Safe/Unsafe drugs

Treatment anecdotes

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Anecdotally speaking

"The plural of anecdotes is data."
(Broadband, ABC Radio National, April 11, 2004)

Most of the responses below are from the people with acute porphyrias. If you have any suitable stories for inclusion please ring our number (03) 9845 2737 and leave a message on the voice mail. Most comments have been said by more than one person.

What happens to your skin?

It's like my family missed out on an extra layer of skin. I knock things and bleed easily. My mum once scratched her leg on a buggy and she got this huge ulcer, which lasted for months.
I can't go out to peg out the washing after 10am or I start getting huge painful blisters.
Sometimes I think its like living in that movie The Others, where you have to hide from daylight.
I only get a patchy rash when I'm in attack. It looks a bit like a heat rash.

What causes acute attacks?

For me, I only get attacks when there are a combination of triggers. For example, one time I went for a walk on a windy beach and forgot to drink enough water [dehydration] on top of forgetting breakfast [fasting] on top of being under pressure because a family member was seriously ill in hospital [serious stress]. In addition to all this, I had a glass of liqueur [alcohol] and I was using cold sore cream [wrong medication]. That night I went into attack. Not enough sleep also puts me at risk.
I have only ever had attacks when they have been triggered by wrong medication.
I tried Tremadol and within 12 hours I was in hospital with an attack.
I have never had an acute attack but I have lost all feeling in my legs after I was on an unsafe drug for a year. At the time, I didn't know it was unsafe.

Do everyday stresses cause attacks?

I watched my wife take the phone call to say her mother had died and I just knew she was going to have an attack. She did.
I did profiling to test a drug and I did not go into attack but I noticed my porphyrin test result numbers were high on days when work was very, very, stressful. I tend to go into attack only when there are combinations of physical triggers.

What about diet?

One book I read said you must have minimum numbers of grams of carbohydrate per day, 300 normally, and 600 when in attack.
I find that if I eat something every two hours and drink at least 2 litres of water per day, then I can usually prevent the pain. I need to exercise to prevent weight gain.
My dietician gave me a list of foods with high carbohydrates and low Glycemic Index (GI).
When it looks like I am headed for an attack I reach for the fast sugars: jelly beans, orange juice, Polycose. However, it is better if I keep up the slow carbs: rice, pasta, bread and potatoes regularly every day or every meal. Old-fashioned oatmeal is good for breakfast.
I eat oranges regularly. They also help prevent constipation.

What about constipation?

Every time my husband gets the [severe abdominal] pain the doctors are convinced he has constipation, they give him suppositories. I could tell them they are not going to work, but they won't listen to me.
He does get constipation as well during attacks. Once they found he was badly constipated and they said, “Well, that is what is causing the pain”. Now I know you get constipation as well during an attack.
I think the doctors should get onto fixing the constipation earlier in an attack. [Peristalsis shuts down, the nerves which trigger your food and your faeces to move through your gut, fail. So, medicines, which work by triggering those nerves into spasm, don't work.]

Can you safely have an operation?

When I am going for an operation I try to make an appointment with the anaesthetist beforehand. It's a delicate task to make sure they know that unsafe drugs can kill me, but not to scare them so much they don't want to do the operation.
One doctor had a new journal article that said this was safe in one person with Porphyria. But I argued strongly until he stuck to Dr Moore's Safe List.
I have had many minor procedures and the anaesthetists get tempted to add a bit of this or that at the last minute, this can trigger an attack. Due to these experiences, I have had a bad run of things, and this has affected me in many ways.
It is hard to convince some doctors to put up a sugar drip and keep it running the entire time from when I begin fasting. When I do get an anaesthetist who will religiously stick to Professor Moore's list from www.uq.edu.au/porphyria they receive my gold award and I can go into an operation with much less stress.

What about nausea?

We are recommended not to have Maxolon but it is usually standard in hospitals it is hard to refuse. Stemitil was okay, however, in a long attack, I had a bad reaction to too much of it, which apparently is fairly common. They wanted to try Ondanzatron. My wife rang up the manufacturer who was not prepared to say it was safe. Now I have very small doses of Largactil just for the nausea.
In one long attack I was given a very slow bag of a low dose of Largactil and maintained that until I could eat enough so that lack of food wasn't triggering extra escalations of the attack.
Getting on top of nausea early seems to be a real key to getting over an attack.

Have you noticed a pattern to your trips to hospital?

If I get to hospital and get a 10% dextrose drip in within 2 hours I am in casualty for approximately 6 hours. If I delay getting there for, say, 7 hours then I am in hospital at least 2 weeks. The nausea gets so bad I lose the ability to eat. My bladder and bowel have shut down. I have had seizures and sensory loss. On one occasion I had a code blue and was in ICU.
I had one attack that went for 9 months and they told me that I just had to get used to the pain. I now understand why lots of people with Porphyria commit suicide.

How were you diagnosed?

I was tested because my mother had it. I didn't want to know about it for years, but now I know I could have prevented a lot of the complications if I had learned more.
I had an accident, which caused a seizure, and my Porphyria was triggered by the anti-seizure drugs. It was months before one specialist thought of testing for Porphyria.

My cousin has had a bad time with the Porphyria. She told me to get tested but my doctor said to wait until I had symptoms. My cousin was really angry with that doctor. She says my grandchildren are at risk.
I got tested in the country and was told it was negative. I found out later that the country lab didn't have testing facilities so must have just pretended they did the test. When I thought I was negative I started taking the pill. Now, years later, my test porphyrin numbers are really high and I have to be very diligent to avoid attacks.
It was over 20 years of a truly horrible time before someone thought to test for Porphyria. Now I want to help in any way so no one else has to go through all that I have had to put up with. If it were not for my rock of a husband I don't think I would have made it.
I don't know what all the fuss is about - I was diagnosed years ago in a family screening, I drink and I smoke and I've never had an attack.

How do you find a good doctor?

I like the doctors who admit they don't know much about Porphyria and just follow Professor Moore's website to the letter.
I have had lots of doctors who told me it was all in my mind until I wondered if I was crazy.
I was told by one doctor not to let it rule my life and get on with it. I wonder if that's what he says to the people with diabetes. I think its like diabetes, the more you learn the more you can do.
I hate it when they say “Well, it's not on the unsafe list so we will try it on you and see what happens”. I've had attacks from this and I'm sick of being a guinea pig.
When my doctor wants to find a new drug for me, we do it when there are no other risk factors happening, and we do a proper trial to see if my porphyrin numbers start to go up.
I like the younger doctors - they admit they don't know much and say “Tell us what you know and we will work together as a team.”
In all the years I have had Porphyria, I only remember two really good doctors. Both were wise older doctors. One took my coat and hung it up like I was a lady, not just a specimen.
After years of trying to avoid going to a doctor unless I really had to, we finally found a good physician who sees us as a family. He talks to us like we are human beings first and seems to accept that we are not making it up. He really does listen to what we have to say then explains how he sees it. Then we agree on a plan of how we can best manage.