Poděbrady Is Not Just for the Heart

Congress president Peter Salaj from the Prague Institute of Haematology and Blood Transfusion (ÚHKT) once again attracted interesting participants, and every listener found something of interest.

There was talk about hepatitis C, but also in detail about the results of total joint replacement surgeries at the Na Bulovce Hospital in Prague. They already have several years of extensive experience there, and Radovan Kubeš, the lead surgeon in the Czech Republic, presented what he and the entire orthopaedic department there have accomplished during that time.

The great progress in surgery was clearly confirmed, but so was the view that joint replacement is the very last and irreversible step that should be delayed as long as possible. According to local statistics, the first surgery has an average lifespan of 15 years, while the second - the first revision surgery - only five. Therefore, such a procedure is appropriate only at a certain age, certainly not too young. Participants from both Czech and visiting Hungarian orthopaedic surgeons agreed on similar findings.

The benefits of pharmacokinetics were once again confirmed, and patients at all centres should request it. The uniform prophylaxis schedule is now outdated, and an individualised approach brings benefits not only to those directly concerned, but also to the financial side of things.

A large part of the programme was devoted to presentations on paediatric care. In the Czech Republic, by the end of 2014, there were 1,066 registered individuals with hemophilia of any severity. Data from 698 of them are collected in the ČNHP (Czech National Haemophilia Programme) registry. 234 of these individuals are children. Data is entered by 17 centres across the Czech Republic.

A feared and serious complication of hemophilia treatment is the development of a FVIII/IX inhibitor. The presence of an inhibitor carries serious clinical complications and difficulties for the hemophilia patient, including life-threatening bleeding. Treatment of inhibitors is complicated and economically demanding, whether it concerns stopping acute bleeding or therapeutic approaches to permanently eliminate the inhibitor. Most commonly, inhibitors develop during the first 50 exposure days of coagulation factor concentrate administration, usually between the 10th and 20th day of application. It is therefore a dreaded complication for paediatric haematologists. The goal of FVIII/IX inhibitor treatment is complete elimination of the inhibitor. The success rate of immune tolerance induction therapy is more common in hemophilia A (50-90%), less common in hemophilia B (15-30%).

Currently, the standard for paediatric hemophilia patients in the Czech Republic is prophylactic therapy with FVIII/FIX. Recombinant products are commonly used for treatment, and some hemophilia patients in clinical studies also use drugs with prolonged effect. Care for hemophilia patients is multidisciplinary and currently also focuses on psychological, orthopaedic, and physiotherapy aspects. The number of bleeding episodes on prophylactic therapy in children has significantly decreased. Therefore, musculoskeletal involvement is minimal in most children. It is therefore necessary, among other things, to change the method of assessment, as previously used tools are no longer sufficiently sensitive. Globally, the Hemophilia Joint Health Score (HJHS) is currently the most commonly used tool for paediatric hemophilia patients. It is therefore useful to understand this scoring system and learn to use it as effectively as possible. Results can be used to compare the condition of hemophilia patients not only between individual centres, but also between individual countries. Physiotherapist Marie Katzerová's presentation introduced the HJHS, scoring of the most common target joints and overall motor activities used in daily life. She mentioned its advantages and did not overlook its disadvantages. The presentation was supplemented with practical experience using the scoring form in everyday practice (28 children examined). It also included a short illustrative case study of a hemophilia patient who was examined over one year, always during the summer rehabilitation stay for paediatric hemophilia patients. HJHS examination should become a routine part of hemophilia patient check-ups at every centre.

Because a large portion of the ČNHP Coordination Council members came to Poděbrady, they used the opportunity for an official meeting. Besides the regular working agenda, the most important conclusion for Society members was one initiated by our organisation itself. The Czech Hemophilia Society Council has long been striving to influence the knowledge of doctors and other emergency service workers, as well as intensive care departments, particularly emergency admissions. It turned out that in emergencies, ambulance services do not transport hemophilia patients to specialised centres, but to catchment hospitals. And the level of knowledge of emergency doctors is lamentably low even in large hospitals.

A working group was therefore established which will create a document by the end of January outlining the ČNHP's requirements for emergency medical services, hospital management, emergency departments, and trauma centres. The Society considers this matter extremely important and is pleased that the results of long-term efforts are finally beginning to materialise. One can only hope that the recipients will adopt the document as their own and that this crucial field will continue to function as needed.