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I Wanted Clusters!

NOT CLUSTERS

I am not sure why men can’t listen to what you say to them. I mean, I make eye contact, I move my mouth really slowly just in case my hubby is actually deaf and has somehow managed to get through life reading lips and it is discovered one day and everyone goes “wow that is an amazing story” and they make one of those mini movies they send around to your email with music and fluffy kitties and people pretend they have something in their eye while they type in the names of 343 of their closest friends to send it on to so that they don’t die a horrible death, lose all their money or have a loved one die in a car crash.  You don’t want to pass on having something incredible happen in exactly 34.3 minutes.  I also worry about whether someone makes his life story into a movie … “Alan Keller” and he comes off looking like a complete saint and because every movie, no matter how short, needs a villian .. . They identify me.  So I speak very slowly ….

I said, “Get the cereal that has the granola clusters … NOT the flakes.   See they are almost exactly the same box and almost the same cereal but they are not.  One has flakes.  One doesn’t.   Say NO to flakes.  Do you understand? “  and I showed him a picture of both cereals from a flyer AND the empty box of the actual cereal that I wanted.  “Do you have it, or do you want to take some notes or something?”

He waved me away with his hand and said something about having better things to do and did I think he was stupid.  I used to answer that question but then he told me it was a rhetorical question and there was no need to answer.  I answer anyway … in my head.  In my head he never talks back.  It is easier that way … for everyone.

He left for the grocery store.

I know,  what the hell was I thinking??? But every once in awhile, usually after either a couple glasses of wine, great sex, or a shot of morphine, I get really euphoric and think anything is possible.  Oh,  and  I love everyone.  I can’t really share what actually happened to cause this particular moment because I signed court documents promising to stop discussing our sex life in these blogs.

I know, I Know … you already can see what happened.

He sooo came home with flakes.

Yup .. and I am like .. “WTF? What are these?”

He is like .. “Cereal,“ ’cause he is quick like that.

And I am like, ” But I told you clusters, NOT flakes?”

‘”No you didn’t.”

‘”Yes I did?”

” No you didn’t.”

” Yes I did.”

And then, he  takes the box out of my hands and says, “Let me see,” and he actually squints his eyes and reads the ingredients like the big picture on the front of the box with the flakes falling into the bowl are confusing him or something. So he hands me back the box and says “I can’t see the difference.”

“Umm …well you see this?”  I was pointing to the picture on the box he bought, “THIS is a flake.  Note the flat, flakey shape? And this thing on the box  I wanted,  that is all round and big and NOT flat or flakey like? THIS is a cluster.”

He shrugged.  Like a “who cares” shrug … and I am like “you did NOT just shrug at me.”

And he is like, “Well I don’t see what the big deal is.”

And I go, “None!! No big deal at all! And I am sure you will enjoy eating these for breakfast and then going back to the store and getting me my clusters.” And I pulled out the bread mixing bowl and dumped the whole box in it, poured milk over it and got him some honey.  “Open,”  and I placed a heavy laden spoon full of flakes in his mouth.

So he comes to me later and asks if he can ask a question and I say, “no.”

He asked anyway because men just keep talking even when the evidence indicates they should make another choice.   He was fearless.  No sense of the dark doom stalking him in our kitchen.  I suppose to be fair, I  wasn’t facing him when I said it so legally, in a court of law, he could make a case for he didn’t hear me.  Unfortunately my rules seldom concern themselves with boring details like legalities.

“What do you have against flakes?” he asked, treading over the dead bodies of fearless angels . . . most of them men.

“They taste like something grandpa used to feed the cows.”

” Well how am I supposed to know that, I have never tasted cow food.”

“Yes you have.”

” Nope never.”

I picked up his uneaten bowl of cereal and pointed to the flakes and the little dingy grey pellets fighting to stay afloat in a sea of milk, “Cow food! BTW you have to finish that before you can go to golf.”

He thought about it for a moment, “You ate the cow food at the farm?”

“NO this smells like the cow food did?”

“How do you eat a smell?”

I gave him the look and he shrugged again and said I was being ridiculous. I don’t think he appreciated my mooing at him as he walked away.

See, this is why I like the idea of using hand puppets when trying to explain things to men.  Not only can you simplify communication and illustrate your point, you have the added bonus of having your middle fingers of both hands extended at all times.  I think expecting them to understand words is just too big a stretch.

Sometimes You Just Have to Pee in The Sink.

out of order

The other day I was working on an art piece and I needed a screwdriver.  My husband had recently moved everything out of the attached garage to the large garage/shed in preparation for our painting the floor this weekend so all the tools were under lock and key.  The key was the issue, it was somewhere safe . . . on his key chain, with him, on the golf course.  I couldn’t put off what I was doing until he got home so I found a nail file that worked.  Later I needed to hammer a nail in.  I had plenty of options to use in place of the hammer;  I used the heavy handle of an older knife.  I was intent on what I was doing, and I made use of whatever was around me to get the job done, even though what I used was not normally used for that purpose.  Success!

It pretty well sums up my approach to life.  I tend to make my own arrangements.  I work with what I have to get the job done.  I don ‘t think I have ever had an “incomplete” or that anyone has ever had to talk to me about deadlines.  I always get my work done and, I tend to thoroughly enjoy myself while I do it.  Even with the most mundane task, I have learned  to make it into something else entirely.

I can remember being a  kid and having to shell peas endlessly one summer and I transported my mind out of that and became the princess that had to save her people and thwart the wicked witch by out shelling all competitors.  I had a whole fantasy with characters and conversations that played out while the afternoons whiled themselves away.  At times the story took over and I lost myself in my own imagination and at times I was trying to beat a certain number of peas shelled in a minute.  I adapted that practice as I grew up.  Fantasy stories fueled by my imagination gave way  to personal challenges that had nothing to do with the task at hand, but had everything to do with pushing me.

We tell ourselves the stories that either make what we are doing unbearable or, doable.  Shelling peas was mind numbing and I would have been overwhelmed with the amount of peas that had to be done and the number of summer afternoons sacrificed doing something I hated.  I changed what the task was by changing how I thought of it and making it into something I could use that did serve me.  Finding a way to make things relevant to my life was my job.  I never had to settle with just being bored … unless that was my choice and it never has been.  I try to use what I am given in life, even when the tools suck.

We reach the point in our lives where we start to have time to examine where we are.  We have the family, we have some stability, we have routine . . . and we start to wonder what else there is and if our bright flame has already burned down to embers.

What, in life, is ever going to be as magical as our sport hero status, our homecoming queen win, emancipation, our wild and crazy nights out with friends where the soul purpose was to completely have fun without any constraints, landing a great job, our falling in love, getting married, getting our first new car, our house, our kids ?

Why do we feel so bittersweet about everything that we have done and completed?  Why do we look at what has been and consider what is ahead of us and lean towards depression?

It is a point we all reach somewhere in our time line.   We have journeyed a long way from our childhood and usually we find ourselves with one foot in our parents plan of life and maybe only a toe in our own dreams of what life was going to be like.  Worse, we sometimes see ourselves drowning in the very life we swore we would never choose.  How we got there seems irrelevant to the bigger concern, how do we escape?

We know we don’t have the luxury of just scrapping everything and starting over because we have responsibilities.  It can make us bitter.  I can make us want to give up completely.  We do that because we feel powerless and because we look at it as something that was done to us.  Sometimes it becomes easier to blame the world and the people around us than to step up to the plate and realize we made the choices that brought us here.  We may have done it by simply not paying enough attention, or we may have consistently made choices that were the easy way or the wrong way,  but it is all on us.   Taking personal responsibility for ourselves is the only way to take our power back.  The good news is that once you accept you got yourself here, you must also begin to understand that you can use that same power to change things.   We don’t have to carry on with an unhappy life as if we have no choice.  We still have choices.

The second reason we feel defeated is because the distance between what we want or need and where we are can seem impossible.  How do we get from here to there when we feel so trapped?

You can’t just grab the wheel of a boat and jerk it sharply in the direction you want to be going.  You have to slowly correct it by making a gentle and sweeping arc that turns you towards your intended destination.  It is the same in life.  You begin.  You get in touch with who you are and what you want, even if it is just the feeling.  Then you make the corrections slowly, working with the boat, against the wind and seas and all else that factors into your move, as you make a wide sweeping turn.  Once in the process, the obstacles morph from stumbling blocks into stepping stones.

You can do that with your life.

You can look around you and start focussing on what you have and how those things can serve you instead of only worrying about what you do not have.  You can stop those things that no longer feed your soul.  You can rid yourself of constant contact with people who do not support and enhance your life.  You can learn new skills, you can live this day with the heart of the person you want to be, changing habits and acts that no longer serve you.   You can connect with those people and experiences that you now realize mean something.

The TV character, McGyver was respected because whatever he got himself into he could improvise and get himself out of the situation.  He didn’t understand the word “can’t.”  He didn’t worry about the perfect solution.   He knew himself and he knew his own abilities.   Knowing yourself affords you that same confidence.  It gives you options, and the most exciting point of all of it is, these are options that are about you.  These are options that bring you tremendous peace, or joy.  You can live with even failure and tragedy when you can own them.  If you know there was nothing more you could do, that you did your best, that you did not intend something … and you love yourself . . . you can get through it.

You  get to a place where you are so sure of who you are that the circumstances of your life are just tools you have to work with.  If the screwdriver is not available, you make use of the nail file.

“Sometimes you just  have to pee in the sink.”  Charles Bukowski

TFFT: Compassion is Powerful, Find Out Why.

Compassion hurts

It is hard to be tender hearted.  Despite the best efforts of my grandparents who raised me, my teachers, all the cruelty of the world and the mean heartedness of some people, I have survived.  I stopped fighting it as a young woman when I finally realized that despite the pain, it was also my greatest gift, affording me sight and understanding that not everyone else had.

But compassion is not just a gift, it is a necessity for our lives, and especially for the future of our children.

The ability to feel compassion means you have a connection to the world around you.  It is what causes us to stop from bullying someone when we have been bullied.  We can remember how painful it was for us so we can understand the hurt our actions will cause someone else.  It is what allows us to respond with a hug and kind words when we see that someone is depressed.  It is what can even spur us into action to do away with injustices.

presence

 

But there is an even greater power in compassion.  Not only do we know understand that the emotions we hold in our bodies have a direct impact on our DNA and the life affirming or life denying chemistry that is released in our bodies, we know that compassion has the most  powerful impact of all the positive emotions.  (See Emotions Can Change Your DNA.)

Dr. David Hawkins in his book Power vs. Force created a Map of Consciousness of the effect of emotions on the energy level, which can be measured through applied Kinesiology.  He shows how the presence of just one person who resonates at the higher levels of compassion and empathy are capable of positively impacting the energy of the masses.  You can test out and try many of the things he talks about in your own life.  It is a fascinating book, well worth a read.

We all have had experience with how the energy of a positive or caring person can alter a room for the better and how someone who is depressed and negative can seem to suck the life right out of people.  I have experimented myself by noting the change in my office staff with a change in the emotion I hold in myself.  I have learned the power of learning to catch the negative emotions as they happen and channeling myself through simple exercises and practices to a more positive place.

 

Inner Peace

 

It has brought me such joy to realize I can enjoy what is natural for me instead of being an emotional football that is at the mercy of how others treat me.  It has given me a sense of purpose and a firm resolve to honour what my heart asks of me.  As I work to improve my ability to overcome my many failings and shortcomings I am moved that in doing so I can make a difference in the world around me and in the lives of those people I love.

Looking at the world and others with compassion has helped me feel more connected, more forgiving, more willing to do what may seem difficult and beyond my reach.  I know that all that is needed for all of us to find peace and joy is simply for us to love one another, support one another as each of us find our own ways and determine what our journeys are about and how we will journey them.  Defeat is what happens when you focus on the impossible.  Success is what happens when you find one thing that you can do right now and do it.

Pray For Peace. Compassion One Heart At a Time.

 

It is easy today to point to people in other countries and situations and talk about their shocking lack of compassion.  How could they kill innocent children like that?  How could they do that to women?  How could that woman have done that to her child?

But I live in a world, in my small corner of life, where there are people living their lives without compassion.  Their happiness seems predicated on making other people suffer.   These are not random acts that occur that one could say it was a mistake, they didn’t mean it …. these are systematic, prolonged efforts to destroy other human beings with unkindness.  These are people who are not only openly practicing hate in their own lives, they are teaching their children how to do the same.

Peace is not something that has to happen in a third world country or at the neighbour’s house.   It has to begin within our own hearts and govern our actions.  It has to start with the ability to act with compassion in all things.  We can be firm, have principles, effect laws . . . with compassion.

Because the only other choice is for people to join together to cut out the cancers in our society, to start saying “No” to the people who fill our lives with their hateful words and conduct.  I know for me, once there is a clear pattern of behaviour that says, “no matter what I choose to continue to act with malice and hate.  It is my intent to continue to hurt and destroy others.”  I am out of there.  THEY have to find their hearts.  THEY have to effect change in their lives.

Choosing peace, choosing compassion is as much about what we do not choose.  There is no place in a world of peace for people whose view of life and love is so narrow that they require people around them to hate others in order to be “loved” by them.  This is the mindset of the sheep herd.  This is the mindset of bullies.  This is the mindset that leads to war and death.  It is not something “over there” it exists all around us and we have to start saying “no” to it in our own lives, and making sure it no longer has any place to be comfortable.

 

 

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Moses, Briefcases, and Crinolines. A Sunday School Take Down.

Moses

While other kids were getting their “Strawberry Shortcake” back packs I was insistent on a briefcase. I had some important stuff and no way was I going to trust it to some pony that sparkled or that dimwit Barbie.  I firmly believed that if you were going to be taken seriously, you had to dress the part.

It was really effective in church. They would herd us into Sunday School class – I am serious … this bell would sound and  this guy who looked like Moses leading the Israelites (only we were more miniature and in fluffier dresses)would take us through the wilderness (the pews) to the promised land of milk and cookies.  It would be years before I found out that “Moses” was a woman and that yes, women could have facial hair like that and really ugly brown sandals.

Sunday School was basically a babysitting service for parents.  I am not sure any of them were really that committed to church. They kind of picked and chose what parts they wanted to follow which was usually the required outfits, singing (albeit off-key) most of the words in the hymns and standing and sitting when required. After that it was like a free for all, make up your own rules as you go. They rejected the hard stuff like loving one another, not drinking themselves stupid the night before, and honesty.  In fairness some of them may have just forgotten some of it.  There are 10 rules and 6 days in between reminders and if is hard when you really really want your neighbour’s wife.  I think they just liked the idea of getting a break for a couple of hours on a Sunday morning – who cared if they all had to dress up and sing to get it.  They were puppets jumping up and down and responding to the old lady with the purple hair waving her arms in the air.

They would get the kids into the basement, closing the door to protect the parents, and make us sit on little painted chairs like somehow we would be excited to sit some more just because we were lower to the ground. People really underestimate the intelligence of children when they think they can be distracted with some bright colours and smiling, cut-out suns. First we had to perform – singing “Jesus Wants Me for A Sunbeam” – which I could never figure out. I thought Jesus knew everything and if he wanted sunbeams he should have just made us sunbeams.  I actually had a few suggestion on who to sacrifice first.  I dreamt about it …

“Aria where is Biff and what have you done with him?”

“Nothing!”   And I would go to the window and point up into the night sky.  ” Jesus wanted him for a Sunbeam … so he went.  He gave his life to the Lord.  You should be proud.”

Also – it should be noted that none of my dreams concluded with me and my grandfather alone in my bedroom with Mr. Belt.  For years that was how I could tell the difference between reality and fantasy.  Fantasy did not have a “Mr. Belt.”  The leader of the fantasy party had outlawed belts long long ago and there were never any elections.  Fantasy world is a dictatorship where my butt is NEVER under attack. Reality sucks.

So we sang to Moses about being Sunbeams and Wise men and Foolish Men who were builders. I struck construction off my list of possible careers early on – just to avoid that thin line between ending up a wise man or a fool. We would have to do all these songs with actions in them because singing about fishing without the casting and reeling in action can actually put people into a coma. I tried really getting into it and making it more realistic by screaming once when I hid “this little light of mine” under a bushel which is evidently your hand. Why can’t they just say “hand?” How are little kids supposed to know what they are even talking about? Anyway I screamed because come on people, if you put a candle under your hand it is going to burn.  How many times had I been told NOT to put my hand over the birthday cake candles because I would get burned?  And then there was Moses smiling and singing and telling us to put our little light under our hands.  So I screamed that my hand was on fire and I stopped, dropped and rolled.  I just want to put that out there that I was stopping and dropping and rolling long before that became a famous saying.   Later my butt was on fire and I learned never ever to get carried away with the song actions. Sunday School song actions must be contained.  A good rule of thumb is if what you are about to do gives you that “whee” feeling in your stomach like when you go up and down hills really fast in the car … probably not a good idea.

Notice how the teacher always thinks it is a fun idea to divide the kids in half and do a “sing off” to see who wins? Ya well don’t get sucked into that. There is never a prize.

And then our singing would be done and we were given cookies and milk.    I refrained from clapping my hands together and barking like a seal.   I did however balance a ball on my nose once in hopes of a second cookie but Moses failed to see the talent. I was not being a good Israelite. Moses insisted on realism in her class, she would make us Israelites if it killed her and seriously what was I thinking? How many Israelites are Harlem Globe Trotters or working at Sea World?

When the teacher was ready to get into the meat of the lessons and we were all sitting around the table, I would slap my briefcase down on the table and pull out a couple of stone tablets, and my thick black rimmed glasses.  I would clear my throat often and squint at the teacher, then frown.  Squinting and frowning are a lost art.  You have no idea how effective they can be as social tools.  I am campaigning Facebook to include those as optional responses to postings.  We are not one trick ponies here.  We can “like,” of course, but we can also “squint” and “frown.”  I can’t be sure, but I think it was effective.  “Moses” was definitely thrown off but I was never sure if it was the tablets or the 3 year old with a briefcase that made him pee his dress.

I eventually just took over my whole Sunday School Nursery class. I wore Moses down. I gave myself a standing ovation often. I would stand on the table and twirl my fluffy dress and swing my briefcase… just so they knew I meant business. I would steal the cookies from the other kids and stuff them into my mouth before “Moses” could catch me and make me give them back. Eventually I learned to stuff them in my panties and that way I could enjoy them later and Moses never crossed that line. I think it is in the 10 commandments.

I was heady with power.

That’s what a good briefcase can do for you.  You have to grow up a bit to be able to learn restraint.  Oh … and also to be able to tell a girl “Moses” from a boy “Moses.”  AND understand you can buy your own cookies and eat the whole pack without having to share with any of the other Israelites, or going to church, or wearing underpants.

Some day Harvard is going to do a study to understand the impact of fluffy dresses and a briefcase on brain cells.  I am sure they will find they have been the downfall of many a good woman.  I blame all those crinolines for the insanity of my childhood . . . and Moses of course.   That’s my story and I am sticking to it.

TFFT The Pain of Letting Go

let go

 

Letting go can seem like the end of the world.  What will happen to us if we let go of something or someone that has been such a big part of our lives, especially if the letting go is not what we want to do?  Our penchant, especially as women, is to love.  We all paint pictures in our heads, tell ourselves stories about who we are, what we are supposed to do with our lives.  We can race ahead of our decisions and imagine the negative outcome of our actions.  We hear our families disapproving voices, we relive all the Sunday School Lessons, we feel the disgust of strangers who don’t even know us … judging.   Are we bad people when we finally choose ourselves instead of bleeding ourselves dry for people and situations that are not healthy and may never be?

Letting go can be easier if it does not involve a family member because we have strict codes of conduct.  Parents must never give up on children.  But children grow up and like all of us, they become responsible for their actions.  We have to accept they are who they are and sometimes our kids do not choose us.  Sometimes our families turn their backs.  Sometimes it is our partners.  Even when we have compassion or understanding for criminal behaviour, we cannot condone the criminal acts or allow it to continue.  Sometimes in our lives we have to say, “enough.”  We have to let go and choose ourselves because there really is nothing we can do to help or change someone else  and most importantly, because we owe it to ourselves.  Sometimes OUR soul needs us to stand up and fight for us.

 

value

 

There can be tremendous healing in choosing ourselves and refusing to buy into or live the negativity anymore.  There is power in a “no” that is for ourselves.  We can step away from the damaged cancerous hole in our lives and move on and towards the light.  We can take the lessons, we can love and let go without anger or resentment . . . not towards any other person, and not towards ourselves.  We can find strength in our decision.  We can become kinder, more loving people.  We can grow.

No-one can truly understand our journey.  Our lives will be full of people who will never try, who are not interested, and yes, they may judge us but those people would have found something else to judge us, regardless of our personal choices.  The issue is never what they are talking about, it is only their need to judge other people and to be unkind.  We are the only ones who know who we really are.  We are the ones who know our heart.  Try listening to it.

 

Kathy Kinney

Choosing to let go can feel like it is only about endings and pain.  It can seem like it is cruel to other people.  But letting go is not about ending as much as it is about moving forward.  It is about love.  Loving yourself enough to choose life and light and loving the other person enough to refuse to participate in what is unhealthy and ugly.  Relationships are give and take.  They work because two people connect in some symbiotic way.  Love works because you give to each other.  It cannot work when the love is one sided.  That is where one person always looks after the other and the other only cares about themself.   That means one person is being neglected and if that person is you, it is not healthy, not ever.  Sometimes in letting go you free that person to find their way back to wholeness and sometimes you simply move on and find others who will love you and honour your journey.  And with each step you take towards wholeness, you will find peace.

Promise to My Husband Before He Died.

Promises

The ALS ice bucket challenge is all over the internet.  Lots of people are doing it in half measures (no ice – which I suppose is proof of how cold the water actually is)and some are doing it without any real understanding of the purpose behind it.  Such is the power of the internet.  We don’t even have to understand something in order to line up to do it because everyone else is.

I watched one of the video’s of a young man who understands ALS from a different view than most of us.   Then I spent a night tossing and turning.

I was impacted by the pictures of him tending to his mother.  It brought back too many memories of the 3 years my husband suffered before he died.  It also brought back conversations we had where he asked me to make sure that I shared his story so that this did not have to happen to any other families.  That was kind of how he rolled.  He hung on way beyond anyone’s expectations because he was worried about the kids and I.  He took his friends out for coffee or lunch to tell them he was dying because he could not bear that they would “hear it” casually from someone else.  He wanted people to know he knew he was dying and that he would be ok.  He wanted them to know he appreciated them.  He wanted his suffering to create understanding and compassion and maybe even force changes for other Acute Intermittent Porphyria Sufferers.

And then he died.

And life moved forward and even within the family there was this attitude of, “stop talking about it, move on,”  like that was the healthy thing to do . . . like that was more noble than honouring his wishes or caring about other people who are currently living the hell we once did.  Our lives became comfortable.  Denial was so much easier.  Everyone has a choice as to how they want to process what happened and what they do with the information and we all found our comfort level moving ever further away from the painful memories.

But last night I could not stop thinking about my husband and how he suffered and what he asked.  His children have children.  Within that one short generational step the knowledge and understanding of the disease is almost completely unknown.  Yet, the children have the potential of developing the disease or being silent carriers.  Their children could be silent carriers for generations before the disease pops up again.  One daughter is in the nightmare her father was where she was diagnosed and since that time all the doctors are debating that diagnosis and dragging their feet unwilling, not knowing how to deal with it.  The rest of the family pretty much avoids her, often with eye rolling and impatient tapping of our feet.  We have become the people who left my husband in tears as he struggled with trip after trip to the hospital in ambulance with a pain that was not silenced, not even with all the morphine, marijuana, and dozens of other drugs they gave him.   When we aren’t thinking about our own family, it is highly unlikely we are thinking about other families.  I had failed to keep the promise.

I did it because it was easier then dealing with the responses from people who were sick of hearing about it.

But my promise was never to any of them.  It was to my husband.  I am here and he is gone.  I have the privilege of walking out into the sunshine to get away from unhappy thoughts, or of turning the channel.  He does not.  He asked very little of me, not in life and not in death.  How did I get from where I was to here where I have allowed others discomfort to keep me from doing what I promised?

ALS occurs in one in 30,000 I think the young man said in the video.  AIP is about one in 50,000.  The Canadian Foundation, so desperately needed to provide resources for sufferers and to compile a list of medications that should be avoided, had to fold due to lack of funding.  I was on the board for a few years and those few women worked tirelessly on a shoestring.  There was no government funding.  We constantly had doctors say they would help and then do nothing.  But we did hear from the families who were suffering.  The stories were all the same.  Problems to get diagnosed, and then constant debating over the diagnosis from every doctor they came into contact with.  Inconsiderate, rude, unkind treatment at the ER.  Accusations of suicide attempts and drug seeking.  Family and friends who accused them of malingering.  Loneliness.  Desperation. And pain.  So much pain.    The point is the Doctors do not know anything about AIP.  They tell you that.   And the nurses, even less.  Most have not heard of it.  One doctor told us they spent all of 15 minutes on it in medical school and were told to forget it because they would never see it.

The Canadian Foundation had to close down, not because there was not a need, but because those people could not fund the foundation all by themselves and everyone else was busy giving to the annual cancer run/fund/dinner/etc.  Everyone knows someone who has/had cancer.  We know what it looks like and how to respond to it.  Very few people know someone with AIP.

What is Acute Intermittent Porphyria?  There are lots of complicated medical jargon explanations, but as simply as I can … the body naturally produces porphyrins as part of the heme in the blood.  The liver has several enzymes that aid in changing the porphyrins into heme but if one of those enzymes are missing this cannot happen and  you end up with porphyria.  There are several different types of porphyria; the others involve skin rashes and reactions to the sun.  When an enzyme is missing, the porphyrins do not get changed into heme and they build up in the body and attack the nervous system.  People often have very dark or purple coloured urine during an attack.

Symptoms in AIP can be variable. They include:

    1. Abdominal pain which is severe and poorly localized (most common, 95% of patients experience)
    2. Urinary symptoms (Dysuriaurinary retention/incontinence or dark urine)
    3. Peripheral neuropathy (patchy numbness and paresthesias)
    4. Proximal motor weakness (usually starting in upper extremities which can progress to include respiratory impairment and death)
    5. Autonomic nervous system involvement (circulating catecholamine levels are increased, may see tachycardia, hypertension, sweating, restlessness and tremor)
    6. Neuropsychiatric symptoms (anxiety, agitation, hallucination, hysteria, delirium, depression)
    7. Electrolyte abnormalities (Hyponatremia may be due to hypothalamic involvement leading to SIADH that may lead to seizures).

[6] Unlike other porphyrias, rash is not typically seen in AIP.    Source Wikipedia 

If it is known that someone in a family has AIP, and their enzyme value is low in red blood cells, other family members who have inherited a deficiency of PBGD can be identified by measuring the enzyme in their red blood cells. Latent cases so identified can avoid agents known to cause attacks. However, in some AIP families, PBGD is normal in red blood cells and is deficient only in the liver and other tissues. Falsely low values sometimes occur due to problems with collecting and transporting the sample.  Source American Porphyria Foundation 

I am suspecting that our family has some mutations of AIP for two reasons.  The first is the issue with the testing and the second is that normally AIP sufferers are somewhat like MS in that they have attacks and then periods of time in between where they are fine.  My husband had almost constant attacks for the entire 3 years before he passed away.  My daughter has a bit more of a reprieve between attacks and hers are not as severe as her father’s were.

Hospitalization is often necessary for acute attacks, particularly if nausea and vomiting have prevented adequate oral intake. Medications for pain, nausea and vomiting, IV hydration, and close observation are generally required.

Glucose and other carbohydrates can help suppress disease activity, are given by vein or by mouth, and are part of initial treatment. Intravenous heme, however, is both more specific and effective than glucose and should be started if the patient’s symptoms fail to improve within 36 hours. Heme is sold as Panhematin®, from Recordati Rare Diseases. Most hospitals do not stock it. Therefore the pharmacy must be notified at the time the patient’s admission to initiate a request for air-freighting enough medication for 5 days of treatment. Generally, shipping will take at least 24 hours.

Panhematin, is the only commercially available form of heme for treatment and prevention of acute porphyric attacks in the United States. Heme arginate, which is marketed in other countries as Normosang® (Orphan Europe), is another preparation for intravenous administration. The main side-effect of Panhematin® is irritation of the vein used for infusion (phlebitis). This is avoided by slow infusion through a large caliber vein or central line. Adding human albumin to the heme solution also may reduce the risk of phlebitis. (Directions for preparing Panhematin® in this manner can be obtained from porphyria specialist and is included in the Primary Care Physician/Emergency Room Kit.) Heme therapy is indicated only if an acute attack of porphyria is proven by a marked increase in urine PBG. It may be useful also as preventive therapy for people with frequent recurrent attacks.

During treatment of an attack, attention should be given to salt and water balance. Harmful drugs should be stopped. These include barbiturates, sulfonamides, and many others (see the Acute Porphyria Drug Database). Attacks are often precipitated by low intake of carbohydrates and calories in an attempt to lose weight. Thus dietary counseling is very important (see below). Premenstrual attacks often resolve quickly with the onset of menses; hormone manipulations may prevent such attacks.

AIP is particularly dangerous if the diagnosis has not been made and if harmful drugs are administered. The prognosis is usually good if the disease is recognized and if treatment and preventive measures are begun before severe nerve damage has occurred. Although symptoms usually resolve after an attack, some patients develop chronic pain. Nerve damage and associated muscle weakness can improve over a period of months or longer after a severe attack. Mental symptoms may occur during attacks, but are usually not chronic.

Wearing a Medic Alert bracelet is advisable for patients who have had attacks. People who are asymptomatic carriers of the genetic trait may choose not to wear a bracelet but should be prepared in any medical encounter to advise their care-givers of medications that are risky in AIP.  It should be remembered that AIP patients can develop other diseases, and symptoms will not always be due to porphyria.   Source American Porphyria Foundation 

If you know the disease exists in the family there is actually quite a bit you can do to make sure that the disease never develops.  Like many things we can discover in our DNA, the potential for the disease is not a fait accompli.  The biggest part of it is avoiding the many drugs that can cause it to start.  The PROBLEM is that it requires a vigilance that most people are not willing to put in.  It requires educating yourself and your doctors.  It will involve some fights when doctors want to argue about having to actually think about what they use for you instead of writing a script that they hand out to everyone else.  It requires some self discipline – avoiding drugs and alcohol, no fasting or dieting, etc etc.  These are things you have to put in place from the beginning and people would rather gamble with the outcome.  The fact that if it goes south they will spend the rest of their life asking why they made that gamble, is not a tangible reality.  How do you convince young people NOT to drink or do drugs because of a maybe?  So most people, like in our family, choose not to deal with it, or to pretend it never happened.  And I am not just talking about our kids, I am talking about HIS family and their children.

Getting genetically tested can seem to be the way to go, once you are cleared you can not worry about your kids having the illness BUT it is expensive and now we have so many reports about botched testing and even talk that there is much more to DNA than we once thought which might make some of those results suspect.  Any testing is a helpful clue and of course, everyone has to make their own decisions.  The purpose of this is not to judge or point fingers, it is only to inform and open discussion.  AND aside from the testing, so you find out you are cleared in your family … what about your siblings?  Your life is good to go and to heck with them?  If family is not going to support one another, what hope do we have of eliciting the compassion of strangers?

 

What can we do?

Please remember that there are lots of rare diseases that you have never heard about that have no big funding campaign and no government support.  That means there will be very little research being done to help the families suffering.

Don’t assume, just because you have not heard of a disease, or because the person is not wasting away or looking really sick, that they are not sick.  Don’t assume because they are able to work in the garden Tuesday after being in the hospital Saturday night and having the whole family called in because the doctor says it does not look good,  that the person wasn’t dying Saturday night.

Be supportive of people who have a chronic, unknown illness.  If you can’t offer help, at least do no harm.  I remember one of my husband’s family phoned him and told him to get off his ass and find a job – just a couple weeks before he died.  Trust me, the person already feels like crap, already beats themself up daily, they don’t need your help.

Consider giving some of your charitable donations to rare diseases.  You should at least be supporting the causes that your own family is too familiar with.  United Way and other organizations allow for you to direct your donations to specific charities.  Look around you at your friends and neighbours.  Ask questions.  People are often happy to talk about what is going on and educate people.  You might be amazed at the courage of the people you have interacted with for years, when you learn what they have been dealing with on a daily basis.

Love one another.  Be the arms that hold someone.  Speak the words that lift and support.  Offer a shoulder, a helping hand.  Give your time.  Give anything you can give.  It does not have to be money, there are hundreds of ways of reaching out and lifting someone’s burden.

 

We have so much power to change the world with just our attention, intentions and actions.  One day we might wake up to that power and actually harness it.  Until then, each of us has the ability to make a difference with your own compassion and your gifts.

I thank the ALS campaign for waking us up.  I am sorry it has taken an ice cold bath to shock me out of my complacency.  I am sorry that I took the easy path and journeyed far away from the painful memories to where I can pretend it never happened.  I am here.  My husband is not.  His life stood for something.  His pain was not something I can ever forget.  I commit to doing more.