SENATE JOINT RESOLUTION

No. 50

STATE OF NEW JERSEY

218th LEGISLATURE

 

INTRODUCED FEBRUARY 5, 2018

 


 

Sponsored by:

Senator  ANTHONY R. BUCCO

District 25 (Morris and Somerset)

 

 

 

 

SYNOPSIS

     Designates last full week of May of each year as “Osteopetrosis Awareness Week” and last Saturday of May of each year as “Osteopetrosis Awareness Day.”

 

CURRENT VERSION OF TEXT

     As introduced.

  


A Joint Resolution designating the last full week of May of each year as “Osteopetrosis Awareness Week” and the last Saturday of May of each year as “Osteopetrosis Awareness Day.”

 

Whereas, Osteopetrosis is a rare, congenital bone disorder that causes bones to be overly dense and prone to breaking due to an imbalance in the bone remodeling process that allows for new bone to be created but not for old bone to be removed; and

Whereas, Osteopetrosis is categorized into three forms: malignant infantile osteopetrosis (MIOP), the most severe form of the disorder with the lowest life expectancy rates and often identified shortly after birth; adult osteopetrosis, far milder than MIOP and diagnosed from adolescence through adulthood; and intermediate osteopetrosis, often considered a severe case of the adult form of the disorder and found in children younger than 10; and

Whereas, Mild cases of osteopetrosis may cause no symptoms or problems, while severe cases of the disorder are life threatening and can impact the entire body; and

Whereas, In severe cases, osteopetrosis can lead to stunted growth, painful deformity, and increased risk of fractures due to the creation of dense, brittle bones; vision loss, hearing loss, and paralysis of facial muscles due to compression of the nerves in the skull; and anemia and lowered immunity due to crowding of bone marrow and the inefficient production of blood cells and platelets; and

Whereas, The adult form of osteopetrosis affects approximately 1,250 people in the United States, while only eight to 40 children are born in the country with MIOP each year; and

Whereas, As with many rare diseases, there is much to understand about osteopetrosis and how to treat those affected, yet research is limited because such a small patient population offers fewer market incentives for developing effective drugs and medical treatments; and

Whereas, The United States Food and Drug Administration has approved only one drug, interferon gamma-1b, designed specifically to delay the progression of osteopetrosis; and

Whereas, For cases of MIOP, a bone marrow transplant is currently the only treatment to completely stop bone and bone marrow abnormalities and to prolong survival; however, an appropriately matched donor is usually available for only 50 percent of those affected and engraftment is successful in about 45 percent of transplants; and

Whereas, Franklin Township, New Jersey is honored to be the headquarters of The OsteoPETrosis Society, a non-profit organization that provides education and support to patients, caregivers, and medical professionals who are dealing with osteopetrosis, while also supporting research aimed at identifying additional treatment and a cure for the disorder; and

Whereas, It is appropriate that New Jersey encourages public awareness of this rare bone disorder and recognizes the last full week of May of each year as “Osteopetrosis Awareness Week” and the last Saturday of May of each year as “Osteopetrosis Awareness Day” in order to call attention to the challenges of treating this disorder; to encourage support for patients, families, and caregivers affected by this disorder; and to promote further research into this disorder and its treatment; now, therefore,

 

     Be It Resolved by the Senate and General Assembly of the State of New Jersey:

 

1.         The last full week of May of each year shall be designated as “Osteopetrosis Awareness Week” and the last Saturday of May of each year shall be designated “Osteopetrosis Awareness Day” to raise public awareness of osteopetrosis, to encourage support for patients and families affected by osteopetrosis, and to promote further research into osteopetrosis and its treatment.

 

2.         The Governor is requested to annually issue a proclamation calling upon public officials and citizens of this State to observe “Osteopetrosis Awareness Week” and “Osteopetrosis Awareness Day” with appropriate activities and programs.

 

3.      This resolution shall take effect immediately.

 

 

STATEMENT

 

     This resolution would designate the last full week of May of each year as “Osteopetrosis Awareness Week” and the last Saturday of May of each year as “Osteopetrosis Awareness Day” to raise public awareness of this rare bone disorder; to call attention to the challenges of treating this disorder; to encourage support for patients, families, and caregivers affected by this disorder; and to promote further research into this disorder and its treatment.

     Osteopetrosis is a rare, congenital bone disorder that causes bones to be overly dense and prone to breaking.  The disorder is caused by an imbalance in the bone remodeling process that allows for new bone to be created but not for old bone to be removed.  Osteopetrosis ranges from a mild, symptom free adult form to a severe, life threatening infant form known as Malignant Infant Osteoetrosis, or MIOP. 

     In severe cases, symptoms can lead to stunted growth, painful deformity, and bone fractures due to the presence of dense, brittle bones.  Vision loss, hearing loss, and paralysis of facial muscles may also occur as the dense bone compresses nerves in the skull.  In addition, dense bone can crowd bone marrow and cause the inefficient production of blood cells and platelets, leading to anemia and lowered immunity.

     There is much to understand about osteopetrosis and how to treat those affected.  The United States Food and Drug Administration has approved only one drug, interferon gamma-1b, designed specifically to delay the progression of osteopetrosis.  Currently, for cases of MIOP, a bone marrow transplant is the only treatment to completely stop bone and bone marrow abnormalities and to prolong survival; however, there are several challenges to this treatment.  First, finding an appropriately matched donor is usually available for only 50 percent of those affected.  With a donor found, engraftment is successful in about 45 percent of transplants.  In addition, a bone marrow transplant cannot reverse any existing damage caused by the disorder.

     The efforts of The OsteoPETrosis Society, located in Asbury, New Jersey, provide education and support to patients, caregivers, and medical professionals who are dealing with osteopetrosis, while also supporting research aimed at identifying additional treatment and a cure for the disorder.  Increasing public awareness will contribute to The OsteoPETrosis Society’s mission and may promote further research into the disorder and its treatment.