. Operation of the program shall be permitted to
be administered by not-for-profit organizations with grants issued by
the department. Participation in any aspect of this program is to be
entirely voluntary. Additionally, the section provides for privacy and
confidentiality protections and exceptions related to the program and
includes reporting requirements.
Section two of the bill adds paragraph (t) to § 207 of the public health
to include Familial dysautonomia, Canavan disease and Tay-Sachs disease
education, screening, and counseling to the list of health care and
wellness education and outreach programs that the department may
conduct.
Section three of the bill provides the effective date.
 
JUSTIFICATION:
Familial dysautonomia (FD), Canavan disease, and Tay-Sachs disease are
serious, and oftentimes fatal inherited diseases. Those diagnosed with
Tay-Sachs disease rarely live past the age of five (ten years with a
Canavan disease diagnosis). While FD is not necessarily fatal anymore
due to advances in medicine, medical care is often very involved and
costly. At present, none of these diseases can be cured, but all have
effective carrier screening tests that hopeful parents can take. An
infant can only be afflicted with these diseases if both parents are
carriers and pass along those genes. With the knowledge a test result
can bring, hopeful parents can more comfortably enter their family plan-
ning stage. These three diseases occur most often in children of Jewish
extraction and of Eastern European origin. One in every thirty Ashkenazi
Jews (those of Eastern European descent) is a carrier of the Tay-Sachs
gene. Couples who are both carriers have a 25% chance of having a Tay-
Sachs child. The estimated cost of caring for a Tay-Sachs child during
their short life is estimated to be $50,000 to $150,000, while the
emotional cost to the family of a Tay-Sachs child is immeasurable. Fami-
lial dysautonomia stymies the development of autonomic and sensory nerv-
ous systems which regulate the function of internal organs and transmit
information from the body to the brain, allowing people to sense things
such as pain, temperature, and changes in body position or muscle
tension. It is estimated that 1 out of every 30 Ashkenazi Jews is a
carrier of FD, and roughly 1 in every 3,600 children of Ashkenazi fami-
lies is born with the disorder. Canavan disease is the most common
degenerative cerebral disease of infancy and rapidly damages nerve cell
growth in the brain. It is estimated that 1 in 40 Ashkenazi Jews are
carriers of Canavan disease.
Voluntary, confidential screening and counseling would be available for
expecting parents and those looking to become parents under this
program. Future parents deserve to know if there is a chance their child
will die shortly after birth from a detectable disease. Preparing for a
family is always challenging and this bill would help to provide more
information regarding these diseases for those who need it.
 
LEGISLATIVE HISTORY:
2023-24: A.1813 - Health
2021-22: A.2303 - Health
2019-20: A.2881-A - Health
2017-18: A.2461 - Reported to Ways and Means -
2015-16: A.126 - Reported to Ways and Means
2013-14- A.314 - Held for Consideration in Ways and Means / S.190 Health
2011-12: A.412 - Reported to Ways and Means/S.738- Health
2009-10: A.1135 / S.7976 - Vetoed Memo 6741
2007-08: A.3907 - Passed Assembly
2005-06: A.2461-A - Passed Assembly/S.5660-A - Referred to Health
2003-04: A.9262 - Passed Assembly
 
FISCAL IMPLICATIONS:
Undetermined.
 
EFFECTIVE DATE:
This act shall take effect on the one hundred eightieth day after it
shall have become a law. Effective immediately, the commissioner of
health: shall make regulations and take other reasonably necessary
actions to implement this act on that date.

Statutes affected:
S7857: 207 public health law, 207(1) public health law