Hemophilia A versus Hemophilia B - Differences and Details

ČSH

At the European Haemophilia Consortium conference in Prague, I had the opportunity to attend an interesting lecture by Brian O'Mahony - President of the European Haemophilia Consortium. The topic interested me greatly, as I myself have a son with hemophilia B, about which it has always been possible to find much less information and which has always been in the "shadow" of hemophilia A. Mr. O'Mahony mentioned several similarities between the two types of the disease: the same inheritance pattern, similar joint and muscle bleeds, and the need for continuous and comprehensive treatment for both hemophilia A and B. Some studies have shown that hemophilia B is clinically less severe than hemophilia A, while other current studies contradict this hypothesis.

The treatment model is similar, although hemophilia B waited much longer for quality treatment. FVIII concentrates first appeared in the 1970s, whereas FIX concentrate was not available until 1992. As for recombinant treatment, FIX also significantly lags behind. Recombinant FIX products appeared in 1997 (three years later than FVIII), and there is only one on the market (while six recombinant products are available for FVIII). Treatment of hemophilia B has always somewhat "lagged behind," and even today, prophylaxis for hemophilia B is used less frequently than for hemophilia A. Unfortunately, economic circumstances can also play a role. To achieve the same level of treatment, a hemophilia A patient needs 1 IU, while a hemophilia B patient needs 2 IU of factor. This information, which Mr. O'Mahony mentioned, was a great revelation for me. And it is not just a larger dose of medication - it is also a higher price. Treatment for a hemophilia B patient is significantly more expensive than for a hemophilia A patient. This can have fundamental consequences in countries where economic resources are strictly limited and where the disease prevalence is higher. As for prophylaxis, hemophilia B patients typically administer medication twice a week, while hemophilia A patients do so three times. The risk of inhibitor development is less common in B; however, if an inhibitor does develop, its treatment is very complicated and it is difficult to eliminate.

The outlook for the future seems quite promising. Although hemophilia B waited longer for quality treatment, research today shows that drugs with longer-acting factor are being developed faster than for hemophilia A. Gene therapy has also achieved certain successes - there are currently five different studies underway that show better results than for hemophilia A.

And the answer to the question of which of them is the so-called royal disease is also a surprising one for me - it is precisely hemophilia B, which many of us probably do not even realize...

Although my observations from the lecture are very lay and experts in the field of hematology would certainly present everything much more eruditely, they nonetheless gave me hope for the future for my son and other hemophilia patients with the same diagnosis. I am glad that I was able to attend the event and be at the center of new findings.

Marcela Rehakova, mother of eight-year-old severe hemophilia patient Jakub