Bleeding disorders: Emergencies in hemophilia

What are emergencies in hemophilia?

Hemophilia emergencies include:

• Head injury/bleeding
• Throat/neck bleeding
• Abdominal bleeding
• Iliopsoas muscle bleeding
• Compartment syndrome

With all hemophilia emergencies, seek medical evaluation immediately.

Remember: Factor first, which means give factor before any procedure or test. This is especially important when you visit an emergency room where the common practice is to do tests before medications are administered. You will need to be an advocate for your child or yourself.

Any severe physical trauma including car accidents should be evaluated immediately even if there are no symptoms.

Head injury/bleeding

All head injuries are considered to be serious. Please seek medical evaluation immediately. These signs can occur following a head
injury or spontaneously (rarely). They can be a sign that bleeding is actually occurring inside the head. They are not always a sign of bleeding, but watch for them and report them if they occur:

• Drowsiness (or change in sleep pattern of young child)
• Irritability/confusion
• Lethargy/fatigue
• Dizziness
• Nausea and/or vomiting
• Seizures
• Dilated or unequal pupils Stiff neck and/or back Headache
• Double vision
• Change in behavior

Throat/neck bleeding

• Bruising in the mouth or under the tongue can spread down the tissues of the neck and block the airway (this can happen after dental work if not adequately treated with factor and/or antifibrinolytics such as aminocaproic acid or tranexamic acid).
• Tonsillitis or "strep throat" often can cause throat bleeding.
• Severe coughing spells or vomiting can cause bleeding.
• Injury to the neck area can cause bleeding which can result in a blocked airway.
• All swelling in this area should be considered to be the result of bleeding unless proven otherwise.

Abdominal bleeding

• Any complaint of abdominal pain should be seen by a health care provider.
• Vomiting blood may be a result of abdominal bleeding.
• Bleeding from the rectum or black, tarry stools may be a result of abdominal bleeding.
• Large amounts of blood can be lost in this area with little or no swelling.

Iliopsoas muscle bleeding (Thigh/groin/hip area)

Large amounts of blood can be lost in this muscle group with little or no swelling. Signs of iliopsoas muscle bleeding are:

• Hip flexion (difficult or painful to straighten leg)
• Groin pain
• Numbness or tingling in quadriceps muscle
• Lower back or flank pain (front of the thigh)
• Toe walking
• Frog leg position with hip flexed outward

Compartment syndrome

Compartment syndrome is an increase in pressure within the muscle, which can cut off the circulation to the nerve in the area. This usually occurs 12 hours to 3 days after the injury. This typically occurs in the forearm, calf or hip area (iliopsoas muscle). Signs may include:

• Swelling and tightness in the affected area.
• Numbness/tingling to the affected area or below (such as fingers or toes).

Surgery may be required to relieve pressure on the blood vessels and nerves. Often this can be avoided by elevating the affected limb and applying ice to the area.

What should I do in an emergency?

Seek medical evaluation immediately. For most emergencies, call the hematology clinic at 612-813-5940:

• During the weekday, ask to speak with the hematology nurse.
• During nights and weekends, call the hematologist on call.

Call 911 if:

• Your child cannot breathe or is unconscious.
• You child has a head injury and has any neurologic changes noted above.
• Your child has uncontrolled bleeding (this is very uncommon).

What else do I need to know?

The following types of bleeds are not always emergencies, but left untreated or treated incorrectly can lead to emergencies.

• Mouth or nose bleeding. Minor mouth bleeding that continues for several days can become serious. Slow, constant loss of blood could quickly lead to severe anemia.
• Hematuria (blood in urine). The cause of this is unknown. It is common in adolescent young men.

Call your hematology provider to discuss a treatment plan if you have these symptoms.

Questions?

This is not specific to your child, but provides general information. If you have any questions, call your clinic.

Reviewed by Hematology 7/2017

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