What is Calciphylaxis? Everything You Need to Know

The Truth About Calciphylaxis: What You Need to Know

Calciphylaxis is the name given to a rare condition of mineralization that affects different parts of the body including blood vessels, skin, bone, and muscle tissue. It happens due to high levels of calcium and phosphate depositing around the body’s veins, arteries, bones, muscle tissues, and organs causing damage and swelling inside them. As the disease progresses, patients experience extreme pain and often have difficulty breathing.

Quick takeaway: What all to consider when you get Calciphylaxis?

• You need to be able to recognize if you have the condition.
• If you do have calciphylaxis, then you need to make sure you treat it properly.
• Make sure you know what causes this condition.

The onset of calciphylaxis begins slowly over time. Patients who have severe kidney failure develop calciphylaxis as their kidneys become damaged because they cannot filter out excess calcium and phosphates in the bloodstream. In addition, people who take certain medications (e.g., steroids) are at higher risk of developing calciphylaxis. The average lifespan of someone diagnosed with calciphylaxis is 5 years. If left untreated, calciphylaxis may cause death within weeks. Fortunately, treatment options exist and survival rates improve over time if caught early enough. Because calciphylaxis can occur anywhere in the body, it is difficult to diagnose. Doctors use X-rays, CT scans, MRIs, and ultrasound tests to determine whether calciphylaxis is present. Once doctors identify the problem, they may recommend surgery or medications to treat the condition.

Affected patients have an increased risk of developing calcification of various organs including kidney stones, heart valve dysfunction, skin ulcers, leg amputations due to gangrene, necrosis, seizures, strokes, brain injuries, blindness, coma, and death. Calciphylaxis affects people who take diuretics (a class of medications that increase urine output) and those who suffer from chronic kidney disease. Other risk factors include diabetes mellitus, hyperparathyroidism, rheumatoid arthritis, use of anticoagulants such as warfarin, liver failure, high cholesterol levels, old age, and excessive exposure to sunlight.

Causes of calciphylaxis?

Several factors contribute to calciphylaxis. High levels of calcium and phosphate build up in the bloodstream when someone has severe kidney dysfunction. Certain medications can cause the buildup of calcium and phosphate in the bloodstream. Most commonly, calciphylaxis develops when excessive levels of parathyroid hormone, vitamin D, prednisone, epoetin alfa, and lithium accumulate in the body. Other causes include chronic inflammation, nutritional deficiencies, diabetes mellitus, hyperparathyroidism, and sarcoidosis.

Who gets calciphylaxis?

A Calciphylaxis occurs when two conditions happen together. First, the body accumulates high levels of calcium, and second, the kidneys fail to eliminate calcium effectively. Both the high levels of calcium and the low kidney function are necessary to trigger calciphylaxis. It is estimated that less than 1 percent of people with end-stage kidney disease go on to develop calciphylaxis. However, it could affect people taking bisphosphonates for osteoporosis.

In 2011, researchers calculated that the incidence rate of calciphylaxis was 0.8% among individuals taking bisphosphonate medication. That means about 4,000 Americans were diagnosed with calciphylaxis while taking bisphosphonate medicine. The study showed that women had a greater chance of calciphylaxis than men. Women make up only 25% of patients receiving bisphosphonate therapy. Researchers believe that estrogen stimulates the kidneys to excrete calcium more efficiently since women produce more estrogen than men. Menopause is also associated with increased risks of calciphylaxis.

While it is not considered cancer, it shares some characteristics with malignant soft tissue tumors. Tissues surrounding calcifications show a combination of necrosis and fibroblast proliferation as seen in cancers.

Symptoms 

Patients suffering from calciphylaxis suffer from symptoms that range from being extremely painful to having trouble swallowing. These symptoms vary depending on where in the body the calcification occurs. Painful ulcers often appear on the patient’s skin, extremities and legs, ankles, and feet. When these lesions break open, pus-filled blisters form as well as an oozing sore. The most serious symptom is constriction of the affected area. Muscle cramps and stiffness set in along with shortness of breath. Here are some of the symptoms associated with calciphylaxis:

• Painful swelling of affected extremities
• Swelling of hands and feet
• Hardening of skin (in advanced cases)
• Ulcers
• Blood clots
• Nausea and vomiting
• Weakness
• Headaches
• Muscle cramps
• Severe itching
• Difficulty breathing
• Fatigue

If you experience any of these symptoms, it is important to seek medical attention immediately. Fortunately, there are many treatments available that can help reduce the risk of developing calciphylaxis.

What are the treatment options for calciphylaxis?

Treatment for calciphylaxis usually involves removing the focal point. A doctor removes the underlying cause and treats the patient accordingly. It is sometimes necessary to remove calcium deposits surgically or to replace them with artificial materials. But for many patients, doctors perform dialysis treatments three times per week until the calcium remains under control. Dialysis helps remove toxins and chemicals from the blood that would otherwise prevent the body from absorbing enough oxygen. To treat this condition, there are a few options available:

• Hydration: The first thing to do when treating this condition is to ensure adequate hydration. When the body lacks water, it cannot properly function. Without proper hydration, blood vessels become brittle and break easily, leading to severe pain and often death if left untreated. If the patient’s kidneys are already failing due to this, then they may have trouble filtering out toxins and minerals. Fluid intake and electrolyte balance are crucial in these cases.
• Diet: A low-calorie diet is recommended for people suffering from this condition. A high-protein diet helps build muscle mass while maintaining bone density. Protein intake should not exceed 1 gram per kilogram of body weight daily. High protein diets increase levels of IGF-1 (insulin growth factor), which is associated with an increased risk of cardiovascular disease. Patients with calciphylaxis should avoid foods containing saturated fats and cholesterol, which promote plaque buildup.
• Exercise: Exercise is extremely helpful in preventing this condition, especially when combined with dietary changes. Regular exercise increases circulation boosts immunity and decreases inflammation and insulin levels. An intense workout can also help flush excess calcium from the body. However, exercising excessively can lead to dehydration and overuse injuries, so patients should monitor their workouts carefully.

Prevention of calciphylaxis

It is possible to treat this disorder without removing the cause. People who are at risk of developing calciphylaxis should receive daily doses of calcium supplements. Doctors can also prescribe drugs called bisphosphonate compounds to reduce the amount of calcium that builds up. Bisphosphonates work by preventing bones from breaking down and reabsorbing calcium deposits. Physicians also recommend treating people who have hypoparathyroidism or severe renal disease.