Bleeding disorders uncommonly dangerous but clinical trials are making headway

• Blood clotting disorders are usually inherited, but can also be acquired
• Clinical trials for bleeding disorders have yielded exciting breakthroughs 

Types of bleeding disorders

Bleeding disorders result when the blood’s ability to form a clot at the site of a blood vessel injury is impaired. If your blood doesn’t clot, bleeding can result in excessive blood loss and can become severe enough to be life-threatening. This can be especially dangerous, for example, for people during surgery and for women during childbirth. 

“The best-known bleeding disorders are hemophilia and von Willebrand disease,” says Livia T. Hegerova, MD, an oncologist and hematologist at the Swedish Center for Blood Disorders and Stem Cell Transplantation. The National Hemophilia Foundation estimates that von Willebrand disease (VWD) affects up to 1 percent of the U.S. population, equally divided between men and women. The Centers for Disease Control calculates that about 20,000 Americans currently have hemophilia. Although blood disorders are usually inherited, sometimes people acquire them during their lifetimes after diagnoses of autoimmune diseases, heart disease, or some types of cancer.

Most of the patients at the Swedish Center for Blood Disorders and Stem Cell Transplantation are referred from their primary care doctor, or from a specialist in blood disorders that may want another set of eyes to consult on the case or recommend a different treatment plan for their patient. “Hemophilia can range from mild to severe,” Dr. Hegerova says. “It and other blood disorders are treated at Swedish because of our high level of expertise and because we have continuing clinical trials in effect.”

Promising breakthroughs

The clinical trials at Swedish are an important part of the process of finding new treatments that make blood disorders easier to manage. Many times, the only way to allow the blood to clot correctly is to infuse the missing clotting proteins (called “factors”) through time-consuming intravenous infusions, sometimes requiring a trip to the hospital or hemophilia treatment center up to three times a week. Dr. Hegerova says about some of the breakthroughs, “We are very excited because there are now brand new therapies being used and more coming very soon.”  

New factor-replacement innovations include:
 

• Injections (in the place of intravenous factor replacements) that can last up to one week
• Laboratory-derived factors that decrease the risk of using human plasma
• Coming soon — injections that may last up to one month

During the clinical trials, Swedish patients concurrently receiving treatments often form support groups where they help each other through their challenges. “Other benefits for patients in clinical trials,” Dr. Hegerova says, “is that they can familiarize themselves with leading-edge knowledge in the field, and they are advancing the science, which helps other people with the disorders. It’s a win-win. Seeing our patients and their families learning and being active and committed to getting better is one of the favorite parts of my job.”

Symptoms of blood clotting disorders

People with hemophilia A or B typically bleed longer than other people due to a deficiency in clotting factors. The danger comes from the fact that these bleeds can occur internally into joints and muscles, or externally from minor cuts, dental procedures, or trauma.

People with VWD have blood that is missing or defective in the von Willebrand factor, a particular kind of clotting protein. People with VWD experience frequent nosebleeds, easy bruising and excessive bleeding during and after invasive procedures, such as tooth extractions and surgery. Women with the disorder often experience heavy menstrual periods that last longer than average, and hemorrhaging after childbirth. 

Complications of blood clotting disorders 

One of the complications of these disorders happens when the patient develops a resistance to their factor replacement therapy. In this instance, the doctors take care to adjust the type and delivery of the factors to produce the best results. 

Another problem is with excessive internal bleeding. As noted women with blood clotting disorders such as VWD experience heavy, painful menstrual periods. Physicians often use hormones to help these women to treat the syndrome.  Dr. Hegerova says, “Hormone therapy for VWD is often hugely beneficial and can greatly improve the quality of a woman’s life.”

Physical therapy is essential for people with bleeding disorders

“As blood seeps into the joints, we often see people with these disorders develop stiffness and degrees of immobility. Physical therapy is an important part of managing blood clotting disorders successfully for our patients,” Dr. Hegerova says.

Learn more about the services offered at the Swedish Center for Blood Disorders and Stem Cell Transplantation, and subscribe to the Swedish blog so you’ll never miss a health update.

This information is not intended as a substitute for professional medical care. Always follow your healthcare professional's instructions.