Have you ever had unexplained severe stomach pain, nausea, and dark urine? Sometimes the cause of this can be a rare condition that none of us have heard of. Since it is so rare, many people don't know about it. Today we are talking about one such disease, Acute Hepatic Porphyria (AHP). Don't worry, we will talk about this simply, in a way that everyone can understand.
Simply put, what is Acute Hepatic Porphyria (AHP)?
Acute Hepatic Porphyria, or AHP for short, is a condition caused by the excessive accumulation of porphyrins, a type of chemical produced in our body, in the liver and other parts of the body. This is not really a single disease, but a group of diseases. When porphyrins accumulate in this way, they can affect the nervous system and cause a number of other uncomfortable symptoms.
Although there is no cure yet for this disease, proper treatment can help control the symptoms and help you live a better life.
Why does this disease occur? Let's take a closer look.
To understand this properly, we first need to know about something very important in our bodies. That is called ' Heme ' .
Heme is an important part of the protein hemoglobin in our red blood cells. As you know, hemoglobin is what picks up oxygen from the lungs and distributes it throughout the body. So this heme is essential for the functioning of places like the liver, muscles, and bone marrow.
Now look, when our body makes this 'heme', 8 types of enzymes help to make that process happen properly. It's like 8 types of spices are needed to make a dish. If for some reason there is a deficiency or weakness in any of these 8 enzymes, the process of making 'heme' stops halfway. What happens then is that the 'porphyrins' that we talked about earlier, which are the ingredients used to make 'heme', start to accumulate in the body .
In AHP, this chemical called ' porphyrin ' accumulates mainly in the liver . The effects of this accumulation are what manifest as symptoms.
| Commonly seen symptoms | A little more about this |
|---|---|
| Severe stomach pain | This is the main and most severe symptom. You may experience an unexplained, severe stomach ache for no apparent reason. |
| Nausea and vomiting | This condition can occur with severe stomach pain. |
| Dark urine | Urine may become dark brown or reddish in color. |
| Skin sensitivity | In some types of porphyria, the skin becomes extremely sensitive to sunlight. |
| Nervous system problems | Severe conditions such as numbness, pain, muscle weakness, and seizures may occur. |
An "attack" of AHP usually begins with severe abdominal pain. This can last for days. In such cases , hospitalization is mandatory . This means going to the Emergency Department (ETU).
Who gets this disease? Who is at risk?
Often, this disease is hereditary . That is, you inherit the genes that cause this disease from your parents.
But the important thing here is that not everyone who has the gene that causes the disease will develop symptoms . The majority of people with the AHP gene can remain healthy throughout their lives without any symptoms.
This is like a sleeping lion. Even if the gene is present, if there is no trigger to wake it up, or "awaken" it, it can remain without any problems.
So what are the triggers for this disease?
This is a very important part. If you or someone you know has AHP, it's essential to be aware of these things.
| Trigger | Important points |
|---|---|
| Some medications | Some painkillers, sedatives, barbiturates, tranquilizers, and birth control pills can be major triggers. If you suspect or have been diagnosed with AHP, do not take any medication without consulting a doctor. |
| Drinking alcohol and smoking | These can easily trigger the disease because they put a lot of pressure on the liver. |
| Strict dieting or fasting | Restricting food intake or skipping meals in an attempt to lose weight suddenly is very harmful to these patients. Not getting the calories the body needs is a strong trigger. |
| Mental and physical stress | Things like severe stress, surgery, and infections can also trigger the disease. |
What should I do?
If you are experiencing any of the symptoms mentioned in this article, especially severe, unexplained stomach pain, on a regular basis , please do not assume that it is normal. See a qualified doctor immediately.
The doctor will examine you and, if necessary, order urine, blood, and genetic tests to determine whether you have the disease.
A balanced diet is very important for someone living with AHP. If necessary, your doctor may refer you to a nutritionist.
Take-Home Message
- Acute Hepatic Porphyria (AHP) is a rare, inherited disease caused by a buildup of a chemical called porphyrin in the liver.
- The primary cause of this is a deficiency in an enzyme required in the process of making 'heme'.
- The main symptom is unbearable abdominal pain. It can also cause nervous system problems.
- Even if you have the gene that causes the disease, if you avoid the triggers that cause the disease, you may not experience symptoms.
- Some medications, alcohol, smoking, strict dieting, and stress are major triggers.
- If you have any of these symptoms or suspect you have AHP, definitely talk to a doctor. Never go out and get medicine on your own.
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