Guía ECF

Sickle cell disease guideline update

In 2009, the Spanish Group of Erythropathology (GEE), under the coordination of Dr. Pilar Ricart, published the guidelines for the management and treatment of patients with sickle cell disease (SCD).

During these years the advances in the knowledge of the disease have been remarkable. This, together with the fact that, with immigration, the number of patients has increased considerably, has motivated us to review and update the previous guide.

Hemoglobinopathy S is the most common hemoglobin variant in the world. Around 20 million people have the disease and approximately 300,000 children are born with SCD each year. And although in Spain the disease was previously a rarity, due to the immigration of people from Sub-Saharan Africa or Central America, we currently have more than 1,200 patients with SCD registered.

Given that it is a multi-organ and complex disease, the guide is addressed with the participation of several specialists such as hematologists, pediatricians, internists, surgeons, traumatologists, ophthalmologists and anesthesiologists.

It consists of 23 chapters in which it has been tried to refresh knowledge and update not only the biology of the disease but also its prevention, diagnosis and treatment, as well as the approach to its different complications.

We have tried to make it clear, concise and didactic. With chapters on the different organs, many of these already reflected in the previous guides, but other new ones that are generating numerous expectations at the present time, such as the new drugs in the treatment of the disease, gene therapy, or the section on the diagnosis and treatment of chronic pain, which has changed the paradigm of pain management.

I hope that these guides, which we have prepared with such enthusiasm, are to the liking and of maximum use for the medical professionals who treat these patients, and a basic reference tool for haematologists, paediatricians, emergency physicians and family physicians.

Our most sincere recognition, mine and that of the rest of the coordinators (Montserrat López Rubio, María Pilar Ricard and Marta Morado), to all the authors, for their work, dedication, enthusiasm and knowledge, which have contributed substantially to improving the edition. previous. We also appreciate the excellent work done by Medea Publishing and Novartis Oncology for sponsoring the printing of these guidelines.

We are sure that this new edition of the guidelines will constitute a fundamental pillar for the treatment of these patients, as it will be of great help to responsible physicians and with the ultimate goal of improving the quality of life and management of people with sickle cell disease.