Living With a Rare Bleeding Disorder

Coping with the diagnosis

Learning you or your child has a bleeding disorder is very upsetting and you may experience a range of different emotions. For some people, it inspires fear and anxiety while for others, being able to put a name to symptoms they have lived with for years can be a tremendous relief. Parents may feel guilty to learn their child has inherited a genetic disease. All these feelings are normal, and e likely to change over time as you learn more about the condition and the impact it will have on your life.

Talking to others—friends, parents, healthcare professionals, and other people with bleeding disorders—can be a great comfort. Learning as much as you can about the disorder will also help you feel more confident and sooth your fears. Get in touch with your local patient organisation or haemophilia/bleeding disorder treatment centre to ask questions and discuss options.

 Living with Factor V Deficiency – Ann Marie McCabe

Healthy Living

The recommendations for healthy living are the same as for anyone with or without a coagulation factor deficiency:

  • Follow a regular programme of physical activity suited to lifestyle and capability. Maintaining adequate muscle mass can reduce the risk of bleeds.
  • Try to avoid contact sports like boxing, Gaelic football, and rugby to reduce risk of serious injury. The most often recommended sports are swimming and cycling.
  • Use high-quality sports equipment appropriate for any sport (helmet, elbow pads, kneepads, proper shoes).
  • Eat a well-balanced diet.
  • Get enough sleep.
  • Drink alcohol in moderation only.
  • Don’t smoke.

 

Prevention

There is no way of fully preventing bleeds but there are a number of things you can do to reduce the likelihood of a bleed occurring:

  • Never take aspirin. Aspirin increases the risk of bleeding by inhibiting platelet function.
  • Consult the healthcare team before taking any herbal medicinal supplements or vitamins and so-called natural products sold over the counter.
  • Take care of teeth in order to avoid having drastic dental care as far as possible. See the dentist regularly. The Haemophilia Treatment Centre can recommend dentists who are familiar with coagulation disorders.
  • If surgery or any other invasive procedure (a procedure in which there is an intrusion into the body with an instrument) is necessary, contact the Haemophilia Treatment Centre in advance in case preventative treatment is required.
  • Wear a Medic Alert bracelet or chain at all times on which the type of coagulation problem is engraved.
  • If planning a trip, let the Haemophilia Treatment Centre know. They will tell you what precautions to take.
  • Always stay in touch with the Haemophilia Treatment Centre.

 

Dental care

Good oral hygiene is essential to prevent tooth decay and gum disease. For people with bleeding disorders, maintaining good dental health is very important to reduce the need for dental surgery, which can be complicated by excessive or prolonged bleeding. People with bleeding disorders should:

  • Brush teeth at least twice a day
  • Floss regularly
  • Use a toothpaste containing fluoride
  • Get regular checkups

 

Any type of invasive procedure, such as a tooth extraction or root canal, can cause bleeding in people with bleeding disorders. The dentist should consult with the Haemophilia Treatment Centre to identify potential risks and properly plan the procedure. Medication may be needed beforehand to help control bleeding and ensure a safe recovery.

Vaccinations

People with bleeding disorders should be vaccinated, but the vaccines should be given under the skin, not directly into the muscle. Vaccines against Hepatitis A and B are particularly important for people that are treated with fresh frozen plasma and any other product that is not viral inactivated. Family members handling treatment products should also be vaccinated, though this is less critical for those using viral-inactivated products.

Medications to avoid

Check all medications with your doctor. Some over-the-counter medications should be avoided because they interfere with clotting. People with bleeding disorders should not take Acetylsalicylic Acid (ASA or Aspirin®) or nonsteroidal anti-inflammatory drugs (such as Ibuprofen and Naproxen) without medical advice.

Have medical information at hand at all times

Carry information about your disorder, the treatment that has been prescribed, and the name and telephone number of your physician or treatment centre. In emergencies, a medical bracelet or other identification, such as the WFH International Medical Card, notifies healthcare personnel of your bleeding disorder.

When travelling, find the addresses and telephone numbers of the Haemophilia Treatment Centres at your destination(s) and bring the information with you.

Special issues for girls and women

Women with clotting factor deficiencies tend to have more symptoms than men because of menstruation and childbirth. Girls may have especially heavy bleeding when they begin to menstruate. Women with clotting factor deficiencies may have heavier and/or longer menstrual flow, which can cause anaemia (low levels of iron, which results in weakness and fatigue).

Women with clotting factor deficiencies should receive genetic counselling about the risks of having an affected child well in advance of any planned pregnancies and should see an obstetrician as soon as they suspect they are pregnant. The obstetrician should work closely with the staff of the Haemophilia/bleeding disorder treatment centre in order to provide the best care during pregnancy and childbirth and to minimise the potential complications for both the mother and the newborn.

Women with certain factor deficiencies (such as factor XIII deficiency and afibrinogenemia) may be at greater risk of miscarriage and placental abruption (a premature separation of the placenta from the uterus that disrupts the flow of blood and oxygen to the foetus). Therefore, these women require treatment throughout the pregnancy to prevent these complications.

The main risk related to pregnancy is a postpartum haemorrhage. All bleeding disorders are associated with a greater risk of increased bleeding after delivery. The risk and the severity of the bleeding can be reduced with appropriate treatment. This treatment is different for each woman and depends on her personal and family history of bleeding symptoms, the severity of the factor deficiency, and the mode of delivery. Factor replacement therapy may be necessary in some case.

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