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 this and if one of those enzymes are missing 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.

Pretty Sure Picasso Has to be Related to Me.

navajo me

After watching this video I started digging through all of our old blankets just in case someone slipped in a Navajo blanket when I wasn’t looking.

They hadn’t.

I do have some blankets with dubious stains on them from the kids growing up. My son would assure you that anything with his spit, pee, or vomit on it should be worth millions.

It isn’t.

I am not sure what drugs I was taking when I packed them up and moved them from Canada to Australia. Maybe it was part of letting go of things in stages. Or maybe I just recognized it would probably be the only thing I would see of my some of my kids from that day forward and hoped it could comfort me in my old age.

It doesn’t.

My hubby pretty much summed up the opinion of the uneducated millions when he came upon me dumpster diving into my own preciously packed “these are precious handle with care” packing boxes. He looked at me, he looked at all the mangled blankets and asked, “What is all this garbage?”

I banned him from the staging area. The fact he had to go to golf anyway does not take away from the authority I asserted when I banned him and the vehicle from the garage so that I could attend this very delicate hunt for antiques that would net me a fortune AND a viral You Tube video.

I found the blanket I knitted when I was expecting my first child. My sister in law and I decided it was the thing to do, seeing as we were both pregnant. We knitted as we ate and threw up and watched TV. You don’t really bond with someone until you have knitted and thrown up with them, let me tell you. We were obsessed with finishing our blankets in time for the blessed arrival. And no, we were not doing drugs at the time, we were just two really sheltered farm girls who were lucky we did not get our asses kicked the first time we used the girls washroom in the big city. I think I knitted sometimes when I was sleeping. We had no pattern, the plan was to knit the crap out of the wool we had and end up with a big square that would be a baby blanket and our child would forever treasure and keep in their trunk and cry over in their older years when they pulled it out and remembered their Disney like childhood and their wonderful giving mother.

That didn’t happen either.

Clearly . . . I am the one with the blanket. I think my daughter kicked it off her the first time I put it over her and gave me a look like, “are you freaking kidding me? What if someone sees me covered with that thing? I want the princess blanket!” Some kids are born with a gifted ability to have a whole conversation with you without ever moving their mouth or speaking words. Those conversations are almost always pretty brutal. I still have scars.

I pulled the blanket out of the tissue paper I had wrapped it in and held it up and wondered if my high school math teacher would be impressed that I finally mastered the whole quadrilateral thing. It was like someone had encouraged Picasso to knit, except the only paint in the knitting box was a kind of dulled pink ( the only pink wool the grocery store carried) and he clearly knitted it in his early years before he had developed any real . . . talent. I tried to contact my math teacher.

He still has me blocked.

I have lots of blankets and things I inherited from other relatives. Man they must have seen me coming. “Look here comes Aria, go get that old ugly throw Aunt Mabel knit, I want to make sure she takes it with her this time. Last time she was here and I gave it to her it ‘accidentally’ got kicked under the fridge.” I was the Goodwill store for the family, except without the nice warm fuzzy feelings people have when they donate, thinking they are doing something nice for someone else. I may have even got the stuff that a Goodwill store would not take. It was either that or I was cheaper than wasting gas hauling stuff to the garbage.

I thought life was a treasure back then and I liked to keep everything, neatly sorted, categorized, packed, cared for. I might have had librarian tendencies, genius savant type of tendencies, except that I was never able to pull my hair back tight enough and forget about that whispering crap. In fairness, maybe my family thought the puce and brown and green throw would one day be a masterpiece and they loved me so much they wanted me to have that possibility in my life.

OK that was just me trying to remember what they taught me in Sunday School. My family were never that kind.

I mean I am sure the Navajo did not know they were making priceless treasures when they were doing their blankets. They probably were just sitting around the campfire pregnant, eating, throwing up … and decided they should weave some blankets. If we were bored in the Seventies, in LA, imagine what they were sitting out in the desert without even TV?

Aunt Mabel’s throw is never going to be worth anything. It is so ugly that even I cannot keep making excuses for it. I might keep it to will to my children one day because … well …a tradition is a tradition and perhaps suffering is best served in a big bowl where everyone has to take a little bit on their plate and eat it without complaining, because we are Canadians after all. We are the poster kids for Polite.

I am keeping my mathematical wonder, dull pink, really ugly, unwanted baby blanket. I like to pretend that someday there will be a video about a family who were desperate for help who found it and when they got to the dump and were about to chuck it out, the dump guy, who is big into antiques and who has real integrity, stops them and tells them it might be worth something. And then they go and take it in to someone who says, “this had to be knitted back in the 70’s with a basic wool that was available in grocery stores, probably by a mom for her baby. Look at how beautifully preserved it is, like it was never even used.” And then they research and people ‘ooh’ and ‘ahh’ over how great the knitting is and they wonder about the originality of the shape I created and speculate I was an artist . Then they hunt for more of my signature pieces and find my other afghans where I start out with a pattern, get bored, and then just make it all up. OR, the doilies that are always lopsided and have made up stitches in them . . . all of them looking like they were never used. And lots of people are suddenly rich because they have an ‘Aria Appleford Original.’ And then, my daughter feels really really bad that she did not appreciate me or her blanket and that weeping sound is the sound of my other children wishing they had been nicer to me.

That will go a long way to heal this deep pain in my heart, once I am dead I mean. It’s not a perfect ending, not like the one where I find a Navajo blanket worth millions, I sell it, get rich, and am able to buy new kids blankets, BUT it will do.

QUOTE

“When did we become so small and so apologetic? Why do we apologize for our humanity? Love what you love, and make no apologies. This is your identity. The most horrendous suspensions of freedom are self-imposed. We imprison ourselves daily, hourly.We have one life, one shot at all the glorious things of life, and we walk about constricted, apologetic, afraid. We have so little time; we have so little space upon which to spread our love and our talents and our kindness. Run toward life fulsomely and freely.

It runs from us so quickly, like a frightened dog or youth or daylight. Chase it and care for it.

Of course art should be about something big. Something terribly big must be at stake. I don’t see this anymore. Our art is becoming terribly polite and apologetic, much like us. It slinks away like a sagging breast, empty of milk or promise or comfort.

We need to get very fervent again. We need to get jacked up.”    Tennessee Williams

Who is Raising Our Children?

parental abuse

Our children have no way of really understanding how their parents sacrificed for them. Especially not in a war being fought in family courts with lies and parents and step parents bidding for children with money and privilege as if they are real evidence of love.

They aren’t.

A wide screen TV, no matter how cool the model, has never made up for the lack of real connection in people’s lives. It is, at best, a temporary distraction. They can never give a person purpose or grounding or ever feed their soul through the long dark night.

There are all kind of real parents out there making sacrifices for their children that their children will never know about or understand. It takes the courage of the real mother in the story of the Bible’s, King Solomon where he had to determine parentage. When he finally said he could not know who the real mother was and that the only solution was to split the child in two, one woman begged him not to do that and said the other woman could have the baby, she would withdraw her petition. King Solomon immediately knew SHE was the real mother. Today moms and dads are doing the same thing all over the world. They withdraw themselves as contenders for the sake of children who are being used as emotional footballs by the other parent. They say, ” no more court,” they accept the child’s hateful words without defence, words fueled by lies and by the insecurity and hate of the other parent. Those victim parents hurt terribly, but they do it in quiet, bleeding from wounds that will never heal. They choose to put the child before their own pain.

No-one is saying all alienated parents are perfect. No-one should be saying custodial parents are perfect. There are no perfect parents. We are all just people who are learning as we go, trying to do our best.

Our children are stressed. Adopting the language of the parent who holds them captive is often a survival skill. They just want to avoid any further drama and pain When seeing the other parent, having any relationship with the other parent, causes the imprisoning parent to fly into a rage that the child bears the full brunt of . . . . it is easier just to say,”Yes, I hate mom/dad. I never want to see them again.”

That way the child gets a new iPhone and everyone goes for ice-cream.

A child learns to give up their feelings for the other parent, but what the imprisoning parent does not see, is that with every chunk of those feelings that are torn from the child, they are tearing parts of the child as well. Issues of being loved, of being wanted by our parents, are the things nightmares are made of. It is essential to our wellbeing to make those connections, even though none of us have perfect parents, even though we all have parents who are going to make mistakes in their lives, who have maybe made BIG mistakes … we still need them.

Smart parents know how to turn mistakes into teaching moments. Those moments carry a lesson and an example of how the child should deal with their mistakes. Only the cruelest of people will take a person’s mistakes and use it to destroy them, especially when their own mistakes are not that much different from the ones they are pointing at.

And that is not to even address the grandparents and aunts and uncles and cousins who have to mourn the loss of the child in their lives, long before the child is even dead. The reality is most of these kids will grow up, and short of some miracle, they may never understand that what they were told was a pack of lies and they were just a pawn in a game of hate for the imprisoning parent. An adult can meet his/her family and have an adult relationship but they have missed out on all the nuances of family that add to our foundation, our sense of who we are. Families share a journey through their formative years. That journey, and the people and experiences we are exposed to form each of our bases for building ourselves and understanding the world around us. You can’t go back and try to add those in later in life. We are stained by our constant thoughts and those are built on our beliefs and life lessons and reinforced with repeated actions. We act in a way that supports our beliefs. Our parents, our family give us those building blocks.

And I wonder about a whole generation of kids raised without their one parent. Those kids being told that their parent is all kinds of horrible and then learning that we deal with life by lying and avoiding tough situations. Kids that have been taught they get gifts and prizes for agreeing with those in authority over them. Kids that think money and things are what matters in this world or that parenting is about being able to give your child “things.”

We all seem so comfortable in the middle of our disasters. There are those who are part of the problem and those trying to make a change and then there is the vast majority of people who are busy shopping, thinking it does not impact them, or that those trying to stop it are overstating it, or just that they need to get to the mall before it closes. Everyone carries on until it reaches a crisis point and then people demand to know what is being done and why didn’t someone do something earlier. None of this finger pointing fixes anything of course. Eventually laws and programmes will have to be enforced to address it, meaning all those who really are not part of this have to live as if it is. We will not help ourselves. We want laws that fix problems. We want programmes to be put in place. As long as we do not have to accept responsibility.

And that is the whole reason we are where we are, with this issue and all others that face us. The answer is not out there. It is not our politicians, or our laws, or even our churches and faith that are going to save us. We have to insist that each of us take total responsibility for ourselves and teach our children to govern themselves with discernment, integrity, and absolute honesty. You cannot compel a man to care, with laws. He must care from within himself. He must care so that he can govern his own actions. You cannot teach people to love by teaching them to hate. Hating others only teaches hate and teaching a child to hate is setting him up for a miserable life.

Those parents who are trying to do the right thing, choosing their children’s hearts even when they sacrifice their own, have no place in our family courts. We continue to hand kids over to parents who are abusing them with Parental Alienation Syndrome. We continue to have coffee with workmates who tell us how they ‘ stuck it to their previous partner and kept him/her from seeing their child,’ and we sit there and congratulate them or say nothing as if this is great. We have family dinners where generations of the imprisoning parent trash the non-custodial parent and feeds into the plotting of how to keep the child from them. If someone were telling us how they were beating their child, would our reaction be the same? Would we laugh? Would we encourage the imprisoning parent to keep doing what they are doing? Of course not.

Parental Alienation Syndrome is Child Abuse.

There will be real and lasting consequences for all of us.

And it is a choice to see what is happening around you, to care enough to do something, or to head over to the mall before it closes.

QUOTE

“Parents rarely let go of their children, so children let go of them.

They move on. They move away.

The moments that used to define them are covered by

moments of their own accomplishments.

It is not until much later, that

children understand;

their stories and all their accomplishments, sit atop the stories

of their mothers and fathers, stones upon stones,

beneath the water of their lives.”

 Paulo Coelho