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 if 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.
How do I know if I’m having another attack?
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 your 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
Will I have to go to the hospital?
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, or if you have seizures or 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, contact your doctor or ask them to provide an after-hours phone number you can call for advice.
What treatments are available at your office?
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
Do I need a phlebotomy?
A phlebotomy is only used in AHP if you have too much iron in your blood. This mineral is important in the creation and maintenance of red blood cells, but high levels can trigger an attack of AHP. 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.
What prescription medications help with AHP?
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 menstruating women. 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.
Are there any lifestyle changes that will help?
AHP is sometimes triggered by foods, medications, and lifestyle choices. 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.
You should also stick with a healthy diet 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, make sure you have a stress relief plan in place. 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. And there’s still a lot to learn about it. So it’s important to keep in touch with your doctor 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.