Hepatology · Acute Intermittent Porphyria

Treating and Preventing AIP Attacks

At a Glance

Acute Intermittent Porphyria (AIP) attacks are treated by stopping the liver's production of neurotoxic chemicals. Mild attacks may be managed with high-dose glucose, while severe attacks require IV hemin. Preventative treatments like Givosiran injections can help reduce frequent attacks.

Managing Acute Intermittent Porphyria (AIP) involves two distinct goals: stopping an active crisis as quickly as possible and preventing future attacks from happening. Because AIP is a metabolic disorder, treatment focuses on “calming” the liver and stopping the production of neurotoxic chemicals.

Stopping an Acute Attack

When an attack occurs, the goal is to quickly lower the levels of ALA and PBG in your system. The medical protocol typically follows these steps:

Removing Triggers: The first step is to stop any medications, supplements, or dietary habits that might be driving the attack ^[1]^[2].
High-Dose Glucose: For mild attacks, doctors may use intravenous (IV) glucose or a high-carbohydrate diet ^[3]^[4]. Glucose works by signaling the liver to slow down the production of the enzyme ALAS1, which is responsible for making the toxic precursors ^[5].
IV Hemin (Panhematin or Heme Arginate): This is the most potent treatment for moderate to severe attacks ^[6]. Hemin is a form of the “finished product” (heme) that your liver is struggling to make. When you receive hemin through an IV, your liver senses that it has enough heme and “shuts off” the production line ^[5]^[7]. This rapidly lowers the levels of toxic ALA and PBG, often leading to symptom relief within a few days ^[6]^[3].

Long-Term Prevention: Givosiran (Givlaari)

For patients who experience frequent, recurrent attacks—often defined as four or more attacks per year—preventative treatment may be necessary ^[8].

Givosiran is an FDA-approved RNA interference (RNAi) therapy ^[9]. Instead of replacing a missing product like hemin does, Givosiran acts like a “dimmer switch” for your genes. It targets and “silences” the messenger RNA for the ALAS1 enzyme in the liver ^[10]^[11]. By keeping ALAS1 levels low, the body never produces enough ALA and PBG to reach the toxic threshold, significantly reducing the frequency of attacks ^[12]^[13].

While effective, Givosiran requires monthly injections and regular monitoring of your liver function and kidney function. Some patients may experience side effects like injection site reactions, changes in kidney markers, elevated homocysteine levels, or liver toxicity (elevated transaminases) ^[14]^[15]^[16]. Routine blood tests are essential to ensure your safety ^[16].

Options for Severe Cases: Transplants

In very rare and severe cases where medical therapies are not enough to manage the disease, surgery may be considered:

Liver Transplant: Because the metabolic defect is primarily located in the liver, a liver transplant can be a curative option ^[17]^[18]. It replaces the “broken” production line with a healthy one. This is typically reserved for patients with life-threatening attacks that cannot be controlled by other means ^[5].
Kidney Transplant: Some patients with long-term AIP develop chronic kidney disease ^[19]. If the kidneys fail (end-stage renal disease), a kidney transplant (sometimes combined with a liver transplant) may be necessary to restore health and prevent further neurological damage ^[20].

The Mechanism of Treatment: A Summary

Treatment	Action	Goal
Glucose	Mildly suppresses ALAS1	Manage mild/early attacks
IV Hemin	Provides end-product (heme) to stop production	Stop moderate to severe acute attacks
Givosiran	“Silences” the ALAS1 gene message	Prevent recurrent attacks from starting
Liver Transplant	Replaces the defective enzyme factory	Cure severe, refractory cases

Common questions in this guide

How are mild AIP attacks treated?

Doctors may use a high-carbohydrate diet or intravenous (IV) glucose for mild attacks. Glucose signals the liver to slow down the production of the enzyme responsible for making the toxic chemicals that cause symptoms.

What is IV hemin and how does it stop a severe porphyria attack?

IV hemin, also known as Panhematin, is a potent treatment for moderate to severe attacks. It provides the liver with the finished heme product it needs, which signals the body to shut off the production of toxic chemicals and rapidly relieves symptoms.

Am I a candidate for preventative treatment with Givosiran?

Givosiran (Givlaari) is typically prescribed for patients who experience frequent, recurrent attacks, often defined as four or more per year. Your doctor can help determine if this monthly injection is the right preventative approach for your specific situation.

What side effects can occur with Givosiran?

Patients taking Givosiran may experience injection site reactions, elevated homocysteine levels, or changes in kidney and liver function markers. Routine blood tests are required to monitor your liver and kidney health while on this medication.

When would a liver transplant be considered for AIP?

A liver transplant is a curative option reserved for very rare, severe cases where attacks are life-threatening and cannot be managed with standard medical therapies. It works by replacing the liver's defective enzyme production line with a healthy one.

Curated prompts to bring to your next appointment.

1.Is intravenous hemin (Panhematin) available at this hospital’s emergency department, and is there a specific protocol for its use?
2.Given the frequency of my symptoms, am I a candidate for prophylactic (preventative) treatment with Givosiran?
3.What are the baseline tests for liver and kidney function we need before I start any long-term treatment?
4.How do we decide if an attack is severe enough to warrant hemin versus just managing it with glucose and fluids?
5.Can we create a written emergency treatment plan that I can give to doctors if I am traveling or at a different hospital?
6.What is the plan for monitoring my liver and kidney health over the long term, especially if I start Givosiran?

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This page explains treatment and prevention options for AIP attacks for educational purposes. Always consult your healthcare provider or emergency medical team for a personalized treatment plan.

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