Educational Module in Cystic Fibrosis Clinical Practice

Educational module in cystic fibrosis clinical practice (Preceptorship in Cystic Fibrosis) based on the University Children's Hospital was held on February 10-11, 2020 in Zurich (Switzerland) for 25 doctors from Eastern Europe (Slovenia, Poland, Hungary, Czech Republic, Slovakia and Russia).

Prof. E.I. Kondratyeva, Doctor of medical science, Head of the FSBI RCMG Research and Clinical Cystic Fibrosis Department, was able to participate in the module from Russia.

The participants discussed the care organizing principles for patients with cystic fibrosis, including diagnosis and modern approaches to therapy, and the use of CFTR gene modulators, in particular. They discussed the use of methods in clinical practice characterizing functional activity of the chlorine channel (CFTR protein function), such as measurement of differential between nasal and intestinal potentials. The Department of Cystic Fibrosis of the Moscow Regional Consultative and Diagnostic Centre for children (SBIH MR MRCDCC) (Mytishchi, Moscow Region) introduced those methods at the FSBI RCMG clinical base in the Russian Federation.

The participants noted that only 6% of children and adolescents with cystic fibrosis had a chronic infection caused by P. aeruginosa. The infection caused by Burkholderia cepacia complex and Achromobacter spp. is found only among visiting patients. The therapeutic regimens for chronic Pseudomonas aeruginosa apply inhaled aztreonam and levofloxacin, not registered in the Russian Federation. Children receive all medical care on an outpatient basis. Prevention of cross infection, early initiation of eradication therapy, and continuous monitoring of patients (4 times a year mandatory) allowed reducing the incidence of chronic infection.

Specialists of the Clinic spoke about methods of radiation diagnosis, noting that they did not use the computed tomography methods, preferring MRI, as a method that proved to be safe for children and highly efficient. The audience learnt about the work of psychologists and nurses.

Particular attention was paid to transition of adolescents from the Cystic Fibrosis children's center to an adult one and to interaction of pediatric and therapeutic teams. Representatives of the therapeutic center informed in detail about lung transplantation for cystic fibrosis and monitoring of patients after transplantation.

The practical part of the module consisted of a demonstration of spirometry, bodyplethysmography, and sweat techniques. Physiotherapists of the Center introduced the equipment used for kinesitherapy. Russian doctors noted that the Center focused on autogenous drainage and used only one of breathing simulators during a session, while everywhere in Russia doctors use breathing simulators and long-term kinesitherapy. These procedures lead to denial or other psychological problems in children.

They generally use a hypertonic solution of sodium chloride to treat children at the Center. They use Dornase alpha less frequently than in the Russian Federation.

Participants from the Cystic Fibrosis Russian centers were active during the discussion and shared their experiences.