Primary sclerosing cholangitis is a chronic and progressive disease that affects the bile ducts and liver by association. Your bile ducts carry digestive fluid, bile, from the small intestine to the liver. However, when you have primary sclerosing cholangitis, scarring forms in the bile ducts, resulting in clogs and the inability to move bile into the liver. This, in turn, makes it so that your liver does not function properly, resulting in eventual failure.
In its early stages, primary sclerosing cholangitis causes few symptoms and may even go unnoticed. However, the longer you leave the disease untreated and allow scarring and blockage of the bile ducts, the more noticeable your symptoms will be and the harder your condition becomes to treat. Therefore, it is important to have primary sclerosing cholangitis diagnosed and treated early to prevent liver failure.
Unfortunately, there is currently no cure for primary sclerosing cholangitis, and there is also no way to slow its progression. There are, however, treatments for the symptoms of the disease as well as treatments to open your bile ducts if they become too clogged. Treatments can also help prevent potential complications of the disease.
While medications cannot cure primary sclerosing cholangitis, they can treat the side effects and prevent potential complications.
One of the biggest risks to people who have primary sclerosing cholangitis is liver and bile duct infections. This happens because built-up bile can stagnate and become infected. To avoid this, you may need to take antibiotics, such as fluoroquinolones and cephalosporins.
To combat the terrible itching that often stems from primary sclerosing cholangitis, you may require bile acid sequestrants. Bile acid sequestrants bind to bile acids and keep them from causing itching.
Antihistamines, such as Acrivastine and Loratadine, are also useful at preventing itching, a common side effect of liver disease.
When antihistamines and bile acid sequestrants are ineffective, you may need to take opioid antagonists to combat itching. Opioid antagonists, such as naltrexone and rifampin, affect the part of your brain that causes itching, thereby reducing it.
Aside from itching, osteoporosis and similar bone diseases are common side effects of primary sclerosing cholangitis. To keep your bones strong and healthy, you may need supplements such as Vitamin D and Vitamin K.
While medications can help with itching and other side effects of primary sclerosing cholangitis, they cannot keep your bile ducts from scarring and clogging. To treat this complication, you may need an operation known as balloon dilation. A balloon dilation is where a deflated balloon is inserted into your bile ducts and then inflated to open up the clogged duct.
A stent placement is similar to balloon dilation in that it involves surgically opening the bile duct. Instead of using a balloon, however, a stent is a tube that sits inside the bile duct and keeps it open long-term.
If worse comes to worse and your primary sclerosing cholangitis progresses to late-stage liver disease, you will need a liver transplant. This is often the end effect of primary sclerosing cholangitis because of how the disease progresses. In most cases, a liver transplant also fixes the damaged bile ducts, which means that primary sclerosing cholangitis should not return. Therefore, while it is the most invasive treatment, a liver transplant is the best cure for primary sclerosing cholangitis.
While you can do everything in your power to treat symptoms and side effects of primary sclerosing cholangitis, there is no way to cure the condition aside from an eventual liver transplant. However, because livers are in high demand and in short supply, liver transplants are reserved for patients who need them most.
In the meantime, you should do everything you can to mitigate the symptoms of the disease and prevent complications.