Isabel Allende has been my favorite writer for years. Her writing is full of magic, fantasy and reality, though I cannot tell when her reality fades into fantasy or back again. So how was I to know that her writing would one day save my life by showing me that I'm really a Zebra.
We have all experienced serendipity, or as I call it, the confluence of event and time. The event has no impact if the time isn't right. Take the time that my stepson, a physician, suggested that I read Paula, by Isabel Allende. Though she is a favorite fiction writer, I refused, as Paula is not fiction, it is about Isabel's losing her young daughter to a rare, genetic disorder. Having almost lost a daughter to attempted suicide in Paris, I could not read about another woman's tragic loss of her child.
Then a year later, I was home with a mysterious illness that was slowly drawing the life from me. This illness had forced me to abandon my career and my life had become about going to doctors, trying to determine what was causing the horrible attacks of pain, episodes of paralysis and a disfiguring skin disorder.
One afternoon, my stepson came over to visit. "How are you feeling?"
"I now believe in voodoo and if I find out who's jabbing pins into my Voodoo image, I'll maim the SOB."
"I brought you a gift, a book, to take your mind off the pain." The book was Paula. I didn't have the energy to remind him that we had previously discussed the book. While medication barely took the edge off my pain, it worked well to dull my judgement, so that night I began to read Paula.
What had caused the death of Isabel's daughter? Reading quickly onward, I finally came across the word Porphyria. What the hell is porphyria? I muttered as I lay the book aside and turned to my computer. As a therapist...I've had patients with every syndrome and disorder known, but I had never heard or seen this word. As I read about this strange disorder...my blistered skin, the pain, the acute paralysis, a myriad of symptoms...all fell into place.
Three years after uttering the dreaded word porphyria to my doctor...I was given the clinical diagnosis of porphyria by a team of doctors who had ruled out every other disorder and disease. There are tests for porphyria but these had to be carried out by special laboratories and my type of porphyria gives normal lab results when I am not in the middle of an attack, a known fact which the expert kept ignoring.
Back to the curious matter of the serendipitous gift of Allende's Paula from my stepson, the doctor. Had Allende not written that beautiful, painful book, had my stepson not unwittingly given it to me at the right moment, I would not be here to write this post...the confluence of event and time. Thank you, Isabel...thank you, Paula.
Note:
Why am I writing about this on a blog that usually deals with bunnies and design? Because I want to document how Tall Husband and I have found a way to control and live with this incurable, genetic, metabolic disorder, so that my children and grandchildren will have a blueprint for surviving and coping, should they have inherited porphyria. Plus, I know there are more of you
Zebras out there, diagnosed and undiagnosed, who need to consider this information.
As I have learned, my type of porphyria (there are eight or more types) has a photosensitivity component: I break out in blisters when exposed to light, especially sunlight or the ever popular fluorescent lighting. There is a long list of medications that a person with porphyria cannot take (the
wrong drug, given in a Madrid hospital, caused Paula's year-long coma, then her death.) In addition to certain drugs, one cannot drink alcohol for fear of triggering a life threatening attack.
Porphyria is a genetic, metabolic disorder and is triggered by environmental events. My triggers were: exposure to cats (an allergen), Atkins diet (persons with porphyria need carbohydrates), and two glasses of Merlot. Bang...I'm in the emergency room with acute paralysis, excruciating pain in every organ, plus deep bone pain; dangerously elevated blood pressure, vomiting and fading in and out of consciousness, and blistered skin. Six-thousand dollars later, we didn't know the cause.
Here's how I handle my type of porphyria:
- Eat 6 small meals a day (every three hours.) At least 60% of intake must be complex carbohydrates, as this will help prevent the build up of porphyrins in the body. A dietitian helps me with this so that I can prevent weight gain.
- Carry glucose tablets with you at all times (they are over-the-counter, in the diabetic section of the drugstore.) These little tablets can prevent attacks if taken at the first sign of an impending attack.
- Carry codeine or another such medication at all times. If the glucose tablets do no stop an attack, the codeine usually will. My neurologist first prescribed the codeine, now my internist does. Never drive after taking this or a similar drug.
- Take NAC to counteract the toxicity of acetaminophen (as in Tylenol) that is in the codeine medication.
- Take a combination of magnesium (for bone pain and muscle spasms), zinc (for skin damage) and calcium. Porphyria depletes the body of magnesium, zinc and calcium.
- Taking Potassium Gluconate, 550 mg, twice a day has stopped the acute paralytic attacks. These attacks can, according to my neurologist, leave one with permanent paralysis.
- Wear hat and gloves even when driving. Sun screen only blocks some of the rays and you can still have skin damage from the rays that get through the screen.
- Plan outdoor activities when the sun is not so intense.
- Use prescription steroid cream on blistered skin.
- Avoid fluorescent lighting. If you cannot, there are filters that reduce the emitted UV of the fluorescent bulbs, thus reducing skin damage.
- Never, ever drink alcohol. Drinking (i.e., ethanol) is dangerous for a person with porphyria.
- Carry a list of "safe and unsafe drugs" with you. Give this list to your dentist and physicians.
- If you are having a full-blown porphyria attack...get to an emergency room so that they can give you the proper medication and prevent dehydration.
- There is the idea that you do not have porphyria unless your urine is purple. Urine usually only darkens during an attack and only after being exposed to light.
- Know the signs and symptoms of porphyria.
- Know that today deaths from porphyria are rare.
- And finally, have the rare, good fortune to have married Tall Husband who has a background in biochemistry and who has learned more about porphyria than any person I've met.
Link:
Addendum September 10, 2010:
A month ago I began taking Tagamet (Cimetidine) (200mg in AM and 200mg in PM) as a prophylactic to prevent porphyria attacks. I have not had an attack in a month...I usually have to fight off two or three attacks a month. Tagamet has even stopped the chronic porphyria pain!
Addendum December 28, 2011:
After being on Tagamet (Cimetidine) for a year, I began having the neurological symptoms of the attack (heart muscle contraction, laryngeal muscles contraction, leg and arm paresis; i.e., feels like heart and larynx are being ripped from my body and arms and legs are paralyzed.) All this without the usual abdominal pain. After some research, I learned that anyone taking Tagamet (Cimetidine) is at risk for B12 deficiency. Began taking B-complex vitamins and the symptoms have decreased.
Addendum April 21st, 2012:
Tagamet (Cimetidine), according to new drug-interaction information, is not to be taken if one is taking Lexapro, which I have be taking for about a year. So, I have stopped taking the Tagamet (Cimetidine) which was causing symptoms of serotonin toxicity.
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