The Opportunity of a Lifetime

11-Year-Old Girl Battling Brain Cancer Signs Record Contract

Eleven-year-old Amy Hosmer got the chance of a lifetime Thursday when she signed with a local recording company and recorded her first single.

PLYMOUTH MEETING, Pa. – An 11-year-old girl with recurring brain cancer happens to be an aspiring musician.

On Thursday, the singer-songwriter got the opportunity of a lifetime when she signed a record deal with a local recording company and recorded her first single.

Amy Hosmer says the song, titled “This Camp,” is about a summer camp she attended at the Ronald McDonald House. She hopes her idol, Taylor Swift, will get the chance to hear her song.

Hosmer was diagnosed with a brain tumor when she was just 2-years-old. Doctors didn’t expect her to make it, but Hosmer is a fighter.

Although the tumor has returned, requiring Hosmer to have surgery next week, she still was able to enjoy the special day.

Hosmer hopes this won’t be the only time one of her songs lands her on the red carpet.

Her new song is expected to be distributed on iTunes in just a few weeks.

You can watch the story in the video above.

A Great Story

Jordan Trunfio, 17, battling brain cancer, surprised by Super Bowl tickets

A Fairfax County teen, battling brain cancer, received a super surprise Thursday night – a trip to the Super Bowl in New Orleans.

Jordan Trunfio got one of the best surprises of his 17 years – Super Bowl tickets.

Jordan Trunfio, 17, a senior at West Springfield High School, thought he had injured his elbow playing football. It was not an injury, but several tumors in his brain affecting the rest of his body. The cancer forced him to undergo radiation and chemo.

Tonight, he thought he he was going to yet another fundraiser to cover his medical bills. But his friends and neighbors were there for another reason.

A local Domino’s Pizza restaurant and ESPN 980 got the tickets for Jordan and his father.

ABC7’s Hatzel Vela was there when Jordan got his big surprise.

Understanding Brain Tumors

Unraveling the Causes of Brain Tumors

There are many types of brain tumors. Unfortunately, the cause behind most of these tumors remains a mystery.

Medically reviewed by Pat F. Bass III, MD, MPH
While an estimated 359,000 people in the United States have aspinal cord or brain tumor, doctors do not yet know much about what causes these tumors. This fact, although disappointing, is understandable considering the remarkable range of differences among these tumors in adults and children.

There are more than 120 different types of spinal cord and brain tumors. These tumors, also called central nervous system (CNS) tumors, are sometimes benign (non-cancerous) and sometimes cancerous.

In addition, some tumor types occur more often in children than in adults. And adult tumors are not the same as children’s tumors.

Brain Tumor: Risk Factors

Certain factors have been found to increase a person’s risk for a spinal cord or brain tumor. These include:

  • Being a Caucasian male
  • Being older than 70
  • Being younger than age 8
  • Having a family history of brain tumors
  • Being exposed to radiation
  • Being exposed to certain chemicals

But just because a person may be at risk for a brain tumor, doesn’t mean they will get one. What these factors tell us is that people who get a brain tumor often fall into the above categories.

Brain Tumor: Genetic Disorders

While researchers do not know much about what causes brain and spinal cord tumors, they do know that some genetic disorders cause brain tumors. But fewer than 5 percent of brain tumors result from these genetic disorders, including neurofibromatosis, a condition in which nerve tissues grow tumors.

That leaves 95 percent of all brain tumors whose cause is not yet known. But research is under way to better understand what causes brain and spinal cord tumors.

Brain Tumor: The Cancer Genome Atlas

“The most exciting new initiative is the Cancer Genome Atlas,” says Andrew Sloan, MD, director of the Brain Tumor and Neuro-Oncology Center at University Hospitals Case Medical Center in Cleveland. The Cancer Genome Atlas Research Network is a collaborative effort funded by the National Cancer Institute (NCI) and the National Human Genome Research Institute of the National Institutes of Health.

The network, whose goal is to discover more about the molecular basis of cancer, recently published the first results of a major, comprehensive study of glioblastoma. Glioblastomas account for 23 percent of brain tumors diagnosed in the United States.

In the study, researchers examined brain tumor samples from 206 patients. They found numerous gene mutations (changes) that occur in glioblastomas (more than 300 are already known). But they also found three genetic mutations that occur frequently but had not been recognized before. The team was also able to identify some of the main biological pathways that are disrupted in glioblastomas.

Johns Hopkins researchers published similar findings from a parallel but smaller study on the same day. “These are very exciting studies because they give us hope: Rather than having to fight the 300 or 400 known genetic defects in glioblastomas, we may be able to focus on far fewer to help us find the cause of these tumors,” Sloan says. “This would make the puzzle much easier to solve.”

Because most glioblastoma patients die within 14 months of being diagnosed, finding a cause of this deadly form of cancer could help doctors design treatments that would enable people to live longer.

Brain Tumor: International Study

Another large project, the Gliogene study, is the largest study to date of a primary brain tumor called a glioma. In the five-year study, an international team of researchers will try to identify what gene or genes are related to their development. A glioma is a common type of brain tumor that grows from nerve cells, called glial cells, which are important to brain tissue support.

If they can find those genes, they may be able to identify a genetic link among the relatives of people with brain tumors and use it to develop new treatment and perhaps improve existing ones. The hope is that one day the findings might even help researchers develop ways to prevent this kind of brain tumor.

The researchers’ goal is to screen more than 15,000 people around the world. Countries that are participating are the United States, United Kingdom, Sweden, Denmark and Israel. If you, or a member of your family, have a brain tumor and you are interested in participating in this study, visit Gliogene: An International Brain Tumor Family Study .

Brain Tumor: Rembrandt Is More Than a Painter

To do accurate research, scientists need a base of genetic information about brain tumors that is properly gathered and stored. Together, the NCI and National Institute of Neurological Disorders and Stroke (NINDS) have created such a database, the Repository for Molecular Brain Neoplasia Data (REMBRANDT). The repository will keep analyses of samples from brain tumors and other data on all types of brain tumors to provide researchers with invaluable information for their studies.

Ivy Foundation Featured on AZ Redbook


A Patient-Focused Approach

Catherine (Bracken) Ivy, founder and president of The Ben & Catherine Ivy Foundation, has traveled the world learning all she can about brain cancer – specifically, how to cure it.

She is determined, through the efforts of her foundation, to find better treatment options and improve the quality of life for patients with brain tumors, especially those with glioblastoma multiforme (GBM), the most common and aggressive form of malignant primary brain tumor. Ninety-eight percent of people diagnosed with GBM live fewer than 18 months.

Because brain cancer is rare compared to many other cancers, research into the disease receives little federal funding, pharmaceutical industry support or media attention. Today, standard of care currently involves removal of the tumor (though surgery most often fails to remove all the cancer) followed by radiation treatments and chemotherapy involving a drug with limited effect for the majority of patients. Sadly, little else remains to extend life expectancy or remission.

This status quo is not acceptable to Ivy. More than anything, she wants the Ivy Foundation to provide solutions – and hope – for people diagnosed with brain cancer.

“I want people with brain cancer and brain tumors to know that there is a community of people working very hard to try and help them,” Ivy says. “We’re not saying we’re going to cure it tomorrow, but a least we’re moving the needle. Our immediate goal is to double life expectancy in the next seven years for people diagnosed with brain cancer. We will never give up until we find a cure.”

Leading the charge

The Ivy Foundation’s overarching goal over the next seven years is to double the life expectancy of brain cancer patients from 18 to 36 months. And in working with TGen, Ivy says she has found three key values that align both organizations:

– Patient-focused research

– Conducting the best research possible in a cost-effective manner

– Making progress immediately

“Those three things are not simple,” Ivy says.

But, she says, the innovative ideas of TGen President and Scientific Director Dr. Jeffrey Trent make her believe her efforts are worthwhile. The two first met at a brain cancer conference in Tucson, and at subsequent meetings Trent outlined research that would help the Ivy Foundation achieve its goal of advancing patient care.

As a result, the Ivy Foundation recently granted $10 million to TGen: $5 million each for two new groundbreaking brain cancer projects.

Discovering why some patients live longer

One $5-million project is titled “Outliers in Glioblastoma Outcome: Moving the curve forward.” This five-year investigation seeks to discover why approximately 2 percent of GBM patients – the outliers – live far beyond the average survival time of 18 months.

“A major challenge with brain cancer is that people survive such a short time,” Ivy says. “If this research enables patients to live longer, clinicians and researchers will gain a better understanding of how this disease works, which will bring us time to study the disease, providing the opportunity to move closer to a cure.”

By precisely identifying the billions of molecular building blocks in each patient’s DNA through whole genome sequencing, TGen researchers hope to discover the genetic differences between those patients who survive only a few months and those who survive longer because their brain cancer develops more slowly.

Using these genetic targets, TGen researchers will identify those patients most likely to benefit from the current standard of care, and those who might best benefit from alternative or new experimental treatments.

First-in-patient clinical trials studies

In the second $5-million project, “Genomics Enabled Medicine in Glioblastoma Trial,” TGen and its clinical partners will lead first-in-patient clinical trial studies that will test promising new drugs that might extend the survival of GBM patients. This multi-part study will take place in clinics across the country and TGen laboratories.

This project begins with a pilot study of 15 patients, using whole genome sequencing to study their tumor samples to help physicians determine what drugs might be most beneficial.

To support molecularly informed clinical decisions, TGen labs also will examine genomic data from at least 536 past cases of glioblastoma, as well as tumor samples from new cases, developing tools that will produce more insight into how glioblastoma tumors grow and survive. TGen also will conduct a series of pioneering lab tests to measure cell-by-cell responses to various drugs.

To get new treatments to patients as quickly as possible, this five-year study will include a feasibility study involving up to 30 patients, followed by Phase II clinical trials with as many as 70 patients. TGen intends to team with the Ivy Early Phase Clinical Trials Consortium that includes the University of California, San Francisco; the University of California, Los Angeles; the MD Anderson Cancer Center; the Memorial Sloan Kettering Cancer Center; the University of Utah; and the Dana-Farber/Harvard Cancer Center.

The results of these clinical trials should not only help the patients who join them, but also provide the data needed for FDA approval and availability of new drugs that could benefit tens of thousands of brain cancer patients in the future.

“It’s a tremendous opportunity to find more solutions for the patient diagnosed with brain cancer,” says Ivy, who also is working to establish additional clinical trials in the Phoenix area, giving local patients more treatment options. “The clinical trials are very exciting because they can impact the patient today.”

More information about TGen

More information about The Ben & Catherine Ivy Foundation

–       Text by Steve Yozwiak, TGen senior science writer

–       Photo courtesy TGen

At top: Catherine Ivy, founder and president of The Ben & Catherine Ivy Foundation, with TGen Deputy Directors Dr. David Craig (left) and Dr. John Carpten (right)

Arizona Mom Shocks the Medical World

Mom Stuns Doctors, Beats Deadliest Brain Cancer

PHOTO: Heather Knies

Heather Knies, shown here with her husband Joe and daughter Zoe. (Courtesy Barrow Neurological Institute)
Jan. 7, 20123

Heather Knies was given a death sentence at the age of 24. She battled not one, but two brain tumors — one of them a grade 4 glioblastoma, the same kind of cancer that killed Sen. Edward Kennedy in 2010.

But today, six years later, she is cancer-free, and her doctors at the Barrow Neurological Institute in Arizona cannot explain it. Her latest MRI is clean, and she is neurologically intact.

The now-32-year-old Knies has not only outlived her life expectancy, she has married and become a mother. Her successful parenthood is remarkable, as intense radiation and chemotherapy can render cancer patients infertile.

Knies’s daughter, Zoe, who is 7 months old, celebrated her first Christmas in December.

Knies’s doctors say that in rare instances, a patient can break the “biological rules.” But most often in those cases, the initial pathology of the tumor was suspect.

In her case, the pathology was “not controversial,” according to her surgeon, Dr. Robert Spetzler, director of the Barrow Neurological Institute at Joseph’s Hospital and Medical Center in Phoenix.

In his 35 years as a neurosurgeon in the United States, Spetzler said he has never seen such a triumph against a stage 4 glioblastoma.

“It’s one of the most malignant tumors there is,” he said. “Invariably it will come back and pop up somewhere else in the brain and it’s uniformly fatal.”

“It’s not unheard of that that a few survive — it’s a bell curve and there are outliers,” he said. “But in her case, not only has she survived, but she is perfectly normal and there is absolutely no evidence of a tumor on her MRI scan.”

Knies has a few of her own theories for why she is still alive today.

“One, being God had a plan for me,” said Knies. “I also had a great team of doctors and wonderful family and friends with a positive attitude.”

“The mind is so much more powerful than anyone can imagine,” she said. “People believe that when they get cancer, it will kill them. But I never once thought that.”

Spetzler said Knies was “on the young side” for a glioblastoma, but it can occur at any age, “even in infants.”

It all began in 2005, when Knies had the first symptom that something was wrong. She had just started a new job as a receptionist at a doctor’s office and was driving home from work.

“Suddenly, I didn’t understand what the dashed white line meant in the road,” said Knies. “I had been driving since I was 15, so I started panicking and called my Mom. She asked, ‘Did you take something?'”

Knies could see, but couldn’t understand what she was seeing.

“I was only 24 and I was having visual problems,” she said. “I can’t even describe them.”

Her boss, a dermatologist, insisted she see a specialist, and an MRI showed a low-grade tumor that was pressing on the visual reception cord in her brain.

“I had just moved to Phoenix from Missouri. I was just out of college and felt like I had the whole world waiting there for me,” said Knies, ever the optimist. “Looking back, it probably grounded me a bit.”

She underwent surgery at another institution, and she enrolled in a drug trial for an oral chemotherapy at Duke University, repeating MRIs every three months.

She says doctors told her to, “Go live your life.”

But in less than a year one of the scans showed the white flairs of tumor growth.

It turned out the new tumor was aggressive — a stage 4 glioblastoma and it was sitting on the right side, touching three parts of her brain: the temporal, parietal and occipital lobes.

The lower-grade tumor had turned into a more aggressive one, which is not unusual in a glioblastoma, according to Spetzler, who took over her treatment in 2007.

“I opted not to have the entire tumor removed or my left side would have been paralyzed — so I asked Dr. Spetzler to debulk it. I didn’t want quantity, I wanted quality,” she said.

When her mother was brave enough to ask how long she would have to live, one of the doctors said about six months.

“For whatever reason, because of being an athlete or just being mad, I wanted to defy him and the medical world and show that no one is a statistic,” Knies said. “I was immediately defiant. I never once thought it would be the death of me.”

The tumor caused massive headaches and vomiting from the pain, and on Friday, April 13, 2007, she went into surgery. “Friday the 13th will never scare me again,” she said.

Surgery was followed by heavy doses of chemotherapy and radiation. Knies will be monitored with MRIs for the rest of her life, but for now, her brain shows no sign of residual cancer.

“I would not feel comfortable calling it a cure,” said Spetzler. “But there is no evidence of a tumor as you would expect with someone who has lived much longer than expected. There is a hole where the tumor was. Her survival is remarkable.”

At her cancer diagnosis, her boyfriend at the time had “freaked out,” according to Knies. “It makes you very insecure when someone tells you up front they can’t handle it — bye-bye.”

But in 2010, she met Joe Knies, now 54, an engineer who was 22 years her senior.

“It didn’t even faze him, and it blew me away,” she said. “He made a good point — we can all die in a car crash tomorrow.”

They married in October while Knies was still undergoing chemotherapy one week each month. She had always wanted children and was warned the aggressive treatments could have damaged her eggs.

“It was almost as scary for me as hearing about the cancer,” she said.

On her oncologist’s advice, Knies decided to undergo in vitro fertilization with a surrogate because of the unknowns associated with cancer and pregnancy.

“I prayed hard,” she said. “After egg retrieval there were only two follicles and the rest were empty.”

In the three days they took to mature, only one was viable. “We had that one, and she is my daughter,” said Knies.

“My husband had never been married before or had kids and his parents thought they would never see the day, so it was a miracle to his mother that he now has a child,” she said.

“Every morning I wake up and thank God that I can feel my 10 fingers and toes and have a loving daughter and husband,” said Knies. “There have been so many miracles. One after another, as my dad said, so many angels must be sitting on my shoulders.”