Do you feel dizzy when you stand up? Could it be Pure Autonomic Failure (PAF)?

We all feel dizzy or lightheaded at some point when we get up from a chair or bed. Most of the time, it goes away in a few seconds. But if this happens to you often, or if you feel like you're about to faint and fall, it may not be normal. Today we're going to talk about a rare condition that causes these symptoms. That's Pure Autonomic Failure, or PAF for short.

Simply put, what is Pure Autonomic Failure (PAF)?

Think about it, there are many processes in our body that occur automatically without our control. For example, breathing, heartbeat, digestion, controlling body temperature, and controlling blood pressure. All of this is controlled by our Autonomic Nervous System. It's like the 'auto-pilot' system in our body.

Pure Autonomic Failure (PAF) is a rare neurological condition that gradually weakens the function of the autonomic nervous system over time. The main thing that happens in this case is that when you stand up suddenly from a sitting or lying position, your blood pressure drops significantly . In medical terms, we call this orthostatic hypotension . But this is not the only problem, PAF can cause many other problems.

This condition is usually seen most often in adults between the ages of 40 and 60. In the past, this disease was also called Bradbury-Eggleston syndrome.

What are the symptoms of PAF disease?

As we discussed earlier, the most common and main symptom is dizziness and fainting when standing up (orthostatic hypotension). However, since this autonomic nervous system controls many organs in our body, the symptoms are not limited to one.

Let's see what other symptoms can be caused by PAF.

In addition, about half of people with PAF may also experience mild anemia.

What is the reason for the formation of PAF?

The main cause of this has been identified as the abnormal accumulation of a protein called alpha-synuclein in our autonomic nerve cells. Like dirt clogging a pipe, when this protein accumulates in nerve cells, their function is impaired.

This protein is deposited in the nerve bundles (autonomic ganglia) on either side of the spinal cord or near the organs. Over time, the number of these nerve cells decreases, and the work of the autonomic system is disrupted. But the most important thing is that in PAF, neither the brain nor the spinal cord is damaged . Therefore, it does not affect things like memory and thinking ability.

Researchers still don't have a clear idea why this protein is deposited like this.

Relationship with other diseases

Here's something important to know. This same protein, alpha-synuclein, is what builds up in the brain in diseases like Parkinson's disease, Multiple System Atrophy, and Lewy Body Dementia. So, some people with PAF (not all) have a chance of developing one of these diseases over time. Your doctor will let you know about this.

How to diagnose this disease?

Diagnosing PAF is a bit of a complicated process, as doctors will first rule out other conditions that could be causing your symptoms, especially the drop in blood pressure when you stand up.

The diagnostic process may include:

• Asking questions carefully about your symptoms and medical history.
• A complete physical examination and neurological examination.

After that, several special tests may be recommended to confirm the disease.

It can be overwhelming to have so many tests, but only by accurately diagnosing the disease can we create the best treatment plan for you.

What are the treatments for PAF?

There is no cure for this yet. But don't worry. There are many things you can do to help manage your symptoms and live a normal life. Your doctor and medical team will work with you to develop a treatment plan that is specific to you.

Orthostatic Hypotension (low blood pressure when standing up) control

This is the main problem. There are both non-drug treatments and medications for this.

• Be careful when changing positions: When getting up from a lying or sitting position, do it very slowly . Do not get up suddenly.
• Water and salt: Your doctor may recommend drinking about 3 liters of water a day. He or she may also tell you to add a little more salt to your food.
• Bed preparation: Sleep with the head of the bed raised about 4-6 inches. That way, you won't feel much of a difference in blood pressure when you get up.
• Compression Garments: Wearing special tight stockings (compression garments) that come up to the waist or thighs helps reduce blood pooling in the legs and keep blood pressure stable.
• In addition to this, the doctor also recommends specific medications that increase blood pressure.

Problems with defecation and urination

• For constipation: You can do things like eat foods high in fiber (vegetables, fruits, bran rice) and use stool softeners .
• For urinary problems: If you have difficulty urinating, you may need to use a catheter. If you cannot control your urine, there are special medications (such as Oxybutynin, Mirabegron).

Sexual immorality

Although there are medications like sildenafil or tadalafil for this, these medications can increase the risk of orthostatic hypotension. Therefore, you should discuss this openly with your doctor and use these only under his or her advice.

What will happen in the future for someone with this disease?

It's difficult to give a single answer about the future of someone living with this disease, because it varies from person to person.

PAF is a progressive disease. But for many people, it happens very slowly, over years, even decades. Some studies have shown that the average life expectancy after a diagnosis of PAF is about 12.5 years. But this is just an average, and it can vary greatly depending on your symptoms and health. So don't get too hung up on those numbers.

If you have PAF, it's important to see your doctor regularly to make sure your treatment is working and how your condition is progressing. If you develop symptoms of Parkinson's disease (such as tremors, difficulty walking), you should tell your doctor right away.