Treatments for acute hepatic porphyria (AHP) vary based on your symptoms and overall health. Managing your condition is key to preventing complications.
However, it’s important to talk to your doctor if your symptoms are worsening or you’re having more attacks than usual.
Consider the following questions as a starting point when you have a conversation with your doctor about AHP treatment.
Despite a comprehensive management plan, an AHP attack is still possible.
Symptoms may occur whenever your body doesn’t have enough heme to make hemoglobin proteins in your red blood cells. The same proteins are found in your muscles and heart.
Ask your doctor if there are any symptoms to look out for that may signal an AHP attack. These might include:
- worsening pain
- abdominal pain
- difficulty breathing
- increased blood pressure and heart rate
Your doctor might recommend a hospital visit if you’re having an AHP attack. Mild symptoms might not warrant hospitalization as much as a severe attack.
You must go to the hospital if you have significant changes in blood pressure or heart rate, seizures, or you lose consciousness. Severe pain may be addressed at the hospital, too.
Once you’re at the hospital, you may be given treatments intravenously to quickly stop the attack. Your doctor may also monitor you for severe complications with your kidneys or liver.
If you’re unsure if you need to go to the hospital, call your doctor or ask them to provide an after-hours phone number you can call for advice.
Many of the emergency treatments available for AHP at the hospital are also available at your doctor’s office.
These are usually given in lower doses as part of a maintenance plan, rather than an emergency medical treatment.
Such treatments include:
- intravenous glucose: helps control glucose levels if you’re not getting enough to create red blood cells
- intravenous hemin: a synthetic form of heme administered a few times a month to prevent AHP attacks
- hemin injections: a form of heme administration recommended if your body is making too many porphyrins and not enough heme
- phlebotomy: a blood removal procedure that aims to remove excess iron in the body
- gonadotropin-releasing hormone agonist: a prescription medication used for females losing heme during their menstruation cycle
- gene therapies: this includes givosiran, which decreases the rate at which toxic byproducts are produced in the liver
A phlebotomy is only used in AHP if you have too much iron in your blood. Iron is important in the creation and maintenance of red blood cells, but high levels can trigger an AHP attack.
Phlebotomyreduces iron stores, which improves heme synthesis disturbed by ferro-mediated inhibition of uroporphyrinogen decarboxylase. Regular blood testing can help ensure your iron is at the right level.
If you do need a phlebotomy, it may be done on an outpatient basis. During the procedure, your doctor will remove some of your blood to get rid of excess iron.
If you have low glucose levels but don’t need a glucose IV, your doctor may recommend sugar pills.
Certain hormone agonists can also help women who are menstruating. During menstruation, you may be at risk for losing more heme.
Your doctor may prescribe leuprolide acetate, a type of gonadotropin-releasing hormone agonist. This will help prevent the further loss of heme during your menstrual cycles, which may prevent AHP attacks.
Gene therapies such as givosiran (Givlaari) may also be prescribed to decrease toxic liver byproducts. The
Foods, medications, and lifestyle choices can sometimes trigger AHP. Minimizing these triggers — or avoiding them — may help support your treatment plan and reduce the risk of an attack.
Tell your doctor about all the medications, supplements, and over-the-counter products you use.
Even an over-the-counter supplement could interfere with your condition. Some of the most common culprits are hormone replacements and iron supplements.
Smoking and drinking can make your AHP worse. No amount of smoking is healthy. But some adults with AHP may be able to drink in moderation. Ask your doctor if this is the case for you.
Try to stick with a healthy eating and workout plan. If you have AHP, dieting can deplete heme and worsen your symptoms.
If you need to lose weight, ask your doctor to help you create a weight loss plan that won’t worsen your symptoms.
Finally, create a stress relief plan and use it. No one’s life is stress free and having a complicated condition like AHP can create further stress. The more stressed you are, the greater the risk for attacks.
AHP is a rare and complex disorder. There’s still a lot to learn about it. It’s important to stay in touch with your doctor and tell them if you don’t think your treatment plan is working.
Talking with your doctor can help them gain insight into your situation and recommend effective treatment.