Section 10 - Cystic Fibrosis Clinical Care Pathway

Studies to date have used different dosing regimens and it is unclear at
present which dosing interval should be used. This protocol is based on the
current best evidence. Of note, there is no toxicology data for the regular use
of azithromycin for more than six months in patients with CF. It is known
that after four weeks, levels of azithromycin in sputum plateau but because of
the long half-life there is the potential for continued accumulation in tissues.
Important side effects to monitor include; hearing and liver function. Liver
function tests are performed annually. Formal audiological tests are not
routinely performed, unless there are concerns regarding hearing. Changes
caused by azithromycin are usually reversible.
2.2.3 Desensitisation
Occasionally, antibiotic therapy is difficult if patients have become sensitised
to a variety of different classes of antibiotics. In these cases desensitisation
should be considered according to GOSH pharmacy guidelines.
2.2.4 Home IVs
Admission to hospital allows for intensive physiotherapy to be given during
this period as well as providing additional opportunity for attention to dietary
intake and calorie counting. Home therapy, however, is less disruptive to a
child’s routine and may be more appropriate, especially in those older
children requiring frequent courses of intravenous therapy (see Sections 2.4).
Continued attendance at school can often be maintained.
The decision to institute home therapy must be made on an individual basis
after careful consideration of the social situation, expected levels of adherence
and ability to cope with the increased burden of care. Thorough parental and
patient education in the administration of IV antibiotics and other treatment
regimens is essential. Parents should be assessed for competency in
administering IV drugs by nursing staff. The first three doses should be given
in hospital. All home IVs should be co-ordinated with the CF homecare
nurse specialist. Patients must be re-assessed after one week of treatment to
monitor progress and antibiotic levels.
2.3 Nebuliser Therapy
There are three different nebuliser systems and one portable compressor in
use at GOSH. The systems are regularly reviewed and updated and changes
can be expected.
31