This document summarizes the results of a survey conducted by the Cystic Fibrosis Trust regarding nutrition and dietetic support for those with cystic fibrosis in the UK. The survey found that 31% of parents and 40% of adults reported having problems maintaining or gaining weight. Additionally, over half of respondents reported issues with digestion such as stomach pains, abnormal stools, and problems managing enzymes. While some centers provided good dietetic support, many respondents felt there was inadequate support, which could be exacerbating nutritional problems for those with cystic fibrosis.

1. Lake E1
, Barnes R1
, Morton A2
, Wolf S2
1
Cystic FibrosisTrust, UK; 2
Regional CF Unit, Leeds, UK
Is enough attention paid to nutrition in CF Centres and Clinics 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).
An important and emotive issue
From the feedback received, it was it was clear that many responders felt very strongly about this
aspect of their health.
“I think diet is probably the hardest aspect of my child’s care. Most people think of Physio and medication in
connection with CF but I find eating and nutrition a constant battle and a never ending process, and in my
child’s case without any great results!”
Parent of six year old
“Even though [her] CF is quite progressed the feeding of her has been the hardest to deal with.”
Parent of 12 year old
Problems with weight
31% of parents and 40% of adults reported putting on or maintaining weight as being an area of
concern.
The table below gives more detail as to the nature of the problems related to weight.
Whilst just over three quarters of parents said their child was growing well, many of these said that
their child was either small for their age or smaller than their peers.
“Yes, but he is shorter compared to his siblings and
peers”
Parent of nine year old
“Youngest is small for age.”
Parent of two year old
It was notable that parents used very emotive
language in this context, seeing the matter of
gaining weight as “a struggle”,“a fight”,“a battle”
and involving a lot of hard work and effort.
“We have to fight to keep him on the 25th centile.”
Parent of six year old
“But it is a constant struggle as she is not a great lover of food.”
Parent of five year old
“He is growing just on the right centile for his weight – however it’s a battle”
Parent of ten year old
Problems with Digestion
54% overall reported problems with their digestion, including ongoing stomach pains, frequent
abnormal stools, blockages and significant difficultly in adequately managing enzymes.
A wide range of specific and general problems were reported, as detailed in the table below, although
as will be noted some responders saw having to take enzymes as a problem in itself.
R E S U L T S
No. No.
Require enzymes 280 Heartburn 7
Stomach pains 278 Problems in the past 7
Abnormal stools 200 Pain after eating 5
Frequent bowel movements 140 Nausea 5
Incorrect enzyme dosage 110 Dehydration 4
Constipation 51 Vomiting 4
Blockages 44 Gallbladder 3
Food Intolerance 35 Problems swallowing 2
Bloated 25 Low vitamin levels 2
Diarrhoea 23 Hiatus hernia 2
DIOS 21 Dismobility of bowel 2
Reflux 13 Piles 2
Flatulence 12 Back pain 2
Effects of medication 11 Pancreatitis 2
When eating out / takeaways 10 Rectal prolapse 2
Indigestion 9 Thrush in gut 1
Slow digestive transit 8 Osteoporosis 1
Stomach acid/ulcers 8 Colitis 1
Soiling / leaking 8 No additional information given 34
Diabetes 7
Not answeredBothNoYes
40%
59%
Is your child growing well?
Not answeredBothNoYes
77%
8%
1%
14%
Not answeredBothNoYes
31%
67%
No. No.
Struggle to gain weight 109 Psychological reasons 6
Problematic when ill 63 Fussy eater 6
Underweight 53 Non CF problems 5
Struggle to maintain weight 51 Not taking Enzymes 4
No appetite 39 Reflux 4
Had problems in past 36 Anorexia 3
Lose weight easily 35 Pregnant 1
Weight fluctuates 34 No additional information given 88
Overweight 30 Treatments
CF-Related Diabetes 12 Supplements 53
Post transplant problems 9 Eat lots 19
When stressed 8 Enteral feeds 57
Do you have problems with your weight?
(Adults)
Does your child have problems with their
weight? (Parents)
Do you have problems with
your digestion? (Adults)
Does your child have problems
with their digestion? (Parents)
Yes
No
Both
Not answered
Don't know
53%
45%
Yes
No
Both
Not answered
56%
39%
Whilst roughly half of those responding to this survey felt that the gastro-intestinal aspects of CF
were well managed and well controlled, half experienced significant problems, which were of real
concern and clearly created a lot of anxiety.
The question as to whether these problems were inevitable and inherent in the condition or
whether better specialist dietetic support would help alleviate many of these issues is an important
one.
As part of the same survey, the level of specialist dietetic input was assessed.This indicated severe
shortcomings in many cases, which could well be a significant contributory factor to these health
problems.
C O N C L U S I O N