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Disease severity degree

Question
Hello, my 2-year-old CF daughter requires since birth very intensive care. The disease leaves her no lull. Today I am very worried about her life expectancy ...
What are the criteria for severity of the illness?
Are there still children who die young?
Thank you in advance for your answer to this dark question ...
Answer
Hello,
Systematic CF neonatal screening was implemented to avoid mistakes and diagnostic delays and to set up a follow up as early as possible in a specialized CF center. The aim is to preserve the "health capital" of your child, especially for normal growth and dealing carefully and promptly with respiratory infections. This attitude is mainly preventive in order to avoid intervening just when complications are obvious. The fact remains that it represents a heavy burden for parents as disease related care adds to the constant attention that every child demands in the first years of life, even when healthy.
The French CF Registry draws "survival curves" according to the patients year of birth. These curves perfectly match the considerable progress made in recent decades, and particularly since the extension of the neonatal screening in 2001 in France: they show that 99.4% of children screened and diagnosed since then were alive in 2011 (the 2012 report will be available late 2013). Deaths of people with CF are now very rare in childhood and their number is becoming close to that observed in the general population.
Rather than speaking of severity (negative) criteria, I would prefer to speak of positive criteria by considering their value as their evolution in time. Out of them, the main criteria used for both clinical follow-up and clinical research, are:
- Good growth in weight and size;
- respiratory exacerbations (infections) well controlled by treatment;
- absence of chronic respiratory infection, including some germs like Pseudomonas, Cepacia, Mycobacterium;
- absence of associated disease or complications;
- good score for quality of life;
- good respiratory function assessed by the results of functional tests feasible from the age of 5 years (or less for specific investigations);
- good chest CT scan score that can detect early lesions (but requires general anesthesia in young children).
Feel free to share your concerns with your doctor in your CF Center: you can make a comprehensive point of the health of your child, question whether or not to adjust the treatment or run special explorations for seeking a complication or problem that could be treated effectively (asthma, gastroesophageal reflux, infection with an unusual organism, diabetes ...).
You can also ask to meet the other team members that could help you find counselling and consider ways to get ones breath back after two difficult years. The commitment of every moment from birth is such that one needs a break to recharge ones batteries. Discussion with other parents may also provide good and benefit. Some CF Centers organize group sessions between young parents for sharing issues and experiences.
Finally, if the concern persists and you want a second opinion, do know that you have the legitimacy to do so, regardless of the competence of the team following your child. It’s a possibility you may speak freely with your CF Center doctor.
Hoping to have answered your questions I wish you happy holidays if they are coming.
Dr. Gilles Rault
PS: For more information, I suggest you consult the Registry Tool Quality - ROQ - 2012 by clicking on the following link [in French]:
pharem.centre-reference-muco-nantes.fr/downloads/page_publique/docs_importants/09_ROQ_2012_LG_13nov2012_Total.pdf

15.07.2013