At some point in our EMT and paramedic training, we learned how dangerous hemophilia and other bleeding disorders can be for trauma patients. We also learned there was not much we could do for them in the field when they are bleeding except transport to the hospital.
I do not think I would be overstating it if I said hemophilia patients scare the heck out of most EMS providers, but newer treatment options can save lives and EMS providers need to be aware of them.
| MORE: On demand: Bringing whole blood to the front lines of EMS
What is a bleeding disorder?
According to the National Bleeding Disorders Foundation, there are many types of bleeding disorders, but hemophilia and von Willebrand disease are the two most common. Hemophilia creates a higher risk for bleeding, because the patient lacks a certain protein, or factor, that is needed as part of the clotting process.
Lacking the protein, the patient’s blood does not go through the normal clotting cascade to respond to injuries (turning blood components into sticky platelets and fibrin mesh to hold the platelets and form a plug over the leaking vessel).
In some cases, patients with hemophilia may even bleed spontaneously into their joints or muscles. The clotting cascade in a bleeding disorder patient is too slow and less effective, allowing any potential for bleeding to become life-threatening.
The most common forms of hemophilia are hemophilia A, where the individual does not have normal amounts of clotting Factor VIII (eight), and hemophilia B which is a deficit of Factor IX (nine).
Von Willebrand disease is the second most common bleeding disorder and occurs when blood does not have sufficient levels of von Willebrand factor or the factor does not function properly.
Who is at risk for bleeding disorders?
The majority of people with bleeding disorders inherited the condition and have dealt with it all their lives, but there are rare cases of bleeding disorders acquired later in life. These cases are sometimes linked to chronic diseases, medications or vitamin deficiencies. Both males and females can have bleeding disorders.
What signs and symptoms do patients with bleeding disorders have?
Both hemophilia and von Willebrand disease put patients at risk for an abnormal response to any situation that involves trauma, surgical procedures, dental work, minor bumps and bruises, and even menstrual bleeding.
The patient may complain of pain, tenderness or a tingling/bubbling sensation. Symptoms may start as a bruise, but progress into a hematoma and further into an internal bleed. A sprain or muscle strain can result in bleeding that can severely damage the joint. It is crucial that if a patient says they have a bleed, believe them!
The life threat occurs when the bleeding continues and definitive treatment is not accessed in a timely manner. In these cases, the patient either loses significant amounts of blood, or they bleed into their brain.
What treatment is available for bleeding disorders?
Once a person is diagnosed with a bleeding disorder, they undergo specific and in-depth testing to determine which of their factors is lacking and to what degree. Some disorders are classified by the amount of the factor that is missing from the blood compared to normal levels.
Based on this information, the patient can be prescribed regular infusions of the needed factor to help them maintain more normal levels.
This is called prophylactic treatment, however, it does not negate the need for an additional factor replacement in the event of an injury or reported bleeding concern.
Often, patients are given doses of their clotting factor to take home and keep in case of an injury and bleeding. Many are even trained to do phlebotomy access on themselves with a butterfly needle so that they can self-administer the protein factor in an emergency. The factor replacement kits are kept in the patient’s refrigerator.
EMS providers should also know about hemophilia treatment centers or HTCs. These are important resources for bleeding disorder patients and offer counselling on living with their condition and the steps they can take to be safe and lead normal lives, as well as what to do with bleeding occurs. HTC provide patients with the factor replacement kits and can also provide kits to hospitals when needed. HTCs may also be an educational resource for EMS providers. Find your regional HTC through this CDC directory.
Treating bleeding disorders: First thing first — RICE
What can EMS do when treating a patient with a bleeding disorder? Rest, ice, compression and elevation are sound advice for any soft tissue injury. Minimizing even minor bleeding into tissue can help reduce swelling and pain, and speed healing. For a person with a bleeding disorder, RICE is even more important to give their body a little extra help clotting and decreasing the likelihood of the injury becoming life- or limb-threatening. BLS skills save lives.
Check the fridge!
Factor replacement kits are extremely expensive and most hospitals do not carry factor replacement kits in their pharmacies. When needed, they reach out to regional hemophilia treatment centers to get doses quickly shuttled over to them. When transporting a patient with a bleeding disorder from their home for any level of emergency condition, EMS providers should consider bringing the live-saving kits with the patient to the hospital. The patient or their family may not think of it in an emergency. Ask them where the kit is kept and make sure it is transported to the hospital with the patient. Let the hospital staff know that the kit was transported as well in case it is needed.
Check local protocols and consult medical control, but some providers may even be allowed to help the patient self-administer the clotting factor to the patient if indicated or start an IV to provide an administration route. The pre-dosed factor is simply reconstituted with a vial of provided saline. The full volume of the reconstituted factor solution is given as an intravenous injection.
While the kits are expensive, they are life-saving. Do not hesitate to bring the kit with the patient or administer it if indicated.
The patient should be transported to the closest, most appropriate hospital, but the patient may have a preference based on previous treatments of their condition and recommendations from their hemophilia treatment center. The patient may also ask that the emergency number for their hemophilia treatment center be called, but this may be more appropriate for the emergency department staff to do. As mentioned earlier, the HTC will be able to provide treatment recommendations as well as deliver needed clotting factor replacement therapy kits or other medications.
What about TXA for bleeding disorders?
Providing a dose of TXA to severely injured trauma patients who are thought to be actively bleeding has become a standard protocol for paramedic providers. Does the presence of a bleeding disorder constitute a contraindication for the administration of TXA?
In a nutshell, TXA slows the body’s normal response to clot formation by breaking the clot down. In normal day-to-day life, it is appropriate for the body to break down clots to as the blood vessel heals itself. In a hemorrhaging trauma patient, giving TXA to slow the clot break-down process limits bleeding and improves outcomes.
A patient with a bleeding disorder is already starting with less clot forming capacity, so it only stands to reason that using TXA to prevent the breakdown of those precious clots is appropriate. Check local protocols. Even if TXA is given, the patient with a bleeding disorder will still require treatment with their factor replacement therapy.
Hemophilia and von Willebrand disease remain relatively rare conditions, but EMS providers may find themselves being called to treat and transport a bleeding disorder patient, especially after a traumatic mechanism. Maintain a high level of suspicion for bleeding and check the fridge!
HTC support
This article was written with some much appreciated assistance from the Hemophilia Outreach Center, which is an HTC located in Green Bay and Wausau, Wisconsin. They provide comprehensive care and 24/7 support to their patients and serve the northern half of Wisconsin and the Upper Peninsula of Michigan. Contact your local HTC for more information.
Stay safe out there.
