Learning About AHP
This article is for people who are living with acute hepatic porphyria (AHP) and their care partners, or anyone who wants to learn more about AHP. The goal of this activity is to help you talk to and work with your doctor and healthcare team about ways to help manage AHP.
You will learn about:
What AHP is and types of AHP
Symptoms, attacks, and complications (additional problems) that may happen
What causes AHP and symptom attacks
Ways to help manage AHP
Making a treatment plan with your doctor and healthcare team and questions you can ask
Test Your Knowledge
What Is Acute Hepatic Porphyria (AHP)?
Acute hepatic porphyria, or AHP, is a group of rare conditions where you don't make heme properly.
Heme is a molecule that contains iron and is normally found in your body and plays a part in many important functions. This includes combining with globin proteins to form hemoglobin in your red blood cells, which carry oxygen to tissues throughout your body.
One of the places where heme is made is in your liver. During this process, other substances or byproducts -- called porphyrins or porphyrin precursors -- are also made. With porphyria, you're missing an enzyme that's needed to properly metabolize and use these byproducts.
Types of AHP
There are 4 types of AHP:
Acute intermittent porphyria (AIP)
Variegate porphyria (VP)
Hereditary coproporphyria (HCP)
ALAD-deficiency porphyria (ADP)
With each type, a different enzyme is missing that's needed to metabolize and use porphyrins or porphyrin precursors. As a result, they can build up and become toxic.
With AHP, these byproducts build up in your liver first. They can then pass into your bloodstream and cause problems in your nervous system and other body systems.
What Causes AHP
AHP is caused by a gene mutation that's inherited (passed on from a biological parent). Genes inside your cells carry information that determines your traits. Changes in genes are called mutations.
But just having the gene mutation isn't usually enough to trigger the start of symptoms, so many people won't develop any. Symptoms, when they do happen, are usually triggered for the first time by hormone changes. Triggers can also include certain medicines, alcohol use, smoking, a diet that's very low in calories, poor nutrition, stress, and certain illnesses such as infections.
What Symptoms Can Look and Feel Like
Depending on the type of AHP, symptoms can include:
Stomach pain, nausea, vomiting, and constipation or diarrhea
Trouble peeing and urine that's dark or reddish
Burning, stabbing, or shooting pain
Numbness, tingling, or pins and needles in your arms, hands, legs, and feet
Chest or back pain
Muscle aches or weakness
Paralysis (unable to move), trouble breathing
Seizures
High blood pressure, fast heartbeat
Feeling irritated, agitated, or restless
Mood changes, anxiety, depression
Confusion, hallucinations
Sleep problems
Fatigue (tiredness)
Skin changes and reactions to sunlight
Symptom Attacks
AHP can affect different people differently. Some may never have symptoms, while other people can have symptoms as attacks that may happen suddenly. Most people who develop symptoms are girls and women (assigned female at birth) between ages 14 and 45.
Some people may also have only 1 or a few symptom attacks during their lifetime. While others can have frequent attacks and chronic (long-term) symptoms.
During an attack, symptoms may last a few days and can get worse before getting better. Symptoms can even last weeks or months, especially if not managed properly. Attacks can be bad enough that you need to go to the hospital.
AHP Complications
Getting proper care and treatment for AHP is important. Attacks and the buildup of porphyrins or porphyrin precursors can cause long-term damage and complications such as:
High blood pressure
Chronic kidney disease (CKD)
Cirrhosis (scarring of the liver)
Liver cancer
The goals of treatment will be to help prevent attacks or stop any as quickly as possible, relieve symptoms, prevent complications, and help improve your quality of life. AHP can be life-threatening, especially if left untreated.
Ways to Help Manage AHP and Its Symptoms
You and your doctor can make a treatment plan together to help prevent and manage attacks and symptoms. Treatment will depend on the type of porphyria, your symptoms, other conditions you have, and your overall health.
As part of your plan, they may recommend:
Identifying and avoiding triggers, when possible, to help prevent attacks. Not everyone will have the same triggers, and yours may even change over time
Lifestyle changes such as not smoking, limiting alcohol, eating a well-balanced diet, and maintaining a healthy weight
Medicines
Glucose, a type of sugar that helps lower porphyrin levels
Surgery, such as a liver transplant (for very severe cases)
Your Healthcare Team
In addition to your family or primary care doctor, you may see different specialists as part of your healthcare team, such as:
Gastroenterologist (digestive system)
Hepatologist (liver)
Hematologist (blood)
Gynecologist or ob-gyn (women's health)
Emergency room team members, in case of an attack
Be sure to tell your team about any symptoms you have and how they affect your daily life. Tracking and recording your symptoms and any triggers and attacks in a journal or diary can help.
Questions to Ask Your Doctor and Healthcare Team
Questions you can ask about AHP can include:
How can we help prevent attacks?
Are there any triggers I should avoid and lifestyle changes I should make?
What treatments are available, and what are their possible side effects?
What should I do if I have an attack?
What should I do if I feel stressed or depressed?
Is there a support group I can join?
Where can I find more information and resources?
Test Your Knowledge Results
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