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The symptoms of hemophilia

What are the first signs of hemophilia in a young child?

Babies have sharp teeth and bite their gums and tongue, often causing bleeding. This and bruises from falls are usually the first signs of hemophilia.

Until the age of 2, bleeding into joints is uncommon. Most bleeds are surface bruises. When babies are learning to walk, they fall frequently and suffer many bumps and bruises.

Bleeding into the joints, soft tissues and muscles is seen more frequently after the age of two.

What are the symptoms of hemophilia in an older child or adult?

Common symptoms of hemophilia are:

  • bleeding into joints (knees, elbows, ankles, shoulders, hips, wrists in descending order of frequency)
  • bleeding into soft tissues and muscles (the ileopsoas muscle around the hip, calf, forearm, upper arm, Achilles tendon, buttocks)
  • bleeding in the mouth from a cut, bitten tongue or loss of a tooth (especially in children)
  • blood in the urine (hematuria)
  • surface bruising.

Are symptoms less severe as children get older?

Yes, in many children, symptoms become less severe as children move into adolescence and young adulthood. The reason for this is not that their hemophilia is any less serious. Factor VIII and IX levels remain constant throughout life. However, hemophiliacs learn to avoid some of the situations that lead to hemorrhages.

What causes the bleeding?

Bleeding is often caused by minor injury - a bump or a slight twist of a joint. However, many hemorrhages, especially among severe hemophiliacs, happen for no apparent reason. This is even truer in joints that have bled often in the past. The more a joint has bled, the easier it bleeds again with no external cause.

Even hemorrhages in the brain often have no apparent cause. Brain hemorrhages are the leading cause of death from bleeding in hemophilia. Therefore it is important to recognize the symptoms of a brain hemorrhage very quickly.

What are the symptoms of bleeding into the brain?

Some of the following symptoms may occur in a person with bleeding in the brain.

  • Persistent or increasing headache
  • Repeated vomiting
  • Sleepiness or a change in normal behaviour
  • Sudden weakness or clumsiness of an arm or leg
  • Stiffness of the neck or complaints of pain with neck movement
  • Complaints of seeing double
  • The development of crossed eyes
  • Poor balance when walking, a lack of coordination
  • Convulsions or seizures (fits).

What other kinds of bleeding are serious?

Any bleeding in a vital area is serious. Important examples are:

  • bleeding in the neck, throat or tongue (this could block the airway)
  • bleeding in the ileopsoas muscle across the front of the hip (this could pinch important nerves to the leg)
  • bleeding in the forearm or calf (this could pinch important nerves to the hand or foot)
  • bleeding in joints, especially knees, ankles and elbows (repeated bleeds in joints can lead to loss of range of motion, muscle loss, and destruction of the joints themselves).

What does a hemorrhage into a joint look like and feel like?

A hemorrhage into a joint, if untreated, goes on for days. This is what happens.

  • The first sign is a feeling of tightness in the joint but no real pain. The joint feels a little puffy to the touch.
  • As the hours pass, the joint becomes hot to the touch. Fully flexing or extending the joint becomes painful. Weight bearing becomes difficult. By this time, the joint is visibly swollen.
  • As the bleeding continues and the swelling increases, all movement in the joint is lost. The joint becomes fixed in a slightly flexed position in an attempt to relieve the interior pressure in the joint. The pain at this point can be excruciating.
  • The bleeding slows after several days when the joint is so full of blood that the pressure inside the joint cavity is equal to the pressure inside the broken blood vessels. Slowly, the bleeding stops and the long process of absorbing the blood in the joint cavity begins.

After several hemorrhages like this, the joint is permanently damaged.