Cystic Fibrosis (CF)

Cystic Fibrosis (CF), rhSP-D in combination with a synthetic surfactant

Cystic fibrosis is an inherited chronic disease that affects the lungs and digestive system of about 30,000 children and adults in the US (70,000 worldwide)[1]. A defective gene and its protein product cause the body to produce unusually thick, sticky mucus that clogs the lungs and leads to life-threatening lung infections. Host defence may be impaired due to the oxidation of surfactant protein D and may contribute to the suppurative lung diseases like CF[2]. Recombinant SP-D has been proposed as a therapeutic option for CF[3]. Inhaled antibiotics are used to combat the lung infections, but antibiotics must be used on an intermittent basis due to concern about the patient developing antibiotic resistance. Patients with CF will require an aerosolized surfactant carrier for aerosolized delivery of rhSP-D, as opposed to neonates who are intubated and administered a surfactant by way of tracheal injection. Airway Therapeutics is in the process of acquiring an aerosolized surfactant product to be the carrier for rhSP-D in the treatment of CF. It is anticipated that the aerosolized surfactant-rhSP-D combination will not only loosen and reduce the volume of mucus secretions due to the actions of the basic surfactant, but also fight infections by way of the rhSP-D. It is anticipated that an aerosolized surfactant-rhSP-D combination will be able to displace much of the need for inhaled antibiotics, and become first-line therapy for all patients with CF lung infections. At present, at least 50% of CF patients in the US regularly use an inhaled antibiotic[4], and that use may be increasing[4].

References

  1. Cystic Fibrosis Foundation. Available from: http://www.cff.org/AboutCF/.
  2. Starosta, V. and M. Griese, Oxidative damage to surfactant protein D in pulmonary diseases. Free Radic Res, 2006. 40(4): p. 419-25.
  3. Hartl, D. and M. Griese, Surfactant protein D in human lung diseases. Eur J Clin Invest, 2006. 36(6): p. 423-35.
  4. Moskowitz, S.M., et al., Shifting patterns of inhaled antibiotic use in cystic fibrosis. Pediatr Pulmonol, 2008. 43(9): p. 874-81.