Acute hepatic porphyria triggers
Acute hepatic porphyria (AHP) is a set of genetic diseases that directly affects certain liver functions. The condition is rare but can potentially lead to life threatening complications and other symptoms that can affect quality of life.
There are four types of AHP. Acute intermittent porphyria (AIP) is the most common type, affecting around 80% of people. Other types include variegate porphyria (VP), hereditary coproporphyria (HCP), and porphyria with delta-aminolevulinic acid dehydratase (ALAD) deficiency (ADP).
There are specific triggers that can lead to acute AHP episodes, and recognizing them is an important part of managing the condition.
The majority of porphyrias are due to a genetic alteration that is passed on from one or both parents.
If an individual carries or inherits an altered gene that causes porphyria, it does not automatically mean they will experience symptoms.
In fact, a small
In people with AHP, the liver’s heme biosynthesis pathway (production process) does not function as expected. This is due to a genetic mutation.
In the liver, an enzyme called delta-aminolevulinic acid synthase 1 (ALAS1) controls this process.
Specific triggers activate ALAS1, and when this happens, heme production is disrupted, leading to accumulation of the toxins delta-aminolevulinic acid (ALA) and porphobilinogen (PBG).
ALA and PBG are associated with acute AHP episodes as well as other AHP symptoms.
If a medication is known to have adverse effects on AHP, it is called a porphyrinogenic. The American Porphyria Foundation has a searchable database of porphyrinogenic medications.
In AHP, hormone fluctuations can trigger acute episodes. These cases are often referred to as cyclic attacks.
This is notably seen in the fluctuation of progesterone during a person’s menstrual cycle, with more than 90% of people with active AHP symptoms being female.
Estrogen and progesterone fluctuate most in the luteal phase of the menstrual cycle, and progesterone levels are at their highest during this phase.
A doctor may measure serum progesterone levels when AHP symptoms begin, as this could help identify the luteal phase of a person’s cycle and how this may relate to acute AHP episodes.
Preventive hemin infusions can help prevent AHP episodes, during which a person must visit a clinic or another healthcare facility for treatment. However, identifying a person’s luteal phase and having the infusion coincide with it may prove challenging.
Although there is no particular recommended diet for people with AHP, there are some things that may trigger acute episodes.
Maintaining a well-balanced diet and avoiding prolonged fasting are important ways to prevent acute episodes.
Eating a larger portion of carbohydrates, such as pasta, bread, and white rice, may benefit some people in the early stages of an acute AHP episode, but more research is needed to confirm these effects.
However, experts do not recommend eating a long-term high-carbohydrate diet, and they do not believe this prevents acute episodes. If weight loss were beneficial to overall health, it would be best to speak with a registered dietitian, who can consider all aspects of health when planning a diet change.
Another triggering factor of acute AHP episodes is consuming too much alcohol, specifically when people binge-drink.
Smoking and substance misuse can also trigger acute AHP episodes, specifically in those who use cannabis, ecstasy, amphetamines, and cocaine.
Seeking help for addiction may feel daunting or even scary, but several organizations can provide support.
If you believe that you or someone close to you is showing signs of addiction, you can contact the following organizations for immediate help and advice:
Experiencing stress, either emotional or physical, is a key trigger for AHP.
This may include daily stressors, physical or emotional exhaustion, a postsurgery response, or an infection, such as the flu or pneumonia.
Managing mental health and well-being may help prevent acute AHP episodes, along with receiving all appropriate vaccinations.
A healthcare professional can suggest the most appropriate care based on an individual’s circumstances.
Understanding and avoiding triggers can help a person manage their condition effectively. The following steps can help raise awareness:
AHP triggers vary for everyone, and recognizing individual triggers is one of the most important steps in avoiding acute episodes.
By taking the time to understand and discuss triggers with a healthcare team, a person can be confident in their symptom management and know the steps to take in an emergency.