Russia is going to have products for children with Pierre Robin syndrome soon


Therapies for blood clotting disorders and products for children with Pierre Robin syndrome will appear in the Russian Federation. This was reported in the press service of the Circle of Kindness Foundation.

“The All-Russian Society of Hemophilia has applied to the Circle of Kindness Foundation with a request to include hereditary deficiency of blood clotting factor XIII in the list of severe life-threatening and chronic diseases, which also includes rare (orphan) diseases.  On June 23, the Foundation added this rare genetic disease to the list of nosologies it supports, and as early as on June 30 it included Human Coagulation factor XIII Concentrate (TN Fibrogammin II) in the procurement list,” the message says.

There are about 60 patients with hereditary deficiency of blood clotting factor XIII in the world. The incidence is about 1 in 2,000,000 and, as a rule, affected children at an early age. Fortunately, the number of such patients in Russia is very low. Fibrogammin II, the drug included in the procurement list, is not registered in the Russian Federation, but researchers have a high estimate of its clinical results, the Foundation noted.

Another pathology, Pierre Robin syndrome, was included in the list. Children with this pathology are born with an underdeveloped lower jaw, cleft palate, and backward displacement of the tongue (“bird face”). The main problem with this congenital ailment is apnea, i. e., obstructive breathing disorder, as a result of which the root of the tongue narrows or completely blocks the patient’s airways.

“To ensure proper breathing of such patients, it is necessary to eliminate the underdevelopment of the lower jaw by compression-distraction osteosynthesis. Therefore, the Foundation included in the list of purchases external fixation compression-distraction devices that reduce postoperative complications. This will help to normalize the patients’ breathing completely,” the Circle of Kindness explained.

The Ministry of Health proposed to allow the Circle of Kindness Foundation to help patients over the age of 18.