Calciphylaxis (kal-sih-fuh-LAK-sis) is a rare, serious disease. It involves a buildup of calcium in small blood vessels of fat tissues and skin.
Calciphylaxis symptoms include blood clots, lumps under the skin and painful open sores called ulcers. If an ulcer becomes infected, it can be life-threatening.
The exact cause of calciphylaxis isn't clear. But people with the disease usually have kidney failure. That's a condition in which the kidneys no longer work as they should. Often, these same people also have received kidney failure treatments such as dialysis or a kidney transplant. Calciphylaxis can happen in people without kidney disease too.
Calciphylaxis treatments include various medicines, procedures and surgery. Treatment can help prevent blood clots and infections, reduce calcium buildups, heal sores, and ease pain.
Calciphylaxis symptoms include:
The exact cause of calciphylaxis isn't known. The disease involves the buildup of calcium in the smallest parts of the arteries in fat tissues and skin.
Many people who get calciphylaxis also have kidney failure or receive dialysis. It's not known why people with kidney failure or people receiving dialysis are at higher risk of calciphylaxis.
For some people, the calcium buildup in calciphylaxis is linked with small organs in the neck called parathyroid glands. If the glands release too many parathyroid hormones, that can cause calcium to collect. But the link isn't clear. Most people with seriously overactive parathyroid glands don't get calciphylaxis. And many people with kidney failure and calciphylaxis don't have overactive parathyroid glands.
Other factors that seem to play roles in calciphylaxis include:
Calciphylaxis most often affects people who have kidney failure. Other risk factors include:
Complications of calciphylaxis include:
Often, the outlook for people with calciphylaxis isn't hopeful. Finding and treating any infections early is key to preventing serious complications.
There isn't a clear way to prevent calciphylaxis. But if you are on dialysis or have low kidney function due to advanced chronic kidney disease, it's important to keep blood levels of calcium and phosphorus under control.
Keeping blood levels of phosphorus under control often is a challenge. Your healthcare professional might have you take medicines with meals. You also may need to restrict certain foods that are high in phosphorus. It's very important to follow your healthcare professional's directions and go to all follow-up healthcare checkups.
If you have calciphylaxis, your healthcare team helps you prevent ulcer infections or other complications. You may need to apply special wound dressings or clean the ulcers daily to prevent germs called bacteria from growing.
Diagnosis involves finding out if calciphylaxis is the cause of your symptoms. Your healthcare professional reviews your health history, asks about your symptoms and gives you a physical exam.
You also may need tests such as:
Often, more than one treatment is needed for calciphylaxis. Treatments can restore oxygen and blood flow to the skin and reduce calcium buildup.
Medicine that helps keep blood from clotting can restore blood flow to affected tissues. These blood-thinning medicines are called anticoagulants. If these don't help, an experimental treatment may be available that can dissolve blood clots in the tiny blood vessels of the skin. This treatment is a type of medicine called a low-dose tissue plasminogen activator.
Another backup treatment option involves breathing pure oxygen in a pressurized chamber. This is called hyperbaric oxygen therapy. It's not available everywhere. A small amount of research suggests that this therapy may help control infections and limit tissue loss while the main treatments take effect.
Wound care is a crucial part of calciphylaxis treatment. So it can be very helpful to have a team of wound care specialists.
Reducing calcium buildup in arteries may be helped by:
For sores to heal, some of the tissue damaged by calciphylaxis may need to be removed with surgery. This is called debridement. Sometimes, tissue can be removed using other methods, such as wet dressings. Medicines called antibiotics can clear up infections caused by germs. Antibiotics can help treat and prevent ulcer infections.
You'll likely be offered medicines to manage pain due to calciphylaxis or during wound care. A pain medicine specialist may need to be involved if you're prescribed opioid pain medicines.
You may start by seeing your healthcare professional. Or you may be referred to a skin doctor called a dermatologist or a kidney doctor called a nephrologist.
Some other healthcare professionals who may become part of your healthcare team include:
Here's some information to help you get ready for your appointment.
When you make the appointment, ask if there's anything you need to do in advance. For example, you may be told not to eat for a certain number of hours before some tests. This is called fasting. It's also a good idea to bring a family member or friend with you. A person you trust can help you remember all of the information from your appointment.
It's also helpful to make a list of:
For calciphylaxis, some basic questions to ask your healthcare team include:
Your healthcare professional is likely to ask you questions such as: