Dear Chairperson Hansen and Committee members:
On behalf of the cystic fibrosis community in Nebraska, we write regarding an amendment to LB1060 allowing parents to opt out of newborn screening for their infant. Newborn screening for cystic fibrosis has radically transformed the course of the disease by allowing treatment to begin as early as possible. The Cystic Fibrosis Foundation asks that lawmakers add language to LB1060 requiring provision of educational resources about the lifesaving benefits of newborn screening and risks of opting out before parents decide to forgo this service.
About CF and the CF Foundation
Cystic fibrosis is a life-shortening genetic disease that affects nearly 40,000 children and adults in the United States, including nearly 290 in Nebraska. CF causes the body to produce thick, sticky mucus that clogs the lungs and digestive system, which can lead to lung damage, life-threatening infections, malnutrition, and other complications. Cystic fibrosis is both serious and progressive; lung damage caused by infection is often irreversible and can have a lasting impact on length and quality of life, resulting in extended hospitalizations, transplant, or premature death. As a complex, multi-system condition, CF requires targeted, specialized treatment and medications. There is no cure.
As the world’s leader in the search for a cure for CF and an organization dedicated to ensuring access to high-quality, specialized CF care, the Cystic Fibrosis Foundation supports the development of CF clinical practice guidelines and accredits more than 280 care programs nationally — including 2 in Nebraska.
Benefits of Newborn Screening for Cystic Fibrosis
Timely diagnosis of cystic fibrosis by newborn screening is critical in order to begin treatment as early as possible and CF clinical care guidelines recommend that full-term infants with clinical manifestations of cystic fibrosis or a positive newborn screen undergo diagnostic evaluation by four weeks of age.
Early diagnosis has always been important and has become even more important in recent years due to advances in CF treatment and the availability of preventive therapies that treat the underlying cause of the disease. Research shows that children with CF who receive care early in life have better nutrition and are healthier than those who are diagnosed later. Irreversible malnutrition due to pancreatic insufficiency and intestinal malabsorption occurs in many CF infants and can begin as early as two weeks of age.
Additionally, respiratory disease onset has been correlated with malnutrition as infection with Pseudomonas aeruginosa can occur early and lead to significant pulmonary disease within the first year of life. Therefore, any delay in the diagnosis exposes CF infants to both increased morbidity and mortality and we urge lawmakers to ensure that parents understand these risks before deciding to forgo newborn screening for their child.
The CF Foundation and the undersigned clinicians from CF care centers throughout the state thank you for considering this important measure.