My nephew has hemophilia A. What precautions can we take?

Question

Hello doctor,

My 11-year-old nephew has recently been diagnosed with hemophilia A after repeated knee swellings. His latest factor VIII activity was only two percent, which I believe is very low.

We were told that he may need prophylaxis, but frequent infusions are difficult for the family to manage. So, our concerns are:

Are there any newer treatment options that last longer or require less frequent dosing?
Also, what special precautions should we take during minor accidents or procedures, such as a tooth extraction, to prevent serious bleeding?

Kindly suggest.

Thank you.

Answer 1

Hello,

Welcome to icliniq.com.

I understand your concern.

Yes, you are right. Newer treatments are available that can reduce the frequency of infusions. These include non-factor therapies and extended half-life factor VIII concentrates, such as:

Non-factor therapy is administered as a simple subcutaneous injection (under the skin), similar to an Insulin injection. Depending on the regimen, it may be administered once weekly, every two weeks, or even once every four weeks. It works differently from factor VIII but is very effective in preventing bleeding episodes and can significantly ease the treatment burden.
Extended half-life factor VIII concentrates are modified versions of factor VIII that remain in the bloodstream longer. Instead of infusions every other day, dosing may only be required two to three times a week, or in some cases, twice a week.

The next step should be to discuss these specific options with your nephew’s hematologist to determine the best approach for his individual case.

Since his factor VIII activity is only two percent, careful precautions are essential. Precautions you must take during minor accidents and dental procedures are-

For minor cuts, apply firm pressure with a clean cloth for 10 to 15 minutes. For superficial scrapes, clean the wound and cover it with a bandage.
During tooth extraction or any dental procedures, patients are considered high-risk and should never be done without prior consultation with their hematologist.
Typically, an infusion of factor VIII is given beforehand to raise his levels adequately and prevent bleeding.
The dentist and hematologist should coordinate to ensure the procedure is carried out safely.

I hope this helps.

Thank you.