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.