Section 10 - Cystic Fibrosis Clinical Care Pathway

Routine monitoring of the pulmonary allograft for rejection and/or infection
incorporates twice-daily spirometry and regular bronchoscopy,
bronchoalveolar lavage and transbronchial biopsy. Acute rejection is treated
by augmentation of immunosuppression with high dose intravenous
methylprednisolone, while further anti-lymphocyte therapy is occasionally
required. Infections are treated according to culture and/or serology and are
most prevalent in the early post-operative period (first three months)
associated with the highest incidence of rejection and, accordingly, most
intense immunosuppression.
The majority of pulmonary infective episodes are due to Pseudomonas species
which often colonise the lung allograft, presumably seeded from the diseased
upper airways and sinuses. Attempts to prevent this colonisation have ranged
from the use of nebulised antibiotics to more aggressive therapy including
regular antral washout and antrostomy.
Management of the non-pulmonary facets of the CF patient must be
maintained, particularly the preservation of gastrointestinal function and a
satisfactory absorptive surface for the essential immunosuppressive drugs.
Adequate pancreatic enzyme replacement, and when indicated aperients and
enteral acetylcysteine are required to satisfy these objectives.
6.3 Survival and Complications
Survival
Survival following lung transplantation for CF is 80% at one year and 60%
at five years. Data from the International Registry suggests that these survival
figures are slowly improving and we suspect this is the case at our centre also,
however, the small number of transplants performed in children in the UK
do not allow us to confirm this as yet. The majority of survivors enjoy a
markedly improved quality of life afforded them by increased pulmonary
function. This enables return to school or college and other age related
activities.
Complications
Mortality in the early post operative period is often associated with episodes
of acute pulmonary rejection and infection, in addition to other
complications related to surgery. After the first three post operative months
the major cause of morbidity and mortality is due to the development of
obliterative bronchiolitis, a progressive obstructive airway disease which may
eventually be associated with a central bronchiectasis and chronic infection.
Its aetiology remains obscure but appears to be immunologically mediated.
Efforts to reverse the pathology with augmented immunosuppression have
been attempted, but once established, the only effective ‘cure’ is retransplantation,
which presently produces poor results and is not practised at
GOSH.
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