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.