Sunday, March 1, 2009

Teens and Cancer: The Problem

When Tyler was diagnosed with cancer, we knew this was going to be a long road. To help Tyler focus on the goal, I decided to put together a book of others who had been through this. I went on a mad search to find everyone I could who had fought Burkitt's Lymphoma and Leukemia. But, as I did so, I found a very disturbing trend. Although we were told Tyler's had about a 60% survival rate, I was finding very different numbers among teenagers. I tracked down the families of 48 adolescents who had been diagnosed the prior year with the identical stage of Burkitt's and on the same protocols. 45 of the 48 had already passed away, most as a result of a relapse very soon after initially beating the cancer. That is nowhere close to the 60% number we were given. So I started digging deeper.
I learned that the numbers quoted are compiled using a formula of "Frequency Data". Both hospitals and insurance providers use this data to determine what treatments will offered, whether or not treatments will be covered by insurance, and even IF treatments will be offered at all. Its primary purpose is to more systematically predict outcomes, manage expenses, control costs, and protect against lawsuits. It is the foundation on which protocols are created. The data is gathered from around the country and put into a formula that is basically PROBABILITY x CONSEQUENCE = RISK.
The formula makes it much easier to automatically calculate risks and predict outcomes. But all formulas based on statistical data have an inherent weakness. Their accuracy is dependant upon large quantities of data. Using limited amounts of data for statistical purposes would be like flipping a coin 3 times to determine the odds of getting "heads". It must be tested hundreds or thousands of times before it is statistically accurate.

So here is the problem. Teenagers and young adults have a biology that is very different that both their younger and older counterparts. And, for reasons still not fully understood, their cancers tend to be far more aggressive, and and tend to react differently to treatments. But this information is rarely documented because of the requirements of "Frequency Data". Teenagers tend to get rare cancers, and their numbers do not satisfy the requirements. Therefore their data is bundled together with other cancers and age groups until the necessary numbers are achieved. Their differences are ignored, because individualized care is considered to expensive. The result is that this groups has the lowest cancer survival rates of any age group below 70.
The U.S. Department of Health and Human Services recently published the results of a 5 year study, Closing The Gap: Research and Care Imperatives for Adolescents and Young Adults with Cancer. The report stated that, although teens have biological issues that are different from children and older adults, there has been little focus this group. The result is that five year survival rates for teens are much lower than those of both childhood and adult patients. The report states “While dramatic survival improvements have been achieved in patients diagnosed at age 15 or younger and steady improvement has been made against a number of cancers common among those over age 40, little or no progress has been seen in the AYA (adolescent and young adult) population. In fact, survival has not improved in more than two decades.”

The top research hospitals are now recognizing the problem, and have begun creating adolescent & young adult specially groups. I contacted every one of them throughout the U.S. and Europe. They all agreed on two points. First, survival rates for adolescents were dramatically lower than the stated statistics (ranging from 5%-20%). Secondly, they all agreed that no mistakes could be made. The common phrase was "with Burkitt's, teenagers will have only one chance to beat it. Relapses are very high and rarely survivable with this age group". But most parents and patients will never be told this information. Almost insurmountable barriers are created to prohibit any individualized focus on treatment.
So what do you do with this information? Here are three suggestions:
1. Make sure your oncologist has an understanding of teenage and young adult biology and how it relates to cancer and treatments.

The truth is that adolescents and young adults have a unique biology. The cancer acts different, and their bodies respond differently to treatment. You can not rely on just "staying the course" of pediatric protocols. The cancer moves faster and acts differently. At the time of initial diagnosis, 80% of teenagers are already stage IV (that number is only 20% in the general cancer population). The chemo and radiation treatments are less effective and have greater side effects. 75% of survivors also experience late term side effects years after treatment. Your oncologist must be aware of this, and willing to discuss it thoroughly. If he says there is no evidence to support the differences, then ask him about the following...

A 5 year study conducted by Children's Hospital of Philadelphia concluded that the adherence to protocols is a leading cause of the poor survival rate among teen cancer patients. The report highlighted "gaps in knowledge", and concluded that "adolescent and young-adult cancers are not well understood and are sorely understudied."

Dr. Michael Caligiuri, CEO of the James Cancer Hospital at Ohio State University was interviewed by the Columbus Dispatch about the poor survival among teenage cancer patients. He said "We only make progress through research, and research for the last five years has been woefully underfunded, I would even say neglected."

Dr. Eugenie Kleinerman of MD Anderson stated, “Cancers that develop in adolescents and young adults have a distribution and biology that is distinctly different from that of cancers in children or in adults, although it’s unclear exactly why.”

2. Make sure your hospital understand and acknowledges the differences with adolescents and young adults.

The average age of a patient in a children's hospital is 7, with 90% below the age of 13. The average age in an adult hospital is 65, with 90% above the age of 40. Teens are an often ignored age group, out of place in either environments.
From personal experience, we found pros and cons at both pediatric and adult hospital. The pediatric hospitals were more willing to use the higher doses of chemo and radiation that children and teens can handle. Also, because cancer moves faster in children and teens, they were faster to respond with treatments, and already had an established protocol for almost every occurrence. The problems we had with them was a incredible resistance to deviate from protocol, and multiple barriers to discuss anything experimental, even when all standard options were exhausted. We found adult hospitals were more willing to think outside the box, but found a resistance act fast, and found a lot of hesitancy to increase doses to the levels necessary in children and teens.
Our best experience was with hospitals that treated all age groups, and had a specially focus on teens and young adults. If you are happy with your oncologist, and he is addressing all your questions and concerns, then stay there. If not, get out. Go to a hospital like MD Anderson or Sloan Kettering that is willing to acknowledge the unique differences in adolescents and young adults.

Karen Albritton, MD, Chief, Adolescent and Young Adult Oncology Program at Dana-Farber Cancer Institute and Harvard Medical School said, "90% of patients seen at pediatric centers are less than 15 years old; likewise more than 90% of patients seen by adult hospitals are greater than 40 years of age. This means adolescents are not the focus of care given by, or research done by, either system. The current binary system of medicine, divided arbitrarily and not biologically between age 16 and 21 does a disservice to those patients at the overlap. This is evidenced by the lack of progress in survival statistics for this population. They have had no change in survival rates in 20 years."
Charlene Liggins of the National Cancer Institutes Office of Science Planning and Assessment said her office is "working to improve knowledge about young people with cancer, but there are far fewer experts in that area than in pediatric cancer and cancer in older adults."
The Biology and Clinical Trials Subgroups of the US National Cancer Institute concluded "One explanation for the relative lack of progress in treating cancer in adolescents is that the biology of malignant diseases in this age group is different than in younger and older persons, not only in the spectrum of cancers but also within individual cancer types and within the patient. It should not be assumed that the biology of cancers and patients is the same as in other age groups, and treatment strategies should be tailored to the differences."

3. Get into a clinical trials.

Survival rates of patients in clinical trials are much higher than those not on a trial. Unfortunately, adolescents have very limited access to clinical trials. The problem comes with the strict requirements on the trials. You must remember that the real purpose of the trial is to gather research. Most pediatric trials cut the maximum age limit off at around 13. The researchers need a "clean" data pool, and they know adolescents have unique biological issues that could taint the results. For similar reasons, adult trials often limit the minimum age to 21. And, since teens primarily get rare cancers, there are not enough active cases of teens with the same cancer to develop a trial. The researchers would never get the numbers necessary for frequency data, and therefore the trial would never be funded.
So what's the solution. Well, first you must find the real problem. You can copy the treatments of a trial without actually being in the trial. The problem is money. The purpose of the trial is to gather statistical information, and it is paid for by those getting the information. If your information cannot be used in the data pool, who is willing to pay for it?
The best hospitals are willing to work outside the box. They will pay for it themselves. MD Anderson calls them "shadow trials". Sloan -Kettering calls them "compassionate trials". The bottom line is that there is always a solution. If your hospital tells you they will not do it, leave. Find the best options. This is your child, and you only have one chance. Even if you do everything right, with the best doctors, the best treatments, and the strongest child, survival is still not a guaranty. You can play the game perfect and still lose. What you can do is make sure you leave no stone unturned.
A recent study at Children's Hospital Pittsburgh of UPMC concluded that teenage cancer survivorship is lower due largely to the lack of access to clinical trials. Most childhood cancer trials have a maximum age of 13, while many adult trials have a minimum age of 21. Is it any wonder that 15-19 year olds have the lowest cancer survival rates? They concluded: "Patients who are enrolled in clinical trials offering the most advanced cancer treatments do better than patients who receive conventional treatment. Adolescents and young adults with cancer are less likely than younger children to be enrolled in clinical trials."

Dr. Peter Shaw, director of the Adolescent and Young Adult Oncology Program at Children's Hospital of Pittsburgh reported, "We've known for several years that older adolescents and young adults don't have the same clinical trial rate as younger patients but didn't know all of the reasons why." Dr. Shaw further stated that this reality "translates into worse survival rates, because clinical trial enrollment is correlated with better survival when it comes to cancer."

In the past year there has been a lot of political talk about health care. Unfortunately, much of it is about managing cost, rather than increasing individualized care. Add to the equation the current economic climate, and I suspect these problems will become much worse before they get better. But remember, there are always options. As parents, it is our job to find them.

And never forget...
--The number of teenagers diagnosed with cancer has increased every year for the past 25 years.

--Teenagers and young adults (ages 15-22) are the only age group that have flat or declining survival rates from cancer.

--In the past 25 years ONLY ONE new cancer drug has been approved for pediatric use. Since kids can handle much more chemo than adults, most treatments are little more than mega doses of adult cancer chemotherapy treatments.

--For reasons not fully known, teenagers experience the highest rate of secondary cancers as a result of the high dose chemotherapy treatments.

--Teenagers have the highest fatality rate of any age group. Their cancers tend to be much more rare, therefore lacking established treatments. Their cancers also tend to be far more advanced when diagnosed.

--At the time of diagnosis in teens, the cancer has already spread in 80% of the cases. That is compared to the 20% in adults.

1 comment:

Heidi said...

I'm trully praying for all of you, may God grant you peace that surpasses all understanding.