How to Prepare for an Acute Hepatic Porphyria Emergency

Why are acute hepatic porphyria attacks dangerous?

If you live with AHP, acute episodes are dangerous because they cause a fast, extreme buildup of the neurotoxins porphobilinogen (PGB) and aminolevulinic acid (ALA).

This can result in a medical emergency, and you may need urgent treatment for symptoms such as severe pain, seizures, respiratory failure, and paralysis.

Early signs and symptoms of an acute AHP attack

The early signs of an acute AHP episode can vary from one person to the next, but severe abdominal pain is the most common. You may also notice a change in the color of your urine to a purple-red.

Other symptoms can include:

• generally feeling weak or fatigued
• muscle weakness
• nausea and vomiting
• limb pain
• constipation
• irregular or rapid heartbeat
• feeling confused
• fluctuating blood pressure
• urinary retention
• psychosis
• hallucinations
• seizures

If you experience muscle weakness, note that it can progress to respiratory paralysis, in which case you may need help breathing.

What to do at the first sign of an AHP attack

Your healthcare team will help you put a plan together for when you experience an acute AHP episode.

At the first sign of an episode, which is often increased abdominal pain, contact your specialist and visit the emergency room as soon as possible.

You will usually need a hemin (Panhematin) infusion as quickly as possible, and it may help your doctor if you can identify what triggered the episode.

Triggers to avoid

While triggers can vary from one person to another, common factors that can cause an attack include:

• Emotional or physical stress: Increased emotional stress, such as exhaustion, and physical stress, such as an infection or response to surgery, may be an AHP trigger.
• Medications: Several medications can cause an acute AHP episode, including certain hormones (estrogens and progesterone), antiseizure drugs (such as valproic acid and phenytoin), and specific antibiotics or anti-inflammatories. Always consult a doctor before starting a new prescription, and you can check the safety of any medication using the American Porphyria Foundation’s (APF) drug database.
• Dietary changes: Avoiding carbohydrates or following a low-carbohydrate diet can sometimes trigger an acute AHP episode. You can create a healthy and balanced diet with the help of a dietitian.
• Hormonal fluctuations: This may be particularly common in females during their menstrual cycle. Some hormonal contraceptives may help, but caution should be taken regarding which specific medications you take, as some can also be triggers.
• Alcohol, smoking, and recreational drugs: Drinking excessive amounts of alcohol in a short period, often referred to as bingeing, can trigger AHP. The general advice is not to drink at all, but if you do, males should not drink more than two alcoholic beverages per day, and females should not drink more than one alcoholic beverage per day. Smoking either tobacco or cannabis should be avoided.

How to prepare for an emergency room visit

You’ll usually have a plan in place that has already been discussed with your healthcare team. This should include your doctor’s contact details, the nearest emergency room, and a list of your current medications.

Wear comfortable clothing, and have a bag ready to go that includes a change of clothes, personal care items, and a mobile device charger.

Preparing a personal emergency plan

Work with your healthcare team to develop a personal emergency plan. Keep this information in a portable kit or folder that includes:

• a letter from your doctor, confirming your diagnosis and treatment plan
• contact details for your doctor and other key members of your healthcare team
• a list of your current medications and information on the importance of drug reactions and AHP
• a list of your known AHP triggers
• contact details for close friends or family members

Don’t worry about gathering too much information. Having everything in one place when you visit the ER may mean a quicker and more efficient treatment experience.

How to advocate for yourself or a loved one in the emergency room

Use these helpful strategies to advocate for yourself or a loved one during an ER visit for an acute AHP episode.

• If possible, arrange for someone to come with you to the hospital. It would help if they were familiar with your condition, so they can discuss it with medical staff if you can’t.
• Let the ER staff know about your diagnosis. You may already have a letter from your doctor for this very reason, including details of a general treatment plan.
• Know that the ER team will still need to do some routine tests. However, feel confident requesting a random (spot) urine PBG test, as it may not be performed automatically.
• Ensure the ER team is aware of the medications you should not take with AHP. You can point them toward the APF’s drug database if needed.
• Let the ER team know that hemin (Panhematin) is the expected treatment for acute AHP episodes. They can administer glucose while you wait for the hemin (Panhematin).
• You should receive treatment for your symptoms. These may include medications for nausea, vomiting, and pain.

Takeaway

If you have AHP, it’s crucial to be as prepared as possible for a trip to the emergency room.

Consider asking someone to accompany you to the ER to advocate for you if you’re unable to do so yourself.

While knowing your triggers can help you avoid acute episodes, having an emergency bag and a personal plan ready can ensure faster and more efficient treatment if an attack occurs.