Appointments at the CF-center: which controls should be done?

Question

Hello!
Our child (7 years, CF, underweight) has an appointment at the CF-center every 3 months. Auscultation of the lung is always done and a swab is taken (according to the infection even more often), once a year the blood is controlled. After first infection with Pseudomonas with i.v. therapy and tobramycin inhalation our child is PA negative again and in a stable health condition. Aspergillus antibodies have been detected in the blood, as well as again and again Staph. aureus in the swab.
My sorrows and question is now, if we probably miss something if no lung function measurment or thorax x-ray is performed, respecitvely which investigations have to be done regularly in order to get everything?
Many thanks.

Answer

Dear questioner,
please excuse the long waiting time.
Your child is presented at the CF-center according to the usual time-intervals in Germany. This is every three months and in case of additional problems at any time. In other European countries, the appointments are also monthly (e.g. Denmark). Furthermore, the recommendation is to do a microbiologiocal investigation (throat swab/ sputum) in case of any infection of the airways.
The possibility in case of a stabe lung function (measured FEV1) to perform further, probably more sensitive, measurements of the lung (LCI lung clearance index) is not standard yet, should however, in case it is available, taken into account. Also the yearly imaging (MRI, CT or thorax x-ray) can give additional hints on structural changes of the lung, without that those changes are reflected necessarily in the lung function test (measured FEV1).
Therefore, you do not miss anything that is standard, probably additions (see above) are sensible.
Yours sincerely,
PD Dr. Ballmann

18.11.2013

18.11.13
One could interprete from the question, that no pulmonary function test is performed at routine visits.
However, there are European guidelines on this topic (Kerem et al. in the Journal of Cystic Fibrosis 2005, 4:7-26:”Standards of care for patients with cystic fibrosis: A European consensus.”) that can be cited as follows: ..."Patients should be seen every 1-3 months, preferably every month. ...Every visit should include a routine physical examination, measurement of weight, oximetry, age appropriate pulmonary function tests and sputum or cough swab cultures. In children, height and in young children also the head circumference should be measured and recorded on percentile charts. ..."
Therefore, the performance of an age appropriate lung function test is recommended at each visit.
If this is not the case, the patient should discuss this point with his CF-center.

Concerning imaging of the lung (chest x-ray or CT/MRI) the guidelines recommend an annual performance of an imaging. Of course in case of any clinical signs of infections and according symptoms or new findings (such as raising Aspergillus antibodies) an imaging of the lungs is necessary in order to clarify the situation.

D. d'Alquen