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.

If wishes were horses…

…then beggers would ride.

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.

And did you forget Her present?

Christmas day has come and the season has essentially passed for this year with little remaining but the clean-up, both at home and at the innumberable retailers staging sales to bring up gross profits (necessary for making payroll and purchasing merchandise for next season) and to rid their inventories of now outdated and unwanted merchandise.

It’s a festival of markdowns, a veritable carnival of cost cuts, and it extends far beyond the malls and big box stores.  Even some of the better Ebay stores are marking down the sorts of items you just cannot pick up at Walgreens or K-mart, things you rarely see on sale, much less almost in the Bargain Basement.

Want to find a few little items for yourself during this whirlwind of post-Christmas shopping?  Looking for some much needed training tools for the boy or girl?  Perhaps you want to reward them with a few new items for their special favorite activities, like a new apron for your sissy or a new harness for your bull?

And how about the household submissives among you?  Need to placate your Owner or Mistress because you dropped Her favorite coffee cup while paying closer attention to the household’s new Wii game than where the coffee table was during a serve?  Or perhaps you just want to suprise Her with a little something extra to show your appreciation for Her affection over the years?  Let me share a few ideas with you that will not cost you a ridiculous amount of money and still show Her how committed you are to Her collar and Her devotion.

Let’s start with apparel.  If you love feminine, nicely detailed, and inexpensive clothing with an exotic, lost in time look about it and a size range from Small to 5-XL, start with Ebay store “Holyclothing Exclusives” for dresses and separates.  If you have issues with Ebay, you can also find the online store at www.holyclothing.com, and they are having a great sale right now with many items as low as $7.99 for hand made tops, skirts, dresses and wrap-style pants.  You want pants that are both sexy and  comfortable?  You and your boy will love you in these.  

I can personally vouch for the quality and selection, as I have several pieces from this seller’s Ebay store.  They run a smidge big on size, and are great for taller Ladies.  And colors!  My goodness, they have colors!  You can set a lot of interesting moods by picking the right color, and basic black is just not friendly to everyone. 

How about impliments of A** Destruction?  Again I direct you to Ebay, but instead of searching for an Ebay store, you need to search for seller SM85 for a great selection of ipmliments.  I have a few that I use and love, including a nice pink and black flogger, a purple and black viper, and for the boy, an impressive heavy black collar that gives just the right headspace for dark, deep scenes.  Again, the prices are just great.  You just cannot, pardon the pun, beat these nice heavy floggers in a variety of colors for under $30.

So there’s a start.  Go forth and shop and enjoy.

Mistress Lila