PATIENT-CENTERED GUIDES

An Interview with
Author-Advocate
Nancy Keene

Lamb:

How long have you been involved with childhood cancer issues?

Keene:

I became a "cancer mom" in 1992 when my three-year old daughter was diagnosed with high-risk ALL (acute lymphoblastic leukemia). My way of coping was to research the disease and its treatment. I became a walking encyclopedia about childhood ALL. Parents and others in my area started asking me a lot of questions and I started explaining, in lay terms, the disease and its treatment.

Mid-way through my daughter's treatment, I was encouraged to write a book for families. At that time, few resources were available. There were introductory booklets from national organizations and there was the technical literature, and nothing in the middle. Since I didn't want the leukemia book to be autobiographical, I focused on explaining the technical information in lay language at a high school literacy level. I incorporated stories from over a hundred parents, children, and their brothers and sisters, in order to show the variability of experience. With that many stories, all readers can find something that helps them in their situation.

Lamb:

Where did you meet other parents?

Keene:

At first, in the institution where I took my daughter for treatment. But we were lucky to only have two weeks inpatient. For the rest of her treatment, we commuted to the hospital, 150 miles each way. So, I didn't have much time or opportunity to connect with other families.

I started a face-to-face support group in my small town for parents of kids with cancer. We were few in number, and had all sorts of different diagnoses and different issues. I also discovered Candlelighters Childhood Cancer Foundation. I avidly read their quarterly newsletters and started to attend conferences.

Then, in 1996, online support groups were created. I discovered a mailing list about hematological (blood) cancers on ACOR, the Association of Cancer Online Resources. The ACOR Hem-Onc list was one of only a few online lists for patients and families, at that time.

The growth of that list, and other ACOR lists, was exponential. More people were discovering and using the Internet. People were searching for more information about the disease or treatment options. Also, people wanted community. Many types of cancer are rare, especially pediatric cancers, and it can be hard to find someone nearby in your situation.

Those early ACOR groups started splitting into more specialized groups. In 1997, groups focused on pediatric cancer began breaking off and forming. The first such group was "Ped-Onc." There are now approximately 20 pediatric groups, serving over 1,500 families. This is incredible community. About 13,000 children under 20 are diagnosed with cancer every year in the US and there are over 1,500 families online on ACOR, alone, talking to each other. Since there are other online discussion groups on other sites, also, this shows that a significant percentage of childhood cancer families have connected to help one another.

There are also now three online groups at ACOR for childhood cancer survivors: one for adolescents, one for adult long-term survivors of childhood cancer, and one for parents. About six hundred people are now on those lists.

I spend about an hour a day on the online support groups. In the beginning, I was primarily sharing experiences and support. Now, I also steer people to resources appropriate for their situation.

Lamb:

How did you start doing advocacy on a national level?

Keene:

After my daughter's treatment, I began paying attention to what was going on politically. I met a lot of nurses, doctors, and parents of children with cancer when I attended national conferences. In 1997, I published my first book for parents, Childhood Leukemia. The next year, I was working with Honna Janes-Hodder on a book for solid-tumor childhood cancers and beginning work (with nurse/experts Wendy Hobbie and Kathy Ruccione) on a book for childhood cancer survivors. During those years, I talked with hundreds of families on the phone, in person, and online.

Archie Bleyer MD from MD Anderson, asked me to be one of the first three parent advocates for the Children's Cancer Group, the largest of the four national groups that developed clinical trials for children with cancer. As parent advocates, we tried to function as a bridge between the professional community-doctors, nurses, researchers-and families. We polled families on what issues they thought were most important. We did that polling through our personal contacts, through 300 Candlelighters Childhood Cancer Foundation face-to-face local support groups, and online through ACOR groups. The patient advocacy committee would prioritize the list of issues that other parents raised and work on two or three issues at a time, to try to bring about improvement.

Two years ago several childhood cancer groups (the Children's Cancer Group, the Pediatric Oncology Group, the Intergroup Rhabdomysarcoma Study Group and the National Wilms' Tumor Study Group) merged into one organization called Children's Oncology Group, or COG. The new chair of COG, Greg Reamon, asked our parent committee to transfer over to COG. Since then, we've added new members to the committee, but have continued to act in the same way: polling many parents and prioritizing the issues they want to see us address.

I also started working with Candlelighters on survivorship issues-answering individual questions, helping with referrals for second opinions, and educating parents about political issues. Kevin Offinger MD and I write a column about late effects of childhood cancer for the Candlelighters quarterly newsletter. The newsletter goes out to 13,000 people, many of whom are survivors.

Recently, I edited the Patient-Centered Guides book, Childhood Brain & Spinal Cord Tumors. I'm very happy that we now have educational materials for all childhood cancers.

National Advocacy Issues

Lamb:

What kind of issues do parent advocates address, in the field of childhood cancer? What new points of view can advocates bring to the medical community?

Keene:

Patient advocates are now in many organizations and federal agencies. The NCI has several patient advocates, as does the FDA.

The COG parent advocacy committee works on a small number of issues related to pediatric clinical trials.

Lamb:

What are some examples of issues that these committees address?

Keene:

One of the first issues we addressed in COG was parents' access to information about their child's clinical trial. Each clinical tral has a document that includes technical information about the trial: the medical studies from which the hypothesis of the trial evolved, the trial design, a description of the method of treatment, etc.

COG did not have a policy about whether to provide the full trial document to families whose children were enrolled in a clinical trial. If a parent asked for the document, some institutions would provide them, many would not.

However, unbeknownst to most physicians, there was an underground exchange of those documents among parents. If a family was denied the document, someone else in the US, Australia, or Canada who had been given the document would send a photocopy to the family.

This secrecy was a real lose-lose situation. If the doctors did not provide the parents with the document, whether out of fear that the information was too technical or policies of the treating institution, the family would lose trust in the doctor. That trust is crucial when a child is going through long and arduous treatment; physicians and families need to be close partners. By getting the information in secret, families also lose having their doctor as a potential ally. Families have no one to go to with questions and concerns.

Not all families want this information, but those families who do should be provided with it. After discussions with the patient advocacy committee, COG changed its policy. On the Consent Form for all pediatric trials, there is now a notification that a copy of the full trial document is provided, if you request it.

The pediatric advisory committee is currently working on developing a public website. Families want "one-stop shopping," so they can find in-depth information that they can depend on for accuracy. There is loads of information on the Internet. Some of it is really great and some of it is full of inaccuracies. I was a member of the task force that developed a grant proposal for a COG public website. I hope it is funded, because families really need a trustworthy website that focuses on childhood cancer.

Increased funding for late effects research is another very important issue for most families. Parents would like more trials that identify late effects from treatment, and that investigate how to test for them and treat those that do arise. This effort has not yet been very successful.

Lamb:

In what other ways do you help parents find ways to have their voices heard?

Keene:

One of our goals as parent advocates is to develop mechanisms for political action. Politics does impact childhood cancer. Trial design, what research is being conducted, funding levels, FDA policies, extending rights in employment and insurance for childhood cancer survivors, etc. are all national questions. We try to provide accurate information so that parents and survivors can voice their opinions to their elected representatives if they so choose.

For example, the FDA has made many changes in how and when drugs are tested before they are given to children, which affects requirements for pharmaceutical companies. We gave parents information about the issues and provide contact information for elected officials, for those who wished to call, write, or visit.

Lamb:

What is the "good news" about childhood cancer, if there is any? Are more children being cured?

Keene:

Treatment for childhood cancer is one of modern medicine's miracle stories: today three-quarters of children diagnosed with cancer are cured, while forty years ago most children died. The reason for that miraculous reversal is that, early on, pediatric oncologists cooperated in developing clinical trials and were very effective in enrolling a majority of children in clinical trials. Over 60 percent of children are enrolled in trials versus 3 percent of adults. Even though pediatric cancers are rare, that rate of enrollment means that there are enough children in trials to rapidly learn about effective treatments. Development of treatments has been rapid and successful.

We are now in the enviable position of modifying treatment for some cancers to decrease the late effects that sometimes develop after treatment. Until this point, research has been focused on cure. Now, researchers are expanding that focus: maintain the cure rates, decrease toxicity, and identify and treat the late effects that are unavoidable. There are an estimated 250,000 survivors of childhood cancer now and this group is growing every year. Survivors are going to live for decades, and helping them with late effects is a very important medical endeavor.

Lamb:

What kinds of cancers do children get?

Keene:

Scientists discuss childhood cancers as "liquids" and "solids." About one-third of childhood cancers are "liquid" - leukemias or lymphomas affecting the blood or lymph fluid. One-third are solid tumors of the brain or spinal cord. The remaining one-third are solid tumors in other locations, such as the eye, bone, liver, kidney, or symphathetic nervous system.

Lamb:

You spoke of a 70% cure rate. What factors influence prognosis?

Keene:

Some types of childhood cancer have survival rates over 90%. For a few cancers, the survival rate is less than 5%. Survival rates for most other childhood cancers are in between these extremes.

There are many factors that impact prognosis in children's cancers. For example, a child's age at diagnosis can make an appreciable difference. The type of cancer, if it has spread, where it has spread, the grade of the tumor, the location of a tumor, and many other factors impact prognosis.

Prognosis is truly individual. Parents need to keep asking questions of their doctor until they understand the type of cancer, treatments, and what their options are.

Treatment

Lamb:

Where are childhood cancers treated?

Keene:

Most children with cancer are treated at one of the 238 institutional members of COG in the US, Canada, and Australia.

Parents of newly diagnosed children can either choose standard treatment (which is the best known treatment) or a clinical trial (if available) which tests that standard treatment against a treatment which may prove to be either more effective or less toxic.

I strongly encourage families to go to COG institutions that treat a large number of children with cancer. This may be particularly important for adolescents and children with specialized treatment needs.

Historically, many adolescents were sent to adult facilities and have been underrepresented at COG institutions. There are studies that show that adolescents treated at adult facilities have lower cure rates. Adult facilities usually don't have the staff or expertise to address the unique needs of adolescents with cancer.

If a child's cancer is a common type with a well-established treatment regime, such as average-risk ALL, a parent would be given the same options for treatment at any COG institution. If you have a child with a rare tumor or one that requires specialized treatment, do some research on where the best place to treat it. Some institutions also have more experience in certain cancers. If your child has a brain tumor, it's probably best to go to a neurosurgeon who has done dozens of similar surgeries rather than to a neurosurgeon who has done one or two.

If your child needs a stem cell transplant, for example, different institutions vary considerably in their experience. Some institutions have been doing transplants for twenty years and some just started offering this treatment.

Institutions may differ in the types of transplants offered, in the services provided, and in methods used in infection control, such as isolating the child; having visitors wear gowns, masks, and booties; or having visitors simply wash their hands.

Just because a method is more stringent does not necessarily mean it's better, as parents might assume. Some methods are used out of habit, not because they have been scientifically tested and found to be better than other methods. If someone told me my child needed a bone marrow transplant and needed to be isolated for weeks, I would research whether or not there was any medical evidence to support that need.

Lamb:

We hear that cancer treatments for adults are getting "easier" with better control of nausea and more targeted treatments. Is this the same case for children's cancer?

Keene:

Yes. There are really effective anti-nausea drugs.

In my experience, more children seem to be given the really good anti-nausea drugs (Zofran, Kytril) than adults. With some insurers, adults are given a cheap and less effective anti-nausea drug, first. Only if they have problems and complain strongly or ask for different drugs, do they get the newer, more expensive, but more effective anti-nausea medications.

In the last ten years, there has also been a big improvement in sedation for painful procedures. Many institutions have recognized the importance of having child-life specialists to help cope with trauma of treatment. Appropriate interventions are more widely used and children are undergoing less trauma, which results in a better psychological outcome for survivors. As recently as ten years ago, several adults would pin children down while they had bone marrow aspirations or spinal taps. Now, most institutions give a short-acting sedative, and the children wake up in ten minutes with no memory of the procedure and no pain.

There is also a tremendous amount of research on targeted therapies. Gleevec, the drug which recently was approved by the FDA for chronic myeloid leukemia (CML), is being tested on some childhood brain tumors. Monoclonal antibodies hold much promise. We all hope that some of them will work, so that future treatments for children will not be as toxic and lengthy as the treatments of today.

Causes of Childhood Cancers

Lamb:

Are childhood cancer rates increasing? How sure are we of the rates?

Keene:

I'm not sure, because there is not a national database that tracks every child who is diagnosed with cancer. The federal government developed the SEER program, where some geographical areas track all cancer diagnoses.

Lamb:

Is environmental contamination linked with childhood cancer?

Keene:

I personally believe that environmental pollutants may contribute to some types of childhood cancer. However, it is notoriously hard to prove that a cancer cluster exists because so few children get childhood cancer. It is almost impossible to obtain statistical significance because of the small numbers of children who develop cancer.

An exception was in the Woburn, MA case described in the book and movie, A Civil Action. In that town, a large number of families drank from a polluted water source; the Harvard Public Health Service did an exhaustive study that concluded that the specific pollutants in that water contributed to childhood cancers.

Another well-publicized area is Port St. Lucie, FL, where many more children than expected have been developing brain tumors and neuroblastomas. Another in Toms River, NJ, has received a lot of publicity.

The problem is that there are probably multiple contributors to the development of childhood cancer. For example, for children diagnosed with acute lymphoblastic leukemia (ALL), the highest incidence is between the ages of two and four. No one knows why. Are children that age more susceptible? As another example, children from more affluent backgrounds, who don't attend daycare, have a higher rate of leukemia. Some researchers believe that viruses may contribute to the development of some types of childhood cancer.

It's natural for parents to feel very guilty and upset, after a diagnosis of childhood cancer. But looking for causes can drive make parents feel crazy.

For families, it is important to remember:

• No one knows why your child developed cancer.

• There is nothing you could have done to prevent it. It is not your fault. If we knew what caused childhood cancer, we would do what we could to prevent it. But, we don't have that luxury yet.

Impact on Families

Lamb:

Childhood cancer has got to be a devastating diagnosis. How does this affect families?

Keene:

It is devastating to every member of the family.

Financially, families take a tremendous hit, even if they are well insured. There is travel, lodging, tutoring, extra services like occupational and physical therapy, and time off from work. The illness can send some families that were just barely making it into financial ruin.

Spiritually, families can have a crisis of faith, although others find that their faith helps them.

The family unit has to be reorganized. If it is a two-parent family, one parent stays at the hospital. Often, one parent quits a job.

Siblings get shuttled around and have problems. They are worried, resentful, and feel abandoned.

The child with cancer goes through a big trauma, physically and emotionally. Sometimes treatments take many months or years. If the child relapses, treatment can be for many years. Children are out of school for lengthy periods, missing both their education and friends. For many cancers, treatments can affect how children learn, in the short- or long-term. Some children have limbs amputated. Some have surgeries that affect their function.

Childhood cancer affects parents forever. If your child dies, you deal with difficult and long-lasting grief. If your child lives, your life is also fundamentally changed. If you are one of the lucky ones whose child has no late effects, the changes can be positive ones. For example, some parents make career changes or put more emphasis on what is really important to them. If you are the parent of a child who has mild, moderate or severe physical or emotional late effects, you spend rest of your life dealing with those.

Lamb:

That sounds overwhelming. How do families get through it?

Keene:

Different people find different ways. I often use a jungle metaphor: we are all walking through same jungle, but we find different paths.

Whatever path parents choose, so long as it doesn't endanger them or their child, is fine. Some choose knowledge; they learn all about the cancer, ask questions, and partner with their doctor in decision-making. Some want to get basic information and make a decision; then they want to trust the doctor and work on healing in other ways. Some want to know only the minimum necessary to sign the consent form and then find support in other ways, such as a support group, a church/synagogue/mosque, online support group, or weekend camps sponsored by cancer communities for families or children.

Still others want to identify needs and work to make improvements, so they become advocates. Some people help others, in an online group, at a hospital, or start a local support group. Some fundraise for research. Some people who have never before exercised, are running marathons to raise money for research.

There are just as many ways to get through it as there are families. Each family figures out what works for them.

Support for Families

Lamb:

It also sounds like an overwhelming situation for friends and extended family. First, how do you know what to say?

Keene:

It's best to just say, "I'm so sorry."

Then, don't say anything else. You're not going to figure out the magic words to make it alright. So many things that people say, backfire. "You're so brave." "I could never go through that." "God only gives people what they can handle." These are not helpful things for families to hear. It's usually better to just say "I'm sorry" and give a big hug.

Then, listen. Take your cue from the family. Whether or not they want to talk about the illness today will vary. If they don't want to talk about it today, it's okay to talk about something else.

Lamb:

How can friends really help the family?

Keene:

It's better not to ask if they need help. Every family with a child who has cancer needs tremendous assistance. Household help is always needed: walk the dog, do the laundry, make dinner, ferry the siblings around. Financial support can be crucial, whether for treatment or car repairs. Emotional support is always welcome.

It is often hard for families to ask for help. It is even harder to supply friends with a list of things they need. Offer to do something specific that you're comfortable doing, such as:

• "The PTA is going to make meals to bring by your house. What is the best time to drop them off?"

• "Can your older child come to our house each Friday night for dinner and a kid's movie?"

• "I walk my dog two times a day. Since you're right down the block from me, I would like to stop by your house, morning and evening, to pick up your dog for walks."

Lamb:

What about support organizations? Who do families turn to, and what can they do?

Keene:

There are a lot of support organizations.

Candlelighters Childhood Cancer Foundation is for any type of cancer. They provide education and support for parents and survivors, and also do national advocacy and awareness work.

The Leukemia & Lymphoma Society is for families of children with childhood leukemia and lymphoma. They provide basic educational materials, including a new Return to School program, and raise money for research.

Other organizations have specific support functions. CHAI in New York provides services for Orthodox Jewish families. Ronald McDonald Houses provide a free place to stay and community for newly diagnosed families.

There are several ways to find local support groups. Candlelighters has a network of 300 local support groups across the country. Leukemia and Lymphoma Society sponsors local support groups. Many Social Service departments at institutions also have support groups.

For online support, go to ACOR at http://www.acor.org.

These are just a few of the many organizations that provide support for families. Extensive lists of resources for parents of children with leukemia can be found at http://www.patientcenters.com/leukemia. For solid-tumor cancers, see http://www.patientcenters.com/childcancer.

Research Directions

Lamb:

What treatment advances are being followed up on?

Keene:

Treatment advances are incremental: each clinical trial gives us a new piece of knowledge. Right now, trials are looking at issues that affect cure rates, quality of life on treatment, and late effects from treatment. Sample issues:

• Timing of drugs. (For example, are there better results from giving a larger dose each three weeks or smaller doses every week?)

• Amount of drug. (Can we give a smaller amount of a drug and still get the same high cure rate?)

• Whether old treatment methods can be modified to reduce late effects. For instance, trials evaluated whether or not cranial radiation was needed to cure most children with leukemia. (Cranial radiation used to be common, now it is used only in a few high-risk cases; it is very neuro-toxic to children.) When my friend Dan Fiduccia was treated for Wilms tumor, 40 years ago, the extensive treatment included 9000 rads of radiation to his abdomen. He died last year from late effects of that treatment. If a child is diagnosed today with stage 1 Wilms, the child might have the tumor removed and three months of mild chemotherapy. The child would have no radiation and probably no late effects. There have been tremendous advances in treatments over the last several decades.

• Biological, genetic and metabolic research is exploding. We all hope these new approaches will dramatically improve cures while reducing toxicity.

Survivors

Lamb:

What other major issues do you see that need more research and advocacy?

Keene:

Survivors of childhood cancer need to form a powerful constituency to address their needs as a group. They can work for more research into preventing and treating late effects. They can also advocate for legislation that will help protect them from insurance and employment discrimination and that will increase education for primary care providers across the continent who will be caring for more and more survivors.

A number of factors result in few survivors getting state-of-the-art care for their late effects.

• Right now, there is no information about childhood cancer survivors in medical textbooks used in US medical schools.

• There are very few CME (continuing medical education) programs to educate pediatricians and internists about late effects.

• There is no centralized database of information where survivors or their healthcare providers can educate themselves.

• There are not enough comprehensive follow-up clinics staffed by experts to care for survivors.

I would like to see childhood cancer survivors, healthcare providers, and treating institutions work together to help survivors make the most of the lives that they fought so hard to win.

Lamb:

What do you say to people who don't want to hear that survivors might have problems? What might survivors gain by knowing that problems are a possibility?

Keene:

My image for this problem is a great, huge brick wall. The wall is the barrier to getting good follow-up for survivors. I'll describe some of the bricks in the wall (and there are many!).

One of the bricks is that survivors often feel the only emotion they should express is gratitude. They are so often told, "You are so lucky to be alive." And they are. They are grateful. At the same time, they also have a other feelings and some problems. Survivors need to be able to say, "I'm grateful to be alive and I need some help with X."

Another brick is that survivors don't have good information about own medical history. In the recent JAMA article Childhood Cancer Survivors' Knowledge About Their Past Diagnosis and Treatment: Childhood Cancer Survivor Study in the April 10, 2002 issue, the majority of survivors had no written summary of treatment. 9% didn't even know what disease they had when they were children; another 19% had some accurate information, but could not tell the precise diagnosis. If people don't know what the disease was or what the treatment was, they cannot give good medical histories to any doctor and cannot get good care. Survivors need a written summary of their disease that includes drugs used, amount of each drug, location and amount of radiation, and what follow-up is necessary. Treating institutions should provide this summary to every survivor.

Another brick in the wall is staying with the same oncologist who provided treatment for the cancer. It is sometimes difficult for a doctor who cures a child and cares about that child, to deal with any late effects caused by treatment. Also, front-line oncologists are very busy keeping up with advances in treatment; it is hard to also keep up with the growing knowledge of late effects. It is better for survivors to go to someone who is an expert in treating effects.

A further brick in the wall that prevents good medical care for childhood cancer survivors is insurance. A lot of follow-up is not covered. This is extremely short-sighted. It is far easier and cheaper to treat survivors early rather than later when a late effect becomes expensive or life-threatening.

For example, I know people at risk for heart damage from anthrocyclines who can't get echocardiograms.

Also, about one-third of girls/adolescents who received chest irradiation as a treatment for Hodgkin's disease get breast cancer before the age of 40. Because childhood cancer survivors are not on the list of those who can get mammograms outside the standard age guidelines, they sometimes can't get these crucial tests paid for.

I am hopeful that we can start addressing some of these barriers, sooner rather than later.