Cystic Fibrosis

7 Drugs classified under this therapeutic system

Vitamins (Multi) A 5,751 IU, C 45 mg, D3 400 IU, E 50 IU, B1 0.6 mg, B2 0.6 mg, niacin 6 mg, B6 0.6 mg, biotin 15 mcg, pantothenic acid 3 mg, zinc 5 mg, selenium 10 mcg, K1 400 mcg, beta-carotene 3 mg, E 15 mg, co-enzyme Q10 2 mg/ml..
LIQ: 60 ml. 0-12 mths: 1 ml dly; 1-3 yrs:
2 ml dly.
Supplement of vits., minerals in CF.
C/I: Pregn., 3 mths prior to pregn.

Vitamins (Multi) A 18,167 IU, C 75 mg, D3 800 IU, E 150 IU, K1 700 mcg, B1 1.5 mg, B2 1.7 mg, niacin 10 mg, B6 1.9 mg, folic acid 200 mcg; B12 12 mcg, biotin 100 mcg, pantothenic acid 12 mg, zinc 10 mg, selenium 75 mcg, beta-carotene 10 mg, E 80 mg, co-enzyme Q10 10 mg..
SOFTGEL CAPS: 60. 10 yrs: 1 cap dly;
over 10 yrs: 2 caps dly.
Supplement of vits., minerals in CF,
malabsorp..
C/I: Pregn., 3 mths prior to pregn.

Monobactams. Aztreonam 75 mg.
VIAL (pwdr. & solven. for nebuliser sol.): 2 ml ×75 mg.  Pts. should use a bronchodilator before each dose of Aztreonam. Short act. bronchodilators can be taken between 15 min. and 4 hrs. and long act. bronchodilat. can be taken between 30 min. and 12 hrs. prior to each dose of Aztreonam. For pts. taking multiple inhal. therap., the recomm. order of admin. is as follows: 1. broncho-dilator 2. mucolytics 3. and lastly, Aztreonam. Adult. & child. 6 yrs and old.: 75 mg ×3/d per 24 hrs. for 28 d. should be taken at least 4 hrs. apart. May be taken in repeat. cycles of 28 d. on ther. follow. by 28 d. off Aztreonam ther. See lit.
Indic. for the suppres. ther. of chron. pulmon. infect. due to P. Aeruginosa in pts. with CF aged 6 yrs. and older. Consid. should be given to official guidance on the appropriate use of antibact. agents.
C/I: Hypersens.

Transmembrane Conductance Regulator Potentiator. Ivacaftor 50, 75, 150 mg.
SACHET (granules): 56×50, 75mg.
FILM COAT. TABS: 56×150mg. Pts. wt.<14 kg: 50 mg granules taken orally every 12 hrs. with fat contain. food total dly. dose 100 mg.
Pts.≥14 kg-<25 kg: 75 mg granules taken orally every 12 hrs. with fat-contain. food, total dly. dose 150 mg.
Pts. ≥25 kg: 150 mg tab. taken orally every 12 hrs. with fat-contain. food, total dly. dose 300 mg. See lit.
Tmt. of pts. with CF aged 6 yrs. and older and weighing 25 kg or more who have one of the follow. gating (class III) mutations in the CFTR gene: G551D, G1244E, G1349D, G178R, G551S, S1251N, S1255P, S549N or S549R.
The granules are indic. for the tmt. of child. with CF aged 2 yrs. and older and weighing less than 25 kg who have one of the follow. gating (class III) mutations in the CFTR gene: G551D, G1244E, G1349D, G178R, G551S, S1251N, S1255P, S549N or S549R.
C/I: Hypersens.

rhDNase. Dornase Alpha 2500 IU / 2.5 ml.
INHAL SOLN: (single-use amps): 30 x 2.5
mg/2.5 ml. Use dly. in conjunct. with
standard ther. Not for pts. under 5 yrs.
or with FVC < 40%.
Cystic fibrosis.

Anticholinergic. Tiotropium 2.5 mcg.
SOL. FOR INHAL. (in a Respimat inhaler):
60 actuations (30 med. doses). 2 puffs comprise one medicinal dose. 5 mcg tiotropium given as 2 puffs×1/d at the same time of the day.
In the tmt. of asthma the full benefit will be apparent after several doses of the medicinal product. In adult pts. with severe asthma, tiotropium should be used in addition to inhaled corticoster.(≥ 800 μg budesonide/d or equivalent) and at least one controller. See lit.
COPD: Indicated as a mainten.  bronchodilat. tmt. to relieve sympt. of pts. with COPD.
Asthma: indicated as add-on mainten. bronchodilat. tmt. in pts. aged 6 yrs. and older with severe asthma who experienc. one or more severe asthma exacerbate. in the preced. year.
C/I: Hypersens. to tiotropium bromide, atropine or its derivatives, e.g. ipratropium/ oxitropium or lactose.