PRINTER'S NO. 3519
THE GENERAL ASSEMBLY OF PENNSYLVANIA
HOUSE RESOLUTION
No.
915
Session of
2018
INTRODUCED BY WARREN, TOEPEL, READSHAW, CORBIN, HENNESSEY, WARD,
DUSH, SONNEY, NEILSON, MURT, CHARLTON, RYAN, LONGIETTI,
KINSEY, HILL-EVANS, McCLINTON, DiGIROLAMO, TOOHIL, KORTZ,
CALTAGIRONE, MARSICO, DRISCOLL, BRADFORD, D. COSTA, BIZZARRO,
GILLEN AND M. QUINN, MAY 17, 2018
INTRODUCED AS NONCONTROVERSIAL RESOLUTION UNDER RULE 35,
MAY 17, 2018
A RESOLUTION
Designating June 4, 2018, as "Batten Disease Awareness Day" in
Pennsylvania.
WHEREAS, Batten disease is a fatal, inherited disorder of the
nervous system which is linked to a buildup of lipopigments in
the body's tissues; and
WHEREAS, Early symptoms of Batten disease tend to appear
between 5 and 10 years of age, including vision problems,
seizures, personality and behavioral changes, educational
difficulties, clumsiness and trouble ambulating; and
WHEREAS, Over time, the health of children affected by Batten
disease continues to deteriorate, which is fatal to patients by
their late teens or early twenties; and
WHEREAS, Batten disease is the most common form of a group of
disorders called the neuronal ceroid lipofuscinoses (NCL); and
WHEREAS, Batten disease and other forms of NCL are estimated
to occur in 2 to 4 out of every 100,000 live births in the
1
2
3
4
5
6
7
8
9
10
11
12
13
14
15
16
United States; and
WHEREAS, Batten disease occurs when a child inherits two
copies of a defective gene, one from each parent; and
WHEREAS, Approximately 440,000 people in the United States do
not show any symptoms of Batten disease but carry disease-
causing mutations in their genome; and
WHEREAS, Individuals with Batten disease can be identified
through diagnostic tests, including blood tests, urine tests,
brain scans, DNA analysis, measurement of enzyme activity,
electroretinograms, electroencephalograms and the prevalence of
fluorescent deposits throughout the body; and
WHEREAS, There is no known cure or treatment to stop or
reverse the symptoms of Batten disease; and
WHEREAS, However, certain symptoms such as seizures can
sometimes be managed as they occur; and
WHEREAS, Special services, including remedial education,
special social support and other medical, social or vocational
services may be effective in managing Batten disease; and
WHEREAS, An early diagnosis of Batten disease and prompt
intervention through treatment and therapy are imperative to
ensure that children with Batten disease can reach their fullest
potential; and
WHEREAS, Due to its rarity, Batten disease can be difficult
to diagnose correctly, which often leads to additional stress
and financial hardship for families dealing with the disease;
and
WHEREAS, Support and encouragement are crucial for helping
patients and families cope with the profound physical and mental
difficulties caused by Batten disease; and
WHEREAS, It is important to acknowledge the difficulties that
20180HR0915PN3519 - 2 -
1
2
3
4
5
6
7
8
9
10
11
12
13
14
15
16
17
18
19
20
21
22
23
24
25
26
27
28
29
30
children with Batten disease and their families face in
diagnosing and managing the disorder; therefore be it
RESOLVED, That the House of Representatives designate June 4,
2018, as "Batten Disease Awareness Day" in Pennsylvania.
20180HR0915PN3519 - 3 -
1
2
3
4