Question

How will you care for a woman with Factor XIII deficiency who is planning for pregnancy?

Hint:

Women with Factor XIII deficiency planning pregnancy should ensure optimal management with Factor XIII concentrate, regular monitoring, and careful delivery planning to reduce bleeding risks.

Answer 1

Step 1: Preconception Care.
Women with Factor XIII deficiency planning pregnancy should receive preconception counseling to ensure optimal management of their condition. Factors to consider include: 1. Factor XIII activity testing: Preconception testing should confirm FXIII levels and adjust treatment accordingly. If levels are low, Factor XIII concentrate should be administered to ensure adequate clot stabilization during pregnancy and childbirth.
2. Genetic counseling: If there is a family history of FXIII deficiency, genetic counseling may be recommended to assess the risk of transmitting the disorder to offspring.

Step 2: Pregnancy Management.
During pregnancy, regular monitoring of FXIII levels is essential, as they may fluctuate during this time. The use of Factor XIII concentrate may need to be adjusted based on these levels. The delivery plan should include preparations for possible bleeding complications, especially during labor, as bleeding risks are higher in FXIII-deficient patients.

Step 3: Postpartum Care.
After delivery, careful monitoring for bleeding and prompt administration of Factor XIII concentrate if necessary is crucial. Postpartum hemorrhage (PPH) risk should be assessed, and appropriate precautions should be taken, including the use of FXIII concentrate during delivery if levels are low.