Operation Success

There’s no doubt surgery can be scary—especially for someone with hemophilia. After being told your whole life to be careful and watch out for bleeding, the idea of doctors wanting you to go under the knife willingly can seem more than a little crazy.

Obviously, it’s better to avoid invasive medical procedures if you can, but sometimes that’s not possible. You may need dental surgery, a tonsillectomy, an appendectomy or an operation to rectify a joint problem.

Fortunately, treatment with various prophylaxis regimens have decreased both general spontaneous bleeding and joint bleeds—thereby cutting down on the need for joint surgeries. That said, sports injuries still account for a large number of surgeries needed by people with hemophilia.

“If an individual is particularly active in sports and is not fastidious in pre-treating himself to make certain his clotting factors are high enough to prevent excessive trauma beyond what a regular individual might also experience, then that’s certainly going to be an issue,” says Craig Kessler, M.D., professor of medicine and pathology, section chief of hematology and director of the Coagulation Laboratory at Georgetown University in Washington, D.C.

Do Your Homework

When it comes to hemophilia, there is no one-size-fits-all advice because so much depends on an individual’s factor levels and health status. “Typical people have 100 to 150 percent function of two clotting factors (factor VIII and factor IX) whereas people with hemophilia can have anywhere from 0 to 20 percent,” says Shane P. Dormady, M.D., Ph.D., medical director for infusion services at El Camino Hospital’s Cancer Center in Mountain View, California. “You need a minimum of 25 percent function of these two blood clotting factors for blood to clot normally. If someone has 0 percent, he will bleed more heavily than someone with 20 percent who will not bleed that much.”

Regardless of percentages and no matter if someone has mild or severe hemophilia, advanced planning is crucial. Surgery should be done in consultation with a hemophilia treatment center where there’s a team of physicians available who are knowledgeable about bleeding disorders. It is also important that the surgeon understands hemophilia and coordinates closely with the hemophilia treatment team.

The facility itself should have a coagulation laboratory immediately available to help in the monitoring of factor levels and there should be an adequate pharmacy or blood bank that can supply the physician with various types of replacement therapies that could be needed to stop bleeding. If possible, schedule surgery early in the week and early in the day for best lab and blood bank support.

Individual preparation is key as well. Obviously the big risk when it comes to surgery and hemophilia is bleeding. “People are going to bleed no matter what, whether they have a clotting factor deficiency or not,” says Dr. Kessler. “You just want to be sure to prevent bleeding that would not otherwise occur.”

When surgery is planned in advance, factor levels should be tested several weeks before the day of the operation. Before, during and after surgery clotting factor has to be administered to ensure the levels are sufficient for blood to clot.

Being in general good health is also helpful for success in the operating room. Keep exercising pre-surgery (unless the surgery is related to a joint problem or your doctor has told you otherwise) and eat a healthy diet. Include sources of protein, and fruits and vegetables, since being well-nourished can aid in recovery. If you smoke, do your best to quit as researchers in Germany have found that people who stopped smoking (or substituted nicotine replacement therapy) at least four weeks before surgery cut their risk of wounds healing poorly in half.

Emergencies Happen

Sometimes no matter how much people plan, emergency situations do come up. Fortunately factor can be administered in a matter of minutes to bring levels high enough to safely perform surgery. However, in order for emergency procedures to be successful, doctors and other treating personnel must be aware the person has hemophilia.

Obviously if the individual is awake he can simply inform them of his hemophilia status. But what if he is unconscious? “The one thing I tell all my patients with hemophilia to have on them is a medic alert necklace or bracelet signifying they have hemophilia, so if they’re in an accident and can’t talk someone will pick up on it and know they have to be given blood clotting factors immediately,” says Dr. Dormady.

The Road to Recovery

Even after the surgery is complete, people with hemophilia still need to be vigilant during the recovery process. “If the blood clotting levels are kept right and the patient is monitored closely by his hematologist as well as the surgeon, things should be uncomplicated, but for sure there’s a greater risk with hemophilia of bleeding postoperatively,” says Dr. Dormady.

Patients must pay attention to their bodies following surgery. Any pain or unusual symptoms such as dizziness require could signal internal bleeding and the treatment team should be contacted immediately. Fortunately, as long as factor levels are kept in the correct range, recovery should be the same as for people who do not have hemophilia and by three to four weeks (if not sooner depending on the procedure) an uncomplicated recovery should be complete.

Ultimately, while the idea of surgery is unsettling, it’s definitely something that, with planning and preparation, you can face with confidence.