Chapter 3: Super Bowl Sunday
I felt better than I had for months when I got back from Cleveland. Off the Keppra my mind cleared and my energy seemed to return. With the official word that I didn’t have epilepsy I was happy. After all, nothing too serious ever happens to me, and all medical problems eventually go away right? This bliss lasted for two weeks, then the blood blisters came.
Ever bite your tongue really hard? So hard that a little bump forms on your tongue and you spend the entire day running your tongue against the roof of your mouth because it feels so weird? I woke up the morning of the Super Bowl with 5 of those sores. They didn’t hurt, but they were ugly. I was pretty sure it was something temporary, so I ignored them. The next day they were bigger. What’s more, my feet looked like they were starting to rust.
It may seem odd to you that having blisters on your tongue and rusting feet would not freak me out. But recall my medical history – all I got was annoying but non-threatening ailments, and this had nothing to do with my brain. Surely this was another annoying, but benign condition. My primary care physician had put me on iron pills to treat anemia (low red blood cell count). Surely, this was just some side effect.
I’d just like to take a minute to address the doctors and med students reading this. Weight loss, fatigue, petechia (the rusting look of the feet), anemia, mouth sores? “Hello, classic lymphoma presentation. You must have had some crappy doctors.” Except for a few things. Remember that up to this point I was presenting with neurological symptoms (seizures, TIA) – not a classic lymphoma. Physical exams weren’t finding huge lymph nodes. And here’s the most important part…I wasn’t being seen by oncologists or hematologists.
What has become painfully obvious to me, is that once you are directed to a given medical specialty, your problem instantly becomes one that that specialty handles. On my first trip to the ER, they thought stroke, so I was handed over to cardiology. What does a cardiologist look for? Strokes. Can’t find one? No problem, must have been a TIA that leaves no evidence. Second ER visit? Handed off to the neurologist. Can’t talk? Looks like a seizure, sounds like a seizure, must be a seizure.
Too many hospitals have become like the three blind men and the elephant. One blind man grabs the elephant by the tail and says, “It is a snake.” The other grabs the leg of the elephant and says, “It’s a pillar.” The third blind man grabs the elephant by the ear and declares, “It is a fan.” Depending on the version of the story you read the elephant then either stomps on all the men, or some wise person sees the entire elephant, and makes fun of the disabled for their limitations (it is not a very politically correct story). The point is extreme specialization has advanced doctors’ abilities to understand and treat disease, but it has come at the expense of a sort of professional myopia.
This is not limited to doctors. Academics have found the same trap. The more specialized they are, the more rewarded they are by that specialized field, and the less able they are to work across scholarly boundaries. There is this sort of unspoken rule in academia: divide pond until biggest fish within you are (best if spoken in Yoda voice). So I’m not just an information scientist, I’m a library and information scientist. I don’t just study libraries, I study reference librarians, not just reference librarians, but digital reference librarians that answer astronomy questions on Tuesdays…you get the point.
Back to my story. On the third day when the blisters in my mouth started growing on the inside of my cheek my wife made me call the doctor. He looked at the blisters and feet and said it might be the iron pills he had prescribed and ordered yet another blood test. So off I went to get my blood drawn…again.
The next day I was sitting having my car serviced when I got a phone call from my wife. “Dr. M has been trying to get you all morning. He says your platelets are very low and you have to go to the emergency room now.”
My wife picked me up, and took me to the hospital (the same hospital where they first diagnosed me with seizures). They did a standard blood test (a CBC, or complete blood count) and found I had a platelet count of 4.
Please let me take a moment for a little hematology lesson. While there are a bunch of different cells floating around in your blood stream, the three basic types are:
- Red blood cells: these carry oxygen around the body and get rid of waste materials.
- White blood cells: these are a series of different cells that fight infection and repair the body; and
- Platelets: these cells help repair blood vessels and stop bleeding.
Now each of these cells have a normal range (a count of how many cells they find in a sample of your blood…hence the “count” of “complete blood count”). Normal white count is between 4 and 10, Red count is 4.6 and 6.1. Platelets are 150-400. I had a platelet count of 4. This is not good.
The reason I was getting mouth sores was the same reason my feet were “rusting.” Small hole were forming in my blood vessels (this happens all the time) and small amounts of blood were leaking into the skin. Normally platelets plug up these holes, but I didn’t have enough, so the blood kept leaking and formed blisters and petechiae (red splotches under the skin).Why the feet? You put a lot of stress on feet by walking. Why the mouth? Lots of blood vessels with thin cell walls there.
Now this might at first sound more annoying than dangerous so why the urgency to get to the emergency room? As the ER attending doctor put it “I could give you a hard ping to your head, and you might die of internal bleeding in your brain.” He had my attention.
So here I was again in the ER waiting for the next crazy diagnosis. I was not disappointed. “Mr. Lankes, we believe you have ITP.” Wait for it. “Idiopathic thrombocytopenic purpura.” Did I mention I took Latin in high school? No? Well I did…I failed the course miserably, but I did get enough out of it to know what “idiopathic” meant. From the Oxford English Dictionary:
“relating to or denoting any disease or condition that arises spontaneously or for which the cause is unknown.”
And so I laughed. “So, in English, I have low platelets,” thrombocyte means platelets…I did have to ask that, “and you don’t know why.” I don’t think the doctor appreciated that. It turns out a lot of folks have ITP, and there is a simple treatment: steroids. High dose steroids and the platelet numbers bounce back.
So, because my platelets were dangerously low they admitted me to the hospital and started pumping me with steroids. And my platelets went down further. This is not good. They got down to 2, and the doctors started getting worried and looking for some other cause. They upped the steroids – nothing. They suspected that my immune system was killing the platelets for some reason so they gave me Intravenous immunoglobulin (IVIG). This is a cocktail of immune components of hundreds of blood donors pooled together. The idea is to confuse my immune system long enough to let my platelets recover. Nope.
To drive the point home to me on how dangerous this was I need to jump ahead a few weeks towards the end of my hospital stay. One afternoon as I was talking to my wife I felt an odd bulge on my hip. I pointed this out to the nurse, and she looked. As she was watching my hip…well my butt really…the bulge got bigger. She took out a magic marker and tried to draw a circle around the bulge, but it kept growing and growing. For the next week every time I saw a nurse or doctor, I had to turn around and drop my drawers so they could check my lumpy butt. My brother-in-law who teaches biology to pre-med and pharmacy students, asked me for pictures he could show his class because they were discussing the lymph system. I respectfully refused letting him show pictures of my inflated butt to undergraduates.
Now I try to see the humor in ending up with a huge purple bulge that ran from my butt down my thigh, but, it was a spontaneous internal bleed. Had it happened in my head or heart, I would be dead. How did the bleed start? Did I bump something too hard? Did I sit down to quickly? No idea. Now back to the story.
At this point I need to introduce another character in our unfolding story: Dr. S. Dr. S to me was a like a mix of Dr. House from the TV show “House” and The Shadow. He was clearly brilliant and always seemed to show up in my hospital room at 2 a.m. alone. While all my other doctors were trying to figure out why my counts weren’t going up, Dr. S was looking back through my medical records. He found this one small entry when I was admitted for my speech problems about an enlarged lymph node in my neck that couldn’t be biopsied because it seemed to disappear on its own. Dr. S found it again and the team decided it needed to be biopsied and a CAT scan was in order.
Here’s how I first learned I may have cancer. As the ITP treatments did nothing, the doctors began to suspect some form of blood cancer, and probably some form of lymphoma. In the CAT scan my lymph nodes throughout my chest and abdomen were enlarged, my spleen was enlarged and both my spleen and my liver looked to have spots on them. A lymph node biopsy and bone marrow biopsy were scheduled. It was late on a Thursday afternoon when an oncology fellow (read advanced med student that my wife and I would come to refer to as the Bringer of Doom) told me that I probably had cancer. I remember it distinctly. My wife was in the room and we both went white as ghosts. All I could think about was “I’m going to die.”
Without any time to let that sink in, Bringer of Doom also informed they had to do a bone marrow biopsy right away as the lab was about to close. Within minutes of being told I had cancer my wife was whisked out of my room, I was lying face down on my hospital bed and the Bringer of Doom was kneeling on my back poking me with needles.
Here is one of the most poignant moments I have had in my life, and frankly, if you take nothing out of this book but the following story, I would be very happy. As the fellow lowered the bed for more leverage to push into my skeleton she requested a steel needle to bore into the crown of my pelvic bone. A nurse who had been taking care of me came to the head of the bed, and with one hand gave the fellow the steel needle, and with the other took my hand.
For the next 10 minutes that nurse asked me about my job, my kids, my wife, where I liked to travel, anything to keep me talking. Meanwhile the fellow continued to lower the bed for better leverage. She was getting direction from another doctor…it was the fellow’s first bone biopsy – great. As soon as the fellow removed the needle, the nurse let go of my hand, walked down to the lounge where my wife was losing it, and told her, “You are planning his funeral, stop it.”
For the doctors and med students reading this, let me be very clear. You do not simply say it is probably cancer, and then move on. You do not separate spouses within minutes of such a diagnosis. You do not put figuring out the diagnostic puzzle before being human. The lab results could have waited; at least waited a day for the lymph node biopsy to be done. You are treating a human being, a human being that needs comfort, direction, and assurance. I apologize if I still sound pissed off, but this still pisses me off.
The nurses knew what was going on, because they are always there after the procedure, and have to help pick up the pieces. That may be part of their job, but humanity is part of yours. Taking time, listening, assuring your patient is mentally ready for the next steps is not a challenge to your skills, or a questioning of your work, it is a means of connecting to a patient, and that connection is essential to involving the patient in their own treatment; and that is the future of health care. Call it patient-centered, call it caring, I don’t care. It is important – more important than the timing of a damn bone marrow biopsy.
Let me contrast this to how a diagnosis is delivered one floor above my hospital room in the Children’s Hospital. The patient is there, and the parents are there and the doctor is there. After the diagnosis is delivered the doctor brings a nurse, and a librarian up to speed on the diagnosis and potential treatments. As the doctor is reviewing the diagnosis the librarian is writing down the terms used: “Hodgkin’s Disease,” “Lymphoma,” etc. After the diagnosis is delivered and initial questions answered the librarian goes back to the Family Resources Center and uses her iPad to do Google Searches on the keywords. The iPad has a unique IP number so the search engine doesn’t filter the results for a hospital. The librarian reviews the top 10 hits for each keyword. She then sits down with the parents and goes through each of the results letting them know what are ads, what are reliable, and why other sites should be avoided.
Why does she do this? What’s the first thing I did after I heard lymphoma? Google every keyword I could think of. Now I’m a trained information scientist (raise nose in arrogant fashion here), but I was still reading everything from definitions to how juicing machines and burning sage would save me. By the way, the children’s librarian doesn’t stop at Google searches. She brings paired books to the family – picture books for the kids (depending on their age), and more in-depth guides for the adults (and, by the way, an Xbox for the kid as something to do while stuck in the hospital).
My point in all of this is that we can do better in health care, and not just by expecting doctors to do this all on their own. We can be more human, but we must acknowledge that the conversation on conditions does not stop when a doctor leaves a room. It will happen on the Internet. It will happen with friends and family. If the doctors are not aware or do not care about these conversations, they are abdicating a huge percentage of how patients come to understand their health and what they need to do.
The good news was that the bone marrow biopsy came back clean, but now they needed a swollen lymph node. There are two ways to biopsy a lymph node. The first is called a needle biopsy because, as you might guess, you use a needle to get into the lymph node and grab some tissue. Needle biopsies are easy, relatively painless, and don’t lead to bleeding.
The problem is that you can’t grab much tissue with a needle. If you really want to know what is going on inside a lymph node, you need to surgically remove the whole node. Since the team was seriously considering the possibility of lymphoma, they wanted the whole thing. Only one problem, cutting open someone with platelets as low as 2 is just asking for death by internal hemorrhage. So the first thing my hematological team had to do was convince a surgeon to do it.
Surgeons Cut, Anaesthesiologists Worry
There is nothing more reassuring to a patient than watching and waiting for internal hospital squabbles. The hematology team (by this time the hematology/oncology team) had to convince surgery to cut me open. This took two days. Together the hematologists and the surgeons came up with a plan. Pump me full of platelets through transfusion, ship me to surgery and hope for the best.
A little more hematology for you. I mentioned the three basic types of blood cells: reds, whites, and platelets. Turns out each of them have radically different life spans. Red blood cells can live for weeks before they need to be replaced by your bone marrow. Whites live days. Platelets live hours. So a transfusion of platelets might raise your levels, but only for hours.
Despite the risks the surgeons agreed to do it, and I was rolled down to surgery. Turns out the surgeons can agree to all the cutting they want, but the final word really comes from the anesthesiologist. It is the anesthesiologist who puts you to sleep and ultimately has to wake you up. They tend to be more cautious. So even though I was now sitting in surgery I had to wait for an anesthesiologist to agree to the platelet plan and get my consent.
Let me paint for you the scene. I am lying on a gurney in a hospital gown and a hair net. A team of four surgeons walk up to me and say “OK, so we are going to make a cut here, right above the collar bone, and go in and grab that lymph node. There is some risk because you have a lot of nerves there, so potentially we could knick one of those and you might lose feeling in your arm…OK? Great.” Now read that quote again, but this time make sure to ignore any punctuation and don’t take a breath.
There is a great line that goes “Surgeons prefer to talk to their patients after the anesthesia has been administered.” This has certainly been my experience. It may have also struck my surgeon, because a few days later he came to visit me in my hospital room. “I didn’t realize that you and I are neighbors. I live right around the corner from you. Here is my card and my personal phone number if you have any problems. I’m glad the procedure went very well.” It was a very nice encounter…I only wish all doctors treated their patients like neighbors.
Still, the surgeons were in. Next up, the anesthesiologist. He sat next to me with a bundle of consent forms. “We’re going to start by giving you a shot to relax you. Then I will be administering gas via mask. Now, if you start to bleed out, I will put a tube down your throat to ensure you can breathe. You see if you begin to bleed internally it will swell the neck and the blood will eventually cut off your trachea. I just want you to know this in case you wake up on a ventilator…sign here.” This conversation was not fast, by the way. The anesthesiologist took a fair amount of time on this. All I could think was, “Could you just throw an ‘in the unlikely case that’ into the conversation?”
I took the shot, breathed the gas and did not wake up in the intensive care unit.
What I did wake up to was a bit of a nightmare/farce. You will note that this juxtaposition of things being amazingly frightening and amazingly humorous is an ongoing theme in my story (if anyone decides to make a movie of my experience I hope it would be Terry Gilliam and I’d like the part of the Bringer of Doom to be played by Eric Idle in drag). Pathologists could now look at my lymph node and tell me what was wrong with me. Except of course, they couldn’t. Normally, if you have cancer of just about any type, 24 hours is plenty of time to identify it. My biopsy? First since the surgery was late on a Friday I had to wait for the lab to open on Monday to do the pathology. Then, as it turns out, my biopsy was “challenging.”
The pathologist took a look. He sent it to the hematopathologist for a second opinion. The hematopathologist sent it to the National Institutes of Health for a third opinion.
Now, take a moment and put yourself in my shoes. You have now been in the hospital for ten days. The doctors have told you that you might have cancer and even put you through a somewhat risky surgery. Your platelets are still dangerously low (like risk for a bleed out in your brain dangerously low). And the doctors are telling you they still can’t tell me for sure what I have. So what the hell was taking so long with my biopsy?
It turns out my lymph nodes are weird (go figure). They are full of granulomas. Granulomas are, as best I can tell, what happens when your immune system gets confused. Normally white blood cells find an invading pathogen and literally eat it. If the white blood cell doesn’t know exactly how to deal with the pathogen, they wrap it up into a sort of sealed bundle, and ship it off to the lymph nodes. My lymph nodes were full of the suckers.
Now here’s the important part. It turns out that the presence of granulomas means absolutely nothing. Seriously, not indicative of anything and not harmful. However, pathologists get really worked up about them in large numbers because
- A large number is very rare, and
- A large number can make it hard to see other things in the lymph node, like say lymphoma.
With Hodgkin’s Lymphoma, however, all you need to see is one special type of cell, called a Reed–Sternberg cell, and the gig is up. You win, you have Hodgkin’s Lymphoma. Guess who won?
Thomas Hodgkin and His Damn Disease
In 1832 Thomas Hodgkin wrote a real page tuner entitled On Some Morbid Appearances of the Absorbent Glands and Spleen. In it Tom, as I call him, described a disease where the spleen and lymph nodes grew abnormally large, and eventually into other tissues killing the patient. Later, Hodgkin’s disease would be classified as a cancer of the lymphatic system (don’t worry, we’ll get there) and be renamed Hodgkin’s Lymphoma. It is related to other blood cancers like leukemia.
If you are ever diagnosed with Hodgkin’s Lymphoma, or really any kind of cancer, you will be inundated with information on what it is, how it works, what kind of cells are involved, and so on. I’m going to give you a few paragraphs, and you only need to read those if you don’t have a better source. These are from the National Institutes of Health.[1]
The Lymphatic System: The tissues and organs that produce, store, and carry white blood cells that fight infections and other diseases. This system includes the bone marrow, spleen, thymus, lymph nodes, and lymphatic vessels (a network of thin tubes that carry lymph and white blood cells). Lymphatic vessels branch, like blood vessels, into all the tissues of the body.
Got it? So lymphoma is a cancer of the immune system. And Hodgkin’s Lymphoma?
Hodgkin lymphoma starts in the lymph system, usually in a lymph node. The disease may be found because of a swollen lymph node in the neck, chest, or other areas.
The disease begins when a lymphocyte (almost always a B cell) becomes abnormal. The abnormal cell divides to make copies of itself. The copies keep dividing, making more abnormal cells that build up.
When white blood cells collect around the abnormal cells, the lymph node that contains abnormal cells becomes swollen. Abnormal cells may spread through the lymph vessels or blood vessels to other parts of the body.
Although normal cells die when they get old or damaged, abnormal cells don’t die. Also unlike normal cells, abnormal cells can’t help the body fight infections.
Got it? So those wonderful white blood cells that are supposed to kill disease mutate and start dividing like crazy.
I would like you to note two things about this information provided to you by your government. The first is that unlike most cancers, lymphoma doesn’t make tumors per se. It is really in the entire blood stream. Other cancers like breast cancer make masses. If breast cancer gets very bad it will spread to other organs and in doing so, they will leave evidence in your lymph nodes and that is very bad. Lymphoma, on the other hand, starts with the lymph nodes. So finding diseased lymph nodes does not mean the cancer is necessarily in the advanced stage.
The second thing I’d like you to notice is how benign the NIH’s write up makes Hodgkin’s seem. It reads like, oh you might have some swollen lymph nodes and these cells won’t be able to fight disease. Ah, poor cells. What it should say is, these cells can make your lymph nodes grow so large they will pop out of your neck and squeeze your heart until you die…or your lungs until you die, or spread to other organs causing cancer of the liver and such….and then you die. I mean seriously, do an image search for lymphoma, but only if you have a strong stomach.
Now those paragraphs I just wrote were not available when I got the final word on my diagnosis. I got a doctor who I had never seen before coming into my room at 9 o’clock at night sitting down next to my bed. “I’m Dr. P, the head of the oncology department. We got the final word on your biopsy and you have Hodgkin’s Lymphoma. We believe it is causing your low platelet count, enlarged lymph nodes, and enlarged spleen. The standard treatment for this is a chemotherapy called ABVD, and we’d like to start in the morning. The nurse will get you some information on lymphoma and the chemo. Do you have any questions?”
Now to be fair, that interchange was not as curt as I present it and the head of oncology did take the time to work with me. Dr. P answered my questions and was very professional:
How advanced was it?
From a CAT scan it appeared to be widely distributed throughout my chest and abdomen and possibly had started to grow on my liver and spleen. That meant it was advanced and aggressive. However, it had not affected the bone marrow and that is good.
Aggressive?
Lymphoma can be aggressive or slow growing. Aggressive is actually easier to treat.
What were my chances?
Hodgkin’s Lymphoma is very treatable, with 90% of people being cured through treatment. I was young as cancer goes, so I had very good chances. Given the distribution, and the possible involvement of the liver, I had about a 60-70% chance of a cure.
Would I lose my hair? I think I asked that because one was supposed to be worried about such things.
Actually you don’t lose you hair, it just becomes brittle and breaks off at the skin. Will you lose yours? Don’t know. Everyone is different.
Then I was left alone. My wife was at home, and I now had to call her and let her know.
I love my wife. I met her the very first night of college. I remember seeing her for the first time. She was saying goodbye to her parents in the lobby of our dorm, and she was the most beautiful woman I had ever seen. I remember her long hair. And her Italian complexion to a born and bred Midwesterner seemed exotic. I pursued her for weeks until she agreed to go out with me. She is the most amazing wife and woman. She is a fabulous mother to our two children. All that I have accomplished is because of the solid loving foundation she has provided.
That night I cried not because I had cancer, but because I had to tell her I had cancer. I don’t remember what I said or anything about the conversation, but thinking back on it I need to take a break from writing and be by myself for a bit. I am sure, however, I told her at least now we know what it is.
Notes
[1] http://www.cancer.gov/cancertopics/wyntk/hodgkin