International Day of Acceptance

Happy International Day of Acceptance!

Today, and every day, I challenge you all to inclusion, acceptance, and understanding. I challenge you to make a new friend, communicate in a new way, expand your knowledge and experience and above all, be kind. Ask questions, do research, and do not ignore differences. Lets start 2018 with more open dialogue because everyone deserves to feel loved and accepted.

Here’s what some other people say about inclusion and acceptance:

“Coming together is a beginning: keeping together is progress; working together is success.” -Henry Ford

“Love isn’t a state of perfect caring. It is an active noun like struggle. To love someone is to strive to accept that person exactly the way he or she is, right here and now.” -Mr. Rogers

“You are imperfect, you are wired for struggle, but you are worthy of love and belonging.” -Brene Brown

“The world is no real home without all of us here.” -Brian Andreas

 

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?

 

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!

 

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:

• Oh, Thats Why I’m So Tired (Oh TWIST)
• The Chronicles of a Millenial Spoonie
• Brilliant Mind Broken Body
• EDS Bloggers Section of Chronic Illness Bloggers
• Annabelle’s Challenge
• My Stripy Life
• A Life Not As Advertised
• The Chronic Illness Diary of a Young Adult
• Nutritiously Natasha

Vlogs:

• Christina Doherty
• Hospital Princess
• Life with Stripes
• Amy Lee Fisher
• Not Your Average Zebra
• Ehlers Danlos Girls
• Cheyenne Love
• Embrace the Brace
  

 

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:

• The Ehlers-Danlos Society is a great resource for information, guides, and awareness. They also have information to help with diagnostics, emergency information, and education for medical professional.
• NIH on EDS
• National Organization for Rare Disorders on EDS
• Ehlers- Danlos Syndrome C.A.R.E.S. Network
• RareConnect Forum for EDS
• Medscape Info with genetic and pathophysiologic information
• Compiled resources from OHTWIST Blog

Research:

• EDS Society Current Research
• Clinical Trials
• Vascular EDS Research partnered with C.A.R.E.S

Books for EDS and related conditions”

• Joint Hypermobilty Handbook by Brad Tinkle. He also wrote another book but it is very hard to get your hands on but can be found here. This is a very useful handbook covering common issues of EDS by body system. Great to share with healthcare providers or to just understand your body more.
• A Guide to Living with Ehlers-Danlos Syndrome (Hypermobility Type) by Isobel Knight
• Living Life to the Fullest with Ehlers Danlos Syndrome by Kevin Muldowney PT. This book describes a physical therapy protocol by a well respected physical therapist.
• Special Like Me… Ehlers Danlos Syndrome by Janelle Shannon LCSW is a children’s book about a boy with EDS to help children with EDS cope. If you have Amazon, there is a free e-book avaliable.
• Never Bet Against Occam: Mast Cell Activation Disease and the Modern Epidemics of Chronic Illness and Medical Complexity by Lawrence Afrin M.D. I HIGHLY recommend this book to anyone diagnosed with a mast cell disorder, especially since it is a rather new condition and there is limited information out there. Afrin is one of the leading researchers/providers studying and treating mast cell disorders and his writing is thorough and easy to understand language (verified by people who aren’t me for those of you that snickered).
• POTS- Together We Stand: Riding the Waves of Dysautonomia by Jodi Epstein Rhum. I haven’t personally read this one but its on my list per recommendation by a friend.

 

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.

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.

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

I moved… bear with me…

Hi all, I will be continuing my blog here for various reasons. I will eventually get my posts moved over here but until then here is a meme to hold you over until the next post!