PULMOZYM

Active material: Dornase alpha
When ATH: R05CB13
CCF: Mucolytic drugs, used in cystic fibrosis
ICD-10 codes (testimony): E84, J15, J44, J47
When CSF: 12.02.01.01
Manufacturer: F.Hoffmann-La Roche Ltd. (Switzerland)

Pharmaceutical form, composition and packaging

The solution for inhalations clear, colorless or slightly yellow.

Excipients: sodium chloride, Calcium chloride dihydrate, water d / and.

2.5 ml – sealed plastic (6) – multilayer foil containers (1) – packs cardboard.

Pharmacological action

Mucolytic drugs, used in cystic fibrosis. Is a recombinant human dezoksiribonukleazu (DNazu). Dornaza Alpha is obtained using a genetically engineered version of human natural enzyme, which cleaves the extracellular DNA.

In cystic fibrosis accumulation of viscous purulent secretions in the respiratory tract plays an important role in the violation of the external respiration function and the worsening infectious processes. Purulent secret contains very high concentrations of extracellular DNA, which is a viscous polyanion, released from decaying leukocytes, which accumulate as a result of exposure to infectious factors.

In vitro dornaza Alfa hydrolyzes the DNA in the sputum and significantly reduces viscosity patients with cystic fibrosis.

Pharmacokinetics

Systemic absorption dornazy Alpha after inhalation aerosol is low.

OK DNaza is present in human serum. Inhalation of Alpha dornazy in doses up to 40 mg for a period of up to 6 days does not lead to a significant increase in the concentration of DNazy in serum compared to normal endogenous level. The value of dornazy in serum Alpha concentration does not exceed 10 ng / ml.

After the appointment of Alpha dornazy the dose 2500 ED (2.5 mg) 2 times / day for 24 weeks of concentration of serum DNazy did not differ from the averages to therapy (3.5± 0.1 ng/ml), that testifies to the low systemic vsasavanii and small cumulation.

In cystic fibrosis average concentration of Alpha dornazy in sputum, through 15 minutes after inhalation drug dose 2500 ED (2.5 mg) approximately 3 ug / ml.

After inhalation concentration of Alpha dornazy in serum rapidly declining.

Testimony

-symptomatic therapy in combination with standard therapy of cystic fibrosis in patients with an FVC not less than 40% from the norm.

Pul′mozim can be used to treat patients with some chronic lung diseases (bronchiectasis, COPD, congenital lung development in children, chronic pneumonia, immunodeficiency states, proceeding with the defeat of the lungs, etc.), if a doctor to assess the mucolytic effect of dornase alfa provides benefits to patients.

Dosage regimen

The drug is prescribed in form of inhalations, who spend using the Jet nebulizer.

Dose 2.5 mg (consistent with the contents of the drug 1 ampoule – 2.5 ml nerazvedennogo solution, 2500 ME) 1 time / day. Some patients over 21 year of the drug may be more effective at the time of appointment 2 times / day.

Most patients have an optimal effect can be achieved with constant daily use Pul′mozima. The results of the research, in the course of which dornazu Alpha appointed in discontinuous mode, show, that after the cessation of therapy effect of the drug (improvement of lung function) quickly disappears. Hence, patients should be advised to the daily use of the drug without interruption.

Complex therapy should continue, including chest physiotherapy.

In acute respiratory infections while therapy Pul′mozimom you can continue to use the drug without any risk to the patient.

Terms of use of the drug

The solution in the ampoules is only intended for a single use of the inhalation.

Pul′mozim cannot be diluted or mixed with other drugs or solutions in the capacity of the nebulizer. Mixing the drug with other drugs can lead to undesirable structural and/or functional changes of Pul′mozima or other components of the mixture.

The entire contents of one ampoule must be poured into the container of a jet nebulizer/compressor type Hudson T Up-draft II/Pulmo-Aide, Airlife Misty/Pulmo-Aide, modifications Respigard/Pulmo-Aide or Acorn II/Pulmo-Aide. Pul′mozim you can enter Jet-nebulajzerom/reusable type compressor Pari LL/Inhalierboy, Pari LC/Inhalierboy or Master, Aiolos/2 Aiolos, Side Stream/CR50, Mobil Air or Porta-Neb.

Ultrasonic nebulisers are not suitable for the introduction Pul′mozima, tk. they can inactivate dornazu Alpha or have invalid aerosol characteristics.

Patients, Unable to breathe through the mouth during the entire period of inhalation via nebulizer, can use the nebulizer Pari Baby with tight facial mask.

The patient must be warned about the need to adhere to the manufacturer's instructions for operation and care of nebulajzerom/compressor.

Side effect

Unwanted reactions when treating Pul′mozimom rarely occur (< 1/1000), in most cases, mild, are transitory in nature and do not require dose adjustment.

From the hematopoietic system: acute lymphocytic leukemia, aplasticheskaya anemia.

CNS: seizures, migraine.

From the senses: conjunctivitis, imbalance.

Cardio-vascular system: tachycardia, bradycardia, cardiac arrest.

The respiratory system: pneumonia, bronchitis, respiratory tract infections, incl. caused by Pseudomonas, pharyngitis, laringit, voice alteration (hoarseness), breathlessness, rhinitis, the deterioration of lung function, decline in respiratory function, respiratory insufficiency, hemoptysis, pneumothorax, pneumorrhagia, throat polyp, cough, increased bronchial secretions, alveolitis, gipoksiya, bronchospasm, productive cough, pleural pain in the chest.

From the digestive system: dyspepsia, stomach ache, hematemesis, hepatic failure.

Dermatological reactions: rash, itch, dermatitis, purpura.

Allergic reactions: hives, angioedema.

Reproductive system: miscarriage, complications during pregnancy, spontaneous abortion, medical abortion.

From the body as a whole: fever, weakness, malaise, death.

Patients, experiencing adverse effects, match the symptoms of cystic fibrosis, can, usually, continue to use Pul′mozima. Adverse events, which led to the complete cessation of treatment Pul′mozimom, We observed in a very small number of patients, While the frequency of interruption of the therapy was the same in appointing placebo (2%) and dornazy alpha (3%).

Dornaze Alpha antibodies detected less than 5% patients, However, none of them they were not IgE class. Improvement in lung function was observed even after the appearance of antibodies to dornaze alpha.

Contraindications

- Hypersensitivity to the drug.

FROM caution the drug should be used during pregnancy, lactation (breast-feeding), in children age 5 years.

Pregnancy and lactation

Safety of Pul′mozima in pregnancy has not been established. The drug should be used with caution and only in cases, when the intended benefits to the mother outweighs the potential risk to the fetus.

Unknown, whether Alpha dornaza stands out with breast milk in humans. The drug should be used with caution during lactation (breast-feeding). Given the minimal system suction dornazy alpha, don't expect measurable concentrations of Alpha dornazy in human breast milk among women.

IN experimental research animals not received data about violation of fertility, teratogenic properties or on the impact of the drug on fetal development. Lactating cynomolgus macaques have, treated with high concentrations of Alpha dornazy/in, were detected in low concentrations of Alpha dornazy in breast milk (<0.1% from serum concentrations).

Cautions

Against the backdrop of Pul′mozima it is necessary to continue the regular monitoring of the patient.

After the start of therapy Pul′mozimom, like any spray, lung function may be reduced, and discharge of sputum increase.

Efficacy and safety of Alpha dornazy in patients with FVC less than 40% from the norm is not installed.

Use in Pediatrics

Experience of application of Pul′mozima in children under the age of 5 years limited. We found no differences in safety profile in children under 5 years and older age group from 5 to 9 years. After the end of treatment have not been identified to increase the frequency of serious adverse events in children under 5 years, receiving Pul′mozim. Assign children up to Pul′mozim 5 years should only, If the potential advantage to improve lung function exceeds the risk of respiratory tract infections.

Effects on ability to drive vehicles and management mechanisms

On the influence of the drug on a patient's ability to drive, and other potentially hazardous activities not available message.

Overdose

The cases of drug overdose not registered Pul′mozim.

IN experimental studies on laboratory animals shows, that a single inhalation formulation in doses, greatly exceeding recommended (to 180 time), well tolerated. Oral administration of Pul′mozima rats at doses up to 200 mg / kg are also well tolerated.

In clinical studies, the drug was prescribed to patients with cystic fibrosis in the dose to 20 mg 2 times / day for 6 days or 10 mg 2 times per day by intermittent scheme (the two-week reception, a two-week break) for 168 days. Both dosing regimens were well tolerated.

Drug Interactions

The product is compatible with standard drugs for treating cystic fibrosis (antibiotics, broncholytics, digestive enzymes, vitamins, analgesics, SCS for systemic and inhalation use).

Pharmaceutical incompatibility

Pul′mozim is an aqueous solution without buffer properties. It should not be diluted or mixed with other drugs or solutions in the capacity of the nebulizer. Mixing can lead to undesirable structural and/or functional changes of Pul′mozima or other components of the mixture.

Conditions of supply of pharmacies

The drug is released under the prescription.

Conditions and terms

List B. The drug should be stored in the dark, inaccessible to children at 2 ° to 8 ° C. Shelf life - 2 year.

A single short action of high temperature (no more 24 hours at temperatures up to 30° c) does not affect the stability of the drug.