Living on The Edge

To put it simply, having a Rare Mast Cell Disease sucks big time.

For those of you who aren’t on my Facebook you may have wondered why I haven’t blogged for a while.   Since going to Toronto for my book signing of Finding Heart Horse at the Hay House I Can Do It Conference, I have been hospitalized twice.  Two weeks apart, I headed to the ER in anaphylaxis and unable to breathe.  Along with that I had been light-headed, teary, in terrible bone pain, nauseous with vomiting and diarrhea (at the same time unfortunately).  Add into the mix a sensation of bugs crawling up the back of my neck into my scalp, flushing, chills, so fatigued I could hardly drag my body outside to take my dog out.  The list goes on and on.  You think you can manage it, ignore the symptoms or blame them on something else, such as I was doing, thinking it was my low iron.  Mast Cells play havoc in every organ and system including the brain, tricking you into rationalizing the pain, the fatigue, the dizziness, the tears.  Eventually, the inevitable happens.

It’s difficult for people  to understand because when you see me, except in a major flare I look healthy.  The outside is deceiving.  Inside, my organs are paying a huge price for late discovery and diagnosis due to lack of medical information.  I’m adopted.  That’s a whole blog in itself, about the need for medical information for adoptees.

In Canada, 1 in 12 people live with a diagnosed rare disorder. There are approximately 7,000 types of rare disorders documented in Canada. This translates to nearly 3 million Canadians diagnosed with a rare disorder, one of which is Mastocytosis. These numbers do not include countless Canadians suffering without a diagnosis. To put this in some perspective, our organization is aware of approximately 300 people of all ages in Canada diagnosed with a form of mastocytosis.

Mastocytosis is a rare disorder (or disease). Medical research articles alternate between classifying it as a “disorder” or a “disease”, depending on the researchers’ focus on the various forms or types of mastocytosis. We refer to it as both alternately in an effort to be inclusive for all patients, regardless of type, form, variant or subvariant named within the mastocytosis definitions.

Mastocytosis and Mast Cell Activation syndrome affect all systems, organs and tissues of the body. In particular, skin, liver, spleen, lymph nodes, bones and bone marrow, lungs, gastrointestinal system, eyes and blood are the most documented as adversely impacted by these diseases. Research exists but much more needs to be done to further identify and understand the less studied effects of mast cell disorders on the heart, brain and female reproductive system.
The signs (eg.skin spots, unusual lab test results, etc.) appear due to an over-abundance of mast cells either limited to the skin or internally, or both. The symptoms occur when mast cells are triggered to degranulate. Mast cell degranulation is a normal response of the immune system trying to protect the body. However, these disorders trigger mast cell activation (ie. degranulation) with or without apparent or valid trigger. Things such as foods and drinks, extreme temperatures in water or air, emotional and physical stress – these are only a few examples – can trigger mast cell degranulation for these patients. If the patient has both an over-abundance of mast cells, in addition to having excessively active mast cells (ie. degranulating when they should not), then the symptoms and illness become more severe, prolonged and life threatening.

http://www.mastocytosis.ca   The above description was copied from the Canadian Mastocytosis site.  It has a wealth of information for patients and family members.

http://www.tmsforacure.org/welcome.php   A great site that provides up to date research, support, information for both patients and families.

I don’t want to focus on creating a blog just about my illness but it is part of who/what I am and depending on how stable my mast cells are, it dictates a good part of what I am able to do..even blogging.  I want people to be aware of those around them that may suffer from various invisible illnesses.  There are many, and if you ask your friends, I have no doubt you will find several with the more common ones such as Fibromyalgia or CFIDS.  Understanding “The Spoon Theory”is helpful to those you may have in your life that are ill.

Click to access BYDLS-TheSpoonTheory.pdf

There are also many support groups online and on Facebook and I have to say for both mastocytosis and adoption these groups have been my lifeline.  I’ve heard it said that online friends aren’t real…I’m here to tell you they are as real and important as your friend across the street.  After years of communication and coffee by Skype, they are a support system of compassion and knowledge and understanding that is so important when one is isolated and ill or struggling with coming to grips with issues only faced by one that is walking the same walk.

I’m going to include some pictures and then move on to another blog post.  I just wanted you to know, I’ve been thinking about you even while

 Living on The Edge.

IMG_3484IMG_3481IMG_0001I have pages of pictures but you get the idea.  It’s helpful for those of us with rare diseases to chronicle symptoms by photo to help the physicians who aren’t familiar with something unusual.  I also keep a journal with foods eaten and symptoms along with meds taken.

When I go into the hospital, it’s always after using at least 2 epi-pens myself.   The Advanced Life Support medics arrive, as I have a tendency to crash quickly with low O2 sats.  They start the IV’s, usually administer more epinephrine and ventolin along with O2 and benadryl IV.  This last admission, was 12hrs. I was headed for the ICU and intubation, fortunately at the last-minute…. after 10+ 2mg epi-nebules, multiple ventolin treatments and the TMS Protocol, which is IV Benadryl, Solumedrol, Ranitadine, administered twice, my symptoms began to subside.  My O2 sats were running low at 70-80 and when 90 on room air I was allowed to go home.  Recovery time is usually a week or so from one of these episodes.

Life is Living on the Edge.

  Each day is a gift whether you have an illness or not.

 Make the most out of your 24hrs.

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Living with a Rare Disease – Support Rare Disease Day 02.28.14

Rare Disease Day is coming up in the next week.  It’s an annual day recognized around the world to help raise awareness among the public and decision makers about rare diseases and their impact on the lives of those that live with them daily.

This year, CARE is the theme for 2014 and oh my, they couldn’t have picked a better theme than that.

www.mastcellaware.com

This is the logo from http://www.mastcellaware.com, a great resource for anyone wanting to learn about mast cells, what they do and how they misbehave in those of us suffering from this rare mast cell disease.  I was looking for a video, and had in fact planned on doing one of myself this week but unfortunately, the mast cells won.  Even a simple picture of what I looked like this week would not do justice to how I have felt.  It’s a  frightening place when you live alone and are too ill to think clearly and make the proper decisions for your health.  It took me being in severe pain for several days to recognize, with the help of my mast cell peers and my Respirologist that I either needed to be in the hospital or take a few large doses of prednisone to break the reaction.  You would think after this long and having experienced this debilitating muscle spasm type pain before, I would know that.  It’s a deceiving process this illness.  You always think you can manage until its at the brink of anaphylaxis.  My O2 sats having been running low, in the 70’s and my iron keeps dropping.  I know that has some bearing on my thinking processes but ultimately, I’m the only one that has to make those decisions and I’m here to tell you it’s not easy.

There are many great links to learn if you go to any of the sites I have posted previously: http://www.tmsforacure.org, http://www.mastcellaware.com, http://www.meandmymastcells.com, mastocytosis.ca and many others.

word cloudI was going to list the symptoms but last night all I could do was play with word cloud letting it create an artistic list.  There are so many, so different, so unique to individuals its almost impossible to describe.  When I had the last nasty chest wall muscle spasm my immunologist said it wasn’t related because he wasn’t familiar with it. Of course it arrives with various other mast cell symptoms but is a new one for me and the most prominent in these flares.  Well, I’m here to tell you it totally was related, just another way my body was telling me loud and clear that my mast cells had exploded spewing forth hundreds of chemicals into my body, and this time my upper body muscles couldn’t handle the load.

I was in so much pain (and I know I have a high tolerance to pain) that I couldn’t move.  Each movement was excruciating and only the must do’s got done..the rest of the time was spent with a heating pad, medication, and meditation trying to just get through the moments.

We all try so hard to stay positive and caring.  I can say, that besides adoptees, I don’t think I’ve met a kinder, more compassionate group of people than those that have touched my life through mastocytosis.  I have never been my diagnosis and I’m not going to let my life disappear into the abyss of labels.  Some days are harder than others and the mast cells win.

A rare disease is defined differently in different countries.  I the USA it is defined “rare” if it affects fewer than 200,000 people.  The issue with rare diseases is the lack of scientific knowledge and the quality of information often results in delayed diagnosis.  I have lived with this my whole life and I can identify each and every escalation point where my disease jumped up a notch.  If you read my books you will know there were many traumas, each one setting off another step and cascade of symptoms and finally with the discovery of my biological family my mast cells went over the edge due to the stress.  It took many years of putting the puzzle together myself to finally find the answer to both biological and genetic factors leading to a diagnosis.  Unfortunately, I had to go to the states at the time for accurate testing which brings up the need for increased funding for testing in Canada and quality care resources.

I often wonder, what my life would have been like, had it not been always tainted by the “outward” signs of mast cell disease.  The emotions that had nothing to do with depression, the surgical complications, the many heat/exertion related physical disasters nearly killing me one time in the Jungle of Thailand.  I wonder how my adoption reunion would have gone if I had known what was going on besides the normal reunion roller coaster ..was that my mast cell disease had taken over and i was beyond exhaustion and in such a place of physical reactivity. It’s too late now to go back and change all of that but I often wonder. What if I had had proper treatment?  Would things have gone differently? Would my organs be fully functioning now if I had known 25yrs ago?  The answer to all of those questions is of course things would have been different.  Looking back I can see clearly, unfortunately, that’s looking back and I can’t allow myself to go there to often as it can’t be changed.

It’s important that those who care about people with “different” illness’ ..be it in mental health, addiction, rare disease.. support not only the person but the fight for better support and care.

To live with systemic mast cell activation disease is frightening, isolating, confusing and frustrating.  I’m sure I could list many more things.  Isolation is difficult, friends are lost in the shuffle from being active to having days dictated by how you feel.

 Reach out and let people know you are thinking of them.  Pay attention if you don’t see or hear from them.  They may be in trouble with no one around to notice.

I refuse, on okay days, to let this get me down, to let this define what I am and what I can do and be…..on good days.

Treatment

Above is only a partial list of treatments we must deal with .  I feel like the nurse I used to be, when each morning I line up my pill cups and pour the daily meds, only this time, they are all for me.

The purpose of this blog is to educate, to encourage everyone to become aware and knowledgeable about rare diseases and the people who live with them.

Kindness