Journal

Friday, May 9, 2008 10:00 AM EST

Hi - Thanks for checking in! We are doing well. We are very excited for the start of a wonderful summer full of fun!

Jason is doing very well in Kindergarten. I think he will be sad and miss it when it ends. I know I will. He starts first grade next year. Wow! Besides a recent painful ear infection and ruptured eardrum, Jason is doing great. He has really become Daniel’s big brother – looking out for him and playing so nicely with him. I just love to see it!

Daniel is doing just great! He has finally become just another happy kid! People always ask how he is doing and I am grateful that they do. I am overjoyed to answer that he is doing great!

Daniel is scheduled to see the neuro-ophthamalogist at CHOP this month for a check up on his Horner’s Syndrome. I don’t even know what they are looking at because as far as I know, Horner’s Syndrome has no effect on his vision. You can barely notice that his right pupil is smaller than the left. We credit this doctor, Dr. Liu, as the one who enabled us to find Daniel’s cancer early. He found the Horner’s and told us to come back and get a repeat MRI in 6 months, which is how we found the tumor in his neck. When we returned 6 months later, even he wasn’t aware that he was the one who decided to keep an eye on it. After Daniel presented with the Horner’s they began to study and even go back and recheck other kids who had Horner’s to determine if any of them could actually have a cancerous tumor that was causing the Horner’s. Daniel was one of the kids in the study. I am sure that Dr. Liu’s work may have saved more lives.

Daniel is scheduled to have his last 6 month checkup with Dr. Maris on July 2nd. We will spend the whole morning there getting bloodwork, urine test, EKG and Echo for heart, and hearing tests. We will see Dr. Maris and expect to hear him say that we do not have to return for 1 year. We will, instead, be introducing Daniel to the Cancer Survivorship Program at CHOP. Hooray!

Children's Hospital of Philadelphia
Cancer Survivorship Program

Fifty years ago, most children and adolescents survived only a short time after the diagnosis of cancer. Today, nearly 80 percent of children and adolescents with cancer are cured, thanks to new research and treatment. In the 1970s, when children with cancer began to survive, Anna Meadows, MD, began to question what their quality of life would look like after cancer treatment. She and her colleagues began researching late effects, the long-term side effects of cancer treatment.

In 1983, under the direction of Dr. Meadows, The Children’s Hospital of Philadelphia started the Cancer Survivorship Program, the first of its kind designed to care for and track long-term survivors of childhood cancers.

The Cancer Survivorship Program team is a leader in identifying, treating and preventing the late effects of cancer treatment. Children who survive cancer may experience problems with their heart, lungs and other vital organs; they may experience growth problems, cognitive delay or infertility. Because of their pioneering role in studying and addressing the special needs of survivors of childhood cancer, team members have been instrumental in changing medical practice and modifying cancer treatments to reduce long-term adverse effects. The Cancer Survivorship Program at Children’s Hospital of works with survivors to maximize their health and well-being. The team members — doctors, nurses and psychologists — provide expert care to survivors of childhood and adolescent cancer who are experiencing or may be at risk for late effects of cancer treatment.

Our program provides follow-up care to hundreds of long-term cancer survivors each year. Since the cure rate for newly diagnosed patients is so high, we expect this program will continue to expand. Our newest offering is a Multidisciplinary Cancer Survivorship Clinic that enables survivors to see physicians and nurses from Hospital departments such as cardiology, endocrinology, pulmonary, nutrition and psychology all on the same day.

The Cancer Survivorship Program seeks to:
· improve the health and well-being of childhood cancer survivors by promoting continuity of care
· provide referrals to specialists as needed
· offer psychological counseling
· transition patients to adult care when ready
· educate patients, parents and healthcare professionals about the long-term effects of cancer treatment


SOME GREAT NEWS!

Researchers Find Gene Location That Gives Rise to Neuroblastoma, an Aggressive Childhood Cancer

--First Finding of Origin of a Puzzling Pediatric Tumor—

PHILADELPHIA, May 7 /PRNewswire-USNewswire/ -- Using advanced gene-hunting technology, an international team of researchers has for the first time identified a chromosome region that is the source of genetic events that give rise to neuroblastoma, an often fatal childhood cancer.

The investigators found that the presence of common DNA variations in a region of chromosome 6 raises the risk that a child will develop a particularly aggressive form of neuroblastoma, a cancer of the peripheral nervous system that usually appears as a solid tumor in the chest or abdomen. Neuroblastoma accounts for 7 percent of all childhood cancers, but due to its aggressive nature, causes 15 percent of all childhood cancer
deaths.

"Until now we had very few clues as to what causes neuroblastoma," said pediatric oncologist John M. Maris, M.D., who led the study at The Children's Hospital of Philadelphia, where he is the director of the Center for Childhood Cancer Research. "Although there is much work to be done," added Maris, "understanding this cancer's origin provides a starting point for developing novel treatments." The study team reported its findings in today's Online First version of the New England Journal of Medicine.

Neuroblastoma is the most common solid cancer of early childhood and has long been known to include subtypes that behave very differently. Some cases strike infants but spontaneously disappear with minimal treatment, while other cases in older children may be relentlessly aggressive from the start.

Researchers at Children's Hospital and colleagues in the multicenter Children's Oncology Group have for decades analyzed tumors for characteristics such as amplified levels of a cancer-causing gene and deletions of chromosome material. They used those tumor peculiarities to classify neuroblastoma into risk levels that guide oncologists toward the
most appropriate treatments. "Properly defining risk level helps us to avoid the twin pitfalls of undertreating or overtreating any given child with neuroblastoma," added Maris.

However, little was known about genetic events that predispose a child to developing a neuroblastoma tumor. In roughly half of neuroblastoma cases, the cancer is not discovered until it has spread widely in a patient's body, so understanding how a tumor originates may allow oncologists to design earlier and more successful interventions.

In the current study, Maris's team collaborated with Hakon Hakonarson, M.D., Ph.D., director of Children's Hospital's Center for Applied Genomics, to analyze blood samples from approximately 1,000 neuroblastoma patients, as well as samples from some 2,000 healthy children recruited through the Children's Hospital network. A DNA chip analysis performed at the genome center identified three single nucleotide polymorphisms (SNPs) -- changes
in single bases on the DNA helix. Out of over 550,000 SNPs studied, those SNPs were much more common in patients with neuroblastoma, compared to the controls. The three SNPs occurred together on a band of chromosome 6 designated 6p22.

The researchers repeated the analysis in blood samples from additional groups of patients and control subjects from the U.S. and the U.K., and confirmed their finding that variants in the 6p22 region were implicated in neuroblastoma. There are two genes in the 6p22 region, but their functions are largely unknown.

"We are doing further studies to understand how these relatively common genetic changes translate into increased risk of cancer," said Maris. "Ultimately, they probably cause subtle changes in gene expression during early development, interacting with other genes yet to be discovered. This suggests that neuroblastoma has complex causes, in which a series of genetic changes may occur at different sites to combine into a 'perfect
storm' that results in this cancer."

The researchers found that patients with these at-risk SNPs on chromosome 6 were more likely to develop aggressive neuroblastoma. The initial changes on chromosome 6 in all their body cells eventually led to the genetic abnormalities seen in tumor cells in high-risk forms of the disease.

Because their finding reveals only the first step in a series of molecular events, added Maris, it would be premature to do prenatal genetic testing for the SNPs on chromosome 6. His research team will continue to perform genetic analyses, in search of other gene changes that interact with those SNPs. One data source will be 5,000 tissue samples in Maris's lab -- the world's largest collection of neuroblastoma samples, drawing on
decades of research into the disease by Maris, his colleagues and predecessors at Children's Hospital.

"This discovery lays the foundation for learning how these initial changes influence biological pathways that lead to neuroblastoma," added Maris. "Understanding those pathways may guide us to new and better therapies that precisely target this cancer." Hakonarson added, "This study represents one of many ongoing projects to which scientists at The Children's Hospital of Philadelphia are committed, and we anticipate several comparable discoveries will be made in other common and equally complex pediatric disorders, such as autism, asthma, ADHD and diabetes."

The National Institutes of Health supported the study, along with grants from the Alex's Lemonade Stand Foundation, the Center for Applied Genomics, the Abramson Family Cancer Research Institute and the Institute of Cancer Research, located in the U.K.

Among Maris's and Hakonarson's co-authors were several collaborators from The Children's Hospital of Philadelphia and the University of Pennsylvania School of Medicine; the Institute of Cancer Research in Surrey, U.K.; the University of Birmingham, U.K.; the University Federico II, Naples, Italy; the University of Rome; the Children's Hospital of Los
Angeles; and the University of Florida.

About The Children's Hospital of Philadelphia: The Children's Hospital of Philadelphia was founded in 1855 as the nation's first pediatric hospital. Through its long-standing commitment to providing exceptional patient care, training new generations of pediatric healthcare professionals and pioneering major research initiatives, Children's Hospital has fostered many discoveries that have benefited children worldwide. Its pediatric research program is among the largest in the country, ranking third in National Institutes of Health funding. In addition, its unique family-centered care and public service programs have brought the 430-bed hospital recognition as a leading advocate for children and adolescents. For more information, visit http://www.chop.edu.

SOURCE The Children's Hospital of Philadelphia

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