Table 5 Quotes relating to defining the severity of acute attacks

“Severity is variable. So, [my wife] had multiple attacks in a single year and […] she’s been diagnosed with porphyria since 2008. So, we’ve had 100 of these things. I tend to group them into ones that have required hospitalisation and ones that haven’t required hospitalisation […] Ones that have required hospitalisation – most of the time […] were sort of […] what I would call, ‘the massive attacks’ or ‘the mega attacks’. [My wife] has had lots of ‘little’ attacks, that haven’t required hospitalisation, where she’s just had lots of pain and, sometimes with the pain she’d also get sickness.” (Caregiver C)

“For myself it would be amount of symptoms that I’m having at one given time. So, if I’m having abdominal pain, diarrhoea, migraine, pain in my limbs, shooting pains, kind of insomnia that would be […] quite an average one for me. If I’m then starting to get into psychiatric territory and migraines and collapsing, with all of those other symptoms […] you now have to go to the hospital, get someone involved.” (Patient D)

“If it is a very minor attack, which they kind of tend to be now, my doctors have told me “if you feel you can manage it at home, do so” – this sounds really bad, I know, but a lot of the people I know with porphyria don’t want to go to A&E every single time because it just feels like a battle with the staff at A&E. If it then becomes so bad that I cannot function, then yeah I need to go to hospital.” (Patient D)