CF center care

Question

Hello, my daughter is followed at a certain CF center.
I am in contact with other parents whose children are followed in other CF centres. We discuss together the ways how the monitoring of our children is proceeded.
I realized that the monitoring of my child was not as regular as others’.
Her last appointment was in March, the next will be in August. The last sputum examination was in November 2012. In other CF centres it is scheduled every two months.
Similarly, the CF centre has not considered it to be useful to write an "Individualized Homecare Plan" for the childcare centre. So I asked my non-CF specialist paediatrician to complete one.
I wonder whether this lack of regularity in monitoring my daughter could have adverse effects on her future. Then, should I require more regular monitoring, or must I have full confidence?
Thank you for your answer.
Regards

Answer

Hello,
The October 22th 2001 decree of the French Ministry of Health defines specifications which must be met by a centre to be acknowledged as a “qualified CF centre”: the centre which follows your daughter is one of them.
Setting up an "Individualized Homecare Plan" is clearly a mission of the CF centres and is particularly justified when enrolling in a childcare centre or when moving to another school.
The monitoring by a multidisciplinary CF team has been specified in the National Protocol for Diagnosis and Care in CF (NPDC-CF) published in 2006 by the French Health Authority and is regularly updated. It recommends a minimum of 4 visits per year at the CF centre. This frequency can be increased because of age (once a month up to the age of 6 months and every 2 months up to the age of 2 years) or of a health event.
The purpose of regularity in monitoring is to preserve a good health status by detecting events that are more likely to evolve favorably if treated early. This is the case of bronchial colonization by new microbials (as e.g. Pseudomonas) which can be totally asymtomatic but nevertheless require:
- an early treatment to prevent or delay chronic colonization;
- and the implementation of hygiene measures to prevent cross-contamination between patients.
This is why the NPDC-CF advocates the creation of a systematic bacteriological examination (throat swab or sputum culture as soon as the child can spit) at each visit.
These recommendations may not be strictly enforced in particular situations related to:
- either the disease, in the case of CF borderline forms (also called CFTR-related disorders) combining mild clinical signs and an intermediate or negative sweat test: if the monitoring can be spaced, it must nevertheless be maintained at a steady pace, a shift to a classic form of CF being possible;
- or a difficulty in the CF centre organization due to a temporary lack of professional resources.
I may suggest you to :
- firstly, discuss your daughter’s health situation and monitoring with her CF doctor;
- secondly, if you do not get a satisfactory answer, contact the representative of your CF Center parent group if it exists
- ultimately, contact the Committee for Relations with Users and Quality of care (CRUQ ). This committee, established by the Law of March 4th 2002, exists in every public or private health care institution. Decree of March 2nd 2005 specifies the tasks, composition, operating rules and the procedure for handling complaints and claims.
Hoping this can help,
Cordially,
Dr Gilles RAULT

13.08.2013

20.08.13
On a European level, there are detailed guidelines on the topic ”Standards of care for patients with cystic fibrosis: A European consensus.” published by Kerem et al. in the Journal of Cystic Fibrosis 2005, 4:7-26. Here a short citation on the question of how frequent patients should be seen by their CF-team: "...patients should be seen every 1-3 months, preferably every month. Newly diagnosed infants or patients with severe disease should be seen more often and those with mild phenotypes or atypical CF may be seen less often, every 3-6 months...."
D. d'Alquen