Please note: While some information will still be current in a year, other information may already be out of date in three months time. If you are in any doubt, please feel free to ask.

Nutrition

Question
Hi

My daughter - 3 years old - is under good care in the Warsaw Mother and Child Institute - cystic fibrosis department. She doesn't need enzymes.
Every day she takes 2 ml AquAdeks witamins, 6 - 10 ml 10% NaCl, inahaltions from 4 ml 5%NaCl and loung clearing by hand two trimes a day.
So far she is healthy but I have some questions about using diet supplements every day- extra minerals, synbiotics and omega-3.

As far as supplements - our doctors say - natural diet will do the job - but it is hard to make a 3 year old eat as much as recommended. The only thing they prescribe is AquAdeks and salt.

I trust our medical stuff in many ways but also feel that in some ways they are behind to the more developed countries.
I had contact with parents who had to go abroad with their children - and they confirmed this.

I can use diet supplements on my own - but would like to hear the opinion of other peaople or medical staff.

Also, I would like to know if it is recommended to use The Vest .
Thank you
Answer
Dear Parent
Many thanks for your question. Whilst we are unable to give specific recommendations for individuals we can advise you to contact your CF medical team if you have any specific concerns about your daughters’ growth and weight. Appetites can be erratic in childhood. Some days children will eat everything, other days they appear to eat almost nothing. This is quite normal and provided that your daughter is growing and gaining weight there is nothing to worry about.

A balanced diet is vital for all the family. This includes dairy products, grains and starchy foods, fruits and vegetables and protein sources such as meat, poultry and eggs. Since toddlers with CF will often eat the same amounts as other toddlers, fortifying the diet should help to provide the additional calories required for a child with CF. The following tips may be helpful:
• Offer whole milk with meals and try whole milk puddings and yoghurts.
• Add cream to cereals and desserts
• Add margarine or butter to vegetables
• Add butter and cream to mashed potatoes
• Add extra cheese to pasta dishes and on top of pizza
Further information can be obtained in the following fact sheets.
‘Eating Well with Cystic Fibrosis’ ( by Carolyn Patchell). This is available from the CF Trust website www.cftrust.org.uk
‘Nutrition for your toddler with Cystic Fibrosis’ (one to three years) Available from the Cystic Fibrosis Foundation www.cff.org
If children with CF are unable to maintain weight gain and growth other forms of nutritional support may be required. This could be in the form of milk or juice based supplements. For a few children overnight tube feeding may be required. Should either of these be necessary your CF doctor or dietitian with you the options available.

If however your child is a picky eater the following tips can sometimes be helpful.
• Establish a regular routine for meals and snacks.
• Sit down at the table and eat as a family so that meal times become a social event.
• Avoid making a fuss or push your child to eat food. If children are forced to eat they will soon learn how to control the situation by being more difficult.
• Avoid discussing your childs’ eating in front of them.
• Praise children if they eat anything, evenif it is only small amounts.
• Give smaller portions of foods. You can always give more.
• Encourage your child when eating but do not let mealtimes drag on. Set a meal time limit of 20 or 30 minutes.
• Do not offer a second meal or snack if the meal is refused. Take the meal away without comment and allow at least 2 hours between meals.
• Be consistent with your approach to feeding, that way your child knows what is expected of them.

In relation to your question to use of omega 3 supplements, a recent review concluded that there is currently insufficient evidence on the safety and effectiveness of fish oil supplements for people with CF to recommend routine supplementation.
Oliver, C et al ‘Omega-3 fatty acids (from fish oils) for Cystic Fibrosis (review). The Cochrane Collaboration, published in the Cochrane Library 2010 issue 10 (www.thecochranelibrary.com)
Finally synbiotics refer to nutritional supplements combining prebiotics and probiotics in a form of synergism examples being:
• Bifidobacteria (probiotic) and fructo-oligosaccharides (FOS, prebiotic)
• Lactobacillus rhamnosus GG (probiotic) and inulins (prebiotic)
I have been unable to find any literature relating to use of synbiotics in Cystic Fibrosis. However there have been in recent years a few small pilot studies looking at the effects of Lactobacillus GG (a probiotic) in the treatment of pulmonary and intestinal inflammation in children with Cystic Fibrosis. Preliminary findings suggest that this may reduce pulmonary exacerbation rates in CF and may have a preventative potential for pulmonary deteroriation. Furthermore intestinal inflammation, a major feature in CF is reduced by probiotics. However it is important to emphasise that these few studies are dealing with a small number of children with Cystic Fibrosis and different doses of probiotics were used. Therefore we can conclude that there is currently insufficient evidence to allow us to recommend routine use of probiotics in children with Cystic Fibrosis.

The Vest is a form of airway clearance and there are many alternatives. There is currently insufficient evidence to allow us to recommend one form of airway clearance over another. Recent information on many forms of airway clearance can be found in the following references:
Flume et al. Cystic fibrosis pulmonary guidelines: airway clearance therapies. Respir Care. 2009 Apr;54(4):522-37.
Bott et al. Guidelines for the physiotherapy management of the adult, medical, spontaneously breathing patient. Thorax 2009;64:i1-i52
These recommendations highlight that there is no evidence to support the use of one airway clearance technique over another. Choice of treatment often depends on patient preference as well as accessibility and cost of the adjunct (e.g. Vest).

Kind regards
Dee Shimmin
Lisa Kent

References
Bruzzese, E. et al ‘Intestinal inflammation is a frequent feature of cystic fibrosis and is reduced by probiotic administration’ Aliment Pharmacol Ther ,2004 20: 813-819.
Bruzzese E et al ‘Effects of Lactobacillus GG supplementation on pulmonary exacerbations in patients with cystic fibrosis: a pilot study. Clin Nutr 2007 26 (3) 322-8.
Infante, P et al ‘Improvements of intestinal function in cystic fibrosis patients using probiotics’ Ann Pediatr (Barc) 2008 69(6): 501-505.
Weiss B et al ‘Probiotic supplementation affects pulmonary exacerbations in patients with cystic fibrosis: a pilot study. Pediatr Pulmonol 2010 45(6) 536-40.







11.03.2011
The answer is edited by: Dee Shimmin