Without a Sound

 When a glass or a ceramic plate is dropped, it makes a loud noise as it crashes on the ground.  When a window shatters, a figurine falls off a shelf, or a picture falls off the wall, it makes a noise.  Why not, then, a heart when it breaks?  It doesn’t seem right that something so devastating, so life changing, can occur without a sound.   

A lot of people have asked me over the past few days what my thoughts are about what happened with Brittany Maynard, the 29 year-old woman who is being referred to as the face of “Death with Dignity” after she chose to hasten the end of her life after she was diagnosed with Glioblastoma Multiforme (GBM).

GBM is something about which I, unfortunately, have personal experience and knowledge, as it is same diagnosis that my dad had.  I am not a physician, but I will do my best to provide accurate and relevant information about this diagnosis below as I think it’s important to know the facts about this diagnosis before jumping into the arena of discussion about Brittany’s case:

GBM is a primary brain cancer, which means it originates in the cells of the brain, as opposed to a secondary brain cancer, which begins elsewhere in the body and then spreads – or metastasizes - to the brain.  Of the many different kinds of primary brain cancers, all of which are rare, GBM is the most common … and the most aggressive in adult humans.   It is considered to be incurable, and, in the vast majority of cases diagnosed in the U.S., survival after diagnosis is less than two years.  The average life expectancy for an adult diagnosed with GBM is 14 months, and, as my family discovered when my dad died only ten weeks after his diagnosis, sometimes even that is too much to hope for. 

When a mass in the brain is discovered by a scan and a biopsy of the tumor is taken and analyzed in the lab, there are two things the pathologist looks at: 

• the type of brain cell from which the tissue came, which gives the tumor a name like GBM
• the speed at which the cells are dividing, which results in an assignment of a grade for the tumor, such as Grade III [3] or Grade IV [4].  In general, Grade I means that the tumor tissue is “low grade,” or very slow-growing, whereas Grade IV indicates that the tumor is growing at a very aggressive rate. 

To make things even more confusing for most of us, although Glioblastoma Multiforme is a primary type of cancer, there are two types of glioblastomas, the first of which is called “Primary, or de novo, GBM.”  This kind is the most common form of glioblastoma and is highly aggressive from the moment of discovery.  The second type of GBM (misleadingly also classified as a primary brain cancer) is called Secondary GBM; this kind begins as a lower grade tumor and then as it develops gets "upgraded" eventually into a GBM. Secondary GBM tumors have a longer, relatively slower growth pattern – but they are still considered to be very aggressive in that they are very difficult to treat.  This type of brain cancer, which accounts for about 10% of all GBM’s, tends to be found in people 45 and younger.

In the words of the neurosurgeon who handed down the news of the cancer to my family: “All GBM’s are Grade IV.”

                 

Some reports in the media about Brittany Maynard’s case seem to be confusing two different parts of the pathology, though: Tumor grade is not the same as the stage of a cancer. Cancer stage refers to the size and/or extent of the original tumor and whether or not cancer cells have spread to other sites. Cancer stage is based on factors such as the location of the tumor at the time of diagnosis, the size of the tumor, and the number of tumors (or "tendrils") present. There are even more scales of different types used for determining the grade and/or stage of certain types of cancers like pancreatic: suffice to say that there is so much information about cancer in general that it's not difficult to see why there is a lack of awareness - and some confusion - amongst the media and the general public.  In fact, the same goes for the medical community: as far as I know, except for the oncologist and a few nurses on the oncology floor of the hospital ... and maybe the neurosurgeon, the majority of the medical professionals that treated my dad knew little to nothing about GBM. Unfortunately, I don't think this was atypical; not long ago I went to a doctor as a new patient for a check-up and, when she asked me about my family history, of course I told her about my dad's diagnosis of GBM.  "Was he sick for a long time?" she asked me, and when I told her no, that he was in great shape, in fact he was in training for a triathlon, she responded, "Oh, so he probably caught the parasite from swimming in a lake?"  Um, no, GBM is not a parasite, I told her.  But I digress ...

GBM tumors can occur in any area of the brain but are most often found in the frontal and temporal lobes [My dad’s tumor was in his right parietal/temporal lobes].  One characteristic of GBM is that these tumors tend to be infiltrative, meaning they spread into other parts of the brain (usually very quickly).  I remember the neurosurgeon explaining to us that GBM tumors, unlike most tumors that have smooth edges, have irregular borders – and then for effect he held up his left arm with his elbow at a 90 degree angle so that his left hand was dangling down in the air, and then he used his right hand to point to the fingers of his left which he said were very similar to the multiple “tendrils of tumor” in my dad’s brain, “finger-like projections, if you will,” he said. 

As the neurosurgeon also informed us on the day of my dad's diagnosis, although GBM can spread throughout the central nervous system (the brain and the spinal cord), it almost never spreads to other areas of the body.  

What the doctor didn’t tell us – what no medical professional did – but what I discovered through spending a lot of time researching on my own is that there are certain “prognostic indicators” with GBM, essentially things that speed up the ticking time bomb, shortening the already shortened life even faster: there are a few that did not apply to my dad like pre-existing health conditions and lack of family support – and at least one that did, which was the surgical removal of more than 95% of the tumor [called "the extent of tumor residual"].  One other factor that affects prognosis, one which we never got the chance to find out about in my dad’s case, is a genetic mutation that involves a gene called the MGMT gene.  People with GBM who have had their MGMT gene shut off by a process called methylation have prolonged survival rates. Unfortunately, as in my dad’s case, testing for this gene is not automatically done at all medical centers, and so many people (as occurred in our case) cannot use this as a piece of information for treatment planning or the end-of-life decision-making process.

It is important to be aware that not all GBM’s have the same biologic abnormalities, which is likely the reason that different patients respond differently to the same treatment. And so, even with the amount of information in the media about Brittany's medical condition, there are some things to which the general public is not privy, and some of those things probably affected her prognosis.

Although this information may seem boring or irrelevant to most people, I feel it is important to have access to it when considering Brittany Maynard’s case.  The other thing that I think it’s important to know is that, unless you have been intimately involved in caring for someone with GBM, there will always be certain things that you don’t know - things that may make the choices of Brittany and others look different to you.  As I’ve said before, GBM is a different beast from other cancers, even when those cancers have spread to the brain. 

In the next post, I will try to express my thoughts about some of things that have been discussed about Brittany’s case.