Purple Hat Day!

If any of you watched the Kentucky Derby last weekend, you saw some of the traditional Derby Hats on the women attending the event. My, weren’t they grand? And such attention-getters!

And for the more international of you, what about the hats that you see at some of your more genteel sporting events? The special races at the Ascot and other tracks, as well as Wimbledon draw people with a unique fashion sense expressed through equally unique headwear.

Now ask yourself why should only the horse breeders, high-stakes bettors, and various levels of royalty have all the fun? Why not attract a little of that attention away from the sideline Johnnies and focus it on the very worthy cause of Porphyria Awareness?

I declare Friday, June 14 officially Purple Hat Day! That is five weeks from today, which should give everyone enough pain-free and attack-free time to put together a chapeau worthy of Churchill Downs! If you have porphyria, care for a person with porphyria, or just want to support a friend with porphyria, this is your opportunity to promote Porphyria Awareness in a wacky, creative, fun, and entertaining way. If your dog supports Porphyria, then by all means, your dog should have a hat, too!

Ideas:

Don’t think you have to run to the craft store or department store to buy a bare hat to start your hat. I’m sure you have all sorts of things around the house that you can use.

An old baseball cap or trucker’s cap is a great place to start.

A large cardboard circle with a hole in the middle large enough for it to set on your head is easy to cover and decorate. They make a great platform for the big Victorian monstrosities.

Use the same cardboard circle, but move the opening to one side, or to the back for dramatic “Modern Art” looks.

Cut pieces from felt and stitch them together into a little top hat that you can attach to a headband, and you have the beginning of a stylish Fascinator!

Have an old gardening hat hanging in the garage? Put it to use.

See an old straw fedora at a garage sale? Could look great!

Have a friend who has a few old bridesmaids hats laying around that are just in the way? Now you have hats for the whole family!

Still at a complete loss? Rummage through the scrap bag and find enough stretchy t-shirt fabric to make a 1930’s Hollywood Vamp Turban. Va-Va-Voom!

If you’re truly creative and gifted and have a little time on your hands, crochet one!

Now just Go Wild! Glue, stitch or staple feathers, beads, sparkly old necklaces or earrings, lace bits, ribbons and bows from old gifts, netting, scraps from your last sewing project, old silk flowers, anything you have to your basic hat to make it beautiful, wacky, provocative, sweet, silly, goth, artsy or homespun. Make it something to attract attention and raise awareness and maybe even make someone’s day.

Then what?

On June 14th, get a photo of yourself or your support team in your hats, that’s what!

If you’re the shy sort who can’t imagine wearing a huge purple headpiece out in public, take your photos in the privacy of your own home or back yard. No one will have any problem with that.

If you’re more the devil may care, exhibitionist sort, then by all means go forth and get your photos out in the park, in the grocery store, riding your bicycle, waiting at the bus stop, having dinner at a restaurant, watching the kids play softball, on the back of hubby’s (or wifey’s) motorcycle, at the farmer’s market, walking the dog (I hear Pugs love to wear hats and be photographed for a good cause, as do Beagles, just so you know). The sky’s the limit.

We will set up a way to send your photos to a single email account so that we can put them into a photo montage video for the Porphyria group’s YouTube channel. The more photos we get, the better the video will be and the stronger the impact it can have for the Porphyria Community.

June 14! Five Weeks From Today! Budget your strength and see what you can create in five weeks! I, for one, am really looking forward to seeing all of your hats. I’m going to do my best to have a good one, and I have already committed to wearing it to work that day.

International Rare Disease Day 2013!

International Rare Disease Day will take place this year on February 28.  Individuals, unaffiliated groups, small foundations and large, well funded organizations will all be participating to one degree or another to raise awareness of not just the rare disease, but hopefully of the men, women and children who fight them every day of their lives.

We have one month to prepare for this event this year.  There have been many, many advances in 2012 and these first few weeks of 2013, and Rare Disease Day is an excellent opportunity to reach out to local media with your stories, your struggles, your victories, and your hopes with the new AIP therapy rapidly approaching clinical trials in Europe.

• Don’t be afraid to approach the television or radio stations in your city or town.  Many of their on-the-air personalities and anchor persons are active on Twitter or Facebook and actively solicit news stories.  Find out who the station health reporter is, as well the science reporter, human interest reporter, and assignment editor for each local news outlet. 
• Approach reporters on Twitter, Facebook, and through the email usually provided at their station websites.  Be persistant, but be oh, so very nice about it. 
• Offer to sit down and talk about being an adult with a rare or orphan disease and the effect it has, not only on you but also on those around you.

There are many ways in which you can participate and raise awareness, not all of them as difficult or stressful as reaching out to the media could be for most people. 

• Be a strong presense on online at Facebook, Twitter, Instagram, Google+, Yahoo groups, and any other online community you frequent. 
• Post a small video on the We Are Porphyria channel at YouTube. 
• Link the existing video on our channel in as many online communities as you can. 
• Use your artistic and crafting skills to make Rare Disease/Porphyria bracelets, anklettes, or necklaces.  Old blue jeans make marvelous bases or blanks for embelishment with buttons, scraps of ribbon or lace, bits of unused quilting materials, paints and dyes (add bits of purple to specify Porphyria). 
• Can you crochet?  Knit?  Does your neuropathy let you do that a little these days?  It doesn’t take many stitches to make a bracelet.  Put together a handful of them in an afternoon so you can embelish them over the next several days.

You don’t need to spend a great deal of money to make an impact on Rare Disease Awareness.  Small projects that you can pick up and put down as needed to accomodate your physical capabilities are perfect.  Put yourself out there, even just a little bit, and make a difference for yourself and for every other Porphyrian.

The more things change, the more they remain the same.

Do neurologist offices make it a point to hire front desk personnel who just are naturally intolerant to elderly or moderately incapacitated people, or do they budget for that as manditory training?

Do physician offices have contests and give incentives for the largest number of times they can get a person to ask the same question without ever giving an answer to the actual question?

Is it part of med school to teach all of the little, fresh faced, innocent pre-doctors how to make the most of a confusing case by throwing every known unrelated test in the world at it instead of actually using a bit of critical thought and basic judgement?

Are obscure tests taught in med school, not as examples of how far we have come, but as things they should still try first just because new tests could diagnose faster and with fewer total office visits?

Does “pain management” typically end up meaning “managing to get your pain patients to go elsewhere” even when you know the pain is real and the cause genuine?

Does it feel like every time you go to the doctor, your encounters with the actual doctor become fewer and shorter?

Are you happier when the only person you interact with during an office visit is the nurse who actually did read the information you sent on your illness and actually was intrigued enough to do more googling on her own?  And don’t you hate it when she leaves for greener pastures and you have to start all over with another nurse or PA?

Have you ever felt like taking a package of hearing aid batteries to an office visit to hand your doctor when she acts as though she didn’t hear what you asked or answered?

Do doctors sometime re-order tests, not because the results were inconclusive, but because they verified a diagnosis they didn’t want to accept because it meant more work than a different result might?

Seriously.  Ever wondered?

 

 

Deeply Disappointed.

I am taking a brief respite from the Porphland Survival List posts to engage in a bit of catharsis therapy.  indulge me a moment while I take off the tender and diplomatic kid gloves and replace them with the heavy, black,  vicious, verbal sap gloves.

There.  Ready.  I feel much better about this now.  I have dainty, tender hands and I don’t really feel like carrying around deep purple bruises for two weeks.  And these tend to be very good at keeping the proverbial gore out from under one’s jewelry and nails, a good point to know when one’s ultrasonic cleaner is on the fritz and someone has absconded with one’s nail brush for dealing with canine oral hygiene.  I do regret not being able to find the violet ones.  I love and miss those beautiful sap gloves.  They always went well with my glasses.

I want to say to each of you that at this moment I am incredibly angry with the way the boy has been treated by what once was his primary care physician.  After 3+ years of depending on her and her staff to provide adequate medical care for the boy, building what I perceived as a good working relationship with her through her staff, and doing my best to be proactive at home with nutritional and supportive care to minimize our use of her office time or hospital stays, we were delivered this coup de grace during his last quarterly appointment:

She does not believe he has porphyria of any sort and will not in the future provide accepted and necessary therapies or treatments based on a diagnosis of porphyria.

Those are not her exact words, but after having heard them as through read from a script on two separate occasions, that fateful day in an exam room and a subsequent late evening telephone call, you can be relatively sure that they are incredibly close to, and do sum up both the gist and the tone of her actual statement.

Interestingly enough, she offered no explanation of his symptoms, no alternate or theoretical diagnosis, and no indication of what she, as his Primary Care Physician, intended to do to find an easier to toss asside a more appropriate diagnosis.  What she did make perfectly clear was that until one of us magically shat one out and hung it in the Louvre in a rhinestone frame, there would be no hope of getting him any sort of maintenance therapy.

No D-10 infusion will be available to him as an outpatient, either in the hospital patient services area or in the big new freestanding infusion center in the neighboring upscale bedroom community.  No prayer of a chance of ever getting any sort of heme therapy, even if we as a household could afford it.

Under rather pointed and repeated questioning about her intentions toward making a diagnosis and what we needed to do to facilitate arriving at that diagnosis, she made very clear her belief regarding his illness:  it is either literally or figuratively all in his head.

(And a great deal of it is in his head, at least if you count up all of the damage he has sustained for lack of treatment.)

 

• It is a neurological issue or disorder that is somehow causing him to urinate the color of raspberry iced tea,
• It is a neurological issue that makes him consistently intolerant to medications and chemicals known to be porphyrogenic,
• It is a neurological issue or disorder that makes him intensely sensitive to relatively normal fluctuations in both his blood sugar and electrolyte levels.

It fascinates me no end how a person will dismiss the obvious in favor of the unknown when there is some incentive or motivation to do so.  What I would like to know is exactly what that motivation is.

There is the probability that the insurance company has brought pressure to bear to ignore the differential diagnosis and demand either a.)  the 1984 test results be presented to them by the hospital where the tests were conducted, by the researcher who conducted the tests, by the insurance company which used legalities to seize the records from the hospital, or by the boy who has tried repeatedly to get copies of the results only to be told they simply no longer exist, or b.)  we somehow manage to pick the absolute optimal time and circumstance for getting another round of testing completed and get new, improved, and conclusive results which one already knows will be disputed immediately however strong the results are.

There is also the possibility that the physician is streamlining her practice and purging the patients with more obscure, more difficult, more time-consuming, or require more effort to manage in order to concentrate on patients which can be scheduled routinely for maintenance more like the service center at a large automobile dealership.  It is, after all, much easier to hire staff qualified to provide regular oil changes than it is to have a staff prepared to do a valve job.

I do not know the ultimate reason for the indifference or for the complete disregard for a patient’s basic rights as a person.  The boy has the right to a reasonable expectation that his illness, disease, disorder, whatever it is called today, will be taken seriously and treated seriously and not second guessed when every sign, every family history, every symptom, every quirky reaction to triggers and stimuli both physical and psychological, every mason jar of light reactive urine sitting on the porch ledge point directly and conclusively to only one preliminary diagnosis:  An Acute Porphyria.

We know the family history points to AIP.  His sibling has a diagnosis of AIP.  His father has a differential diagnosis of AIP.

And what on earth is the reason for continuing to utilize a test which has been proven to be so horribly inaccurate that it is now not used as a diagnostic locations outside of the United States?

How many wonderful, talented, intelligent and loved men and women are losing their lives at this very moment, one nerve cell, one breath, one attack or crisis at a time  because of cross-purposes, apathy, arrogance, lack of education, intentional ignorance, ineptitude, poor planning, lack of time management, poor use of resources, lack of staff training, and the unavailability of practices, laboratories, and facilities with any expertise in diagnosing at the state level, not some arbitrary regional division?

Why does NORD and the NIH and the FDA not establish and enforce a requirement that every state-run or state assisted university with a medical school and a research laboratory must designate that laboratory as the state testing site responsible for the testing of all specimens submitted for the diagnosis of a rare disease or disorder by physicians and/or private patients residing within that state?  By placing all rare disease/disorder testing in central state university operated labs and diagnostic centers, you not only help people who cannot get to regional sites, you also educate more than three or four new people every year in diagnostics and give them practical experience.

Why don’t they require facilities such as these so that people already in physical, emotional and fiscal distress are not bound by the current system of rare disease and disorder testing and expertise being sequestered and contained in small core groups at a few  regional centers?

Why aren’t physicians who withhold treatment from patients with only a differential diagnosis held accountable when these patients finally do get a diagnosis that is “good enough”?  Shouldn’t they bear some of the responsibility for the losses these patients suffer while waiting with no hope of even a bag of saline?

I can’t write any more.  The more I do, the angrier I get, and I, at least, feel an obligation to the boy, which is more than I can say for his hematologist.

Vacations can be vacations again.

I do realize that today, August 20, 2011, is outside of the generally accepted vacation season for most people these days.  School starts their fall semesters ever so much earlier each year.  Employers don’t want to ‘give’ any more hard-earned vacation time than they absolutely have to and try to dole it out a few days at a time.  Spouses and other significants find it more and more difficult to coordinate schedules (see above).  Add to that the late summer heat that makes it almost unbearable for mere mortals to travel, especially for those who have any sort of health issue.

But it is also the start of the “I must get [tested][diagnosed][treated][enrolled in this study] season.  With little Bucky and Kitty going off to third grade, so too are their parents trudging back to grad school, and when you have people in grad school working on research, you have Research Studies and Clinical Trials.

Oh, joy of joys!  Soon thousands of sleep deprived, white-coated supplicants at the sacred altars of Knowledge, Enlightenment, and Fifty Cent Beer Night will be scurrying about, clipboards in hand, ID badges at the ready, eager to interrogate, poke, pinch, probe, and remove the bodily fluids of willing victims Believers.  And you, too, could be among the called, the chosen, the sacrificed.  You could be a Participant!

Or you could decide after a dozen failed attempts at a diagnosis not to trust your local lab any longer with something that important, make arrangements to drive a thousand miles or more to go to a more reputable lab, and take your specimens in their “sealed, original packaging” to be deposited appropriately after you arrive.

Whatever the reason for your travel, you want to make the best of it, not only for you but also for your traveling companions.  More importantly, you want to make certain that you arrive at your destination alive and not in need of immediate hospitalization.  To help accomplish this, here are some suggestions based on my household’s experience on a mid-July 5,000 mile road trip across the northern plains, high desert, several rivers and two mountain ranges during one of the hottest Julys in 100 years.

1.  Make sure that your pain meds are easily gotten at all times.  It does not help you to have them tucked safely and securely away in your locked suit case inside a locked trunk traveling at 75 miles per hour across the South Dakota landscape at any time of the day or night.  If you are that afraid of losing your meds, carry a second smaller set in your handbag or backpack or manbag and lock the bulk of them away in your luggage.  Just make sure if you do that you refresh your mini-stash before starting the next day.

2.  Make sure that you have some sort of tranquilizer or stress relief medication with you.  We all know, often from painful personal experience, that stress can be a very powerful trigger for porphyria.  It can be a worse trigger than some medications because unlike meds that require your conscious participation to build up in your system, stress can often times be out of your control.  You cannot control a vicious thunderstorm that strikes with a vengeance at 3 am on a dark highway in Minnesota.  You cannot control the stress it causes either.

3.  Make sure you have ziplock and wet cloth cold packs in a cooler of ice so that you can get to them and swap them out as needed.   I make these to keep around the house, to take in a cooler or insulated bag, even to take out into the yard when we are doing yard work or spending outdoor time with the dogs.  They are compact enough to keep in a small cooler or soft sided bag with those blue plastic frozen ice replacements.

Use an oversized washcloth or microfiber cleaning cloth or even a cotton bar towel or hand towel.  Fold it so that when you slip it into a 1 gallon size ZipLock freezer bag, it lays flat and fills no more than the bottom half of the bag.  Try to keep it as flat and bump-free as possible so it is more comfortable to you when you use it.  Pour about a cup of water into the bag.  press all of the air you possibly can out of the bag and seal it tightly.  Lay it flat to freeze it.  Stack several of them in your cooler under a layer of ice.  Keep a few close by in the passenger compartment of your vehicle.

4.  Keep a variety of juices and Gatorade on ice. Restock it as soon as it starts to thin out.  It is so important not only to keep yourself hydrated, but to keep yourself properly hydrated with fluids that satisfy your body’s need for electrolytes and carbohydrates.  Juices and Gatorade are, for the most part, the only safe and consistent fluid carbohydrate and/or electrolyte replacements easily and inexpensively available on the road, even in the most obscure gas stations along the most desolate stretches of interstate.

They are safe for washing down your medications.  They can be kept at room temperature until you drop them into the cooler.  They travel well, even over the Continental Divide.  The best part is that you can pretty easily replenish your “back stock” by finding a grocery store and picking up an 8-pack or two (often for little more than $5 per).  Some of the big, superstore type of truck stops even have them in multiples.

5.  Keep glucose tabs with you. You might not think they have much value, but they can get you through until you can get to an ER.  Put a bottle in the glove box, in your suitcase, in your carry-bag, and have one or more of your traveling companions carry a spare.  The backpack of our household Service dog holds one.

And for goodness sake, do not be stingy with them.  One tab is not going to do anything.  In a hard-hitting, fast onset attack, you’re going to need to take them 4-6 at a time, preferably with one of the cold Gatorades you have on ice in the cooler with your coolpacks.  Glucose, Gatorade, and cool packs across the back of the neck, over the shoulders, and on the center of the chest.

6.  Keep all of the medical records you have with you.  If you have access to the disc from your last MRI or CT study, take that as well.  Put these things together in a ring binder or what we old folks called an Expanding Folio or Wallet.

Make sure you have a page with all of your doctors with their office number, answering service number, fax number, address and specialty.  Also make sure you have the same information for the hospital where you receive any treatments or have been hospitalized or tested.  It is also a very good idea to have a page of links to any porphyria-centric sites that you feel necessary for finding the information necessary for treating you properly.

And don’t count on a flash drive to be nearly as effective as hard copy files.  Most hospital IS departments won’t even allow personal data storage devices to be plugged into the hospital network, so all of the information you carry that way is completely inaccessible and worthless.  They just aren’t as effective as a dramatic device or prop, either.  You cannot whip a stack of flash drive out with a flourish, start turning pages on the blanket of your gurney, point out from the depths of your medical records exactly what you have and how to treat it, and provide them with the phone number of a nationally known specialist (if you are lucky enough to have one) who will vouch for the fact that you cannot have barbiturates to treat your “imaginary pain.”

*sarcasm off*

You only get that with Paper.

7.  Pack a carry bag.  Michael has a WWII ammo bag, a 1920 Irish Army backpack, and a leather backpack that he can keep his meds, records, and a beverage in whenever we leave the truck or hotel.  Depending on how he feels, how much he plans to carry, or how far we will be away from home, he will pick one bag or another.  Any one of these bags will carry his records, his meds, a spare small bottle of Gatorade, a bottle of glucose tabs,

8.  When you plan your route, don’t just look for hotels. Make sure you know where the major hospitals are as well.  This is pretty self-explanatory.  Most mapping websites will show the hospitals along a route with a little tweeking in the settings.  If nothing else, you can search separately and make a list to print out.

Another thing to actively search out when planning your route are easy to get to malls, pet food big box stores that welcome leashed pets (especially if they have an in-house vet), and chain grocery stores.

9.  Don’t forget your cell charger. If you can get a car charger, get one.  A car charger is much more difficult to lose than one you plug into a hotel wall outlet.  Once you have plugged it into the lighter port in your dash board, you really have no reason to unplug it and remove it from the vehicle.   The last thing you need is to be 20 miles from civilization with no charge on your phone.

10.  Keep a box of carb rich foods.  It is oh so important to ensure that you can keep your blood sugar levels at a consistent an healthy level.  Glucose tabs, as important as they are for a swift kickstart when you are dancing on the line between attack and non-attack, are not meant for healthy maintenance.  That you have to do with diet.

Fruit cups, whole grain crackers, cookies, peanut butter, even dry cereals that are easy to eat with one’s fingers. Frosted mini wheats are good for an easy food, as well cracklin oat bran.  The pieces are large enough to pick up and eat like popcorn, but they are also small enough to be easy to eat with liquids such as milk or juice.

11.  Be the passenger. Don’t add the stress of driving to your trip if you have a competent driver with you who is willing to do the driving.

Most people wouldn’t dream of offering to do a bit of relief driving for the pilot on a cross country flight.  And most people wouldn’t give a serious thought to taking over the bridge of a cruise ship.  Do the same thing while riding in the car.  If the person driving doesn’t ask for help, don’t feel all sad and upset that they don’t.  Let them enjoy the drive.  Enjoy the ride.

12.  Try to keep as closely to your normal eating/sleeping routines as you possibly can. If you do like we did and shift it 12 hours, shift the routine as well.

13.  Avoid fast food. Picking up a few easily eaten things in a grocery close to the road is so much better for your system than the convenience of a BigMac or Taco Bell.  You can easily pick up the makings of a salad, some pre-cleaned and cut fresh fruit, even a bit of steamed fish fresh from the fish counter sometimes.  Toss in a small baguette from the deli, a drink, and perhaps a sweet roll or slice of pound cake from the bakery, and you have a lovely meal.  And for goodness sakes, don’t skip meals.

it doesn’t take that long to assemble a good healthy meal from even a small grocery if you know what you’re looking for.  It certainly is quicker to put together than going through the ritual of waiting to be seated, ordering, then waiting to be served that you go through at most sit-down places.

14.  If you have a gps, make sure it has as current info as it can. If not, mapquest like a mad fool before you go and take contingencies into account. If you can’t do that, get a good atlas and learn how to read it.

15.  Don’t do like we did and do 700 miles a day. That can be too much driving for some drivers, and entirely too much time in a car for the passengers and any pets or service animals, when you are not feeling well.

If you must do your trip as quickly as possible and have no choice but to do very long days, balance that car time with some walk time.  Try to do at least 15 minutes of time outside the vehicle every two hours.  More time is even better if you can.  Balance is good. Spread it out and relax.

#8: Get a Kick-ass Partner

As a member of a few Porphyria groups on Facebook, I have encountered a diverse group of people, some of them very much individuals, others not so much.  Each of them, with the exception of the non-affected caregivers, friends and family members, deals with one or another of the porphyrias each and every day.  Some do it very well.   Others, not so well.

The fiercely individual and independent people in these groups eagerly share quite a bit of information, take responsibility for their own day-to-day lives, read everything they can get their keyboards wrapped around, and maintain a strong sense of and pride in their individuality.

But one trait you will notice in most of them is the recognition and acceptance of the fact that no one can go through porphyria alone.  Each of them has developed a strong partnership with another.  They have found a Kick-Ass Partner or a group of Kick-Ass Partners for their journey through Porphland so that someone always has someone elses’ back.

Having a partner is your best way to having someone close to you who really does have a pretty good understanding of not just the mechanics of the disease, but also of its effects on you as a person, as a living, breathing, feeling, thinking human being. 

A partner in porphyria knows about the frustration of finding a qualified doctor, much less getting that doctor to truly understand your symptoms.  He or she knows what it’s like to wake up in the night three times because you didn’t eat enough the day before and your body is demanding carbs before it lets you sleep.  He or she knows first hand those moments when the big buggy masses start buzzing around your field of vision, the ringing in your ears turns into a monsoon on a steel roof, and your gut begins to feel like an extra in the movie Alien.

He or she knows how hard it is to get coworkers to understand that just because you look perfectly fine, you are struggling to keep your arms and legs from flailing about as though you were a pinned bug.

Your Kick-Ass Partner could be in the same bed, or on the same block, in the same city, or state, or could even be thousands of miles away and only visible as a line of type and a small icon on your monitor.  He or she stands up for you when people act like fools, and stands up to you when you return the favor and play the fool.

A Kick-Ass Partner has always got your Six. 

One thing you may notice about people who have a strong, independent nature and a partner who reflects that is that they tend to be pack people, not herd people.  They may have pretty strong opinions that are not shared and they will discuss them candidly, but it doesn’t drop to the level of nonsense. 

They work for the betterment of themselves and of the pack, but not if it means blind alleigence or group-think.  You won’t find sycophants in Kick-Ass Partners.

You certainly won’t find sheep.

Sheep are tasty.  They certainly don’t make good Kick-Ass Partners.

Sugar, from the kitchen to the spa.

In my reading today, I came across the complaint that common depilitories are just not acceptable or safe for individuals with Porphyria.  And it makes perfect sense that they are not when you look at the labels on some of these products. 

Whether it is a caustic chemical that you slather on/rinse off, or a pricey, high-end boutique wax with a bone china lined, sterling silver custom heater for maintaining just the right temperature to get just the right shade of redness post-wax, these things are just jam packed with nasty, harmful ingredients that can make you miserable for days, weeks, even the rest of your life depending on the level of damage any attack leaves behind.

Is beauty worth braincells?  Are those smooth, hairless bits enough to make you endure all of that trauma?

Personally I find it a poor trade-off.  But what’s a person to do?

Try sugaring.  It’s ancient, tried and true, and has nothing in it that you, personally, didn’t put there.  You control the ingredients.  You control the outcome.

And it’s sooo hard to make and requires just a ton of equipment…not!

1.  Start with a heavy stainless steel or glass saucepan.  I have one of those glass “Visions” pans that works beautifully.  It has good, heavy sides and a heavy bottom so that the heat is distributed very evenly and consistently.  That’s important for this.  It also has a spout for pouring which makes it really convenient and much safer when handling molten sugar.

2.  Pour in your ingredients: 

• 2 Cups Sugar
• 1/4 Cup Water
• 1/4 Cup Fresh Lemon Juice

Seriously, that’s all of the ingredients.  Sure, you can make changes to that if you really want to.  You can use a little echinacea infused into the water if you want, and I’ve seen it infused with chamomille, lavender and tea tree oil in the commercially produced sugaring kits.  But for our purposes, keep the ingredients as simple, pure, and non-chemical as we can at this point.

3.  Stir over medium heat and make sure it is fully disolved.  Once it is disolved, it will slowly start to bubble. 

4.  Turn down the heat to a low setting and let is simmer very slowly until it reaches what candy makers call a Soft Ball stage.  By the time it reaches this stage, it should  be a lovely deep amber color, darker than honey bur lighter than dark maple syrup. 

For those of you who have handy dandy candy thermometers, that is 235 to 245 degrees Farenheit.  You metric folks are on your own on that conversion.  Way too many years since high school chemistry.

5.  Pour it or ladle it into very clean, very dry glass jars.  If you do it while it is very hot, it will pour  better than it would at a cooler, thicker, more stubborn temperature.    Make sure the rims on the jars are very clean.  No drips if at all possible. 

Drop on the lids, put on the rings (don’t turn them down hard, though), and let them cool a bit.  Once they are cool enough to actually grasp without glass-blowing gauntlets, tighten down the rings.

I use fabric strips for sugaring that are heavier than muslin but not quite as stiff and hard as duck/canvas.  Cotton is better than blended, and anything not natural is a bad choice for making strips.  Make at least 2 dozen (preferably 3 dozen) that are about the size of a dollar bill or a little bigger.  Don’t make them too big or you end up causing yourself more discomfort in the long run with too many do-overs. 

You should also make about a dozen or so that are about 1 inch by 4 inches for more, shall we say, precise hair removal.  Some things are just too tender and delicate for large scale deforestation.

The strips need to be rinsed well after use and hung to dry.  You really don’t want to put them through a washing machine or a dryer.  Just soak them until the sugar disolves, rinse off the hair, wring and hang to dry.  Keep them clean until you use them again.  They will last a long time if you take care of them.

WARNING!  Do not use on your pets.  Cat and Dog waxing are illegal in most areas and can result in steep fines.  Refrain from any pet waxing unless your pets are human.

#17: Pack your glucose

One of the things that researchers have found about porphyria attacks is that glucose levels in the body both influence and are influenced by porphyria.  Have an attack, your levels drop.  Let your levels drop, it causes an attack.  It’s like a vicious loop.  A really bad record with a scratch.  Even a bit like early Phillip Glass where he manipulated the spoken word.  Over and over.  And over.  You know when it starts you’re not going to like it, and the longer you let it go, the worse it gets.

This is one of the things that you do have a small amount of control over in your day to day life.  If yours is like my household, you keep cookies, various breads, ice cream, fruit, juices, pudding cups, etc around so that there is always something easily found to eat and potentially stave off an attack before it reaches disasterous proportions.

But what do you have when you leave the house?  What do you have out in the yard, or in the car, or on the bus?  Where can you reach quickly and find the sweet relief of a sugary bit on a dark and deserted highway between far flung exits?

Why, your backpack or purse, of course.

How you accomplish this is entirely up to you, but in the great scheme of things it is simply the responsible thing to do for yourself and for whomever else is around you at the time of a sudden onset attack, regardless of the type, sub-type, or variety of porphyria.

What we have done is place the large bottles of glucose in the glove compartment of our vehicle, in his wife’s or my oversized purse, and in the vintage 1920 Irish Republican military backpack or Vietnam era ammo bag he carries when we leave home. 

The good thing about the glucose tabs made for diabetics is that they are stable and require no refrigeration or special handling.  When he begins to have that bad feeling, he takes six of them in quick succession with gatorade or juice.  One tablet is worthless.  Six can make a difference.

Another thing we keep in the vehicle when we are going to be away from easy access carbs are the packs of crackers and peanut butter or cookies that can be found boxed by the dozen in any large grocery or big box store.  Not only do they contain a decent number of carbs in a pinch, they also tend to have a bit of sodium and can have a calming influence on a tender tummy.

Graham Crackers and ‘Nilla Wafers can easily survive a short life trapped in a ziplock bag in a backpack or purse.  Granted, vanilla can be a triggering substance in some people, so your own self-knowledge will tell you if this is the carb for you.  If not, there are other options that do as well:

• Ginger snaps (good for nausea too),
• Wheat Thins,
• Powder Sugar Donuts,
• Reeses Peanut Butter Cups,
• Kit-Kat Bars,
• Hard Candy.

Now remember, this is the list we use in my household.  These may or may not work for you and yours.  No one knows better than you what you can and cannot tolerate.  The important thing is to have something, and to have it in quantities that will make  a difference.  One LifeSaver will do nothing.  A roll of them can take the edge off of your carb need.

But what about something to wash it all down?  What about something that can bulk your carbs and bring down your core temperature enough to slow down the carbohydrate metabolism that causes your attacks?

The obvious answer is cold, sugar rich beverages, but there are actually quite a few to consider.  What we have found is  that the smaller bottles of gatorade travel well and can be popped in the freezer for a while before putting them in your pack.  They replace electrolytes, very important for the AIP porphy, and pack a decent sugar load.  Also, by freezing them first you can also use them as ice packs to bring down skin temperature.

Gatorade also makes a really good product called “Recover” which looks and tastes for all the world like chocolate milk.  It is a post workout carb replacer for athletes and worked really well when used in my household.  It is not quite as versatile as the frozen bottles of traditional gatorade, but really does the trick.

Juices work really well, but you have to watch which juices you drink.  Some juices are, for lack of a better term, sugar metabolizer boosters.  They tend to be advertised as juices for dieters and such.  And as good as they taste, and as much as you like them, it’s really not a good idea to have them packed in a Glucose Rescue Bag.  Acai, pomegranite, mangosteen and others actually seem to boost metabolism, which is great if you’re trying to lose weight and lower your blood sugar.  Leave these at home for small rewards if you cannot live without them completely.

Juices we have had very good luck with are simple things:

• Apple,
• Pear,
• Pineapple,
• White Grape (within limits if there is nothing else available),
• Peach (especially the thick, sweet nectar),
• Coconut Water (sing its praises),
• Mango.

Make sure that any juices you pack for your survival kit are refrigeration-free and safe at room temperature until opened.  Read the lables and make sure they are as low in additional chemicals as you can possibly find.

#2: Question Every Medication

One thing you have to remember with AIP and, indeed, with all of the porphyrias is that persons who live with it daily are incredibly sensitive to many chemicals.  Even beyond that, some individual elements in their pure form are specific triggers.

What medication is anything but a chemical?  A glorified, designer made, highly researched, rigorously tested, mass advertised, and often time insanely priced chemical of dubious effectiveness and the potential for both healing and harm, sometimes simultaneously, often to people with no documented history of reactions to medications or other chemicals.

You can do your homework when finding a primary care doctor, and you can make sure you’ve dotted every I and crossed every T when setting up your team of specialists.  But as much as you do to find the best possible fit, you have no guarantees that the team you assemble is as knowlegable about or experienced in treating your porphyria as you would hope, and certainly has less experience than you do.

You want to believe in them.  You want to know that they would never prescribe something that would harm you or even potentially kill you.  I mean, the first rule of Doctor Club is “Do no harm.”  But often times it comes down to two things:

• What their favorite meds for specific ailments are, and
• Just how much time they have between patients to do a proper search online.

So how do you know that you can trust your physician not to fall into that chasm of ignorance?  Honestly, you can’t.  You simply cannot trust them to always make the proper choice when prescribing medication or even in suggesting OTC medications for you.  You have to be pro-active and prepared before a medication is prescribed.

There are many good porphyria drug lists online, some of which are compact and complete enough to print and place in a folder that you can, and should, take with you to every doctor appointment.  Merck Pharmaceutical’s list is one of them, and gives you a very good starting place.  It gives you the opportunity to know before you leave the office if the scrip in your hand is appropriate.

You can also insist that you be allowed to go online to one of the more complete lists in the presence of the Doctor or his assistant to check for the appropriateness of the medication he or she is suggesting.  I have done that myself for my one, and his neurologist was extremely open to taking me to a computer and going through medications to find one that was non-porphyrogenic.  Of course, some will not do that and require a different tack.

For those situations, you have to look things up before you go.  You know why you are going to the doctor before you go, and generally you have an idea of what sort of medication, if any, is going to be suggested.  Do you need an antibiotic?  Are you stressing and feel you need an anti-anxiety medication?  Are you concerned about your pain management and want to discuss moving from your current med to something that might be more effective? 

Before you leave for your appointment, go to a porphyria drug list site and research a few.  Eliminate the ones with red flags, and make notes.  Be prepared when the doctor says he wants to put you on Drug A.  Better yet, give him a few options that you have already looked up and found to be safe, or at least relatively safe since everyone’s chemistry is different and affected by meds differently.

Take control.  You simply have little other choice than to take control over what you allow to be put in your body.

#9: Nut-up and med-up

One of the biggest issues for my submissive over the last year has been adequate and appropriate pain management.  In the past, when his attacks would last a day or two, he was able to manage with aspirin taken on a regular basis.  Even at its worse, he could manage it with an ocassional low dose of generic hydrocodone prescribed by his primary physician. 

However during this last year and its unrelenting series of back to back attacks, it has become increasingly difficult to manage his pain.  Many physicians’ hands are tied, preventing them from adequately addressing the pain.  Any relief they can provide is temporary at best.  Finding a specialist in pain management who is acceptable to one’s insurance can be nearly impossible, especially when the diagnosis is not conclusively confirmed by a difficult to perform and easily misinterpreted biochemical test.

Even when adequate medication was prescribed, it was difficult to get him to take it at truly therapeutic levels.  Was it a matter of masculine pride to tough out the pain?  Was it a fear of dependence on the narcotic component of his medication?  Perhaps it was a bit of both.  Regardless, the outcome of this self-deprivation remained the same:  unrelenting and increasing levels of pain and a steadily worsening damage to his person.

To most people, having a “reasonable” amount of pain is of little consequence to their overall health and well-being.  Even short periods of stronger pain are not detrimental if well-managed.  Most of us deal with various degrees of pain every day, including chronic pain from old injuries or old age, and suffer no long-term effects from it. 

The same cannot be said of pain in the person with acute intermittent porphyria.  Pain is most certainly the enemy.  Pain is, ultimately, a trigger for increasing attacks by virtue of the fact that it causes stress.

Stress is a huge trigger for porphyria attacks.  Even the mild stress that results from a sleepless night can accumulate over time to a level which can trigger or worsen an attack.  Because stress is such a strong trigger, it is important to manage it to the lowest consistent level possible. 

Remove the things which trigger stress, and you remove a porphyria trigger. 

Remove porphyria triggers, and you potentially prevent attacks or lessen existing attacks. 

Prevent or lessen an attack, and you give the individual an opportunity to recover and heal.

Sounds logical because it is logical. 

Just as it is important to maintain a healthy diet which is free from those foods which are known triggers, to keep a regular and adequate sleep schedule, and to avoid chemicals and medicines known to trigger attacks, it is equally if not more important to prevent stress as much as is possible.  One way to prevent a sizable portion of this stress is to make certain that pain is managed to a level which prevents stress.

This  brings us to the crux of Rule 9:  Nut-up and Med-up.  Misplaced medicinal machismo and self-denial are nothing less than self-destructive behaviors for the person with AIP.  If one does not have control over some of the other of life’s stress-makers, one can at least have some control over the stress caused by the pain of both an attack and its aftermath.

If you have a physician who has a firm grasp on the type and level of your pain and has prescribed medication adequate for managing your pain, then follow instructions and take these meds As Directed.  Don’t skimp, skip, or otherwise ignore your physician’s orders. 

Rule #9:  Nut-up and Med-up.  Take what you are given, as instructed, to eliminate as much pain as is possible, to eliminate as much pain-related stress as is possible, and to help you prevent, lessen, and heal from AIP attacks.  If you will not do it for yourself, do it for those who count on you, love you, depend on your service and affection, and from whom you receive much love and affection.