1. Lake E1
, Barnes R1
, Morton A2
, Wolf S2
1
Cystic FibrosisTrust, UK; 2
Regional CF Unit, Leeds, UK
Is my CF diet making me ill? A question arising from a recent survey conducted by the CFTrust in the UK
Over the past two years, the Cystic Fibrosis Trust has peer reviewed three quarters of the 50 or so
CF centres in the UK. It has been notable that weaknesses in the service, normally reflecting poor
staffing levels, often include inadequate dietetic support.
Consequently, the Cystic Fibrosis Trust initiated a survey amongst adults with CF and the parents of
children with Cystic Fibrosis. We also separately surveyed Specialist CF Centre dietitians.
It is recognised that a poor BMI, especially when combined with a deteriorating lung function, is
associated with a poorer prognosis for those with Cystic Fibrosis.
I N T R O D U C T I O N
We wished to establish the number of people with CF suffering with weight and digestive problems
and whether as part of the multidisciplinary team, there was appropriate specialist CF dietetic
support, given by dietitians with experience, commitment and time to ensure that the special
nutritional needs of those with CF are effectively addressed.
A I M
The questionnaire used was developed by the Cystic Fibrosis Trust in conjunction with the advice
and recommendations of the joint chairs of the UK Dietitians’ Cystic Fibrosis Interest Group:Alison
Morton (Adult Specialist CF Dietitian) and Sue Wolfe (Paediatric Specialist CF Dietitian).
There were two parallel versions of the questionnaire – one for parents/guardians of children with
CF aged 0-15 years and one for adults with CF aged 16 and over.The content of the questionnaires
was very similar, with the parent’s version having an extra question regarding growth.
The questionnaire was sent to most of the adults with CF (3,500) and just under half of the parents
of children with CF (1,800) who were on the CF Trust’s database at the time of the mailing.
M E T H O D
Response rate
The survey amongst patients resulted in a response rate of 21% (595 questionnaires from parents
and 559 from adults).
A significant number reported concerns over the perceived unhealthy nature of the diet they were
advised to eat. They were worried that it would lead to health difficulties in later life. The fact that
they heard repeatedly that a high fat, high sugar diet could lead to heart trouble, cancer and many
other health problems was of concern to them. They felt that as they were beginning to live longer,
these facts should be taken into account in recommending the sort of food they ate. It was clear
that in these areas more information and explanation was extremely important.
Worries about fat content and ÂjunkÊ food
“My dietitian said not to worry what I eat!What about too much junk, what could it do to me? Second
opinion please! I mentioned fatty liver but they say they don’t worry about stuff like that.”
Adult
“I believe that with CF sufferers living longer, the dietitians need to adopt a more holistic approach to their
diet, to ensure that all of them remain as healthy as possible.”
Parent of a 12 year old
“Each child is different and I don’t like the idea of a full fat, high sugar diet. BUT stop CF dietitians
recommending bad fats and calories!!There are good alternatives!!”
Parent of a nine year old
“I have always wanted my daughter to eat a healthy
balanced diet. However the dietitians that we have
come across seem unable to see beyond the high fat
message, even for a child growing as well as mine.”
Parent of a 12 year old
“The CFTrust should look at the long term health
consequences for health of the CF patient and family
of prescribing a high fat diet. People with CF may live
along time now, to the age of heart disease etc.”
Adult
“Also [would like] advice regarding potential disadvantages of CF diet.”
Parent of a one year old
R E S U L T S
These understandable concerns and beliefs indicate the need for and importance of better education
and information in relation to the nutritional needs and digestive problems of those with Cystic
Fibrosis.
In this health and food conscious age, those with CF and indeed those who care for them, including
schools, need to understand the importance of an appropriate diet to maximise the length and
quality of their life. Unless this is addressed and achieved, many children adolescents and adults may
make food choices which are detrimental to their long-term health interest
Sugar and Cystic Fibrosis Related Diabetes
Some were concerned that a diet high in carbohydrates and particularly sugars may well be why so
many people with Cystic Fibrosis developed Cystic Fibrosis Related Diabetes.
“Dietitians always push high sugar food. People with CF are at a high risk of
developing diabetes; therefore pushing high sugar foods is extremely stupid. I
personally have chosen to ignore this advice.”
Adult
“Recently my glucose tolerance test showed that I have IGT and when I was
talking to my nurse about this she said my high sugar diet was a
contributing factor.”
Adult
“I believe too much emphasis is put on eating sugary sweet foods but we
also need to remember that CF is linked to diabetes.”
Adult
“What [food] should I be avoiding to prevent diabetes etc?”
Adult
Eating at school
Given the emphasis on healthy eating in society in general and in schools in particular, many parents
reported considerable difficulty in ensuring their child had an appropriate high-fat, high-calorie diet
whilst at school as well as having easy access to their enzymes. This is something that clearly needs
to be addressed in a robust manner.
“Schools don’t understand. My son won’t take high calorie foods to school, I then have problems getting the
calories in after school that he needs. I have mentioned this to the dietitian.”
Parent of a ten year old
“I am concerned when he starts school as in the School he will hopefully attend, they have a healthy eating
policy which prevents children eating the foods my son needs to eat.”
Parent of a three year old
“We need help on educating schools on the importance of a high fat /calorie diet for a child with CF.”
Parent of a four year old
“I don’t think the importance of CF diet is stressed enough and therefore overlooked so as usual the child
suffers later on in the day or in the evening – the school doesn’t see any of it.”
Parent of an 11 year old
“I feel more support could be given. For example helping by doing school visits as there are many problems
with unhealthy pack lunches and snacks etc.”
Parent of a ten year old
“Mostly when he forgets Creon with packed lunch … Dinner ladies tend to segregate him when they are in
his lunch box.”
Parent of a seven year old
“There have been issues at School in understanding Creon requirements”
Parent of a six year old
C O N C L U S I O N