FOLSE and HUDSON with patient AMANDA SAXTON Photo By St. Jude Children’s Research Hospital / Peter Barta

Nick Dustman knew he had to live a healthy life. Diagnosed with Hodgkin lymphoma at age 16 in 1985, Dustman’s treatment was radiation to his chest area. After treatment ended he resumed his life, knowing that he should pay close attention to his health. He biked 10 miles a day and ate well; he was the picture of health. And then, in 2009, he wasn’t.

Like all childhood cancer patients treated at St. Jude Children’s Research Hospital in Memphis, Dustman received notice of the St. Jude Lifetime Cohort Study that looked at late effects of the therapies received by the children treated there. After signing up, he made appointments to return to St. Jude from his home in Florida to be interviewed and tested.

Dustman recalls the discussion he had with Tim Folse, director of the St. Jude Lifetime Cohort study after all the scans were in. “He said, no biking, no lawn mowing, nothing,” Dustman says. “He told me to go home to Jacksonville and find a good cardiologist and have a heart catheterization.”

Dustman asked Folse why was there so much focus on his heart, and he explained that research had shown that radiation to the chest area damages the heart, but the changes may not appear for decades.

“He explained that they now knew that radiation to my heart had aged it,” said Dustman. “Instead of the heart of a 40-year- old, I had the heart of a 60-year-old.

By the time Dustman joined the St. Jude Lifetime Cohort Study in 2009, Melissa Hudson, director of the survivorship programs at St. Jude, had been researching late effects of childhood treatment for a number of years in collaboration with some 26 other centers that treated children with cancer.

A number of possible late effects had already emerged, including second cancers, cardiovascular problems and a host of other issues both physical and emotional depending on the treatment, the cancer’s location, the kind of cancer and whether the children had also received chemotherapy.

When it became apparent that a more substantial long-term, follow-up program was needed, Hudson began the Lifetime Cohort Study to follow St. Jude’s patients, many of whom had been treated decades earlier. The study allowed for identifying populations at highest risk and developing models that would allow for targeted interventions.

Indeed, the first patient in the study was enrolled in 2007; he had been treated for Hodgkin lymphoma in 1962.

As findings emerged around late effects, treatments began changing or being adapted to reduce such issues for children currently being treated. Doctors began carefully planning individual therapy to take into account the severity of the cancer as well as the risks of serious late effects, Hudson says. Radiation plans avoided exposure to nearby normal tissue, and surgery was planned to protect organ function.

But the need to understand the problems for the existing population of children, estimated at around 400,000 who were now adults, remained. Indeed, a study released in 2013 indicated that of those adult survivors already assessed, 98 percent had one or more chronic health conditions, with 67.6 percent having a severe disabling or life threatening condition.

Other outcomes indicate accelerated aging in some adult survivors of acute lymphoblastic leukemia, memory impairment among those who received cranial radiation, and an overall increased risk of premature death — all of which point to the need for lifelong follow up, a challenge when primary care physicians for the most part are unaware of these outcomes or others related to survivorship.

One of the things that surprised me when we started seeing the adult survivors was the undiagnosed laboratory abnormalities that showed they were already getting changes in organs, or they already had changes,” Hudson says, meaning the community providers weren’t looking for the problems in the survivors, unaware that such late effects even existed.

“Survivors need to know about their cancer history,” Hudson says, “because they have to advocate for their own health needs.”

Tim Folse had been in family medicine for 20 years in the Memphis area before joining the St. Jude Lifetime Study in the spring of 2008, six months after enrollment began. Today he runs the clinic where some 3,200 adult survivors are assessed and followed. In addition, St. Jude has now included children, meaning the waiting room may have 5-year-olds to 75-year-olds.

The St. Jude Survivorship Division provides a Survivorship Care Plan for each patient where all the details of their treatment and potential problems are listed.

For the children who have undergone treatment within the past 10 years, he says, verifying treatment information is much easier because the records are all electronic.

For the adults, it requires going into the records kept by hand for treatment details that occurred 20 to 50 years in the past.

The Survivorship Care Plan follows guidelines set by the Children’s Oncology Group, the oversight group that coordinates many of the studies now being conducted across the country (see sidebar). Folse says it’s amazing to look at those who were treated and are now in their 60s or 70s because they received radiation that was often much higher than the doses given now.

“And with the proton beam used now for some cancers, radiologists can limit the radiation to the tumor and thereby avoid damaging nearby tissues,” he says.

When Nick Dustman came in for initial follow-up testing, Folse says his heart problems wouldn’t have been found by his internist at home since he was asymptomatic.

“We did coronary CT studies on a limited group of participants who have received chest radiation for Hodgkin lymphoma, and the radiologist reading Nick’s study showed me the coronary angiogram, and I had never seen one like it. All his arteries were calcified, and he was riding 100 to 150 miles a week on his bike,” Folse says.

“We pieced it together and I made him promise not to touch the bike and to find a good cardiologist at home immediately.”

Dustman did just that, undergoing a 6-vessel bypass three weeks later. Today he is back on his bike and just fine. Folse says he often spends time on the phone with patients’ physicians once they get home.

“It is a ‘shared care’ model,” Folse says. “The local physicians can talk to the providers at the hospital where the treatment was performed, and we provide information to help them care for patients who have been treated for cancer. We explain that some have minimal risk while others have a potential for life - threatening conditions due to therapy. It all depends on the specific treatment. You have to know the details.”

Today Nick Dustman and Tim Folse keep in contact as Dustman continues his follow up.

“I say that St. Jude saved my life twice,” Dustman says

Articles

Feature

Adults who were treated for pediatric cancer during childhood may be more prone to health problems and lasting effects of their treatment. A study at St. Jude is following up with many of these patients. 

Moving On: Adult Survivors of Pediatric Cancers Find Answers

At the age of 29 Dave Dubin heard the words no one wants to hear — you have cancer. Just a few short years later, a second tumor, then a third. It was during that time Dubin learned he had Lynch syndrome, an inherited disorder caused by genetic mutations that increase a person’s risk of developing certain cancers, in particular colorectal cancer.

One in 440 people are Lynch- positive but likely do not know it. Now a three-time cancer survivor, the 48-year-old married father of three and his wife Robin are making it their mission to spread awareness through their foundation AliveAndKickn. People are encouraged to share medical information via the foundation’s HEROIC Registry that can in turn help researchers develop new treatments and conduct further studies. 

 magazine spoke with the Dubins about the registry, their organization and what they hope to achieve as this continue this journey.

Tell us a little bit about your own story and when you were first diagnosed.

 My story goes back to 1997 when I was first diagnosed with colon cancer. The history of colon cancer ran in the family, so, to an extent, it wasn’t a super surprise, although no one expects it at age 29. That’s when I had my first surgery, but it continued through 2007 when I had a second tumor and then in 2009 when I had a third tumor. The first two were colon cancer, and it wasn’t until the second tumor was diagnosed when I had genetic testing. I was diagnosed with Lynch syndrome and because of that, I had additional scans and essentially other “parts” checked, if you will, and that’s when the kidney tumor was discovered.

As a result of that experience, you are working to build awareness of Lynch syndrome through your organization, AliveAndKickn. What are some of the priorities of the organization?

Statistically, Lynch syndrome affects 1 in 440 Americans, yet 95 percent don’t know about it. So, there is this huge group of people who could be affected by Lynch syndrome that if they knew about it in advance, could really do something about it. We don’t have the celebrity survivor per se, so I felt it was imperative for me to shout it from the rooftops and spread the word. It just takes some knowledge, some awareness and some funding.

What would you tell a potential member of the registry about the advantages of being a part of a registry like this?

We’re actually very excited right now. We just launched our HEROIC Registry. It’s the first of its kind. It’s patient-centered, but essentially the goal is to aggregate data for Lynch syndrome. It can range from uploading data from electronic medical records, to social issues, to sharing best practices. The technology is wonderful: because patients feel comfortable that there is security around the information they’re entering, they’re comfortable sharing it. And so far we’re very excited in that we theorized that people who would enter into registry would be similar to me in that they would be comfortable sharing their data with not only their own physicians, not only their own hospitals, but also the clinical research world. We are looking for the miracle cure, if you will, something that is really going to make a difference tangibly, and so far so good.

 We are using a platform developed by the Genetic Alliance. We are one of several disease advocacy organizations that are working on a PEER (Platform for Engaging Everyone Responsibly) platform. Thus, it is highly secure, and every patient who enters the registry goes through a series of privacy directives where they get to choose what data is seen, how much of it is seen, and who gets to see their data

What are some of the issues that arise when a family member has Lynch syndrome, and how do you make the decision of when to talk to your children about it and when to begin screening?

Participants in the registry get to elect whether our organization or researchers recommended by our organization can see a higher level of data than researchers generally who may be coming in to use data within the registry. They choose how much information they want shared and with whom. That gives the patients a level of comfort to know that we are taking their medical data and information very seriously, and we want to make sure that this information is used properly to further research and to connect the patients to research. We’re hopeful that we are going to connect the patients and the researchers in a way that isn’t being done yet.  

 There are a lot of discussions within the community about that specific topic, because some people are eager to have their family members tested and some aren’t. It’s definitely a struggle. We have been very open with our children about Lynch syndrome and what it is, what it means. Because we have the AliveAndKickn foundation, they hear about it all the time. They also have grown up with their father as a cancer survivor their entire lives, since Dave was first diagnosed with colon cancer when our oldest son, who is now 20, was just 15 months old. It definitely impacts them, and they understand from a very young age.

We know that Lynch syndrome increases the risk of colorectal cancer. What are some of the other ramifications for individuals who have it?

We try to give them age-appropriate information as they get older. Our 20-year-old is a Lynch-positive previvor, and he got his testing done just as he turned 18. It’s definitely tough at 18-years-old to find out that you have an 80 percent lifetime risk of developing cancer. It’s a lot to take in, but he’s handled it really well. To me, the most important thing is really keeping the communication open and educating your children, your adult children and other family members on why it’s so important to have this information and what can be done to keep yourselves healthy whether it’s done via annual screenings or other preventive measures that can be done over time. Both of Dave’s second tumors were caught very, very early at stage 1 because he was being screened so carefully. It’s not a fun thing to be a three-time cancer survivor, but to know that we were so careful that his tumors were caught early, he didn’t have to have any chemotherapy. We were able to have them surgically removed and really positive outcomes. That’s what’s most important, keeping on top of everything.

 The original title was hereditary nonpolyposis colorectal cancer (HNPCC) because of colon cancer, but it has been revised to just identify it as Lynch syndrome because of the percentages for endometrial cancer, which can be up to a 60 percent cumulative lifetime risk. So part of our goal is to make sure that people know that it is an “equal opportunity” syndrome. We know of women who will have a hysterectomy prophylactically because of the percentages, but it also has implications for other areas such as the liver, pancreas, kidney and bladder. There is no proven link to breast cancer, but it’s also something we keep an eye on — even men.

What advice would you give to a Lynch syndrome previvor?

There are five different genes that are considered part of Lynch syndrome. It is an autosomal dominant mutation which means that you can inherit the gene from one parent. It does not have to be present in both parents, and any individual has a 50/50 chance of inheriting that gene from one of their parents. There are five different genes, and within each gene, the risk percentages for different kinds of cancer vary. One of our goals with the registry and gathering more data and doing more research is to really get a clearer understanding of what the associated cancer risks are for the individual genes for the various cancers because they do vary depending on which gene you’ve inherited. But almost across the board, the syndrome presents an extremely high risk colorectal cancer, up to an 80 percent lifetime risk. In most of the genes, you have up to a 60 percent lifetime risk of endometrial cancer and then varying risks for other cancers, GI, reproductive and urologic tracts among the most common.

The best thing you can do is get information and really start the process early and get screened early. This is the goal of the registry — to try and make sure that people know about it beforehand. The goal is to not have people find out about Lynch syndrome because they had a tumor at an early age, but to share information and that means sharing information from your physicians, sharing information with your family.

 The key points are that you need to have an understanding. For people out there who may be afraid of genetic testing and really knowing those results, there is so much you can do with that information to keep yourself healthy. It’s really like managing a chronic disease. It’s a lifelong commitment to understanding your health, your family history and your medical history to make sure you’re utilizing all of that information to keep yourself and your family members healthy.

We hope people will contact our organization before they have a tumor. We want to be in that mode, and that’s really the goal of the registry. The events that we do between soccer and bubble soccer are for awareness, but the ultimate goal is to get people in before they need to be in. We want them there first.

Heal magazine spoke with the Dubins about their organization, AliveAndKickn, which raises awareness and empowerment for those with Lynch syndrome. 

AliveAndKickn: Raising Awareness of Lynch Syndrome and Empowering Those It Impacts

As a six-time NBA Champion, 19-time All-Star, six-time Most Valuable Player and the league’s all-time leading scorer, Kareem Abdul-Jabbar is regarded as one of the greatest professional basketball players of all time. He is familiar with the concepts of discipline and overcoming adversity, but not only because of his athletic career.

In 2008, Abdul-Jabbar was diagnosed with Philadelphia chromosome-positive chronic myeloid leukemia (CML) after experiencing hot flashes and sweats. He was treated with two targeted therapies — tyrosine kinase inhibitors: Gleevec (imatinib) and Tasigna (nilotinib). Last year, Abdul-Jabbar underwent a quadruple coronary bypass surgery on his 68th birthday but recovered fully. Today, he is a spokesperson for Novartis, the pharmaceutical company that manufactures both drugs that allowed him to survive cancer.

Why did you want to come to this meeting of oncologists to speak?

When fist diagnosed, what did you know about CML?

I had no idea about the disease. I thought leukemia was something exotic that happened to other people. And then, all of a sudden, it happened to me. 

Do you think that's how most people think of it?

What has your experience been like fighting CML? Do you think your background as an athlete affected your outlook?

Do you keep up with current CML research? Are you familiar with what's going on?

Are there things you're excited about in the field?

What is your advice for people who have CML and are just receiving the diagnosis?

Personally, have you been able to continue living a normal life since your diagnosis?

What are some of the obstacles that remain regarding leukemia and awareness of this cancer?

In March 2016, Abdul-Jabbar was asked to speak at the 20th Annual International Congress on Hematologic Malignancies®: Focus on Leukemias, Lymphomas and Myeloma, a meeting of over 125 oncologists and other oncology professionals. At the meeting, Abdul-Jabbar spoke with Heal about surviving CML, his role as an advocate and his advice to patients and survivors.

My goal was to let the people in attendance know what my story was, how having leukemia can affect your life and the things you can do about it. In talking about all this publicly, I want people to know that we’re all vulnerable to cancer in some way. Many people have the chance to beat this cancer, if you do it the right way and follow the instructions of your doctors — they’re seeing really good results with new drugs.

I think so, probably. Unless you have an indication that you have a problem early in your life, you don’t expect it to come down on you later on in life. You think you passed that point. But these things can happen at any time.

My history as an athlete has given me the discipline to do the things I need to do to maintain my treatment regimen. Some people just don’t want to be bothered — they’re too caught up with the ordinary things in their lives. You need to deal with cancer, be aggressive and respect the fact that it can kill you. If you don’t do that, it will be a lot harder. Fighting cancer requires that you allow medications and other treatments to work by doing what the doctors tell you and following through.

I’m relatively familiar with what’s going on. I try to stay current with what’s happening in preparation for the public speaking engagements I do.

I think that the methods they used to discover Gleevec and Tasigna — that template is being applied to other cancers. They’re having some success treating other cancers with the same approach of finding out the genetic component and treating the genetic abnormalities. It’s very personalized. People used to think leukemia was just leukemia and now they’re finding out there’s a lot of different types and subtypes of the disease. The more we know, the easier it is to find treatment.

For people who are just finding out that they have this disease, I would suggest they absolutely follow the instruction of their doctor. They know what they’re doing. The success with new drugs makes the jobs of doctors more pleasant. Some of the doctors I’ve talked to used to say that they’d do their best with treatment, but patients would still die. And now, physicians are treating people and those people are going on with their lives and forgetting about their disease. That’s the way it should be.

Yes, I have. It doesn’t intrude that much. I take the time to visit my doctor on a regular basis and take my meds, but beyond that, it hasn’t been much of a burden.

I think incorrect information and lack of information is an issue. The more informed people are, the easier it is to achieve things we want to achieve in combatting these diseases.

Basketball MVP and cancer survivor Kareem Abdul-Jabbar sat down with CURE for an interview about his experience battling CML. 

Spring 2016

Moving On: Adult Survivors of Pediatric Cancers Find Answers

AliveAndKickn: Raising Awareness of Lynch Syndrome and Empowering Those It Impacts

NBA Legend's Message to Patients and Survivors of Cancer: Listen to Your Doctors