Breathing Room

An update... Crisis... Resolution

All -

Because I am on some very serious painkillers, it is difficult for me to write. I have pasted an update that my mother e-mailed around to her friends. Soon I will take it down and replace it with a first-person version, but a lot of people have been asking for updates, so here is a third-person version.

“Dear Friends,
There’s a good reason for the saying “it’s not over till it’s over”…  
So it was with Dan - just when we thought the worst was behind us.  After 12 radiation sessions out of a total of 14 prescribed, we had to hospitalize him for severe burn to the esophagus - the cummulative effect of radiation.  The pain was so severe that morphine did not help and the painkiller he got, Dilaudid, was several times stronger.  He was in the hospital for a week, lost 10 lbs., and is still not able to eat even soft foods.  
It was a harrowing time. The pain could not be controlled through regular IV push, so they put him on a pump where he could release the drug every time he felt the pain coming on, in addition to a baseline that was continuously delivered.  He could not speak (it hurt) and the only thing that he could take in was water.  
Dan was released Thursday last week, completed the 2 remaining treatments and is now recuperating at home.  He is very weak, but slowly improving.  The only food he’s able to have is very thin vanilla milk shake that I mix with protein powder, multi-vitamin, and other supplements.  He is on Methadone (they weaned him off of the Dilaudid) which is also an opiate. This will take many weeks, during which time the esophagus will hopefully heal.  We won’t know for another week or so if the last 2 treatments will worsen the situation again.
This child has gone through more torture in 9 months than most people go through in a lifetime!  I have no words to describe how strong he has been through it all, despite the horrible pain and the fog of drugs.  He had the doctors in stitches with the tale of a comedy of errors on the part of the nurses one night, which really was a particularly tough night for him.  I would have fallen apart a long time time ago if he had not been the way he is. “

9 months ago

Medical Update: 3rd Official Opinion, Clarification

I am writing from Penn Station in sunny Baltimore, MD. There is a big art festival here today, and the town is clearly bracing for a massive flood of humanity, but it’s early enough that no one has showed up yet (not even the exhibitors). So here I sit, enjoying the calm before the storm.

Before I go investigate a car shaped like an old-fashioned telephone, I figured I would post a medical update.

Issue 1: Tumor Histology Controversy: when I was first hospitalized, a sample of my tumor was taken through an incision in my neck. The biopsied tissue was analyzed by the pathology lab.

By far the most important question was: is this lymphoma? Even without a microscope it is possible to know that a mass is lymphoma. I know this because I was conscious while they sliced my neck open, and the head surgeon described how to differentiate between teratomas, neck and glandular cancers and lymphoma to a resident, using me as an exemplar. Once “yes/no lymphoma” is resolved, the logical question becomes “which kind of lymphoma” – and there are several: Hodgkin’s, Non-Hodgkin’s, Burkitt’s, B-cell Lymphoma, Mucosa-Associated Lymphoid Tissue (MALT) Lymphoma, and others. It is actually fairly easy to distinguish Hodgkin’s from other types of disease. For decades, scientists knew that Hodgkin’s existed, and knew that radiation and certain kinds of chemicals (based on chemical weapons used in World War I, interestingly) would kill it – but they did not know whether it was a cancer. This is why it is sometimes called Hodgkin’s Disease, rather than Hodgkin’s Lymphoma. Two scientists resolved this by noting that under a microscope, it was possible to see very large, distorted cells that impacted and distorted nearby healthy cells. These abnormally large cells were named after their (independent) discoverers and are called Reed-Sternberg cells. You can see them here:

11 months ago

But even after the pathologists have differentiated a Hodgkin’s mass from other kinds of lymphoma, there are several sub-types of Hodgkin’s Disease. Curiously, there was a time when the sub-types were viewed as different diseases and treated as such. Currently the thinking is that there are 5 distinct sub-types, three of which are quite rare, and all of which can be treated basically the same way. When my tumor was analyzed and a decision was reached on the sub-type, it was called “mixed cellularity.”

When I saw the lymphoma team at the NIH, they examined me and went over my records, and also submitted leftover tissue from my biopsy to their pathology lab. Since the NIH uses biopsied tissues for research, and it is critically important that they assign the right tissues and patients to the right studies, they have a pretty good lab. In fact, the NIH doctors claimed that the world’s premier Hodgkin’s pathologist works in the lab (although that doesn’t mean he worked on my tissue). On Thursday, I got a call from a doctor at the NIH who said that the lab results came back as “Nodular Sclerosis” sub-type, e.g. not mixed cellularity.

This is a good news/bad news situation. On its face, it is good news. Nodular Sclerosis is the “classical” presentation of Hodgkin’s. Survival rates are higher, and recurrence rates are lower for patients with Nodular Sclerosing Hodgkin’s. Mixed ceullularity is considered a negative prognostic indicator. When a patient under 40 has a large tumor in the chest, it is nearly always Nodular Sclerosis-subtype. Since I am under 40, and I had a large tumor and it came up as mixed cellularity, it was perceived by my doctors as a very strange, aggressive form of the disease. So the bad news is… the lab was wrong, and my doctors were operating under a false assumption, for reasons that were never explained to me.

I reacted to this with in the stoic, tactful manner you have all come to expect from me: at my latest doctor’s visit I asked “how the f*** is it possible that they got this wrong?”

Apparently, oncologists are not used to hearing the f-word. The answer, it turns out, is pretty simple. Only about 1% of a Hodgkin’s tumor is made up of abnormal cells. This is one of the reasons that the disease baffled everyone for so long. It seemed like people were dying of a condition where their normal immune (lymphatic) tissues kind of bunched up into clumps and stopped working.  The implication of this fact is that if you take a Hodgkin’s tumor, and slice off a chunk of it and put it under a microscope, it will inevitably contain a bunch of different kinds of cells, including a very large number of healthy cells. Therefore, it is easy to mistake other sub-types for mixed cellularity. Such errors are not necessarily indicative of incompetence. What probably happened is that by sheer coincidence, the section of tumor used to determine the histology (cell-type) initially did not happen to contain sclerotic cells. When the NIH did their analysis, they either used a more tissue, or got “lucky” and saw a bunch of sclerotic tissue.

My follow-up question, “is this screw-up going to f***ing kill me?” was also greeted with amused reassurances. Mixed cellularity diagnoses are treated as negative prognostic indicators, but there’s a bit of a chicken-and-egg problem with treating it as a straightforward indicator. That is because mixed cellularity tends to appear in tumors that have left the upper chest (e.g. advanced, metastatic disease), in older patients, and in AIDS patients. It is not clear that higher death rates among these patients are the result of mixed cellularity per se (and not their age, AIDS, and/or advanced disease).

Issue #2: Polarized Disagreement about My Treatment: You might recall that my doctor at Georgetown was adamant that a) I will have a recurrence of my cancer without radiation and b) radiation is worth the risks, while the NIH doctors said a) I am very unlikely to have a recurrence and b) radiation is really dangerous. The wide gulf between their opinions was, to some extent, my and my mom’s fault – we knew that my oncologist follows an aggressive treatment philosophy and that the NIH has been leading the charge to scale back the use of radiation on Hodgkin’s patients.

Nonetheless, I was surprised at the vehement, borderline-hostile nature of the resulting disagreement. For a non-scientist, it is very frustrating to see two teams of doctors who read the same studies, have the same degrees in the same field look at the exact same data and reach the opposite conclusions. Since I work in the social sciences, I’ve unthinkingly accepted a somewhat idealized portrayal of the “hard sciences,” where one can perform controlled, randomized experiments. I expect hard answers from hard scientists, and it infuriates me when I don’t get them (insert childish foot-stamping here).

One of the first things I demanded when I met the doctor who supplied my third (tie-breaking) opinion was an explanation of why the other two opinions were so different. The first answer was disturbingly… sociological: doctors can get sucked into dogmas that are similar to ideologies. When you think cancer should be treated with a hammer, all the tumors look like nails. The second answer was a bit more disturbing, because it involves a medical mistake (see #3).

Issue#3: Bulky Or Not? One of the core arguments made by the NIH doctors was that my disease did not meet the “bulky” standard for automatic radiation treatment. It is standard practice to use radiation on tumors that are large enough to take up 1/3^rd of the maximal diameter of the chest, or are greater than 10cm in diameter. Because bulky disease is harder to cure and more likely to recur, the rationale for radiation is strong in such cases. The NIH doctors claimed that my tumor was 8.5cm at the most, so it didn’t qualify. Hence if this argument is correct, even a conservative/aggressive oncologist wouldn’t be justified in irradiating me. If you read the “informal opinion” e-mail I posted here, you will note that the doctor specifically refers to my disease as “non-bulky.” Thus, both “no radiation” recommendations assumed non-bulkiness.

It was not until I went to see the oncologist who wrote the informal opinion at Massachusetts General Hospital this Friday (see: http://www.massgeneral.org/cancer/locator/search_clinician.asp?id=488) that this got cleared up. This doctor gets super high marks from me for asking me to tell him, from start to finish, how I perceived what had happened to me, from beginning to end. Other doctors consult files (written by other doctors) and might even read parts of the file aloud and ask the patient to confirm them. But this particular doctor put away the files (which he had read even before meeting me) and asked for a first-person narrative account. When I provided it, he realized that he had made a critical mistake: he based his opinion on the wrong tests.

This requires an explanation, which I will offer forthwith - feel free to skip this paragraph if you want to. In a normal case of cancer, the earliest, the best and most comprehensive scans are the ones used for staging. The initial scan is used to determine the stage of the disease prior to treatment, and follow-up scans are used to measure the effects of surgery/chemo/radiation. In my case, this did not happen. When I arrived at the hospital, my major veins and arteries were being twisted around by a big tumor, and it was compressing my trachea: my airway was 4mm wide. (Thought experiment: imagine spending two weeks breathing through a straw less than half a centimeter in diameter. That was me circa 12/2007) This presented a number of very serious health risks. To avoid brain damage, hypoxia, death, etc., even before they knew what kind of cancer it was, they were doing things to shrink the tumor and open up my airway. I got high doses of steroids and two radiation treatments. Only after my airway had been cleared up, did they formally stage the disease.

So, my first staging scan happened after I had been treated with steroids and radiation for about a week. As a consequence, the tumor had already shrunk from its maximum diameter in the staging scan. So, the Mass General doctor who wrote the informal opinion re-measured the tumor, checking its diameter in my first (non-staging) scan and found that it was well over both the 1/3 of chest diameter and 10 cm. requirements for bulkiness.

When he factored bulky disease into his analysis, his conclusion changed. He now thinks that to not perform radiation would be a serious gamble. He estimates a 15% probability that I will have a recurrence if I do not undergo radiation (or, an 85% chance I am “cured”). Survival rates for recurrent Hodgkin’s are about 50%. Hence, without radiation, there is a 7.5% probability I will die. No one has ever performed a study comparing the survival and recurrence rates of patients with bulky disease who receive radiation and chemotherapy to chemo alone. However, the fact that no one was willing to do such a study is suggestive – no researcher thinks it is worth the risk. Furthermore, none of the long-term radiation side-effect studies account for technological innovations in radiation therapy that have occurred in the last decade. So no one really knows what my odds are in either scenario (e.g. the hard scientists are guessing about my life/death). Either way, this doctor, who clearly doesn’t hesitate to counsel against radiation, now thinks I should get radiation. It is unclear whether the NIH team would change their conclusions if they realized they were looking at the wrong scan. I’m not sure if that really matters: if they reevaluate the evidence in light of bulkiness and change their minds, I should disregard their negative opinion and get radiation. If they don’t reevaluate, that suggests that they are ideologically opposed to radiation (e.g. biased) and I should get radiation.

I’m not sure what I will end up doing, but I’ll probably decide soon. If you have a strong opinion, feel free to e-mail it to me, and I’ll thank you and feel free to accept… or disregard it.

Sidenote: thanks to everyone who has e-mailed me recently – I’ve been too caught up in stuff to respond, but I do plan to get back to you.

11 months ago

Medical Update - 3rd (but unofficial) opinion

I recently sent my medical records on to a third hospital for a tie-breaking opinion. I’ll get examined by this doctor soon, so you’ll all hear about that in a few days. But I wanted to post the INFORMAL PRELIMINARY opinion e-mailed to me. Since it’s speculative and unofficial, I have excluded the doctor’s name and identifying information.

I’ve reviewed the medical records and scans, and am certainly delighted to see evidence of a complete remission following ABVD therapy.  Obviously, I cannot render official impressions and recommendations without seeing, speaking to and examining [you], and so my impressions here are informal and based on the following assumptions:   It appears to me… that you have stage IIB nonbulky classical Hodgkin lymphoma and completed 6 cycles of ABVD chemotherapy at full dose, and have achieved a complete remission. I see that at least one dose of ABVD was delayed due to a neutrophil count of 700, but I am assuming there were not frequent dose delays (it is my practice to never hold ABVD regardless of how low the WBC is, which is quite safe and does not require the use of Neulasta).  I am also assuming that a PET/CT scan performed after 2 cycles of ABVD showed resolution of FDG avidity, which is what is stated in a note by Dr.XX, but I do not have a mid-treatment scan to review, only the pre-tx scan from December and post-tx scan from June.

The most important thing to recognize from my perspective is that the prognosis here is excellent, regardless of whether additional therapy is provided or not.  This is based on the fact that limited stage Hodgkin lymphoma is quite curable in general, but also because a negative PET scan after 2 cycles of treatment has been shown to be the most powerful predictor of a favorable outcome.  Recent Italian data has demonstrated a 95% chance of cure if the PET scan is negative after 2 cycles, and this analysis included patients with more advanced stage disease than yours.  The real question is how to best achieve both long term survival along with reducing treatment-related risks.  This is a critical consideration where mediastinal radiation is concerned.

The standard therapy for limited Hodgkin lymphoma for many years has been chemotherapy followed by involved field radiotherapy.  Radiotherapy in young people, however, has raised concerns due to the significant long term risk of second cancers including lung cancer, leukemia, sarcoma, throat cancers, thyroid cancers, breast cancers and more.  By 15 years after completion or radiotherapy, the chance of dying from such a secondary cancer eclipses the chance of dying from Hodgkin lymphoma, and the overall chance of developing a secondary cancer after radiation for Hodgkin lymphoma is approximately 25% by 30 years after radiation (you’ll only be 55)!  Further, mediastinal radiation induces a significant amount of premature heart disease including coronary artery disease and valvular heart disease, with a recent analysis showing that greater than 20% of patients treated with mediastinal radiation develop heart disease within 15 years, and I suspect this is an underestimation. There are other consequences too, including potential for lung fibrosis, decreased salivation and tooth decay, and thyroid dysfunction.  Given these observations, 2 randomized trials have evaluated chemotherapy alone with ABVD versus combined chemotherapy and radiation.  These trials have found an approximately 5-7% increased risk of relapse with the omission of radiation, but no difference in overall survival.  The reason there is no difference in overall survival (death rate) is that many patients who relapse with Hodgkin lymphoma may be cured with second-line therapy.  This data also implies that 93-95% of patients definitely do not require the radiation and are therefore needlessly exposed to toxicity. These studies also have not matured to the 15 year follow up stage where secondary malignancies and heart disease in the radiotherapy-containing arm would begin to affect the data.  As such, both strategies appear safe and effective in the short term, while secondary complications due to the radiotherapy would almost certainly be reduced with the chemotherapy only approach once enough time passes to observe this.

Given this emerging data, it is my practice in young people to avoid radiotherapy, particularly to the mediastinum, if at all possible.  This is only an acceptable approach for limited stage non-bulky disease, since bulky disease patients (disease measuring greater than 10cm or a third of the maximal diameter of the chest) have never been included in the aforementioned trials, and still should receive radiation.  That does not appear to be the case with you… It appears to me that you have nonbulky limited stage disease that achieved a complete PET response after 2 cycles of ABVD, denoting exquisite chemotherapy sensitivity.  You then received 4 additional cycles of chemotherapy with post-treatment scans demonstrating a complete remission of the disease.  Based on the available data, I think that the inclusion of radiation now is unlikely to improve your chance of long-term overall survival, which is obviously excellent, but is likely to increase your risks of long-term toxicity including heart disease, lung disease, and secondary cancers.  Given your young age, the focus now must be on both curing the Hodgkin lymphoma and protecting you from additional adverse problems down the road.  Accordingly, my informal recommendation is to not undergo radiation therapy— I think it is likelier to harm than to help, and think you have an extremely high probability of being cured today with no further therapy.  Instead, you should throw back a few beers with your friends and family and celebrate your remission and excellent chance of long term good health, and feel proud of yourself for what you’ve just come through.

11 months ago

Cat? Or vengeful Russian woman? You decide.

I keep telling people that my cat is smarter and meaner than me. People who haven’t lived with her don’t believe me. So I took some pictures to prove my point.

This illustrates just one Anastasia Cycle: cuddling —> anger at unscheduled end of cuddling —-> retaliation —-> gloating.

11 months ago

Shameless Plug for My Favorite Book #5 - Clumsiness

Of all the thousands of books I have read, Chuck Amuck is my favorite. It is by Chuck Jones, one of the geniuses behind the Golden Age of Warner Brothers cartoons (that would be Daffy Duck, Bugs Bunny, and Elmer Fudd, not Minnie Mickey and a Duck with a speech impediment).

My favorite part of my favorite book is a description of where Wile E. Coyote (one of the author’s creations) came from. I think these are the two best-written and most entertaining paragraphs I have ever read.

“The Coyote is a history of my own frustration and war with all tools, multiplied only slightly. I can remember that my wife and daughter would start to weep bitterly and seek hiding places whenever they saw  me head towards the tool drawer, if only to hang a picture. I have never reached into that devilish drawer without starting a chain of errors and disasters of various but inevitable proportions. Like any other man, I would rather succeed in what I can’t do than do what I have successfully done before. I have never reached into that drawer without encountering one of those spiny things you stick flowers in. We don’t keep that thing in that drawer, but it is always there. I consider it a good day when I get only one spine under a fingernail. I tried to get the spiny thing out of the drawer once, but found out that the last time, when it had stuck to four fingers at once and been in fact lifted a few inches out of its nest in the resulting shriek, it had fallen on a tube of glue, puncturing the tube and affixing itself to the drawer for all time.
I have tried lackadaisically from time to time to remove it and have succeeded in breaking: a rattail file, a kitchen knife, three fingernails, a pair of manicure scissors, an eggbeater (in one of my more fanciful efforts), and a window, when the tail of the rattail file separated from the rattail file.”

11 months ago

Shameless Plug for My Favorite Book #4 - Stupidity

The man, Ray Katz, in the story is a “business manager” at an animation studio. Having encountered other individuals with similar titles, I am in no way surprised that he is a complete idiot.

“Anyone but Ray Katz would gather then that flipping a sheaf of drawings is the classic test for the animator. Having completed a series of drawings for a given piece of action, before the in-between drawings are added, the animator becomes his own test camera by flipping his drawings, just as he did in grammar school. However, this constant and repetitive flipping by every member of every unit, sixty or seventy people, continued to be a mystery, a puzzlement, an enigma to Mr. Katz.
Eighteen years of observation eventually bore fruit: Mr. Katz decided to avoid licking this whole matter of flipping by joining it. He too, would flip.
No sooner said than done. One fine memorable morning, with the enormous confidence born of sheer ignorance, he strode into our music room, where two directors and story men had joined our composer Carl Stalling to go over the score of a soon-to-be-recorded film. The music of this film, thirty or forty pages of bar sheets, rested comfortably on the desk top as Ray Katz walked in, determined to become one of the boys: if they could flip, he could flip. Casually, he picked up the unoffending music score and, under the fascinated and glazed gaze of those present, moved to the window for better light and carefully flipped the music score two or three times. Then, nodding and grunting his appreciation of the artistry therein, he departed, clucking to the group on his way out. The term “dumbfounded” found new meaning that day, as did “delight.”
And that is how it came about that every succeeding music score was presented to Mr. Katz to be flipped for his endorsement and his professional and artistic approval.”

11 months ago

Shameless Plug for My Favorite Book #3 - On Failure

When (not if) you fail, you are in good company - Charlie Chaplin, Peter Sellers, Jack Benny, Wile E. Coyote, Sylvester the Cat, Elmer Fudd, and (my favorite) Daffy Duck are loved precisely for the universality of their failures. Failure is the most human and humanizing of experiences.
“Father loved his children but hated having a family. He became belatedly aware one dismal rain-struck morning of the painful reality of his fatherhood: that he was up to his hips in children, and unless he wanted to blame those selfsame hips which were, after all, responsible, he must do something to rectify matters. In short, get out.
Now, all that remained for him was the technical difficulty of how to do so. We were far from a wealthy family. Indeed, a history of Father’s fiscal meanderings would make a valuable contribution to the “What to Avoid” chapter of any “How to Succeed” textbook. In short, he didn’t have enough bread to supply bread for his family for the next 12 to 15 years, much less provide all the gear, from garter belts to saddle shoes, necessary to see the self-respecting child through high school and perhaps, college.
The only solution seemed to be to strike it rich. Then he could run for the hills, secure in the knowledge that his family was fiscally secure…
Opportunities for immediate wealth were a dime a dozen [in 1920’s California] - and worth approximately that. Father tried them all, plus some introductory ideas of his own: he formed companies that attempted to sell avocados when people called them Alligator Pears and thought of them as either poisonous or Communistic, or both; he offered vineyards for sale when Prohibition was in full astringent swing; he took a short option on a place called Signal Hill and tried frustratingly to grow geraniums there for the Eastern market, only to discover that his floral non-fecundity was caused by crude oil saturating the soil. Where flowers should have blossomed, crude-oil rigs sprouted instead, long after Father’s geraniums and options had withered away.” 11 months ago

Shameless Plug for My Favorite Book #2 - Mother's.Uncle's Advice

My mother says this to me all the time. It is both absurd, and insightful.

“A dear uncle told me once, when I was deep in despair at some injustice by some bureaucrat, scholastic or familial, “Chuck, they can kill you, but they’re not allowed to eat you.” Exactly why this statement has since stood as the cornerpost of my determination to live my life as a life and not as an apology, only Ralph Waldo Emerson could have explained. And I hadn’t read much Emerson when I was eleven.”

11 months ago

Shameless Plug for My Favorite Book #1 - Selfishness and Honesty

This is a description of the author’s 6th birthday. Since I pulled a nearly identical move at the same kind of party and our birthdays are one day apart, I empathize:

“I was immensely proud – it seems to me that all my life I have taken the most pride in things over which I have little or no control. Even though I had older sisters, it never occurred to me that anyone had ever become six years old before, and the splendid cake, candles bravely ablaze in salute to my maturity, was ample evidence that I had entered into manhood.

Having blown out the candles, and, as a side benefit, managing to send most of the smoke up my little brother’s nostrils, I was handed the knife, my first baton of any kind of authority in six misspent years, and was told to cut as large a piece as I liked. At this point Daffy Duck must have had, for me, his earliest beginnings, because I found to my surprise and pleasure that I had no desire to share my cake with anyone. I courteously returned the knife to my mother. I had no need for it, I explained; I would simplify the whole matter by taking the entire cake for myself. Not knowing she had an incipient duck on her hands, she laughed gently and tried to return the knife to my reluctant grasp. I again explained that the knife was superfluous. It was impossible, I pointed out, with incontrovertible logic, to cut a cake and still leave it entire for its rightful owner. I had no need and no desire to share.

My father thereupon mounted the hustings (he was nine feet tall and looked like a moose without antlers to me) and escorted me to my room to contemplate in cakeless solitude the meaning of a new word: “selfish.” To me then, and to Daffy Duck now, “selfish” means “honest but antisocial”; “unselfish” means “socially acceptable but often dishonest.” We all want the whole cake, but unlike Daffy, and at least one six-year old boy, the coward in the rest of us keeps the Daffy Duck, the small boy in us, under control.

“You may cut as large a piece as you want” is a dangerous euphemism. There is a prescribed wedge on every birthday cake that is completely and exactly surrounded by corporal punishment. Exceeding these limits by even a thousandth o fan inch brands one as “selfish.” From my seventh birthday on, I learned to approach with judgement sharper than a razor’s edge this line, without cutting the “un” from “unselfish” to “selfish.” I learned very little about social morality, but a great deal about survival, and this, after all, is what Daffy Duck is all about.”

11 months ago