Art of Oncology

We are drafting an article for the Journal of Clinical Oncology for the Art of Oncology section. “Art of Oncology publishes personal essays, reflections, and opinions in the Journal of Clinical Oncology, giving readers a chance to reflect on important aspects of practice and help shape our professional discourse.” We hope it will make an impact on practicing physicians based on Raina’s experiences. I hope you will visit the article when it is published and come back to us for comments.

Here is what we are working on:

What Did We Learn?

She was giving. Friends and family, life and love, were important. Leaving happy memories and hopeful thoughts were what she wanted to do for us. We still find little notes. “Hi Dad,”  it says on a sticky note. “Limitless undying love….”, the Beatles were one of her favorites.

It has been 3 years since Raina passed away at age 27 from histiocytic sarcoma-an extremely rare and deadly cancer. Honestly, we still have not come to terms with her loss. We still shed tears. We still get angry. We still question. The pain is still overwhelming at times.

Knowing what she was facing over the three year battle she endured, she often said that, “Just think….they are learning a lot from me. “  Her mindset was that whatever she had to face, we should learn from it, and help others. So, what did we learn? Much could be said, but we wish to offer the following to the oncology community, on behalf our beloved daughter and sister.

Teaming Across Institutions for Rare Disease Care

The diagnosis was difficult to come to, from pancytopenia to histiocytic sarcoma took weeks. Even then, it was uncertain what to call it: malignant histiocytosis or histiocytic sarcoma (HS).  You quickly come to realize the true meaning of uncertainty when such a rare disease is diagnosed. To put it into perspective, only 53 patients in Raina’s age group over a period of 19 years have presented with HS.

In such rare cases, dependable established protocols, as compared to other diseases, are not there. Even the pathology may be difficult to understand interpret. Analogs and comparisons must be used to set the courses of treatment. The literature is sparse in comparison to more common diseases. The , and the knowledge pool is held by just only a handful of physicians across the country. When you sit down with your oncologist, the person in whom you will entrust your life, you already know from your own research he or she may not have all the answers.

Second and third opinions, perhaps at places far away, will become necessary. But, beyond that, you will need a team to pool the knowledge of the disease and the course treatment. The experts will be willing, and your oncologist may be as well. But, but, this may not come so easily. It must be fostered.

It’s not that the medical community is unwilling to collaborate. There are forums for sharing, journals and professional associations, yet. Yet, there are constraints and barriers too. What Treatment options you can place your hope on in may not yet be found in the open existing literature, or even in a well-structured clinical trial. There are geographical and time constraints for a small pool of experts. And, you can sense there is a competition at the institutional level arising from the desire to make a breakthrough in cancer treatment. Hence, the patient and family must reach out, foster a team approach, and make their wishes known that pooling knowledge is a priority to them in the planning and execution of treatment.

Genetics and Genetic Testing

This is not a technical article of course. But, patients and family now know that the course of hopeful treatment may depend on the genetic tests that define possible defects, mutations, alterations or fusions.  Cancer is in some sense evolution run amok.  Yet, there is of course at present just the tip of the iceberg that is known. What are the important cellular pathways that affect disease progression? How do we prevent an alteration that is identified with disease from occurring? Answering these questions is the hope for the future. Press onward and forward.

In very practical sense to take advantage of what is known depends on effective testing.  That is effective gene sequencing of the disease.  Yet, it takes weeks to get a result to inform decision making and there is still much uncertainty about techniques, such as blood biopsies. The practicalities of testing must advance as well as the basic research. Dependable results are needed quickly. The limitations of testing also need to be understood by the broader community affecting medical decisions. Let us not forget basic statistics. As we advance the state of the art, so too, we must advance the infrastructure. Training of those will affect decisions in the broader community is also important, so decisions can be made quickly.

 Expectations of Your Oncologist

As treatment moves ahead, your frontline oncologist becomes one of the most important people in your life, in taking on cancer as the enemy. You place your trust in them. They become a part of your broader family and you began to care about their wellbeing too. There is a myriad of doctors affecting and participating in your care when you are admitted to the hospital for treatment, or complications: the hospitalist, the attending, pain management, etc. But, in your mind you, however you take comfort in knowing that your oncologist is guiding everything. This His or her ongoing involvement gives you confidence and reassurance and hope that are vital to coping day to day. with the days as they unfold.

When treatment is not working any longer, honest communication becomes essential in the end. We, the patient and family, need to know what we can hope for and what we need to prepare for. It cannot be said in terms of options presented by palliative care, chaplains or social workers. What is to be expected needs to come from your the primary oncologist; the person in whom you’ve invested developed deep trust.  It The message may not be easy, and it may need to be said more than once, but. And, when the end finally comes, you want them close. The person with whom you fought the this battle with should be there in the end. It is a lot to ask.

“Time, he’s waiting in the wings, he speaks of senseless things[1].” 

No one has the right to waste one precious hour of what you have, in the face of your as you battle with cancer. From the minute you are diagnosed, your awareness of time is heightened. Yet, decisions about the course of treatment that should be taken next, sometimes become unexpectedly bogged down and take away some of the precious time you have. Insurance providers get in the way and impose constraints that often may take weeks to resolve. This needs to change through the collective will efforts of the patients and oncology community.

Third party reviews and decision makers are now the norm in allocating insurance benefits. There may be multiple parties: one to approve cancer treatment and another to approved hospitalization and testing. Inconsistencies will occur that and this serves no one and wastes valuable time. One party in the insurance decision process should not require a course of action and another disapprove the same. One party in the decision process may require genetic tests to approve medications such as trametinib, while another disapproves those same tests.  One drug maybe be rapidly approved for a course of 12 months, while another with better efficacy and acceptance across the community of experts, is disapproved.  When there is no apparent effective rationale for these decisions, you cannot endure the waste of your precious moments. If one approver suggests a course of action and another disapproves the same, how is a patient to understand the inconsistency?  How can you understand an insurance disapproval for a drug that is recommended across the community of experts?  You are aware your life depends on the decisions being made.  Inconsistencies in decision making of multiple parties in insurance benefit allocations are a detriment to patients, precipitate delays, slow access to treatment and generally produce poor outcomes. 

The Last Day

Our last day with her will never be forgotten. She had endured so much in three years, from chemo to transplant, and to the deep skeletal pain with recurrence of HS. There was a rapid decline of her state of health as HS took over her body. There were massive infusions of sugar, intubation and desperate attempts at resuscitation and her final moments of consciousness in which she could not say those last words.

Near the end, we remember she had to carry around an infusion pump in her little backpack purse. Her pain was great; yet she endured it. But we also remember she played Chopin beautifully and always smiled. We can’t change that she Raina lost her battle and we lost her. But, we can learn the lessons she taught us, to carry on her call to help others and advocate  and perhaps we can do something, collectively for those who fight on.

[1] This is a line from an old David Bowie tune. Raina loved all kinds of music.