This is a composite of reportings from a range of people with multiple acute attacks of Porphyria.

“She had an attack. In Casualty she was given three bags of ten percent dextrose* & non stop stat. Needed less morphine. Six (6) mg of largactil stopped the vomiting. We were home in under five hours.

They took electrolytes during the second or third bag without stopping the dextrose.

At home she ate five pieces of toast with vegemite, had a cup of tea with sugar then slept for twelve hours. The next day she had no pain - didn’t even need Panadol.

On other occasions when the electrolytes have been low she had a bag of saline run in parallel so that the dextrose didn’t get stopped. Two pump lines going into one vein.

The day after her attacks we do a de-brief. We talk about what worked and what could be done better. This was how we got better at working with the doctors so fewer things went wrong.”

* Good overview references regarding the use of 10% dextrose and haem-arginate include:
Thadani H, Deacon A, and Peters T, (London), “ Diagnosis and management of porphyria,” British Medical Journal, Vol 320, 1647-51, 2000
and Kauppinen R, (Helsinki), “ Porphyrias”, the Lancet, 15 Jan 2005

“If it all goes right I ask for two or three days off work and take it easy for a week. People at work can’t tell that I was so ill two days before. After this I go on a high-prevention lifestyle for at least a month.”

Tips from a collection of de-brief talks:

• “We got to casualty early – within an hour of the pain starting. She was triaged as urgent and got the dextrose started a few minutes later as the staff knew that the quicker its started the quicker she is out of there.”

• “It’s hard to get a vein. Veins are difficult, they have shrunk, possibly with dehydration. A good experienced casualty nurse can usually get a bung in. The only thing I hate , if they stop trying to get one. Even though we can’t have lignocaine, I know the pain is nothing compared to Porphyria pain.”

• “Some doctors have tried 5 percent dextrose. It doesn’t work properly. It seems as if, with the biofeedback loop, it takes 10 per cent, enough to kick the loop out. It needs to be continuous and fast enough and concentrated enough. 10% dextrose works and even sometimes a 50% push, though its hard on the veins.”

• “6 mg of chlorpromazine (Largactil), that is 1/8th of a dose by injection, or ¼ tablet, stops the vomiting and sometimes settles the panicky feeling. We can’t have Valium or Maxolon. These two are on the unsafe list and likely to make the attack worse. Stemetil is on the safe list but too much of it sometimes has funny effects.

• “Too much Largactil too early makes me too disorientated to cope – but I accept a small dose at the point after the worst of the pain and if I have begun vomiting.”

• “We use Betadine instead of alcohol swabs for drip sites etc. Alcohol is on the unsafe list. A sugar bung is a good bug-breeder but if the attack is stopped quickly it’s not a problem.”

• “If the dextrose doesn’t stop the attack, or signs of neurological problems are appearing, we start asking for haem-arginate *. & If given early enough, Haem-arginate makes the difference between one week in hospital or five weeks as well as months to recover. Or worse - permanent damage.

• “They say Haem-arginate won’t reverse an established neuropathy. But we also know that even brain lesions and paralysis can reverse if an attack is stopped.” **

• “Neuropathy for me starts with my feet - ‘socks’ one at a time.”
  
  * Thadani et al ibid and Kauppinen ibid 2005
  
  ** King PH, Bragdon AC, “MRI reveals multiple reversible cerebral lesions in an attack of acute intermittent porphyria", Neurology, Vol 8 , 1300-02, 1991
  and Auchincloss S, and Pridmore S (Hobart), the Lancet

• “Oral carbohydrates can work as a preventer but once into attack, absorption doesn’t seem to work (even peristalsis fails).

• “Constipation is a dehydrator and is part of an attack. But laxatives that depend on peristalsis don’t work. It’s best to stop the attack.”

• “Recovery and prevention lifestyle: a simple one is 2 hourly boiled rice and lots of drinks of water 2- 6 litres per day (this is in addition to any other drinks – tea, coffee and especially coca cola seem to dehydrate) Other carbohydrates rice, pasta, potato, bread with minimum preservatives are good when the rice gets too boring.”

• “We have found a good family dietician and a good family pharmacist are real helps with prevention. Our pharmacist checks everything we have whether the doctor has written the script or it’s the ingredients in over-the-counter cough syrup etc. This pharmacist has seen me so sick from past attacks when the effects lasted for months. We don’t take medications that aren’t on the safe list unless we are in a low risk time and do a monitored trial according to the testing regimen.”

• “In the de-brief after an attack, go through all the possible triggers. First and foremost is medication. The second is dehydration, thirdly fasting (eg. skipped breakfast) is looked at. Then there is womens’ own oestrogen peak time and severe stress and even exhaustion. Often, and usually, we find a combination of factors. It is interesting that there are usually many hours (maybe 12 hours) between the trigger and the start of the attack – it seems to take time to build up the biofeedback loop. Once, when I was given a slow acting drug that has to build up in your system, it didn’t seem to give me an attack straight away but I found I was having more frequent attacks ‘til I finally went off that medication. Smoking works like that too.

• “Of course alcohol is also a trigger. Some people think they can get away with drinking if it is managed but all you need is another little trigger to tip you into attack and then you are always more susceptible for future attacks.”

• “I used to go drinking when I was younger, but after one period of being sick with a bad prolonged attack, now my body can’t even have one glass or I pay for it with so much pain. People don’t believe the pain can be that bad.”

• “I think of an attack as having two tipping points: one into the biofeedback loop and another out of it. To “kick out” of an attack dextrose needs to be “thick” enough and fast enough. 5% is not enough and at least the first 2 bags needs to be stat. Third or subsequent bags can be slowed but not stopped or the attack builds again. In an ‘at-risk’ phase sometimes it’s something little that tips you into an attack.”

• “One thing that used to go really wrong was that the vomiting used to really get out of control. Fine-tuning the dose of anti-emetic made a difference and especially getting to the hospital quicker.”

• “I had an attack where pain wasn’t such a problem but I went ‘gar-gar’. My family had trouble convincing the doctor that it was Porphyria. Eventually, a nurse who knew me and knew what I was normally like, persuaded the night doctor to give me dextrose. The next morning I woke up normal. I told her she saved my brain.

• “Another time a doctor didn’t want to give me dextrose as my blood pressure was high. I explained that Porphyria can make your blood pressure go high and if we stopped the attack it would go down again. I told him I would rather take that risk. We settled on me taking beta-blockers along with the dextrose.”

• “Rule of thumb for daily dextrose: 300 mg dextrose daily non stop as a preventer eg. for an operation or other fasting periods, and 600 mg to get out of an attack or until the Haem-arginate arrives. (We remind the staff to check electrolytes because hyponatremia is not nice.)”

• “I know some of the other people who have acute attacks say the dextrose works best for them if its run stat, as fast as possible. I like the first bag fast but the second bag a bit slower, so that a two bag infusion lasts 3 hours. If it is too fast for me, I get a migraine. But to work properly it must be continuous, with the second bag ready to hook up straight away.”

• “Until better management was achieved, too many fits were happening. Bad Porphyria can cause fitting or huge doses of pethidine but electrolyte imbalance seems to be a big reason.”

• “On the other hand insufficient pain medication is a big problem in an attack. High doses early seemed to work better than trying to hold back on the opioids. After the first wee, pain seemed easier but exhaustion makes it harder to manage. And if the pain is controlled and you can get sleep then you can get over the attack. “

• “It seems that the hormones that are a by-product of severe pain, feed and escalate an attack. I want enough pain relief so that the attack stops. If I can get to sleep it off, I need no medication at all until the next attack. But if they don’t give me enough pain relief I seem to go into a rumbling attack for weeks with pain that just wearingly lingers round. I end up having more medication that way.”

• “I have heard that your metabolic rate slows in sleep so sleep becomes a treatment for a Porphyria attack. But you need to be out of pain to get proper sleep.”

• “Pethidine works better for most of us but we started switching to morphine when the dose gets too high.”

• “I read* that dependence is unlikely in Porphyria if the attacks are treated and stopped.”
  
  * Professor Elder, University of Wales porphyria pages

• “It is much better now that they know to get the next bag of dextrose ready before the last one runs out. In the past, the attack used to build again while we waited for the next bag.”

• “Sometimes in a really bad attack I was put in recovery and on all the monitors. I can get really “tachy” in an attack (really fast heart rate) or I can black out on sitting up, if my blood pressure suddenly drops. Or, if the pain is incredibly bad and I need a lot of medication. Also I used to have fits before we got better at managing attacks.”

• “One time when casualty was really busy they put me in a recliner chair and got the dextrose going. I was so grateful for this rather than having to wait until a bed was available. The attack was stopped before I became a serious emergency case with the prospect of weeks in hospital. The next morning I went home.”

• “One day I would like to see that anyone having too many attacks has a team (like diabetes or a heart coaching team) who goes through their lifestyle searching for triggers and how to prevent them. I had a good hospital dietician who helped with managing diet. And another team offered yoga lessons to reduce stress. Whatever it takes.”

• “The obvious triggers are medication, dehydration, alcohol and fasting. The subtle ones, the ‘last straws’, are harder. From passive dope smoking to some cough mixtures, alcohol mouth wash, contraception and maybe even oestrogen in soy milk or sulphur dried sultanas or dehydrating from too much coffee and coca cola. Or when the Council is using bitumen in your street.”

• “I know that I can die in attack. Or go paralysed. Or have other long term or permanent damage. My doctor knows that with all the things we have learned, I can get treated, not have to be admitted to hospital and then I can go back to uni.”

If anyone has any additions to these annecdotes, or finds something different, please let us know. It may help someone else. The editor.