When Marie Hiagler had her first child, Brianna, she was born just like any normal child. Soon after her birth, however, her mother found that something was not so normal–Brianna spit up whatever she drank. She also threw up whatever she managed to swallow.

Her confused doctors said the child suffered from terrible reflux. Brianna failed to progress like other infants her age; she never was able to crawl of even roll over. After Brianna turned 1, a doctor found calcification in her brain area and diagnosed her with cerebral palsy, but the child didn’t gain weight and had a much smaller head than children with cerebral palsy. Eventually Brianna was diagnosed with an aggressive form of a rare premature aging disease called Cockayne Syndrome.

Cockayne syndrome affects no more than an estimated one in 100,000 babies, and those with the disease may live into their 30s. Because of the rarity of the disease, it is difficult for doctors to diagnose, especially if it is mild.

"Nobody ever sees them enough to be good at diagnosing," says Dr. Will Sorey, an associate professor of pediatrics at UMC. The disease is caused by a defect in the body’s ability to repair damaged DNA. "We don’t think about our bodies constantly falling apart. Every cell except the central nervous system is constantly repairing itself. With Cockayne Syndrome, that repair does not occur, so those who have it appear decades older."

While a test can now determine whether someone has the faulty gene on chromosome 4, experts say Cockayne is such a rare disease that treatment options are not on the horizon.

The University of California Children’s Hospital in San Francisco has opened a new department that focuses on the infant brain.

It is the first facility of its kind in the United States. The department brings together specialized treatment for infants who show signs of brain damage at birth – and are at-risk for developing cerebral palsy, mental retardation and other cognitive disorders – with clinical research.

The new Neuro-Intensive Care Nursery (NICN) is a state-of-the-art newborn care unit designed to host clinical trials and bring the latest cutting-edge treatments to patients.

Newborn brain damage is the leading cause of mental retardation, developmental delay and cerebral palsy in the U.S., according to Rowitch. Among all babies born very prematurely, five to 15 percent go on to develop cerebral palsy, and 25 to 50 percent develop cognitive disorders or a learning disability. The cost of treating cerebral palsy alone exceeds $35 billion annually, according to the March of Dimes Foundation.

The opening of the NICN comes at an important time, as the last few decades have seen an increase in the number of infants who show signs of brain damage shortly after birth. This increase is primarily due to a corresponding increase in the survival rate of extremely premature infants – known to be much more susceptible to brain damage than full-term infants.

Although advanced neonatal care has enabled doctors to keep preterm infants alive, there is currently a lack of therapies that prevent or diminish brain damage in these cases. There is hope that the work done at the NICN will play an instrumental role in the development of new therapies for premature babies at high risk for brain damage.

Our country is failing hundreds of thousands of its youngest citizens on the day they are born, according to the March of Dimes.

In the first of what will be an annual Premature Birth Report Card, the nation received a “D” and not a single state earned an “A,” when the March of Dimes compared actual preterm birth rates to the national Healthy People 2010 objective.

The only state to earn a “B” was Vermont.  Eight others earned a “C,” 23 states earned a “D,” and 18 states, including Texas, plus Puerto Rico and the District of Columbia got failing grades of “F.”

The National Healthy People 2010 preterm birth objective is to lower the rate to 7.6 percent of all live births.  Latest available data (2005) show that the national preterm birth rate is 12.7 percent. In Texas, the rate is 13.7 percent.

The March of Dimes says that in 2009, Report Card grades will reflect state actions taken that have the potential to reduce preterm birth rates in future years.

Preterm birth is the leading cause of death in the first month of life in the United States.  The preterm birth rate has increased about 20 percent since 1990, and costs the nation more than $26 billion a year, according to the Institute of Medicine report issued in July 2006.

Babies who survive a premature birth face the risk of serious lifelong health problems including learning disabilities, cerebral palsy, blindness, hearing loss, and other chronic conditions including asthma. Even infants born just a few weeks too soon have a greater risk of breathing problems, feeding difficulties, hypothermia (temperature instability), jaundice and delayed brain development.

The March of Dimes is the leading nonprofit organization for pregnancy and baby health.  Its mission is to improve the health of babies by preventing birth defects, premature birth and infant mortality.  For the latest resources and information, visit marchofdimes.com or nacersano.org.

You can see a map showing how all stated ranked on the March of Dimes’ website, www.marchofdimes.com.