Alphabet Soup that is Mast Cell Disorders

Once you’ve racked up enough diagnoses and spent enough time in hospitals the lingo starts piling up and it can get confusing but I held on. Until I got to mast cell disorders and mmmaaannn it is alphabet soup up in here. MCAD, SM, ASM, IgE, HSCs, H, UFH, DAMPs, PAMPS, ATP, TNF, and more! The good thing is you could read one article about this stuff, memorize it and sound REALLY smart rattling it back off at some party. Furthermore, mast cell disorders are not well known, relatively newly discovered, and effect each patient differently. Simple right?

 

diagram of the role of histamine

So here is my attempt to spell (badumchh) it out for you guys. If you haven’t already, read my intro posts to mast cells here.

Types of Mast Cell Disorders 

  • Mastocytosis (rare)
    • Cutaneous Mastocytosis
    • Indolent Systemic Mastocytosis
    • Systemic Mastocytosis with clonal hematologic non-mast cell lineage disease
    • Systemic Smoldering Mastocytosis
    • Aggressive Systemic Mastocytosis
    • Mast Cell Leukemia 
    • Mast Cell Sarcoma
  • Mast Cell Activation Disorder (MCAD)
    *note: Mast Cell Activation Disorder and Mast Cell Activation Syndrome (MCAS) are the SAME disorder BUT they are not the same as the term “Mast Cell Disorders” which is an umbrella term for Mastocytosis and MCAD*

Difference between Systemic Mastocytosis (SM) and Mast Cell Activation Disorder (MCAD)

*note again: I don’t know enough about other forms of mastocytosis to formulate anything useful*

When looked at basically, the main difference between SM and MCAD is MCAD patients have the normal amount of mast cells in their bodies, they are just hyperactive and hypersensitive vs SM patients have too many mast cells throughout their bodies and they build up in various organs and cause issues. Symptoms of both can be identical and like most conditions, follow a spectrum from mildly affected to severely affected and can easily be life-threatening. High risks for both include anaphylaxis, severe bleeding from heparin release, seizures, organ damage from mast cell accumulation, even multiple system failure in severe cases. 

Common Treatments and Therapies

*note: I do not know of everything, this list is by no means exhaustive*

  • H1 Antihistamines (stabilize one type of histamine, mainly hives, headaches, nausea): hydroxyzine HCl (Atarax), diphenhydramine (Benadryl), cyproheptadine (Periactin), cetirizine (Zyrtec), levocetirizine (Xyzal), fexofenadine (Allegra), loratadine (Claratin), desloratadine (Clarinex)
  • H2 Antihistamines (stabilize a different type histamine, mainly GI): famotidine (Pepcid), Nizatidine (Axid), Ranitidine (Zantac)
  • Mast Cell stabilizers: cromolyn sodium (Gastrocrom), quercetin, luteolin, asprin (if allowed)
  • Leukotrine Inhibitors (respiratory): montelukast (Singulair), zafirlukast (Accolate), zileuton (Zyflo)
    *if you don’t feel like these are fictional sci-fi names by now… just wait*
  • Xolair (omalizumab): this is an injection that was originally marketed for asthma but works to desensitize the body. Must be taken at an office every time, can be very expensive and hard to get covered by insurance, can have different reactions at different times. That being said I know people who have had amazing results from it
  • Epinephrine injection (EpiPen & AuviQ) for anaphylaxis or anaphylactoid reactions
  • Diphenhydramine injection (Benedryl IV/IM) for those who react poorly to epi or as a first response for reactions or if patient can’t take oral medications

All right folks. Think that’s enough to chew on for now. More later to come later!slot hanging on to pole with text "my energy level on any given day is equal to that of a sloth on Ambien"

 

When Life Gives You Lemons… Put On Your Mask and Gloves

Living with a mast cell disorder is like being a human and trying to live on Mars, you’re living in a world that is dangerous, unknown and clearly not made for you to be there. It is becoming really really good at adapting because your health and well being depend on it. It can be tricky, but it is not impossible.

Mast cells are a type of white blood cell that play a major role in immune function and allergic reactions. Mast cells are formed in your bone marrow and are located throughout your body but with clusters in your marrow, stomach, skin, heart, lungs and brain. These cells are mediator cells meaning that they contain certain hormones and chemicals and the release of such chemicals/mediators can happen when your body senses something is wrong and triggers the mast cells. This is commonly called mast cell activation.

Mast cells carry several different mediators and each mediator has an effect on the body. The most common and well researched are the mediators histamine, tryptase and heparin. Histamine release is the driving force behind allergic reactions and the well known symptoms that come with those (hives, rash, swelling, headache, GI upset). When you take benedryl, you are taking a class of drug called antihistamines which help reduce and calm down released histamine. Important clarification, mast cell reactions are not the same thing as “true/IgE mediated allergies”. Unlike “true allergies” mast cell reactions can be triggered by different things at different times and triggers can be things like heat and emotions.

mc mediators.jpg
Mast cell mediators and the symptoms they cause

Mast cell disorders are systemic and complicated disorders that can vary greatly from patient to patient. For me, my mast cells have reacted to everything from coughing to the sun, to peanuts one day and not the next. I have had more anaphylactoid reactions then I can count and have to carry several emergency meds with me at all times. I wear masks in public because cigarette smoke and perfume are some of my worst triggers. Yet despite all these precautions and more, I still maintain a constant reactive state and always run the risk of spontaneous anaphylaxis. Sometimes it isn’t even possible to figure off what started a reaction… it is frustrating to say the least. That being said, it makes you a master at adapting and very very aware of chemicals in foods and products. This is a really big topic to chew off so I plan to do several posts about it if I don’t bore you all silly.

mcas meme.jpg
Bad Luck Brain Meme: Develops and allergic reaction to his allergy medicine.

Majoring in Chronic Illness Management with a Minor in Applied Nursing

Seriously guys. Every time I hear those commercials for continuing education that say things like, “get credit for military experience!” or “use job skills to get an accelerated degree!” I think… man… if only they gave out degrees keeping yourself alive with chronic illnesses. I have a lot of spoonie friends and every single one of them should get to add C.P.P. (certified professional patient) to the end of their title in addition to an honorary nursing degree for many of them. For those of you who don’t get a peak into this aspect of my life, here are some of the behind the scenes tasks needed to function.
Medication Management
This takes up SO. MUCH. FREAKIN. TIME. You have to make sure all your meds are cleared with all your specialists because they don’t chat, you have to make sure no new meds will kill you (allergies, interactions, effect on other conditions), you have to get the script, send it to the pharmacy (assuming they have it), pick it up or set up shipment, keep track of refills, separately order all your OTC meds and supplements, sort out medication or check if medication is presorted correctly, make sure any ED staff, EMS, or doctor can access what prescriptions you are on at any point in time, fight insurance, pay bills, likely deal with 2-3 pharmacies, infusion companies, specialty pharmacies, mail order pharmacies, and actually remember to take them and have rescue meds with you at all times. But yeah… just that. Add secretary and applied pharmacist to your CV.
Appointment Scheduling, Cancellations, and Heckling
This one might be my LEAST favorite. Doctors and other medical professionals have a HUGE range in availability, responsiveness, attitudes towards collaboration and remote patient contact. I hate talking on the phone. I hate talking to people I don’t know in general. People find it hard to understand me and I have a hard time understanding them. It isn’t a great combo. But I do it because I have to. Generally, my team falls into two categories: can see you within the week or can see you in the next 3-9 months. While I totally get the deal with both types, it can be more than frustrating for something to come up in between your 3-9 month follow ups. I am currently playing phone tag and portal spamming with my GI teams because things need getting done but have been less than successful.
As mycharliequinn so aptly explains going to the doctor as a spoonie: “going to the doctor when you’re chronically ill is weird. It’s like imagine everything in your house is on fire, and you’re standing there and the fire department come[s] in like, describe the fire to me and maybe we can find out what caused it and put it out. And you can’t just say everything so you’re like… well the fire in the curtain is the biggest but the fire in the photo albums might be doing the most damage also the fire in the couch is really inconvenient. Occasionally the fire guy is like, well your tv is on fire so it might be electronic- fireitus but that would cause other things like fire in the DVD player. And you’re like, oh yes. That’s been on fire for years. I forgot to mention it because it’s always been a relatively small fire. It’s right next to the bookshelf which has much more fire. And then the fire guy is like, oh. I wouldn’t worry about that, book shelf fire just happens sometimes.”

 

Attending your “Weekly” Appointments
For me, I am currently on a “rather empty” schedule with weekly PT and Aqua PT sessions and biweekly infusions. Just getting those scheduled and being able to get to them and participate takes a lot of spoons and most of my schedule is based off these events.
Decide When to Brave Emergency Departments
Practically have an algorithm this one now but can be tricky, especially considering the “luck of the draw” on if your ED team has even heard of your conditions, actually pays attention to your chart, or can do anything to help. Chronic illnesses often leave you in this limbo where you’re not about to drop dead but not okay enough to stay home and NO WAY can get in to see your 3-9 month doc who manages that kind of stuff. It can be awkward for everyone.

I’m invisible… You can’t see me!

       Lol but actually in case you couldn’t tell by the cheeseball title, I plan to ramble on about invisible disabilities because it’s invisible disabilities week! It’s pretty self explanatory but an invisible disability is any disability that can not be seen and has no major visual manifestations. Examples include: diabetes, depression, EDS, lupus, schizophrenia, anxiety, Lyme etc. Some examples of visible disabilities might include: Down Syndrome, spinal cord injury, amputation, blindness, or muscular dystrophy.

 

It can be hard feeling so sick on the inside and looking “fine” on the outside. You also have to deal with people challenging your right to accessible parking spaces, store scooters, and even medical care. Since there is usually no way to tell the difference between someone with an invisible disability (ID) and a faker, I usually go with,”be kind, everyone you know is fighting their own battle”. One in every 5 Americans has a disability and many disabilities are invisible. Many of us get comments that we are faking being sick, when in reality, most of the time we are faking being well. We put on a smile and continue on with our lives despite the lack of energy, the intense pain, and feelings of hopelessness. To those of you who are close to me, you have seen me with my guard down, but for others you may have no idea I am in pain every day of my life. I don’t mention this for pity or to complain but more so in attempts to open up a dialogue, even if just an internal one. I want take this post and this week to spread awareness, compassion, and answer people’s questions if they have them. The love and support that y’all have given in various ways and modes truly amazes me.

Since I kinda missed EDS awareness in May except a blog post, I’m going to try and make an ID/EDS etc awareness video… Eventually. If I do, I will post the link here.

WARNING: Might just be one long rant…

        Okay… so I wanted to touch on a bunch of different things that don’t have another home. I’m feeling very brain foggy/disorganized so if none of this makes sense… then it’ll probably at least be entertaining.
#1: How long it takes to get anything done in the medical world
Let me preface this with I ABSOLUTELY know this is not all on the medical professionals. There’s shit with insurance, documentation guidelines, hospital rules and regulations, and just overweighted caseload that influence this issue. But seriously, it gets ridiculous.
Some examples:

  • I met with a new GI July 22nd. He seemed on top of his game, familiar with my conditions, and agreed that action needs to be taken to get by nutrition etc back on track. He had lots of medical history, former testing, current summary… everything he needed. He ordered a SmartPill test (camera pill you swallow and it takes pictures and does testing as it goes through your GI tract… pretty cool) and an upper and lower scope (sticking cameras up your arse and down your throat…yum). I had already had a gastric emptying test to diagnose gastroparesis (delayed stomach emptying), but we had no idea the motility of my intestines or if they absorb any of the nutrients. Fast forward 4 weeks until my scheduled SmartPill test swallow. I had to go off all GI meds for a week (which means not only more of the daily yuck but near constant acid coming up my throat), get a ride to the hospital, fast beforehand, take off work etc etc. I get there and they’re (nurses) doing the pre-procedure checklist. They get to question #3 “do you have any implanted devices?”… “yes… but the doc knows about it… we talked about it”  “Let me give him a call”  *comes back 10 minutes later* “I’m sorry, the implant disqualifies you from doing this test, I have to cancel it… the doctor will give you a call (lol jk)”. Okay, so waste of time. I messaged my doc later (shock and awe… he never called) and asked if we could schedule the only other test one can do to get the same data as the SmartPill. His response: we will talk about it next appointment. So you’re thinking aight… that’s okay. Wrong. Not only is his first available not until mid-November, but his office won’t schedule any follow-up appointment until after I complete, and get the results back from the scope scheduled for September 14. So basically I’m SOL until at least December timeframe…. assuming that visit won’t just mean going over EGD results and him ordering the same test I asked him about months earlier. Don’t worry guys…it’s only nutrition.
  • It took my PT 3 weeks to write and sign a sentence saying “patient needs new custom wheelchair back to maintain posture and for support”.
  • It has taken my doc 5+ weeks (he was on vacation for one but still) to write and sign a prescription, note, and LOMN saying “patient needs custom bilateral AFOS”.
  • I’ve been waiting for records from one doctor for 13 months despite several verbal and written reminders to him and his staff.
#2: My two cents on the Epipen bull:
          As someone who is literally allergic to life (sunlight, heat, cold, stress, talking, meds etc) and must always have Epi on my person and has unfortunately had many uses of the autoinjectors I am pissed. But not just by Mylan (jerk face mcugly butts) jacking up the price of Epipens (500%), but the fact that I have multiple friends who have to make the choice between life-saving medicine, therapy, and treatments and feeding themselves or their kids.
And we aren’t even talking about those without insurance. Most of my friends are lucky enough to have some form of health insurance (even if its crappy) and are still drowning in bills and medical debt. There is not a single day that there isn’t a medical bill arriving at my house and that is just ERs, hospitals, and doctor’s bills. Plus medicine, devices, PT, testing, surgery, and prolotherapy (not covered by any insurance as it is “experimental”). I have been incredibly blessed with kick-ass insurance (although still a PITA) and to be able to stay afloat in the bills. Despite all kinds of preventative and prophylactic treatments, there is still only so much you can do to keep ahead of things and emergencies still happen. Epipens also only are viable for a year. They come in a 2 pack but most need more to keep one at school, one in the car, and one on your person. For most, it is now cheaper to go to the ER for anaphylaxis treatment than to use their Epipens. Honestly, all I got is “fuck you Mylan”. No excuses, epinephrine is cheap, autoinjectors are relatively cheap. Not to mention their tax evasion strategies.
#3: Depression
Since the people who 1) even read this blog and 2) read all the way til now and aren’t asleep from boredom are probably the people who care, I saved this one for last. As some of you have noticed, I’ve been dipping into another depressive phase. Depression and me are buddies now so it’s cool. It is also normal and a big part of chronic illness so don’t go panic on me. So don’t be alarmed if I’m a) super clingy and annoying or b) super avoidant and grumpy gills … that’s kinda just how it goes. See previous post RE: things you can do to help.
Aight. That’s all for now….

But You Are So Young…

          But you are so young… words that pierce a hole right through my sternum. Yes, I am young but unfortunately chronic illnesses don’t ask for ID. Yes, I am young but I know kids, young adults, adults, and seniors with chronic illnesses. Nobody tells a diabetic kid they can’t be sick because they’re too young! Yet we (spoonies of various illnesses) get it all the time. My passport and birth certificate concur that I am 21. I am young but anyone who has heard the snaps and cracks of putting my joints back in place before I get out of bed each morning would swear that I am 80. I am young but take more medication and supplements than my grandparents. I am young but I wake up each morning to face a monster that knows my name, my fears, and my limits. I am young but my calendar is that of a retiree: volunteering with doctors appointments and physical therapy to fill in the gaps. I am young but I have friends my age and younger fighting for their lives and few that lost their battle already. I may not look sick, or I may, but I feel sick. I feel these disease nestle into every nook and cranny of my body. I put on a smile to mask the pain and refuse to slow down for the fear that I will be eaten whole by the flames. As Plato said, “be kind, everyone you know is fighting a hard battle”. While many of these comments aren’t said with ill intent, they can be destructive to someone not quite back up on their feet. I suggest going with a complement not related to pain, illness, or weight for the general population. My heart broke the other day when a fellow friend with gastroparesis (stomach paralysis) told me (after losing a lot of weight do to sickness and malnourishment) her mom had told her she looks better “lighter” after she had just been talking to her mom about how she’s really struggling with her GP. Pro-tip: Try crawling around in our bodies in your mind. If you were in constant pain and feeling defeated, would you want someone to tell you “you don’t look sick!”? Even if with good intentions the answer would probably be no. You can always ask instead how we are doing or if we have seen any good movies recently (chances are if it is a spoonie the answer is YES…we watch a lot of Netflix… 😉 ).

Harper Lee