Abstract
Haemophilia can be a good way to compare health care in socialist Czechoslovakia and the present Czech Republic. The Communist regime did not have the money for expensive treatment, and it did not matter to individuals.
He did not care that many hemophiliacs had lived their lives as invalids. Children did not have access to effective treatment, which is paradoxically protected from the AIDS pandemic that hemophiliacs in Western Europe have badly hit.
The Iron Curtain worked in many forms. Shortly after the Velvet Revolution, we were able to provide our patients with effective concentrates of missing coagulation factors, and after 2000, in most hemophiliacs, they performed a prophylactic treatment that made their lives comparable to healthy children.
Endlessly bleeding wounds in oral cavity and joints damaged by haemophilic arthropathy, bleeding spontaneously and invalidating children are endlessly a memory. The co-operation of pediatric haemophiliac centers within the Czech National Haemophilia Program and the reporting of the number and types of bleeding in a common database guarantees the same level of modern care for all children.
Median 3 bleeding per year in severe haemophiliacs is evidence that the haemophilia care system works. Children and their parents soon learn to administer intravenous treatment separately so that prophylactic application of concentrates 2-3 times a week, as well as therapeutic treatment at the first bleeding manifestations, are at home.
Every year organized summer camps for haemophilia, their parents and siblings contribute greatly to this. Treatment in centers guarantees professional dental, surgical, rehabilitative care.
Paradoxically, moderate haemophilia who are not on prophylactic treatment may have a more severe joint disorder than children with severe haemophilia due to a delayed response to joint bleeding. An improved level of care for haemophilia leads to a steady increase in treatment costs, which is offset by their full involvement in society and their expected life expectancy.
Hemophiliacs are no longer suffering from HIV infection or chronic hepatitis C. The exception is individuals with an inhibitor who have failed immuno-tolerant therapy, who are more likely to bleed and respond to treatment with substitutes slower.
This difficult situation can help solve the early onset of immunotolerant treatment and, in its failure, prophylactic treatment with substitute preparations bypassing the role of factor VIII or IX. The cost of this treatment increases to astronomical heights.
Further improvement in the quality of life of haemophiliacs will provide prolonged half-life preparations which will allow for intravenous prophylactic treatment to be more tolerable by prolonging the intervals between applications. Gene therapy remains the music of the future.