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Ella's Medical History

Ella was diagnosed with medulloblastoma five days before she turned two. Medulloblastoma is the most common childhood brain tumor, but it is still a rare diagnosis; most pediatricians will only see one or two cases of childhood brain cancer in their entire careers. Ella contracted a very rare, more aggressive variant, called large cell anaplastic medulloblastoma.

Two days after she was diagnosed a neurosurgeon removed the primary brain tumor, but the cancer remained over a large portion of the surface of her brain and in her spinal column. To battle the remaining cancer, Ella began chemotherapy on a Children's Oncology Group (COG) treatment protocol called ACNS0334, undergoing two rounds of Methotrexate, Leucovorin, Vincristine, VP16/Etoposide, Cytoxin, MESNA, Cisplatin and G-CSF. She also had stem cells harvested with the intention of using them in the second part of the protocol.

The chemotherapy worked initially, with over a 50% reduction in cancer after the first round of treatment. But the cancer adapted to the drugs, and an MRI after the second round of treatment showed that the cancer was returning quickly. Ella slipped into a coma within weeks, and passed away a couple of days thereafter when we removed her from life support.

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Disease and Treatment

The name medulloblastoma refers to the location and classification of the cancer. The tumor originated in the medulla oblongata, which is located adjacent to the fourth ventricle of the brainstem. The classification of cancer, blastoma, is of unknown genetic origin, but it can occur in the center of the brain (pineoblastoma), in the nervous system (neuroblastoma) and even in the retina (retinoblastoma). There is evidence that the genetic markers for these types of cancer exist in the patients at birth and work in concert with the genes that control initial brain growth, thus their unusually early presentation in human life.

Pediatric brain tumors are rare, but extremely dangerous to children under three. For instance, standard-risk medulloblastoma is overcome in more than 80% of cases in older children, although the treatments may cause significant permanent side effects. For children under three, such as Ella, the prognosis is much worse. The primary reason is that young children cannot tolerate radiation, and suffer tremendous brain damage from radiotherapy compared to older patients.

Ella's oncologist told us that treatment with the standard dose of radiation would eventually render Ella to a permanent infantile state, likely unable to tie her own shoes and limited to a vocabulary of only a few words for the rest of her life. In addition, the radiation was not a sure cure, and the damage it would cause to non-cancerous cells could cause new cancers that would emerge later in Ella's life.

Because of these consequences, high-intensity chemotherapy is the standard line of defense prescribed by physicians to patients like Ella, even though it's not nearly as effective as radiation and chemotherapy combined. While it offers virtually the only hope in established treatments for curing the cancer, its toxicities have brutal side effects on young children. Oncologists and parents have to decide if the tremendous suffering under chemotherapy and its lower chances of successful outcome outweigh the longer-term, severe, permanent side effects of radiation, which offers a higher chance of a cure.

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The Situation Today

Tragically, there is no third, better choice, unless if you believe in some of the alternatives offered by the shadowy world of south-of-the-border treatment centers and by other "cures" that have no peer-reviewed, scientific substantiation.

Why is there no better choice? Part of the answer is that the rarity of these cancers in children three and younger means that comparatively little attention has been paid to developing new treatments for them. For instance, the main chemotherapy drug that Ella was being treated with, Methotrexate, was first used in the 1950s. While some advancements in brain tumor treatment have been made in recent decades, particularly with stem cell transplants, much needs to be done. For all the danger and side effects we put children through in treatment, we should be able to guarantee them a higher cure rate.

Another reason there is no better choice is that the cure for almost all cancers remains elusive. The study of childhood brain tumors would help to further the understanding of all cancers, since many adult cancer treatment strategies are also stuck in antiquated chemotherapy and radiation strategies that work by poisoning good cells as well as the bad.

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Controversial Relationships

Unfortunately, the incentives may not be there to find the best cures quickly, or even to pay attention to promising therapies that do not offer possibilities for high profit.

The drug companies make millions of dollars from the sales of chemotherapy drugs, and as evidenced by a May 9, 2007 NY Times article, oncologists are not above taking incentive payments for administering drugs, even when those drugs are considered unsafe.

For instance, according to the NY Times article, doctors have been accepting millions each year in kickbacks to prescribe Aranesp, Epogen and Procrit, drugs that the FDA have shown are not proven to be effective and may shorten patients lives when prescribed at high doses, which is a common practice. At one clinic in the Pacific Northwest, a group of six cancer doctors received $2.7 million from Amgen for prescribing $9 million worth of Aranesp and Epogen last year. Johnson & Johnson has been accused of making similar payments.

In addition, even non-profit, advocacy organizations may not be representing patients' interests as objectively as they should. In a January 24, 2008 article, the NY Times raised a question regarding whether the American Heart Association was acting in the public's best interest regarding the drug Vytorin. A press release from the association criticized a study of Vytorin, but did not state that the organization receives nearly $2 million a year from Merck/Schering-Plough Pharmaceuticals, the joint venture that markets Vytorin. How does this apply to cancer patients? Dr. Sidney M. Wolfe, the head of the Public Citizen's Health Research Group, a consumer advocacy group, asserts that the drug industry underwrites many professional medical organizations, which would undoubtedly include some cancer advocacy organizations.

This situation can leave cancer patients and their families feeling as if no one is watching out for them. On one side, the doctors appear to be using treatments that are outdated, inhumane and ineffective. On the other side, there are entrepreneurs and fanatics who are ready and willing to offer their own scientifically and statistically unproven "cures," which are hawked chiefly through personal testimonials and pyramid schemes.

Most oncologists also appear to have no opinion or even interest in nutritional or supplemental treatments, although many parents and adult patients instinctively feel these are important. Instead, patients must wander in a thicket of information mostly found on the Internet and often on sites run by entrepreneurs, some of who are clearly unscrupulous.

Many hospitals have nutritionists on staff, but their knowledge specific to cancer may be limited. Patients can hire independent, professional nutritionists who specialize in cancer treatment, but their fees are generally not covered by insurance.

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Finding a Cure?

Government and consumer monitoring of non-profits accepting donations toward cancer research can be insufficient. While Americans pour money into cancer research through direct donations, fundraising events, the Combined Federal Campaign and other avenues, organizations may not be forthright, or even honest, about disclosing such things as where these donations go.

Recently, the Chronicle of Philanthropy reported on nonprofit managers who exploit the Combined Federal Campaign (CFC), which is a giving program for government employees that generates millions of dollars for non-profits each year. One consultant runs a site that to us appears to look as if it is an official government site: www.bestcfc.org. This site purportedly reveals the "very best cancer charities," but the only charities listed are those of the consultant's clients, who are certified as the "very best" through a certifying organization affiliated with the consultant. Meanwhile, a network of non-profit administrators exploit the CFC by creating shell organizations to increase CFC visibility and then inaccurately represent where donations are directed.

The Chronicle has also shown that reporting by fundraising organizations can be so inconsistent that it is difficult to know the administrative fees associated with running fundraising events, and whether these events are worth the organizations' and volunteers' efforts.

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There is Hope

Despite these hurdles, there is hope. Research on medulloblastoma falls under roughly two categories: clinical research and basic science.

Clinical research is meant to save the lives of patients using tools already in existence, which for pediatric brain tumor patients usually means experimenting with various surgery, radiation and chemotherapy combinations. So, for instance, although Ella was treated in a "new" study, it really was just a new combination of existing chemotherapy drugs. A more radical example includes the daughter of basketball star Derek Fisher, who underwent an experimental treatment in which the chemotherapy was delivered directly into her eye. Another example would be the use of focused radiation, such as a gamma knife, in which cancer cells are specifically targeted instead of the shotgun approach of regular radiation, in which healthy cells are as vulnerable as cancer cells.

These studies are important, and especially in the case of radiation there is great progress being made. But there is also a certain stagnant quality about them, because they keep drawing from a limited deck of cards to treat symptoms. The surgery/radiation/chemo mix offers methods of suppression that sometimes eliminate all cancer cells, but they do not offer a cure that gets to the root of the problem.

That said, they can help to save patients immediately and sometimes yield great gains, and therefore should continue to be pursued. But basic science is where the real key to the cancer cure lies.

Basic science is a misnomer; it's basic because it goes to the very fundamental core of the disease and its relationship to human physiology: it's the study of the disease's genesis and method of dissemination. When that knowledge is furthered, it can lead to what is called "translational research," which takes the new basic science knowledge to lead to a drug or other cure.

Cancer researchers face two very large hurdles: the disease itself can be difficult to understand, and because they involve cells in humans, they are usually very sophisticated at resisting cures. So, for instance, when you hear about someone taking an HIV "cocktail" of drugs, they are actually taking multiple medications that attack the virus in different ways. The virus is plastic enough that if you just presented one attack, it would figure out its way around the roadblock and continue to adapt and reproduce. With a cocktail, you are presenting enough different methods of attack that you essentially back the virus in a corner.

Cancer cells act with the same resilience. They reproduce quickly and therefore adapt quickly. The more basic science that is done, the better researchers will understand the disease, and the more possibilities they will have for actually eliminating it. But right now, basic science research funding in childhood brain cancers is scarce. In large part, this is because childhood brain cancers are rare and therefore not a national priority, but the system is not operating as well as it should, either. The National Institutes of Health, which includes the National Cancer Institute, has had cuts to its programs under the Bush administration, and funding is much scarcer than it should be.

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How You Can Help

Clearly, the system is not working optimally. But the goal is worthy-to cure cancer in children and adults. Please do what you can toward this end by helping in these powerful ways.

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