Basic Aid for Hemophilia

Hemophilia (heem-o-FILL-ee-ah) is a rare bleeding disorder in which the blood doesn’t clot normally.

If you have hemophilia, you may bleed for longer than others after an injury. You also may bleed inside your body (internally), especially in your knees, ankles, and elbows. This bleeding can damage your organs and tissues and may be life threatening. Hemophilia usually is inherited which means that the disorder is passed from parents to children through genes.

People born with hemophilia have little or no clotting factor. Clotting factor is a protein needed for normal blood clotting. There are several types of clotting factors. These proteins work with platelets (PLATE-lets) to help the blood clot.

Platelets are small blood cell fragments that form in the bone marrow—a sponge-like tissue in the bones. Platelets play a major role in blood clotting. When blood vessels are injured, clotting factors help platelets stick together to plug cuts and breaks on the vessels and stop bleeding.

The two main types of hemophilia are A and B. If you have hemophilia A, you’re missing or have low levels of clotting factor VIII (8). About 8 out of 10 people who have hemophilia have type A. If you have hemophilia B, you’re missing or have low levels of clotting factor IX (9).

Rarely, hemophilia can be acquired. “Acquired” means you aren’t born with the disorder, but you develop it during your lifetime. This can happen if your body forms antibodies (proteins) that attack the clotting factors in your bloodstream. The antibodies can prevent the clotting factors from working.

First Aid Treatment

Within a first aid setting it is important to understand how to deal with a person affected by Hemophilia.

Soft Tissue/ Muscle Bleeds

What to look for:

  • A bleed that is increasing in size. Use a tape measure to measure the area of concern every few hours. Compare the right and left sides of the body also. If you do not have a tape measure, check every few hours to see if the area is growing larger.
  • A bruise that is increasing in size. Use a pen to mark the outline of the bruised area, and check every few hours to see if the bruise is growing larger.
  • A muscle that is becoming more firm, tense, or tight. Compare the right and left sides to assess for differences. Can you feel a specific lump? How big does it feel? Can that limb still be moved normally or is movement limited?
  • Pain in a particular area that gets worse with time, even without visible signs to help you locate bleeding.

  What to do:

  • Have the affected person reduce or stop his or her activities
  • Treat with R.I.C.E (Rest Ice Compression and Elevation)
  • Watch closely for signs of improvement, including less swelling, bruises changing colour to green or yellow, and less pain.
  • Talk to the patient as they will probably know how they normal deal with these injuries and may have their and factor concentrate which they can apply.
  • If in doubt dial 111 for advice or 999 if you cannot contol the bleeding.

Joint Bleeds

It can be common for bleeding to occur around the major joints, this bled is internal.  Symptoms include tingling sensations and a feeling of constriction/pain when moving the affected joint.

What to look for:

  • A joint with less movement than normal. Compare left and right joints for movement.
  • Pain or stiffness in a joint, with or without bruising present.
  • Swelling and warmth in a joint, with or without bruising to the area.
  • In childern unusual fussing and crying in an infant or young child and/or refusing to use a limb in
    a way they previously could (e.g. crawling with one straight leg, when previously both were bent when crawling)

What do do:

What to do:

  • Have the affected person reduce or stop his or her activities.
  • Treat with R.I.C.E.
  • Talk to the patient as they will probably know how they normal deal with these injuries and may have their own medication which they can apply.
  • If in doubt dial 111 for advice or 999 in an emergency.

Mouth Bleeds

Mouth bleeds need immediate treatment due to the risk of compromise of the airway.

What to look for:

  • Obvious bleeding from the gums or tongue
  • Bruising on the gums
  • Continual or very frequent swallowing
  • A salty or metallic taste in the mouth

What to do:

  • Keep calm – if the person is a child, try to distract him or her.
  • Have the person eat cold foods, like an ice lolly or ice cube.
  • Treat with factor concentrate according to your clinic’s guidelines.
  • Dial 999  immediately if the mouth bleed happened because of significant trauma.
  • Dial 999 immediately if the patient has taken their factor concentrate and it doesn’t stop within 20 minutes of treatment, or if bleeding episodes continue.
  • Avoid eating spicy foods, as they can irritate gums, especially gums that have already been bleeding.
  • Avoid eating foods with sharp edges (such as taco chips) and using drinking straws, as they can both cause gum bleeds or re-injure gums that have already been bleeding.
  • Watch for pale skin, trouble breathing with exercise, dizziness, and black or dark bowel movements.

Life threatening bleeds

If the patient suffers a knock to the head, neck or chest these can be potentially life threatening if bleedsing occurs.  If you suspect an injury to the head, neck or chest dial 999 immediately.

What to look for with a head injury:

  • Headache
  • Abnormal vision
  • Nausea or vomiting
  • Mood or personality changes
  • Drowsiness
  • Loss of balance
  • Loss of fine-motor coordination
  • Loss of consciousness
  • Seizures
  • Specific to infants: increased head circumference in a short period of time (within days), along with increased irritability and bulging fontanelles (the soft spots on the top of an infant’s skull)

What to look for with a neck injury:

  • Pain in the neck or throat
  • Swelling
  • Swallowing difficulties
  • Breathing difficulties

What to look for with a chest injury:

  • Pain in the chest
  • Breathing difficulties
  • Coughing up blood
  • Pale skin colour
  • Lack of energy

What to look for with an adomen injury:

  • Pain in the abdomen or lower back
  • Nausea or vomiting
  • Blood in urine
  • Black or bloody stool

What do to:

  • Dial 999 immediately
  • If the patient has their factor concentrate then allow them to take it
  • Treat any external bleeding
  • Monitor the paitent and treat them accordingly if they go unconcisous or go into cardiac arrest

Allergic Reactions

It is uncommon for a patient to suffer an allergic reation to their medication but if they do, then follow the standard guidelines for anaphalaxsis.

  • Make sure they stop taking their medication at that point
  • Dial 999
  • Calm and reassure the patient
  • Be perpared to begin CPR if they go into cardiac arrest.

More information about Hemophilia can be found on the Hemophilia Soctiety Website: www.haemophilia.org.uk/