Thursday, July 21, 2011


LOL It's so hot.. Perhaps, as you stumbled in the house, clammy and damp, you said, "Man, it's hot," for what seems like the hundredth time. We know we have and we're tired of repeating ourselves. We figured if our bodies can't be fresh, at least our words can be.

Until the heat peaks in August, try out a hot phrase. They won't make the temperature drop, but they might make you smile. And that's pretty cool.

• It's so hot the birds have to use potholders to pull worms out of the ground.

• It's hotter than a half-bred fox in a forest fire.

• It's hotter than two bears fighting in a forest fire.

• It's hotter than a billy goat in a pepper patch.

• It's hotter than two cats fighting in a wool sock.

• It's so hot that I tied my mule in a field of corn, and the corn started popping and the mule thought it was snow and froze to death!

• It's so hot I saw two trees fighting over a dog.

• It was so hot today I saw a dog chasing a cat and they were both walking.

• It's hot enough to fry an egg on the sidewalk.

• It's so hot outside that you could fry an egg on the hood of my car.

• It's hotter than a depot stove.

• It's hot as love in August.

• It's hotter than a mother-in-law's kiss.

Sunday, July 17, 2011

Pain Levels (EPP): by Victor Mejias

Pain Levels (EPP): by Victor Mejias

In this posting I am going to attempt to document, on a Zero to 10 scale, differing degrees of pain and (with the exception of swelling - refer "note B") other symptoms involved in differing degrees of EPP reactions to sunlight.

The level zero will of course be defined as that state where there is no light exposure effect whatsoever, and a level of 10 will be the complete opposite, i.e. a totally out of control EPP
reaction after a great deal of exposure.

I attempt this in the full knowledge that both the speed of reactions as well as their severity, as hereinafter described, will and do differ from one EPP patient to another and therefore
some may not agree with everything that follows.

However the reasons I am attempting to do this basically are: - The degree of pain involved in EPP reactions has been "sadly under-articulated" in general (to say the least), and therefore not fully appreciated by the medical profession or some EPP parents and relatives.

- To assist all EPP patients (young and old alike) communicate with their physicians and parents/relatives the level of pain and type of reaction they are experiencing.

Several factors for each "Reaction Level"  level will
be included, these are:

a) Pain Description.
b) Skin Sensitivity Rating: On a scale of 1 to 10 skin surface sensitivity to touching, scraping, accidental knocks and exposure to heat etc.
c) "Sleep Disturbance" Rating (if any), from zero to  very significant 
d) A "psychology effect" factor on a "No Effect to the High" scale, whether behavior modifications are sought, levels of    stress experienced etc.
e) An "overall pain" level, also on a scale of Zero to    Very High.

So let's begin at the very base level of "Zero" – i.e. the very inactive base level, defined as no reaction whatsoever.

Reaction Level - 0
a) Pain Description: This is the completely normal (un-agitated) state of the skin prior to any exposure to sunlight.
b) Skin Sensitivity: This also zero, i.e. same as any normal person. Completely pain and sensation free.
c) Sleep Disturbance Rating - Zero.
d) Psychology: Undetectable/No effect.
e) Overall Pain Level: Zero.

Reaction Level 1
a) Pain Description: Light tingling sensation.
b) Skin sensitivity: Rating 1, almost normal.
c) Sleep Disturbance Rating: Zero - Normal sleep pattern.
d) Psychology: Almost Zero - Detectable only.
e) Overall Pain Level: Low - Easily tolerable.

Reaction Level 2
a) Pain Description: Stronger tingling - including the nerves.
b) Skin Sensitivity: Two - getting a little sensitive.
c) Sleep Disturbance rating: Very slight - But normal sleep pattern.
d) Psychology: Still low - But noticeable.
e) Overall Pain Level: Low - Still relatively tolerable.

Reaction Level 3
a) Pain Description: Stinging sensation, light nerve vibration, light chills on the skin surface (particularly when exposed to cool air).
b) Skin Sensitivity: Three - getting sensitive.
c) Sleep Disturbance Rating: Slight - But normal sleep pattern possible.
d) Psychology: Low - But noticeable and still quite tolerable.
e) Overall Pain Level: Low/Medium - Still relatively tolerable.

Reaction Level 4
a) Pain Description: Warmer stinging sensation, nerve vibration, chills on skin surface.
b) Skin Sensitivity: Four - Sensitive to touching, scraping knocks etc.
c) Sleep Disturbance Rating: Still only slight - But normal sleep pattern usually possible.
d) Psychology: Low/Medium - Slightly intrusive but still tolerable.
e) Overall Pain Level: Medium - Still tolerable.

Reaction Level 5
a) Pain Description: Warmer stinging sensation, slightly painful deeper nerve vibration, more severe chills.
b) Skin Sensitivity: Five - Quite sensitive.
c) Sleep Disturbance Rating: Getting significant - normal sleep pattern often disturbed.
d) Psychology: Medium - Intrusive and now barely tolerable.
e) Overall Pain Level: Medium - Barely tolerable.

Reaction Level 6
a) Pain Description: Very warm (almost hot) sensation, painful & deeper nerve vibration, more severe chills.
b) Skin Sensitivity: Six - Getting very sensitive.
c) Sleep Disturbance Rating: Getting more significant - normal sleep pattern usually disturbed.
d) Psychology: Medium - Intrusive and now becoming bothersome, beginning to be stressful, methods of alleviation often sought from this level upwards.
e) Overall Pain Level: Medium/High - becoming intense.

Reaction Level 7
a) Pain Description: Hot sensation, painful & deep nerve vibration, severe chills (if any chills occur).
b) Skin Sensitivity: Seven - Very sensitive.
c) Sleep Disturbance Rating: Significant - normal sleep pattern almost always disturbed.
d) Psychology: Medium/High - Intrusive and now annoying and/or distracting, often stressful, methods of alleviation usually sought.
e) Overall Pain Level: Medium/High - relatively intense.

Reaction Level 8
a) Pain Description: Hot & burn like sensation, painful and very deep nerve vibration, severe chills (if any).
b) Skin Sensitivity: Eight - Highly sensitive, getting painful to touch, quite painful to knocks and scrapes.
c) Sleep Disturbance Rating: Very Significant - normal sleep pattern often/usually severely disturbed.
d) Psychology: High - Intrusive and now very annoying and/or frustrating, stressful in general, methods of alleviation more actively sought.
e) Overall Pain Level: High - Intense.

Reaction Level 9
a) Pain Description: Very hot & burning sensation, deep searing nerve vibration occasionally affecting other parts of nervous system, hot flushes, etc.
b) Skin Sensitivity: Nine - Very highly sensitive, now painful to touch, very painful to knocks and scrapes.
c) Sleep Disturbance Rating: Very Significant - normal sleep pattern almost always severely disturbed (if sleep is possible at all).
d) Psychology: High - Very Intrusive and now very annoying and/or very frustrating, quite stressful, methods of alleviation pro-actively sought.
e) Overall Pain Level: High/Very high - Very intense level of pain.

Reaction Level 10
a) Pain Description: Very hot & burning sensation, very deep searing nerve vibration (deep into the bone marrow) often affecting other parts of nervous system, hot flushes, etc.
b) Skin Sensitivity: Ten - Extraordinarily sensitive, now very painful to touch, incredibly painful to knocks and scrapes.
c) Sleep Disturbance Rating: Very Significant - normal sleep usually impossible until reaction declines in severity.
d) Psychology: Very high - Dominating and exasperating, very stressful, any method/s of alleviation pro-actively sought and adopted.
e) Overall Pain Level: Very high - Extremely intense level of pain.

A. Once again I must re-iterate that the speed at which one patient progresses from
Zero to 10 varies from one individual to another. This is to be expected with any qualitative rating system/analysis like this. So please feel free to respond to this posting with other symptoms that you experience but are not listed above etc.

B. I have deliberately omitted the swelling that often accompanies the above levels due to the vastly differeing levels of swelling experienced by different EPP patients. Those that find
swelling a major symptom are encouraged to pencil a description
of that in as an additional factor for each level above.

C. I probably should add another level (number 11) for those patients who have experienced liver problems, or even a total breakdown of liver function, because there will obviously be
factors involved in such a reaction that are not included in the ten levels above. Maybe one of them can give us an indication of what they went through in comparison?

D. EPP parents who are having trouble comprehending the degree of pain their EPP children are suffering should experiment with a table like this. Younger patients in particular are not able to articulate the level of pain and discomfort they are suffering. Parents will then gain a clearer understanding of what their children are experiencing and therefore whether to commence taking alleviating and/or preventative measures.

E. I included the "sleep disturbance rating" to remind relatives of EPP patients that just because they have gone to bed does not necessarily mean they are getting good night's sleep.

F. To assist communication with non-EPP relatives, all EPP patients (younger patients
in particular) should be encouraged to rate on this 0 to 10 scale events that often occur
in the lives of non-EPP patients. e.g. falling off a bicycle, spraining an ankle or wrist, breaking a limb, getting kicked in the leg during a football match etc. For example, I would rate falling off a bicylce and getting a few bruises and scratches a 2 or 3 (depending on the severity of the fall) which is a long way from the pain experienced in a full blown EPP reaction (9 or 10).

G. Personally, I would rate "normal" sunburn as being between Reaction Levels 1 and 4 on the above scale, well maybe a 5 for very serious sunburn. This is because it is mainly a superficial skin surface phenomenon in my experience. Yes, I have experienced normal sunburn a few times in my life, and it is much, much less painful than the levels of pain achievable by a "full blown" EPP burn.

H. I have never experienced the chills at the higher reaction levels, only up to about the Number 7 level. After a reaction has started subsiding from higher levels, yes for sure, but not at the very top levels, this is probably another reaction factor/symptom that varies from one individual to another.

I. I have a more simplified version of the above on an Excel spreadsheet, which I can send to anyone that needs it, or post in the "files" section of the website if required.

J. In view of the lack of appreciation (by the medical profession etc.) of the type of pain being experienced by the average EPP patient, I had decided to compose a table like this some time ago.

Wednesday, July 13, 2011

Miranda's Advice to College-Age Porphyria Sufferers

You’ve just turned 21, and you have a rare disease that forbids you from drinking. You go to parties where people pull a PBR from a bathtub or do keg stands. People are getting progressively drunker; even shy people who never talked in that English lit class you had are suddenly dancing on a table, and you are standing by, utterly confused. You’re holding a beer. You take a sip, but you don’t actually drink it. You just kind of hold the beer, hoping no one expects you to consume. Later, you give the beer to someone when there’s no more beer left in the bathtub. Someone offers you a cigarette. “I don’t smoke,” you say. Neither does that person, “just when drinking.” You begin to wonder if you’re an alien.

Don’t worry, it’s totally normal if you have porphyria!

I went to a tiny women’s college in mountains of Virginia, where there’s not a lot to do besides drink. American college life sort of stipulates that you make a lot of poor decisions, including possibly getting your stomach pumped. Obviously, I’m being facetious, but I’m also being realistic about the drinking culture, especially where it applies to college life in the United States.

But, guys, as tempting as it is to join the legions of drunk people, eating eggs at an IHOP at 2 am, and dancing on tabletops, remember that alcohol can affect some of the porphyrias, if not all of them. As someone with AIP I could die if I drank.

I’m not judging people who drink in college. A lot of times I envied them because in American culture drinking is such a coming-of-age experience, despite the fact that there’s nothing inherently mature about getting drunk. (We really have a weird relationship with alcohol in this culture!) But I am saying that it’s important to realize that many of us just cannot drink alcohol, and basically, you have to suck it up and get over it.

I could try to provide a list of things you could do as an alternative to drinking, but you know what? It would be unrealistic and ring false. Instead, you should go to the parties and dance and talk to people (I’ve been known to explain porphyria to people who are drunk) and be who you are-- pretend you are completely uninhibited, and you, too, can dance on a tabletop.

--Miranda Dennis

In Praise of Chicory Coffee (and Grandmothers)

As a child and a teenager I was lucky enough to live in the same city as my grandmother, who was a health nut and a bit of a snob when it came to good cooking, especially in restaurants. Up until I was a teenager I had no idea that my grandmother knew how to make sweets like fudge or divinity. I assumed that she got her sweet tooth fix from ripe blueberries and the occasional custard. It turns out she simply chose not to eat rich, unhealthy desserts, except very rarely.

She’s a great example of someone who was able to eat very healthy while maintaining the necessary carbs to prevent Acute Intermittent Porphyria attacks. Now that I’ve grown older and can appreciate what I thought was rather fanatic as a kid (it wasn’t) I like to follow the examples she set in my own health, as evidenced by previous posts of mine.

One thing she introduced me to, which I am forever grateful, is chicory coffee, served with steamed/heated milk and maybe a little sweetener, New Orleans style like at the famous Cafe Du Monde. One day she made it for me, and I got hooked. The great thing about chicory is it negates a lot of the caffeine’s effects; half of the drink is steamed skim milk, leaving only 25% caffeine intake compared to a normal cup of coffee.

I sometimes make chicory coffee with steamed milk as a dessert drink when I have a hankering for sweets. After all, skim milk can help satiate the craving for sweets (something my Grandma taught me, which has turned out to be true). So, I tend to heat up my milk, brew up some chicory coffee, and add agave nectar (or the occasional raw sugar, which isn’t as processed and therefore better for you). Voila, I have a sweet drink that’s not too sweet but satisfies my sweet tooth.

I think people with porphyria should be mindful of how they’re getting those sweets they’re craving. While it may feel great to eat a piece of cake (yum), don’t forget about fruits, skim milk, a little bit of sugar, etc., going a long way. Remember, the healthier we eat, the healthier we are, porphyria or not.

--Miranda Dennis

Saturday, July 2, 2011


Written All over His Face: Rare Disease Offers Clues to How We Read Emotions

People who feel what they see offer clues about how we read emotions and empathize
  • Understanding the thoughts and feelings of other individuals is essential for navigating the social world. But empathy is a complex process, based in part on fleeting facial expressions. Research suggests that we empathize by effectively putting ourselves in others’ shoes: for example, when we observe someone feeling sad, we simulate their experience by activating the same regions of the brain that are involved when we feel sad ourselves.
A study in the Journal of Neuroscience in February bolsters this idea using rare individuals with “mirror-touch synesthesia.” When watching another individual being touched, these people actually feel a touch on the same part of their own body. Neuroscientist Michael Banissy and his colleagues at University College London tested whether this heightened ability to simulate another person’s experience would cause eight mirror-touch synesthetes to excel at recognizing the emotions embedded in facial expressions. They did, correctly identifying 92 percent of the facial expressions tested compared with the 81 percent identified by control subjects. Their success probably stemmed from their simulation expertise rather than a general agility with faces because further experiments showed they were no better than controls at recognizing a person’s identity.
For the rest of us without mirror-touch syn­esthesia, the simulation process is the same but less pronounced, Banissy says. So the next time you find yourself sympathizing with someone who looks sad, thank the part of your brain that feels you frown.
I enjoyed reading this over, when we face trials, illness, over outward expressions can speak volumes and usually we say were doing ok or good!  So be honest with how you feel!
Thanks to Michele for letting me pass this on to you.
Amy Chapman

What is δ-Aminolevulinic Acid Dehydratase Porphyria (ADP)?

What is δ-Aminolevulinic Acid Dehydratase Porphyria (ADP)? ADP is more severe than the other acute porphyrias and can present in childhoo...