The things I took for granted
On change and fighting the good fight
When I was in college, one of my friends had an eye-opening experience when she went home on winter break.
It is getting better, if my current hospital stay is any indication. The word is getting out. More people have at least heard of mast cell disease, know that it can cause sudden, severe anaphylaxis, know that we react to things that most people never do and in ways that might seem strange. No one has made me feel crazy, accused me of being drug seeking or treated me disrespectfully. If they wanted to do something that wasn’t appropriate for mast cell patients, they called to confirm before proceeding.
I know it doesn’t always seem like it, but every time we present for medical care, we are fighting the good fight.
I always wanted to travel. From a very early age, I wanted to go everywhere, see everything, converse in other languages. I spent hours in the library reading about faraway places, whispering phonetically written phrases in strange languages. I never doubted I would make it everywhere I wanted to go.
More the same than different
On emergencies and making a scene
I am a pretty brassy person. (I’m sure this surprises people who don’t know me in real life, as I am so shy on the internet.) I am not easily embarrassed and never have been. I have always been klutzy and loud, and I figured out early on that it was easier to just not be embarrassed by that. Self acceptance. I has it.
However, there is one thing that I get very embarrassed about, and I’m sure I’m not alone. That, my friends, is making a scene, particularly if that scene is health related.
And then there’s this other part, where I worry that I’m making a scene unnecessarily. Like I worry that I’ll call the ambulance and they’ll arrive and be like, “Oh, you’re fine! Why did you call us?” even though I AM ANAPHYLAXING AND THERE IS A CHANCE I COULD DIE FROM LOW BLOOD PRESSURE. Like I said, it’s not logical.
If any of you mast cell people told me you waited forever to give yourself epi, I would be all over you. I would send you pretty infographics about how you should use epi early and quote statistics about how often people accidentally stick themselves with epipens and live to tell about it. Because you should use epi early. It will help you. I know the party line AND I AGREE WITH IT but this fear of embarrassment thing is really strong. And I am sure I’m not the only one who feels like this.
If I’m going to the ER, I better need it
Seeking emergency assistance presupposes that the benefit outweighs the risk. For people like me, this is not always true. People with mast cell disease often have a very complicated relationship with their medical providers, including their favorite local ER. Let me give you an example of how it plays out when I go to the ER.
On the way to the ER, I page the person covering service for my immunologist and GI mast cell specialist. At least one of these doctors will call ahead to the ER to tell them I have systemic mast cell disease and must be treated immediately. They will give orders for immediate IV medications to stop anaphylaxis. I also have a signed letter from my doctor listing emergency treatment protocols, in the event that I can’t get in touch with one of my doctors.
When I arrive, I say the following: “Hi, my name is ( ) and I have systemic mast cell disease. I am in anaphylaxis and need IV meds immediately to prevent going into shock. Dr. (whoever) called about me.” The receptionist may or may not tell me to fill out a form and take a seat. I may or may not have to argue with them. My mother may or may not have to argue with them. Most of the time, I am not looking great by this time, so either way, I am in a bed in the ER pretty quickly.
A nurse comes and puts in my IV. With my terrible IV access, this often takes upwards of twenty minutes. Remember – EVERY time you break the skin of a mast cell patient, you risk anaphylaxis. If I am anaphylaxing, this makes me worse. Getting an IV is dicey for me.
During this time, I speak to three or four other people. They might be nurses, physician’s assistants, interns or residents. An attending physician usually comes over. I tell every single person that I need specific IV meds immediately to prevent shock. I then have to explain this, telling each of them the exact same long, complicated story. They generally react badly, like I am telling them how to do their job. Most of these people have never seen anyone with my disease. Statistically, they may never see anyone with my disease again. I do not have time to wait for them to figure out the exact remedy for someone like me. People like me are directed by our physicians to be forceful and repetitive when presenting to an ER to avoid situations where we wait hours for meds.
If there are any students anywhere on the ward, they will ask if they can observe my (rare and exciting) existence. I always say yes, because medical professionals recognizing mast cell disease can only ever help me.
After half an hour in the ER, I am likely just getting my IV meds. They give them IV push, not infusion, because that is faster and at this point, faster is better. However, giving some of these meds IV push can cause wild variations in blood pressure for mast cell patients. I may or may not have problems related to stabilizing my BP.
Frequently, the reason I end up in the ER is because I had a bowel obstruction that hurt so badly that it triggered anaphylaxis. If this is the case, I am also told to request pain medication to suppress the pain to suppress the mast cell reflex. Most pain medications are not safe for mast cell patients. I am limited to tramadol, fentanyl and hydromorphone.
Telling anyone in an ER that you can take hydromorphone but not hydrocodone or oxycodone is guaranteed to get you an eyeroll and an assessment as a junkie. The fact that this can be easily verified through my medical records (or google) does not help my cause. So I just have to accept the fact that I have been branded as a drug addict. From that point on, I am treated as a junkie for the duration of my stay. People go from being curious and helpful to full of disdain pretty much immediately.
Last summer, the ER wanted to order a CT scan to make sure my bowel obstruction wasn’t turning into something worse. They wanted me to get a pregnancy test and wait for the result before getting the test. I refused because I had been in my OBGYN’s office twice that week and knew that I could not be pregnant. I didn’t want to wait hours longer for a test that I was already going to wait hours for. The physician’s assistant turned to the resident and told him that “she just wants dilaudid, she could care less if there’s a baby in there.” Unbeknowst to me, they called my PCP to let him know that I was “drug seeking.” This sort of things happens pretty much every time I go the ER. It is not unusual for people with mast cell disease.
Please note that this happened at the prominent hospital where I am seen several times a month, a hospital that has a department that treats my disease, and where all of my pertinent medical records reside.
Eventually, the reaction ends and I either go home or am admitted. Funny: as soon as one of my doctors, or their representative, shows up and states that I am being admitted because I have systemic mast cell disease, no one is calling me a drug addict anymore. But they’re not apologizing either. Once I am admitted, I generally have far fewer issues.
The emotional stress of going to the ER has to outweigh the physical stress of my current reaction for me to go. I wish it weren’t that way, but it is. If I cannot breathe, am bleeding freely, or have dangerously low BP, I go to the ER. But otherwise, I’m probably going to wait it out at home.
Experiences like this are the reason why rare disease awareness is so important.
At Least I Don’t Have Cancer
I was engaged in casual small talk with someone when the subject of my illness came up. I showed my PICC line by way of explanation.
“You have cancer?” he asked, voice low.
“I have systemic mast cell disease,” I answered. I knew where this was going.
“Oh. Well, at least you don’t have cancer.” His whole body language changed. It wasn’t cancer, so I wasn’t worthy of his sympathy. Not that I want it, really; but I’m worthy of it, I think.
At least I don’t have cancer.
In the US, people tend to categorize illness into three groups: cancer; physical illness that is not cancer; and mental illness. The amount of respect and understanding you are afforded descends in that order. To most people in the US, having a verifiable physical illness gives me more credibility than someone with mental illness (although it shouldn’t), but less than someone who has cancer. Even if that cancer has been treated successfully and is in remission.
My disease is sometimes considered a cancer, but sometimes isn’t. It depends on the organization. Some hospitals treat it as cancer; some treat it as an immunologic disease; some treat it as a hematologic disease. It’s so rare no one can agree on what’s best.
My disease is classified as a myeloproliferative neoplasm. This is a fancy way of saying that my body makes too many cells in the bone marrow. Know what else are neoplasms? Cancers.
My disease is usually propagated by a mutation in an oncogene. Onco- is a prefix meaning “cancer.” Know what other diseases are caused by a mutation in this same oncogene? Cancers.
If you have involved systemic mast cell disease like I do, and you present to most hospitals, do you know what department you will be seen in? Oncology.
If you have smoldering or aggressive systemic mastocytosis, systemic mastocytosis with associated clonal non-mast cell lineage hematologic disorder, or mast cell leukemia, do you know what kind of therapies you will receive? Therapies designed to treat cancers. This is mostly chemotherapy in doses that are roughly equivalent to those used for – that’s right – cancers.
Cancer is a broad term. It refers to over 200 diseases that involve unregulated cell growth. Hey, know what other diseases involve unregulated cell growth? I bet you do.
There is massive variation in morbidity and mortality among those 200 diseases. The term cancer encompasses diseases like pancreatic cancer, for which 5-year survival is 6.7%, even when identified in early stages, as well as thyroid cancer, for which 5-year survival is 97.8%. When looking at all types of cancers, 66.1% of patients are alive after 5 years. In early stages, some cancers require minimal or no treatment at all. Some cancers have become essentially chronic diseases through innovations in drug therapies. Some offer the promise of remission for the majority of patients. And some are fatal, always.
There is this truth that doesn’t get discussed openly a lot in the mast cell community: that some of us will die from it. While the majority (SM and CM) won’t, life expectancy for SM-ANHMD averages 85 months (see paper here:http://www.ncbi.nlm.nih.gov/pubmed/24465546); for ASM, 41 months; for mast cell leukemia, 6 months. Many of us suffer progressive organ damage and repeat anaphylaxis, and eventually it kills us.
If mast cell disease was classified as cancer, insurance would be more likely to cover my therapies, including reconstructive surgeries, if needed; employers would be less likely to terminate me for illness-related absences; I wouldn’t be limited to a handful of treatment facilities; I would be more likely to have my pain controlled; I would be less likely to be referred to a psychiatrist when my symptoms couldn’t be managed; and I would have been diagnosed faster. Much faster.
There are obviously other aspects to living (or surviving) cancer aside from survival. There are financial issues due to loss of work and the general astronomically high expense of being sick. I know about that, as I have spent more than twice the cost of my education on my healthcare, all while having decent, employer-paid insurance. That’s just what I have paid out, not what I accumulated in debt. One difference here is that there are a significant number of charitable organizations dedicated to helping cancer patients pay for their treatment, or to assisting with incidental things, like transportation, cooking, etc. There are no organizations like this for me in my state. There is exactly one that I know of.
Cancer patients and survivors may also have to contend with body image issues due to disfiguring surgeries and tissue/organ removal. I have multiple scars from biopsies, tumor removal, organ removal, and am the proud owner of a colostomy. I would contend that gives me an idea of what it’s like to be disfigured.
For many cancer patients, there is the chance to become a “survivor.” There is a chance that their disease can go into remission. That will never happen for people with any type of mast cell disease. There is no cure for our diseases. For those that need chemo, it is essentially a lifelong process of on for a year, off for a year, or something to that effect. I personally know someone who has been on chemo for nine years.
The cancer “equivalent” of advanced mast cell disease, which I estimate to be Stage III chronic myelogenous leukemia, provides a 95% chance of 5-year survival. That would be an improvement for many with mast cell disease.
I want to be very clear about something: I do not have a problem with cancer patients or survivors. I feel a lot of empathy for them, especially as we have a lot of shared experiences. We go the same places, see the same doctors, take the same medicine, have the same procedures. I know what it is like to have a doctor tell you how long you are likely to survive. I know what it is like to take dozens of pills a day, to spend 20+ hours a week at the hospital, to be so nauseous that you can’t sleep and so tired that your vision is blurry. I know what it is like to wonder if you will be able to do all the things you want to do before you die.
I have a problem with the idea in American society that physical illness is either cancer or not cancer, and that “not cancer” is inherently less severe. It particularly bothers me because advanced mast cell disease is so similar to blood cancers. But that’s not really the point. Is advanced multiple sclerosis less severe than prostate cancer, for which 5-year survival is 98.9%? Is HIV? Diabetes? The fact of the matter is that some people with HIV take their medications and exercise certain precautions, but otherwise live a pretty normal life. Someone with diabetes, a very well-known and manageable disease, gets a difficult to treat infection and they die. You can’t say cancer/not cancer because disease progression is inherently individual. Two people with the same disease can have completely different experiences.
The point is that disease is disease. It all sucks. None of us deserve any of it. We should all be afforded the same compassion and understanding. I love my life, I really do, but this shit is hard. It is really, really fucking hard.
But, hey! At least I don’t have cancer.
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