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By Dr Dulcy Rakumakoe

Chief Executive Officer


Uncontrolled Bleeding: Understanding the condition of Haemophilia

Proteins present in the blood called clotting factors collaborate with platelets to create clots. Those with hemophilia experience prolonged bleeding, even following a minor injury or wound.


Haemophilia is a condition where the blood fails to clot because it lacks blood-clotting proteins called clotting factors.

When a person bleeds, the body typically pools blood cells together to form a clot to stop the bleeding.

Clotting factors are proteins in the blood that work with cells known as platelets to form clots. People with haemophilia will bleed for a long time, even after a minor cut.

The bleeding may happen internally like in the organs, joints, even the brain, which can lead to death. Treatment is available when the condition is caught on time and includes replacing the clotting factors that the body does not have.

A family history of haemophilia is the biggest risk factor for developing the condition and it’s more likely to be seen in males.

Severe haemophilia cases are usually diagnosed in early childhood. For many, the condition goes unnoticed until a major traumatic experience, such as an accident with injuries or a surgical procedure where one bleeds excessively.

Causes of Haemophilia

Haemophilia occurs when a clotting factor is missing or levels of the clotting factor are low.

  • Inherited haemophilia – one is born with the deficiency of clotting factors.
  • Secondary haemophilia – you are not born with it. Conditions exist that may damage the clotting factors. These are:
  • Pregnancy
  • Autoimmune conditions
  • Cancer
  • Multiple sclerosis
  • Drug reactions.

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Doctor during consultation held in his hand and shows patient laboratory tube with blood. Counseling of transfusion, blood and hematologic diseases and pathology like anemias, cell cancer, hemophilia. Picture: iStock.
Doctor during consultation held in his hand and shows patient laboratory tube with blood. Counseling of transfusion, blood and hematologic diseases and pathology like anemias, cell cancer, hemophilia. Picture: iStock.

Symptoms

The signs and symptoms of haemophilia depend on your level of clotting factors. The disease will be mild, moderate or severe.

Mild means you will bleed excessively after injury or surgery. If your deficiency is severe, you can bleed easily for seemingly no reason.

These are:

  • Nosebleeds without a known cause. Unexplained and excessive bleeding from cuts or injuries, or after surgery or dental work.
  • Many large or deep bruises
  • Unusual bleeding after vaccinations or injections.
  • Pain, swelling or tightness in your joints.
  • Blood in your urine or stool.
  • Unexplained irritability in infants.
  • Sometimes one may bleed in the brain from a seemingly mild bump to the head.
  • Signs and symptoms of brain bleeding include: Severe headache with vision abnormalities.
  • Vomiting
  • Sleepiness or tiredness
  • Sudden weakness or clumsiness.
  • Convulsions or seizures

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Blood sample for hemophilia test, inherited genetic disorder. Picture: iStock.
Blood sample for hemophilia test, inherited genetic disorder. Picture: iStock

Complications of Haemophilia

Complications are based on which part of the body the bleeding is occurring.

  • Muscles: Bleeding that occurs in deep muscle can cause the limbs to swell. The swelling can press on nerves and lead to numbness or pain.
  • Throat: Can cause shortness of breath. Joints: Will cause severe pain and may cause arthritis or destruction of the joint.
  • Brain: Can cause stroke and even death.

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Treatment

The gold standard of treatment for haemophilia is replacing the clotting factor you need.

This can happen during a bleeding episode or on a regular basis at home to prevent bleeding episodes. This can be done by using donated blood or using clotting factors made in a laboratory.

Other treatments include giving Desmopressin, a hormone that can stimulate the body to release more clotting factors. Physiotherapy is very helpful to ease signs and symptoms of internal bleeding, especially for joints. Surgery is needed when there is severe damage.

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Protection, treatment, prevention and patronage health in hematology in blood health against diseases, pathologies, oncology concept photo. Doctor surrounded lab tube with blood with hands on desk. Picture: iStock.
Protection, treatment, prevention and patronage health in hematology in blood health against diseases, pathologies, oncology concept photo. Doctor surrounded lab tube with blood with hands on desk. Picture: iStock.

Lifestyle modification

There are some pain medications that can make the bleeding worse.

These include aspirin, Grandpa headache powder, ibuprofen and other anti-inflammatories. Avoid over-the-counter medications.

Only take medication prescribed by a health professional who knows about your condition. Avoid blood-thinning medications.

Those that prevent blood from clotting include heparin and warfarin.

  • Exercise regularly. Activities such as swimming, bicycle riding and walking can build muscles while protecting joints.
  • Contact sports such as football, hockey, boxing or wrestling are not safe for people with haemophilia.
  • Brush your teeth regularly.
  • Practise good dental hygiene because the goal is to prevent tooth and gum disease, which can lead to excessive bleeding.
  • Get vaccinations. People with haemophilia should receive recommended vaccinations at the appropriate ages, including those for hepatitis A and B.
  • Requesting the use of the smallest gauge needle and having pressure or ice applied for three to five minutes after the injection can reduce the risk of bleeding.
  • Protect your child from injuries that can cause bleeding. Kneepads, elbow pads, helmets and safety belts help prevent injuries from falls and other accidents.
  • Keep your home free of furniture with sharp corners.

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