The rural family physician spends much time with the parents, reassuring them and keeping them abreast of any new developments in the management of this illness. The child experiences numerous acute exacerbations of her condition, pneumonia and septicemia. She frequently needs IV treatment requiring central lines, cut-downs and jugular access. Repeatedly, decisions have to be made as to when she needs intervention and whether the interventions could be delivered locally or require tertiary support. The rural physician remains in close, frequent contact with the appropriate specialists, who also know this patient well.
Antenatal diagnosis is offered but because of religious convictions, is declined.
Because it is too difficult and too costly to see the specialists at the tertiary centre frequently, the care and supervision of care is taken on by the rural physicians and the small rural hospital and staff. There are multiple, long, in-hospital stays for the daughter with CF, frequent physiotherapy, inhalation therapy, and IV therapy delivered via scalp IVs, saphenous IVs and central lines. She experiences recurrent infections, slow malnourishment, constipation and rectal prolapse, all of which are dealt with locally. A decision is made to insert a permanent indwelling line. The line is found to be colonized and is withdrawn.