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Congenital Coagulation Disorders and Bleeding Management Methods

Congenital coagulation disorders represent a group of hereditary haemorrhagic pathologies caused by deficiencies in coagulation factors. Among the recurring symptoms, nosebleeds (epistaxis) are common and can be particularly debilitating for patients. This article aims to detail the main congenital coagulation disorders and provide practical recommendations for managing bleeds, both nasal and minor everyday wounds, highlighting the use of the BleedCEASE / NasalCEASE products.

The Main Congenital Coagulation Disorders


Hemophilia A and B:

  • Hemophilia A: Deficiency in factor VIII.
  • Hemophilia B: Deficiency in factor IX.
  • Symptoms: Prolonged bleeding after injuries, hematomas, spontaneous bleeding in joints and muscles.
  • Prevalence: Hemophilia A (1 in 5000 male births), Hemophilia B (1 in 25000 male births).


Von Willebrand Disease:

  • Quantitative or qualitative deficiency in von Willebrand factor.
  • Symptoms: Mucosal bleeding (epistaxis, gingival bleeding), heavy menstrual bleeding in women, postoperative hemorrhages.
  • Prevalence: 1% of the global population.


Factor XI Deficiency:

  • Symptoms: Moderate to severe bleeding after surgery or injury, heavy menstrual bleeding.
  • Prevalence: More common in Ashkenazi Jews (8%).


Other Rare Deficiencies: Factors II, V, VII, X, XII, XIII:


Symptoms: Vary depending on the deficient factor but generally include prolonged bleeding after injuries or surgical interventions.


Management of Nosebleeds (Epistaxis)


Nosebleeds in patients with coagulation disorders can be frequent and require a specific approach. Immediate and effective management is crucial to prevent significant blood loss.


Recommendations:

  • Compression: Pinch the nostrils for 10 to 15 minutes while keeping the head slightly tilted forward to avoid swallowing blood.
  • Use of BleedCEASE/NasalCEASE packing :
    • Description: BleedCEASE/NasalCEASE haemostatic nasal packing is made of calcium alginate, a natural product, extracted from seaweeds, that promotes coagulation.
    • Method: Gently insert the packing into the bleeding nostril and keep it in place until the bleeding stops. The packing will jellify on the surface to allow a gentle, non-sticking removal after maximum 30 mn.
  • Management of Minor Wounds

Small cuts and abrasions are common and can also pose a significant problem for patients with coagulation disorders. BleedCEASE/NasalCEASE is the product suitable for these situations.


Conclusion


Managing bleeding in patients with congenital coagulation disorders requires specific knowledge and appropriate tools. The use of products like BleedCEASE/NasalCEASE, , is particularly effective for managing nasal bleeds and minor everyday wounds. A proactive and well-informed approach can significantly improve the quality of life for these patients.


References