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"

 

Blogs and Vlogs by EDSers

***At the suggestion of a friend I will keep two running posts to be updated randomly, this one for fellow bloggers and vloggers with EDS and another one for information, research and studies. Please let me know if any links are broken/nonfunctional or suggestions for additions!***

Blogs:

Vlogs:

 

Resources for Ehlers-Danlos Syndrome

***At the suggestion of a friend I will keep two running posts to be updated randomly, this one, for educational resources, studies, etc and another of fellow bloggers and vloggers with EDS. Please let me know if any links are broken/nonfunctional!***

General Information:

Research:

Books for EDS and related conditions”

cartoon of muscle talking to tendons and joints with title "inside my body right now"

 

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.

Me vs Gravity: Life with Dysautonomia

         October is also Dysautonomia Awareness Month! Wahoo! Dysautonomia is an umbrella term for several conditions that result from a dysfunction of the autonomic nervous system (ANS). Many people know of the ANS from health class as being the system that is in charge of the “fight or flight” response, and you’d be mostly right. Yes, the ANS is in charge of “fight or flight” but more broadly, it is essentially in charge of regulating the automatic functions of your body. There are nine different types of dysautonomia: POTS, OI, AAG, Pandys, NMH, NCS, PAF, FD, and MSA. Learn more about them here. For this post I will only be talking about POTS and my experiences with POTS. I have secondary hyperadenergic POTS (hPOTS).

What is POTS?

As stated above, POTS is a neurological condition in which the ANS does not send the proper signals to the body to regulate blood pressure and heart rate (primarily, POTS affects the whole body).  Normally, when a person stands, gravity pulls blood to the lower extremities. The nervous system senses this and sends a message to the blood vessels to constrict, sending more blood back to the heart and head. With POTS, this message to constrict does not get sent. Therefore, instead of the heart rate increasing by a normal 10 to 15 bpm upon standing, it can increase by more than 30 and can even double (when first diagnosed mine went from 68 bpm lying down to 189 bpm and remained that way for 10+ minutes). By affecting circulation, it also affects cerebral blood flow. Symptoms include orthostatic intolerance (dizziness), chest pain, headaches, GI cramps and dysmotility, inability to focus and concentrate for long periods of time, inability to read due to blurred vision, difficulty with recall, blood pooling, extreme fatigue, nausea, tremulousness, insomnia, loss of consciousness. 

What causes POTS?

While there is some knowledge about comorbid conditions that are commonly seen with POTS and certain types of POTS have associated causes with the start of symptoms, it is unknown what is the true cause. For example, one type of POTS can occur post-concussion and we know that but not everyone who gets a concussion gets POTS. There is currently no cure and treatment is based on symptom management. 

Common Misconceptions

  • POTS is caused by anxiety. POTS symptoms can mimic anxiety/panic disorders patients are often misdiagnosed or POTS is missed entirely because symptoms are deemed to be caused by anxiety. See research here
  • POTS is caused by deconditioning. See research here. Exercise (for those allowed by their doctors) can be a crucial part of a POTS patient’s treatment plan but is not caused by deconditioning and cannot be cured by exercise alone
  • POTS is just getting dizzy sometimes. POTS is a complex neurological condition that effects every patient different though almost all, if not all, have multiple organ systems effected. 

Lingo

  • brain fog: cloudy feeling that hangs around and causes you to do stupid shit like flush the toilet three times, forget if you took your meds or just thought about taking your meds, and being unable to form a coherent sentence

What my POTS is like

***Disclaimer: This will by no means be an extensive list of symptoms or experiences and this is just how POTS effects me. There will also be crossover between EDS, GP, MCAD and possibly LQTS.***

       I was formally diagnosed with POTS in 2015 though I had symptoms beginning in early middle school years. To save my fingers I am just going to talk about my current life with POTS and will probably do a more extensive post later, I just tired and lazy.
       Remember the last time you had the flu and ached everywhere, slept all day and don’t remember half of what happened? Now imagine waking up feeling like that every day (and more! yaaay), this is one of my main struggles with POTS. Several factors contribute the fatigue including medication and adrenaline surges/crashes but on any given day my energy level is equal to or less than a sloth on Ambien. I take naps nearly daily, sometimes multiple times a day just to keep up with my peers. I have trouble filtering out stimuli, recalling things, producing speech, following multistep directions, and reading for comprehension.
        Another big problem I have is blood pooling. POTS alone can cause bad pooling but with the stretchiness of my veins from EDS, it is much worse. I also have Raynaud’s syndrome so my hands and feet are always cold! The picture to the left is an example of blood pooling in my hands (on medication). This is what happens if I stand with one hand raised and one arm relaxed for two minutes. The white hand was raised and therefore has less pooling (gravity does all the work). After about a minute of sitting my legs look like my left hand. Now imagine a 11 hour car ride or a 3 hour lecture. This is why many POTSies wear compression stockings and socks to help our bodies circulate blood or why lying down with our legs up helps. 


       While POTS impacts many things in my life it does not mean I can’t do fun stuff, learn, and enjoy life I just need everything to slow down and take breaks. I have very limited energy and most is spent on school, existing, medical appointments, personal hygiene (showers are EXHAUSTING), and keeping myself alive. I have to worry about getting places without steps, lying down in the middle of the mall to keep myself from passing out, obsessing over hydration and medication schedules to maintain baseline and prevent things from spiraling out of control, and pretend to be a functioning adult.

Thanks for reading and make noise for turquoise (dysautonomia awareness color)! 

Sick of Silent Suffering

        Over the past couple years I have become increasingly open about talking,  writing,  blogging,  and advocating about things that are important to me,  including my chronic illnesses but I definitely wasn’t always this way and my family,  for all their wonderful other qualities,  is more the suffer in silence type.  That is how I grew up and it wasn’t until recently I realized the toll it has taken on me.  *Side note:  I do believe that this system works for some people and keep them happier,  I’m not judging.* Back to glorious me. The problem with suffer in silence is that,  intentionally or subconsciously,  it creates a wall between you and “everyone else”.  After all,  how could you possibly know that another person was facing similar challenges as you if they are also silent?  One of my most valued friendships is with someone that I despised in high school because she was getting help for what turned out to be the same condition I had (she hated me back because I flew under the radar). We reconnected after my diagnosis and confessed our mutual hatred for each other and turns out we are very similar people with similar struggles and challenges and we keep each other sane (lol, if you can call it that L.B.N). Yes,  that is a extreme example but hang in there.  
       We are social creatures.  We strive to make connections with others,  find the people who make us laugh and support us,  and teach us about the world. We long to fit in,  to be noticed, or even to be famous.  But who here can say that they have felt it is easier or better or more desired to suffer in silence no matter how big or small the challenge is? I struggled through my education beating myself up along the way for not being able to figure out long division,  for NEVER being able to spell things right,  for trying to fit in harder than I tried to learn. Growing up in an area that prides academic achievement as a critical pillar of your existence,  I felt like a failure even though I “made good grades and had good friends”.  It wasn’t just academically either,  family stress… silence,  friend drama… silence,  depression… silence, and pain… silence.  Then I graduated and moved to Birmingham,  AL.  Which made it so.  much.  worse.  There saving face is critical to social acceptance.  It was suffocating for me and I’m fairly certain contributed to my physical and mental health getting so much worse.  There is a great Miranda Lambert song (oh no Julie… not country.  Yes.  Country.) called “Mama’s Broken Heart” about a breakup and saving face. It “don’t matter how you feel,  it only matters how you look… my mama came from a softer generation where you get a grip and bite your lip just to save a little face”. 
       So I left and moved back here and that is when I decided to stop hiding and start being open about all kinds of things and I have been amazed at where it has lead me.  I have accepted the vulnerability that comes with exposing hurt,  weakness,  and challenges and through it have gained truly amazing friends,  education, healing and perspective.  I no longer feel totally alone in this world which I am remind of even in my worst days by the true connections I have made with others.  I am learning to express myself,  care for myself,  and help others.  I truly believe that vulnerability leads to connection and connection leads to understanding and understanding leads to compassion and compassion… well compassion is pretty powerful stuff.  

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“The irony that we attempt to disown our difficult stories to appear more whole or more acceptable but our wholeness – even our wholeheartedness- actually depends on the integration of all our experiences including the falls. “ -Brene Brown

Life Hacks: Spoonie College Edition

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      So I’m actually starting my 5th year of college going straight (fall, spring and summer except one summer). I have attended three different universities and started out only minimally effected by health problems (mostly GI, migraines) and wound up here, a professional patient needing complex care and supports. While each college had different systems, strengths and difficulties, I learned some valuable things about being successful in an environment that is largely unsuited for those with chronic illnesses. 
Here are some things I have learned along the way, in no particular order and not institution specific: 
    1. Disability services: The process towards getting accommodations is LONG, often repetitive, and stressful. Often, accommodations are generic,  inflexible, and “base packaged” (you probably will get about (max) half the things you need to keep up and be successful and the rest is up to you. Here are some tips to get the most out of the services offered there (and your time and energy):
      1. Start paperwork ASAP, pester those overworked doctors to get their part in before the semester starts as accommodations will never be retroactive. Also, make copies/ scans of every form or piece of documentation that leaves your hands. Things get lost all the time. 
      2. Know which battles to fight. Back to the max 50% of what you need thing, often times you will receive basic accommodation and get some pushback asking for more. Is that note taker necessary for you to succeed or can you work out a way to record lectures, ask a classmate to look at theirs, use assistive technology and save your fight for extended time on tests where you might fail without it.
      3. Send a personal note/make a personal introduction along with your formal accommodations sheet. In my experience, this has helped teachers relate to me, understand how my disabilities effect me and my learning/schooling, and also makes it seem a lot less like taking passes on things and more like “I expect this to happen, I don’t know when or where or how, but here’s a proactive heads up”. It isn’t necessary to name or intricately discuss your diagnoses to be effective.
      4. You’ll have to do a lot of accommodating for yourself/ working 1:1 with teachers. 
      5. In most universities, students with disabilities have priority class registration. SUPER HELPFUL!!!
    2. Housing: While living on campus may at first seem unappealing (again, varies in atmosphere by university), there can be a lot of benefits especially if you can’t drive. 
      1. They are required to have ADA accessible rooms. My room has widened doors, a lower peephole, lower closet bars, grab bars in the bathroom and shower, a fold down shower bench, and lowered light switches. 
      2. Accommodations for single rooms can be made for those with compromised immune systems, PCAs, MCAD, severe GI issues, and other medical reasons. 
      3. You can have a quiet place to rest in between classes, are close to dining (if you eat), and have access to evening events such as clubs, performances, and hanging out with friends without being too far from home or living in the Student Union Building. 
      4. DOWNSIDE #1: If you have problems that can be exacerbated by fire alarms… apparently no one knows how to cook MFing popcorn.
      5. DOWNSIDE #2: Germs spread fast and easy, may not be the safest place if you have a weak immune system without taking precautions.
    3. Class Schedule Advice

      1. What you want isn’t always what is best. Sure, like nobody wants 9 AMs every day… or any day… but for me, the later in the day the more medication wears off, pain increases, spasticity increases, and overall deterioration occurs. Class is already hard enough to sit through, concentrate, learn and remember… don’t make it harder on yourself for a potential 2-3 more hours of sleep. Or flip all that if that’s how your body works.
      2. Register ASAP. Classes fill up, plans change.
      3. Schedule max number possible classes, attend all the first week and then drop as needed. That way you secure your seat, get to meet the professor, see the syllabus, see if there are major barriers in the class (i.e.  Service dog you’re allergic to in a small classroom)
    1. Medical care/ health safety
    1. If you live on campus, tape a folder somewhere visible (wall, by light, dresser) and write EMERGENCY INFO on it super big and put in your medical emergency info, copy of your license/state ID card, insurance card (if you have it), and your school ID/student ID #
    2. Start a file with on campus health. Even if they never care for you it is helpful for them to have your base information in case you need them in an urgent matter, have doctors far away, or just need something simple like a wound cleaned. Most student health offices will be able to do allergy shots, some will even help manage infusions. 
    3. Introduce yourself to the campus chief of police, especially if you have the potential for reoccurring EMS issues like seizures, anaphylaxis, diabetes etc. They are usually first on the scene and can inform EMS.
    4. Wear a medical ID bracelet. Bonus if it has a way to see all your info. See my post on my system here.
    5. If you have asthma, MCAD, or immune problems I highly suggest purchasing a high quality, relatively comfortable mask to wear outside around campus. I pass smokers, high perfumers, and other triggers CONSTANTLY on campus. I use these and love them (recommend the ones with 2 filters for comfort and breathability).
  1. Miscellaneous 
    1. Join one club. Even if you only go twice a year, you may meet some people and you feel somewhat a part of things.
    2. If you live on campus, get to know some people on your floor. They may be good for procrastination buddies, errand helpers, cards against humanity mates, or near family friends. 
    3. Don’t bring everything you own to move-in. Stuff accumulates anyways and it is a pain.
Hope this helps. It can be overwhelming but it IS manageable with the right supports.