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The current and changing world of a cystic fibrosis dietitian

Dietetics

My Health Career is pleased to present a guest blog post from Accredited Practicing Dietitian Nicole Micallef. Nicole coordinates a state-wide cystic fibrosis service (Tasmania).  This role allows her to combine her passions for clinical dietetics, interdisciplinary practice, leadership, chronic condition self-management, and research.  She is currently the Cystic Fibrosis Interest Group Convenor for the Dietitians Association of Australia and is completing her PhD in the area of children’s chronic condition self-management.

“With a little help from health professionals, anything is possible for a person with cystic fibrosis (CF).  People with this condition can – go to university, have successful careers, be parents, write piano concertos, win modelling competitions, play professional rugby, and ride a bike from Paris to Istanbul.

CF is a complex condition that affects many part of the body, mostly the lungs and digestive system. Approximately 3000 Australians have CF. Despite new and continually improving treatments, CF remains an incurable and life limiting condition. A simple cold can lead to a serious lung infection and a lengthy hospital admission. If that doesn’t sound bad enough, to remain healthy individuals with CF spend up two hours a day completing preventative treatments. Being a multi-system condition, optimal management involves many different health professionals and care is usually provided at a specialist CF centre. The core ‘CF team’ consists of a doctor, nurse, physiotherapist, social worker and dietitian.

Good nutrition helps people with CF to fight infections and maintain overall good health.

What does a cystic fibrosis dietitian do?

As a CF dietitian, I ‘custom design’ nutrition treatment plans to make sure that every child in my care receives the nutrients and energy (calories) they need. If a child is not growing well, I may recommend the addition of nutritional supplements to their diet, that they eat extra McDonalds (yes, we currently promote a high-fat and high-salt diet) or the placement of a feeding tube. A lot of my clinical time is spent discussing poo, diarrhoea, abdominal cramps, and farting. Approximately 90% of people with CF have difficulty breaking down their food and need to take pancreatic enzymes; it is the role of the dietitian to adjust enzyme doses to alleviate gastrointestinal symptoms. In addition, people with CF often have additional nutritional needs caused by diabetes, osteoporosis (thinning of the bones), liver disease, pregnancy, transplant or even additional exercise.

How can I see my role as a cystic fibrosis dietitian changing in the future?

Change is inevitable. While I can’t predict the future, there are five trends that I think will continue to change the way that CF dietitians work. These are:

  1. Emerging gene-therapies for CF. In 2013, the Australian government approved Ivacaftor* for people with CF who have a specific genetic mutation (genotype G551D, 5% of the CF population). This drug is the first to address the cause of CF and opens the door for more research into gene-therapies. From a nutrition perspective, overseas data show that once people start on Ivacaftor they gain substantial weight and require less or no pancreatic enzymes. More individualised tailored dietary management based on CF genotypes will be required.

*Ivacaftor is currently not reimbursed for Australians and is estimated to cost $300,000 per year, per patient.

  1. An aging, better nourished CF population. Due to malnutrition being less and less prevalent, I predict that CF dietitians will start recommending a Mediterranean style diet similar to what we currently suggest for the general Australian population. Studies completed in non CF populations, and some in CF, show that a Mediterranean style diet (or its components) reduces inflammation and promotes longevity.
  2. An external quality focus and benchmarking.  Peer review (our colleagues and clients reviewing our work and determining if it is at a high enough standard) and openly published benchmarking results (comparisons of the performance of CF centres) will help to identify better ways of providing nutrition care.
  3. A team based approach to nutrition.  Good nutritional outcomes require the support whole CF team. Great CF teams consist of people who complement each other and push the group’s performance above that that could be achieved by an individual alone. Dietitians have the training and expertise to play a both a decisive role in the direct nutrition treatment of people with CF and in mentoring of the other disciplines involved.
  4. Telemedicine and technology. Many people with CF live far away from their specialist CF centre. CF Dietitians in Queensland and Tasmanian have recently started successfully using videoconferencing to reach patients in regional/remote areas.  Email consultations may also help facilitate communication between CF dietitians and their clients. In addition, increasing technologies (e.g. apps designed for tablets) will empower people with CF to manage their own diet. Dietitians who can embrace and/or develop these technologies will be well ahead of the pack.

Do I have any advice for people considering becoming cystic fibrosis dietitian?

All dietetics courses accredited by the Dietitians Association of Australia will teach you the basic skills to work with clients with CF. However, to secure a job in a specialist CF team you generally require a minimum of 5 years clinical dietetic experience and you need to demonstrate considerable advanced dietetic skills.  Many CF dietitians have additional (post graduate) qualifications in areas such as paediatric dietetics, diabetes education, chronic condition management, research, and public health. Mentoring is available to all new CF dietitians.

Photo: 10th Australasian Cystic Fibrosis Conference. Left to right = Dr Carli Armstrong (intern), Dr Ingrid Els (paediatrician) and Nicole Micallef (dietitian). Written permission to use photo has been provided via email.  

Nicole Micallef B.Bio.Med.Sci (Hons 1), M. Nutr. Diet., G.Cert.Health. (Self-management), APD/AN”

When we first published this article, we had people on social media who asked questions about why a dietitian would be recommending McDonalds! Nicole has clarified this in the comments below. Amanda – Founder MHC :-)

8 replies to “The current and changing world of a cystic fibrosis dietitian”

  1. Clarifying my McDonalds comment

    Individuals with cystic fibrosis require more energy from food (120-150% more than the average person) to maintain their weight and to be healthy. This is due to their increased work of breathing and malabsorption. Also, cystic fibrosis is a condition that affects the movement of salt in and out of cells. Individuals with cystic fibrosis lose up to 6 times more salt than people without the condition.

    McDonalds is a great source of energy (high fat = maximum energy) and salt. But, importantly, McDonalds needs to be included as part of a balanced cystic fibrosis diet that also includes wholegrain cereal products, dairy, fruit, vegetables, meat and healthy sources of fat.

    Please let me know if there are any other questions you would like answered – always happy to help.

    1. I was one of those that retweeted a question posted about using McDonalds, and for me the reason it initially raised eyebrows was that “extra McDonalds” was quite a vague statement as not only do McDonalds now have quite a range of foods, but each meal has multiple components which go beyond just being high fat and salt (e.g. many are also high in sugar).

      Presuming that you mean McDonalds beef burgers; do you use that option as a pragmatic choice likely familiar to the kids you deal with and fairly likely to be accepted and eaten? If so, does the type of fat you need these kids to consume matter (e.g. omega 6, omega 3, saturated, monounsaturated, etc) and is that a reason you would suggest e.g. a beef burger?

    2. Hi Nick,
      It’s great to see so much interest in cystic fibrosis nutrition. Many dietitian’s promote what we call the cystic fibrosis food cube – i.e. eat lots of everything including fats and sugar!!! The reason for this is that we know that, at the population level, higher body mass index in individuals with cystic fibrosis equates to much better health outcomes and a longer life. In 1988, there was a landmark cystic fibrosis nutrition study completed at the Boston and Toronto clinics. The major difference between these centres practices was that the Toronto clinic promoted a high fat diet, whereas the Boston clinic promoted a low fat diet to prevent gastrointestinal symptoms (standard practice at the time). Median age of survival of patients attending the Toronto clinic was 30 years old versus the Boston clinic 21 years old. The results of this study started the promotion of the high fat CF diet.

      As you can expect, with treatment advances the life expectancy of individuals with cystic fibrosis continues to increase (recent publications suggest that individuals diagnosed today have a good chance of living into their 50s and beyond). Many of my dietetic colleagues and I predict that with this increasing life expectancy that individuals with cystic fibrosis may start experiencing lifestyle related complications – e.g. cardiovascular disease (currently not seen in the cystic fibrosis population). In my article I mention that a Mediterranean style diet may help reduce inflammation and promote longevity, but we also know that staple fat in the Mediterranean diet (Olive Oil 60-80% mono-unsaturated/omega-9 fatty acids) also helps to prevent against heart disease. So, the type of fat we promote as cystic fibrosis is likely to become increasingly important.

      I hope that this answers your question.

    3. Thanks for the detailed and super rapid reply, Nicole. I felt absolutely obligated to follow up a little and amongst others found this article that details what I presume are the differences between the Boston and Toronto sample groups that you mention:

      http://europepmc.org/articles/PMC1291914/pdf/jrsocmed00142-0014.pdf

      It details that the Toronto group who “were encouraged to eat larger portions than their peers; to add fat in the form of butter or untrimmed meat, and to have high calorie snacks between meals and before bed.” had improved survival rates over the Boston group who were fed a ” a low-fat, carbohydrate-rich diet”.

      Your CVD prediction does seem a bit sadly ironic but it will be interesting to see whether it is in fact the case, considering there is a growing divergence from the generally assumed connection between high fat (not including processed oils in that) diets and CVD, notable in examples like Sweden’s adoption of LCHF dietary guidelines.

      Anyway, thanks for your time. I actually didn’t know a lot about cystic fibrosis until today so this has been educational and interesting.

  2. Thank you Nicole for clarifying the point you made about McDonalds food being something that can be included in the menu plan for people with cystic fibrosis. We had some people on social media who were questioning it. It really does go against many people’s expectations….. dietitians recommending McDonalds!! Amanda – founder MHC. :-)

  3. Hi Nicole,
    Nice snapshot of working life in nutrition. I’m a dietitian working with adults who have cystic fibrosis. The scope and complexity of nutrition needs for this population continues to be dependent on a number of factors in adulthood. High energy foods, including takeaway foods such as Hungry Jacks, McDonalds etc continue to play a significant role for many adults with Cystic Fibrosis.

  4. Below is my response to another comment raised on social medical…
    “I still struggle to see why McDonalds is prescribed over energy dense food.”

    This person raises a very valid point….I agree, energy dense foods (e.g. cheese, yoghurts, milkshakes with added ice-cream and milk powder, extra oils, margarines/butter, avocado, dried fruit, nuts, chips etc) are great way of adding calories to meet the higher energy needs of people with CF. These foods are also a great way of ensuring a balanced diet.

    As a dietitian you need to keep in mind that the eating habits, behaviours and preferences, as well as the nutrition needs and goals of people with CF will affect what they choose to eat. At every consultation, a cystic fibrosis dietitian will assess the patient’s diet history and asses it for nutritional adequacy before making dietary recommendations. If I identify a nutritional problem during a consultation I then work with the child and their family, in equal partnership, to develop a plan that meets the child’s nutritional goals. Takeaway foods (e.g. McDonalds or Hungry Jacks) are often suggested by families as being an easy and manageable way to increase energy intake.

    How I work as dietitian has changed a lot over the last eight years. When I first graduated I was optimistic that I could get everyone to follow the perfect diet. In practice, influencing dietary and other behaviour change is very difficult – people are reluctant to change. Partnering to find solutions becomes particularly important. This is where my passion for chronic condition self-management started.

  5. Hi Nicole,
    I am a CF dietitian in USA looking for some specific advice.
    Please email me at (email address suppressed by My Health Career) if that is not very inconvenient.
    I appreciate your time and looking forward to networking with you.

    Thanks
    Chaitali

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