Bill Text: NJ AJR75 | 2010-2011 | Regular Session | Introduced
Bill Title: Designates February 28 of each year as "Pompe Disease Awareness Day."
Spectrum: Partisan Bill (Democrat 1-0)
Status: (Introduced - Dead) 2011-05-09 - Introduced, Referred to Assembly Health and Senior Services Committee [AJR75 Detail]
Download: New_Jersey-2010-AJR75-Introduced.html
Sponsored by:
Assemblyman JOHN S. WISNIEWSKI
District 19 (Middlesex)
SYNOPSIS
Designates February 28 of each year as "Pompe Disease Awareness Day."
CURRENT VERSION OF TEXT
As introduced.
A Joint Resolution designating February 28 of each year as "Pompe Disease Awareness Day" in New Jersey.
Whereas, Pompe disease is a rare, inherited, and often fatal disorder that occurs in about one in every 40,000 individuals; and
Whereas, Mutations in a gene that generates alpha-glucosidase, an essential enzyme which normally breaks down a complex sugar called glycogen but which is greatly reduced or eliminated in cases of Pompe disease, results in a buildup of glycogen in the body's cells; and
Whereas, Excessive buildup of glycogen in the body's cells can impair the ability of affected tissues to function normally, and most seriously affects the heart and skeletal muscles; and
Whereas, The age of onset of Pompe disease can vary widely, but the disease is broadly classified into infantile-onset Pompe disease and late-onset Pompe disease; and
Whereas, Infantile-onset Pompe disease is caused by a complete, or nearly complete, deficiency of alpha-glucosidase, and the symptoms begin in the first month of life with feeding problems, poor weight gain, muscle weakness, inability to hold the head up normally or move normally, enlarged tongue, and respiratory difficulties that often are complicated by lung infections; and
Whereas, Most babies with infantile-onset Pompe disease die from cardiac or respiratory complications before their first birthday; and
Whereas, Late-onset Pompe disease can occur as early as the first decade of childhood and as late as the sixth decade of adulthood; and
Whereas, The primary symptom of late-onset Pompe disease is muscle weakness that progresses to respiratory weakness and death from respiratory failure after several years; and
Whereas, Pompe disease may be confused with other chronic muscle diseases such as multiple sclerosis or muscular dystrophy, but the diagnosis can be confirmed by examining the activity of alpha-glucosidase in skin cells, muscle, or blood, and by genetic testing; and
Whereas, Although there is currently no cure for Pompe disease, a recently-approved enzyme replacement therapy, which requires replacement of some of the missing enzyme in the patient's body every other week, has been found to improve the symptoms of some patients; and
Whereas, An individual with Pompe disease requires care from a team of specialists, including geneticists, dieticians, cardiologists, pulmonologists, orthopedists, physical, speech and occupational therapists, and neurologists; and
Whereas, As is the case with all persons affected by rare diseases, individuals with Pompe disease and their families often encounter a variety of problems, including a sense of isolation and difficulties obtaining an accurate and timely diagnosis and effective treatment, and receiving reimbursement for treatment; and
Whereas, Since 2009, the National Organization for Rare Disorders has organized a nationwide observance of Rare Disease Day on February 28, and to coincide with that observance, it is appropriate that New Jersey designate February 28 as "Pompe Disease Awareness Day" to bring attention to patients and families affected by Pompe disease; now, therefore,
Be It Resolved by the Senate and General Assembly of the State of New Jersey:
1. February 28 of each year is designated as "Pompe Disease Awareness Day" in the State of New Jersey to increase public awareness of Pompe disease.
2. The Governor shall annually issue a proclamation recognizing February 28 as "Pompe Disease Awareness Day" in the State of New Jersey and calling upon public officials and citizens of this State to observe the day with appropriate activities and programs.
3. This joint resolution shall take effect immediately.
STATEMENT
This joint resolution designates February 28 of each year as "Pompe Disease Awareness Day," to coincide with the observance of Rare Disease Awareness Day, which is observed nationwide on February 28.
Pompe disease is a rare, inherited, and often fatal disorder that affects one in 40,000 individuals. Pompe disease disables the heart and muscles, and can lead to death before a child's first birthday in the case of infantile-onset Pompe disease. While a recently-approved treatment may alleviate symptoms for some patients, there is no known cure for Pompe disease.
