Are you developing painful sores on your skin? It could be Calciphylaxis!

Have you ever heard of a disease called Calciphylaxis? The name may sound a little strange. But it is a very rare, very painful, and sometimes life-threatening condition. What happens is that calcium deposits build up inside the small blood vessels in your body, blocking them. When that happens, it stops blood flow and your skin and the tissue underneath it start to die. It's like a plant withering away when it doesn't get enough water.

What is Calciphylaxis? Let's understand it simply!

Simply put, Calciphylaxis is a condition in which calcium deposits build up in your blood vessels, blocking blood flow and causing the skin and underlying tissue to die. Just like a water pipe gets clogged with dirt and water stops flowing, calcium deposits build up in the tiny blood vessels in our bodies.

Most often, these calcium deposits occur in the blood vessels that supply blood to the skin and the tissues beneath the skin. However, very rarely, these calcium deposits can also occur in organs inside the body, such as the eyes, lungs, brain, muscles, and intestines.

When this condition affects the skin, it can be incredibly painful . The affected area can also open up and form a sore, similar to a bed sore. As the skin and tissue around the sore die, the damage can spread. Calciphylaxis sores take a long time to heal, and can lead to serious complications.

If this wound becomes infected, the infection spreads throughout the body and our immune system starts to overreact. We call this sepsis . This is the most dangerous and fatal complication of Calciphylaxis.

Are there types of Calciphylaxis?

Yes, there are two main types of calciphylaxis, depending on how it develops.

1. Uremic calciphylaxis

This is the most common type . It often occurs in people who are on dialysis due to end-stage kidney disease (ESKD). This means that their kidneys are not working properly and they need to be connected to a dialysis machine to remove waste products from their blood.

2. Non-uremic calciphylaxis

This type is very rare . It can occur in people who are in the early stages of kidney disease, in people who have recently had damage to one or both kidneys, or (very rarely) in people who have no kidney problems at all.

How common is this condition?

Calciphylaxis is a very rare condition . It affects about 35 out of 10,000 people on dialysis (about one-third of 1%). For example, in the United States, there are more than 1.5 million people on dialysis, so there are an average of about 2,000 cases of calciphylaxis reported each year. The condition is even rarer in countries with a low incidence of kidney disease.

But, surprisingly, women are at a higher risk of developing this disease . Women are twice as likely to develop it as men.

What are the symptoms of this?

Symptoms of Calciphylaxis include:

• Severe pain: This is often a very painful condition. The pain may begin before there is any visible sign of injury or other damage. The affected area may become sensitive to pressure or touch, making the pain worse.
• Skin changes (early stages): These are areas of the skin that turn red, purple, brown, or black. These changes usually look like a mesh. There may be blisters, and the affected skin may become thickened and leathery.
• Sores and ulcers (late stage): Skin changes usually turn into sores, spreading in a star-shaped pattern. These sores often have a foul odor. The dead skin and flesh may turn black, crust over, and peel off.
• Vision problems, internal bleeding, and muscle damage: Very rarely, calcium deposits can occur not only under the skin, but also inside the body, causing other problems. If calcium deposits are in the eyes, vision may be impaired. If they are in internal organs, they can bleed and damage muscles.
• Calcium deposits in major blood vessels and the heart: A common complication of end-stage kidney disease is calcium deposits in major blood vessels and heart valves. The risk of this occurring increases when calciphylaxis develops.

Two main types of Calciphylaxis lesions occur:

When injuries occur, they often occur in one of two ways:

• Central distribution: These lesions are most often found in the abdomen and thighs. They are more common in people with a higher Body Mass Index (BMI) and in women. About 70% of patients with uremic calciphylaxis have these central distribution lesions. In non-uremic cases, the incidence is about 50%. In cases where there are more of these central lesions, the risk of death is higher.
• Peripheral distribution: These are lesions that occur in the extremities of the hands and feet, especially the fingertips. When peripheral lesions are severe, a doctor may have to amputate a finger, toe, or part of the limb to stop the damage from spreading and causing life-threatening complications.

Why does calciphylaxis occur? What are the causes?

The exact cause of Calciphylaxis is not yet known , but research suggests that it is caused by more than one factor. These factors come together to cause Calciphylaxis.

These factors may include:

• Too much parathyroid hormone: When you have kidney problems, your parathyroid glands try to keep your blood calcium levels stable. When they do this, they produce more of a hormone that your body needs. This is called hyperparathyroidism . This can also happen when your body doesn't get enough vitamin D or calcium.
• Kidney failure: Kidneys that are not working properly disrupt the chemical balance that controls calcium and phosphorus in the body.
• Too much phosphorus: Our bodies use phosphorus for various chemical processes, and the kidneys help control phosphorus levels.
• Certain types of cancer: This is most commonly seen in cases of calciphylaxis, which is unrelated to kidney disease.
• Trauma: This can be caused by an injury, a medical procedure such as surgery, or an intravenous (IV) line.

Who is most at risk for this? (Risk Factors)

There are several risk factors and medical conditions that may be associated with Calciphylaxis. They are:

• Kidney problems: The vast majority of people who develop calciphylaxis are in the final stages of kidney failure, as well as those on dialysis.
• Obesity: This condition increases the risk of developing calciphylaxis.
• Diabetes: When you inject insulin every day, the skin at the injection site can become damaged.
• Autoimmune diseases: For example, diseases like Lupus, Ulcerative Colitis, and Rheumatoid Arthritis.
• Liver disease: Alcohol-induced hepatitis, a condition caused by alcohol abuse, can also be a risk factor.
• Warfarin: This blood-thinning medication prevents the body from using vitamin K. Vitamin K is essential for blood clotting. Vitamin K also activates a protein that controls calcium deposits. Many people with end-stage kidney disease do not have enough vitamin K. This makes calcium deposits easier to form.
• Corticosteroids: These medications are given to reduce swelling and inflammation. People who develop calciphylaxis often have recently taken corticosteroids.

What are the complications of Calciphylaxis?

Calciphylaxis can cause complications such as:

• Insomnia
• Loss of appetite
• Depression
• Severe ulcers
• Infections
• Amputation
• Sepsis

How do doctors diagnose this?

A doctor may suspect calciphylaxis after reviewing your condition, symptoms, and a physical exam. This physical exam may include looking at your skin or the areas under your skin and feeling for any changes. They will also ask about your medical history and try to diagnose calciphylaxis. Once a doctor suspects this, they will order medical tests to find out more.

What tests are being done?

A doctor may order these tests:

• Skin biopsy:

The most reliable way to diagnose calciphylaxis is to take a small sample (usually about 4 to 5 millimeters deep) of the skin and the tissue underneath it. A doctor will usually take a sample from the edge of a wound or skin lesion. This sample is examined under a microscope, and certain substances are used to cause color changes or chemical changes to help confirm the diagnosis.

If you already have end-stage kidney disease and have severe lesions with a black crust, a biopsy may not be necessary.

• Lab testing:

Blood and urine tests help diagnose diseases, assess the severity of the disease, and determine the best treatment. The tests that doctors use most often are:

• Kidney function tests: These tests analyze urine to see how well your kidneys are working.
• Mineral and bone parameters: Analysis of calcium, phosphorus, parathyroid hormone, and vitamin D can determine the severity of the disease.
• Liver function tests: When you can't take medications that are filtered by your kidneys, the next option is to take medications that are filtered by your liver. Liver function tests help doctors determine if it's safe to give you medications that depend on the liver.
• Immune system markers: This test looks for signs that your body is fighting an infection. It can also help detect autoimmune diseases.
• Blood coagulation markers: Analyzing how well your blood clots (or does not clot) is useful, as it can reveal the possibility of internal bleeding or other problems.

• Imaging tests:

A doctor may order imaging tests, such as X-rays or bone density scans, but these are not common. Imaging tests are most likely to be done when other tests don't provide answers that can help with the diagnosis.

How is this treated? (Treatment)

Because researchers still don't fully understand calciphylaxis, there is limited guidance on the best treatment. Currently, there is no cure for this condition, but if treatment is successful, your symptoms may go away.

Typically, doctors focus on these things:

Wound care

The main things are to prevent infection and help the wound heal. This may include several advanced treatments, for example:

• Hyperbaric oxygen therapy: This involves placing you in an airtight chamber that contains 100% oxygen (instead of the normal oxygen concentration of about 20%). The pressure in the chamber is also slightly higher than normal. This is a very effective way to promote wound healing.
• Debridement: This is the removal of dead or dying tissue from a wound. This helps prevent infection and encourages the wound to heal. In severe cases, this may require surgery.
• Skin and tissue grafting: In cases where a lot of tissue has been lost, grafts taken from elsewhere in your body can help repair and heal the wound.
• Antibiotics: These medications help stop bacterial infections.

Pain control

Calciphylaxis is an extremely painful condition . It can make it difficult for you to rest and let your body heal. Doctors usually prescribe opioid painkillers for Calciphylaxis.

Sodium thiosulfate

This medicine is given to you through a vein (IV). It can help remove calcium deposits from your blood vessels or other tissues.

Treatment of bone and mineral problems

Keeping your blood minerals in the right levels and balance is very important to prevent complications and problems. This includes keeping your parathyroid hormone levels from going too low or too high. In some cases, surgery may be needed to remove one or more of your parathyroid glands.

Dialysis

This is a treatment that removes waste products from the blood when your kidneys are not working properly.

Nutritional management

It is very important to prevent malnutrition or nutrient deficiencies that can occur with kidney problems and related conditions.

Medication management

It is important to review all medications you take to avoid potential side effects and problems. This is especially important if you are taking warfarin or other medications that increase the risk of calciphylaxis.

Are there any side effects of the treatment?

Your doctor can best explain the possible complications and side effects. Because calciphylaxis is a poorly understood condition, complications and side effects can be unpredictable and vary greatly from person to person.

Some of the side effects and complications that may occur are:

• Infections: This refers to infections that can occur associated with medical procedures, dialysis, or surgery, not infections from wounds caused by calciphylaxis.
• Side effects of sodium thiosulfate: These include nausea, vomiting, fluid balance problems, low blood pressure, and blood acidity problems. Doctors should be aware of these problems, especially in people with heart disease. In many cases, these can be managed by changing the dose of this medication or by treating the side effects.

Can't this be prevented?

Because researchers don't fully understand the causes of calciphylaxis, it's difficult to prevent the condition. However, doctors often recommend minimizing risk factors, such as keeping calcium and phosphate levels normal.

What happens if I have Calciphylaxis? What is the future?

Unfortunately, due to a lack of understanding of the disease, the outlook for Calciphylaxis is generally negative. Many people with Calciphylaxis lose the ability to walk and spend most of their time in a wheelchair or bed.

About half of people with calciphylaxis do not survive more than a year. Sepsis is the leading cause of death.

However, depending on the nature of the disease, the chance of surviving one year may increase or decrease. Here are the conditions and chances:

• Non-uremic calciphylaxis (not associated with end-stage renal disease): People who develop calciphylaxis without end-stage renal disease are less likely to die within a year. In cases of non-uremic calciphylaxis, the risk of death within a year is between 25% and 45%. In cases of uremic calciphylaxis, the risk of death is between 45% and 80%.
• Ulcers and sores: In the final stages of calciphylaxis, skin changes develop into ulcers and sores. The later the diagnosis, the worse the outlook. Only about 20% of people with ulcers or sores survive more than six months.
• Wound debridement (removal of dead tissue): There is evidence that this type of wound care increases survival. The one-year survival rate for those who underwent debridement was slightly over 61%, compared to about 27% for those who did not.

How long has this situation been going on?

Calciphylaxis is a chronic, lifelong condition that currently has no cure . However, in some cases, the disease can go into remission after treatment. Researchers do not know how long it can be maintained in remission.

How do I take care of myself?

It is very important to follow your doctor's instructions as closely as possible with calciphylaxis. You should be especially careful about wound care, keeping any wounds or sores clean and protected from infection. You should also talk to your doctor about the foods you eat, medications you take, and any new supplements or home remedies you are considering.

When should I see a doctor?

Talk to your doctor in these cases:

• If you think or know that you are at risk of developing Calciphylaxis, that's fine.
• If you already know you have this disease.
• If you notice any new painful spots or areas.
• If you notice a change in your skin color.
• If you notice any changes in your skin or under your skin.

Your doctor is the best source of information about your risk factors, what symptoms you should watch out for, and when to seek medical advice. They can identify calciphylaxis early, which can help with your treatment.

When should I go to the emergency room?

If you have Calciphylaxis, your doctor will guide you on when to call their office regarding wound care or when to seek urgent medical attention. The most important things to watch for are signs of infection or problems around a wound. These include:

• Swelling or redness around the wound is okay.
• If you feel heat when you touch the area around the wound.
• If there is fluid coming from a wound and/or a bad odor.
• If a black crust forms on the wound.

What questions should I ask the doctor?

Some questions you might consider asking your doctor are:

• What type of Calciphylaxis do I have?
• What is the best treatment for me?
• Can you connect me to resources that can help me during my illness?
• What is the outlook for someone in my situation?

Take-Home Message

Calciphylaxis can be difficult to understand, as there is limited research and information about it. Talking to your doctor can help you understand it better and know what to expect. Calciphylaxis can be a complex, difficult-to-treat condition, but there are treatment options that can help. If you are feeling stressed about it, talking to a counselor can help you manage your feelings. Remember, you are not alone, and it is important to get the right medical advice and support.

` Calciphylaxis, kidney disease, skin disease, blood vessels, calcium, dialysis, wounds