PRINTER'S NO. 838

THE GENERAL ASSEMBLY OF PENNSYLVANIA

SENATE RESOLUTION

No.

98

Session of

2015

INTRODUCED BY GREENLEAF, HAYWOOD, WOZNIAK, FONTANA, FARNESE,

DINNIMAN, PILEGGI, RAFFERTY, BREWSTER, SCAVELLO, SCHWANK,

AUMENT, SMITH, VULAKOVICH, MENSCH, TARTAGLIONE AND ALLOWAY,

metabolic disorder that is characterized by the inability of the

body to process the essential amino acid phenylalanine, or Phe,

which is found in all foods containing proteins; and

WHEREAS, PKU is caused by a deficiency of the liver-produced

enzyme phenylalanine hydroxylase (PAH); and

WHEREAS, When this enzyme is absent or deficient, Phe

accumulates in the blood and is toxic to brain tissue; and

WHEREAS, Symptoms of PKU can be mild or severe and, in a

child, may include delayed development, behavioral, emotional

and social problems, poor bone strength, skin rashes (eczema), a

WHEREAS, In 1934, PKU was discovered in Norway by Dr. Asbjörn

Fölling, one of Norway's first physicians to apply chemistry to

medicine; and

WHEREAS, In 1961, Dr. Robert Guthrie developed a laboratory

test to detect PKU in newborns; and

WHEREAS, In 1963, newborn screening for PKU was initiated in

the United States; and

WHEREAS, In 1965, the General Assembly enacted the Newborn

Child Testing Act, requiring newborn children to be screened for

six genetic or metabolic disorders, including PKU; and

screening is crucial in helping to prevent major health

problems; and

WHEREAS, When treatment is begun within the first few weeks

of life and adhered to, affected children can expect normal

development and a normal life span; and

WHEREAS, Although there is no cure for PKU, treatment

involving medical foods and medications and restriction of Phe

intake can prevent progressive, irreversible brain damage; and

WHEREAS, Access to health insurance coverage for medical food

varies across the United States, and the long-term costs

Foods Insurance Coverage Act, requiring health insurance

carriers in Pennsylvania to cover the cost of formulas that are

medically necessary to treat PKU and certain other disorders

that prevent children from consuming normal foods; and

WHEREAS, The 2012 Phenylketonuria Scientific Review

Conference affirmed the recommendation of lifelong dietary

treatment for PKU made by the National Institutes of Health

Consensus Development Conference Statement 2000; and

WHEREAS, The American College of Medical Genetics and

Genomics and Genetic Metabolic Dieticians International

control before and during pregnancy to prevent fetal damage; and

WHEREAS, Children born from untreated mothers with PKU may

have a condition known as maternal phenylketonuria syndrome,

which can cause small brains, intellectual disabilities, birth

defects of the heart and low birth weights; and

WHEREAS, Researchers across the United States are conducting

important research projects involving PKU; and

WHEREAS, The National PKU Alliance, a nonprofit organization,

is dedicated to improving the lives of individuals with

phenylketonuria and pursuing a cure; and

therefore be it

RESOLVED, That the Senate designate the month of May 2015 as

"Phenylketonuria Awareness Month" in Pennsylvania and urge all

Pennsylvanians to learn about this disease.