Question and Answer
Every newsletter we encourage you, the reader to submit questions to our expert panel of specialists. Thanks goes out to Dr. Adam Jaffe from Sydney for organising the panel, as well as all of the medical community that volunteers their time to answer these questions.
If you have a question that needs answering – be sure to send it to facebook@cysticfibrosisaustralia.org.au
Question: What’s the easy way to get teenagers to be compliant with their treatment and medications??? Even when they know what happens when they don’t do it or take it.
Thanks to Dr. Donald Payne Answer:
I’ll start by re-phrasing the question so that it is a little kinder on young people with cystic fibrosis.
‘What is the most effective way to help young people with cystic fibrosis adhere to a complex, daily treatment plan?’
There is no single way to address this issue. There are a number of possible strategies to help young people adhere to a treatment plan. Shouting, crying and bribery will help a few, but not many.
Before outlining the options, it is helpful to think about what happens during adolescence. This is the time when young people move from being dependent children to independent adults. This process inevitably involves questioning and challenging others (particularly parents) and experimenting with different choices and behaviours. For all adolescents, these choices may have a detrimental effect on their health. For adolescents with cystic fibrosis, the health consequences of these choices (e.g. smoking, not sticking to the treatment plan) may be especially problematic. It is difficult for parents and health professionals to stand back and watch - and neither should we. However, in this situation it is worth considering what is the most effective way to help young people look after their own health.
There are quite a few published studies, which look at the reasons why adolescents with chronic illness do and do not adhere to treatment. Reasons for wanting to take regular treatment include:
• It allows me to take part in sport
• I can do whatever my friends are doing
• I sleep better
• I’ve stopped a few times in the past and got really sick
Identifying positive aspects of adhering to treatment is therefore important, as these provide some motivation for adolescents to continue with treatment. However, it is also essential to acknowledge the reasons why adolescents may not adhere to treatment. These include:
• I forget (the most commonly reported reason)
• The treatment doesn’t work
• It’s too complicated
• I’ve got more important things to do
• I’m worried about the side-effects
• I don’t need it
• I can’t be bothered
Discussing these issues can help to highlight worries, lack of knowledge or myths about treatment. It can also identify factors which are getting in the way of treatment and provide an opportunity to discuss ways to address them. Health professionals should take the opportunity to talk to young people about these issues on their own, separate from their parents, and give young people some assurance that what they talk about will be treated confidentially and respectfully. Finally, it must be remembered that poor adherence to treatment may be a symptom of significant sadness and depression - common problems in adolescents with cystic fibrosis, which need to be acknowledged and treated appropriately.
Question: What happens to patients after transplant, what symptoms do they still suffer from? How likely is it for a 3yr just diagnosed only because her baby brother has it to have full on symptoms develop?
Thanks to Dr. Glen Westall Answer:
1. Lung transplantation is not a cure, but rather a treatment for CF patients with end-stage lung disease. It aims to improve the survival and quality of life of CF patients with severe life-threatening disease, allowing them the opportunity to achieve life goals that are important to them.
Following the transplant operation children remain in hospital for 2-3 weeks. It takes about 8 weeks for wounds to heal and 3-4 months before a child’s strength returns to normal. During this time an extensive rehabilitation program aims to increase confidence, muscle tone and overall fitness. Children usually return to school about 3 months after the transplant operation. Physical activity is strongly encouraged.
After the transplant, a number of medications are given for the rest of their lives to prevent rejection and infection. While patients require life-long review at a transplant centre, it is expected that they will get back to a normal life following lung transplantation.
2. The future outlook for patients with CF is optimistic and outcomes have improved dramatically in the last few decades. Earlier diagnosis and more effective therapy, including lung transplantation for some patients, may explain the gratifying increase in overall survival of patients with CF. Equally important is attention to nutrition, physiotherapy and early aggressive treatment of chest infections. We now know that a significant proportion of children born today with CF will survive to become adults, and will expect to lead productive careers. Indeed, children born today are now expected on average to live into their 50’s, with many living longer.
Question: As a CF patient, I have always been told to never use an over-the-counter cough medicine, but I never understood why? I understand not using a suppressant, but I don't understand why we can't just use a "chesty cough" expectorant over-the-counter cheap cough medicine (rather than disgusting tasting, expensive Brondecon)????
Thanks to Dr. Dominic Fitzgerald Answer: Over the counter cough medicines have not been shown to help children recover from chest infections. This is independent of any considerations regarding cystic fibrosis. Most children have a disruptive cough for a couple of nights due to a viral infection. The medications have several problems. Firstly they may suppress the cough which is not desirable for children with CF who need to clear their airway secretions to improve their breathing comfort and reduce the risk of a more serious bacterial infection needing antibiotics. Secondly, for all children, they may be given in inappropriate doses which may make them more sleepy, increase their risk of other complications from the medicine such as seizures or delay the time to present to a doctor to have a chest infection or asthma/wheezing diagnosed and the appropriate treatment started. One study has shown that children will do as well with a teaspoon of honey!
Question: Our 1yr old son had 3 neurological ops, including having a VP shunt (from lateral ventricle in brain to abdomen) installed, before then being diagnosed with CF. He recently had his first shunt blockage where he was admitted through emergency which then resolved itself after 36hrs. Is it possible that his CF could cause a shunt blockage - ie could CF impact his cerebrospinal fluid and thus, cause a blockage of the shunt at its top in the ventricles or could a CF plug block the exit of the VP shunt into the abdomen?
When we were in the hospital for the shunt, the neurological team advised they did not know and had never been asked such a question.
Thanks to Dr. Sarath Ranganathan Answer:
'CF affects about one in 3000 people in Australia. Although this makes it one of the commoner inheritied conditions it is not so common that neurologists would know all about it and many doctors need to refer questions about CF to a specialist. There a very few children with CF who also have a VP shunt and the two problems are totally unrelated. Although the thick mucus in CF affects different parts of the body such as the lungs, gut, pancreas and, in boys, the reproductive organs, it does not cause problems in the brain.
The end of the VP shunt does not actually go into your son's gut but lies in the abdomen outside the gut (the peritoneal cavity) and is not affected by him also having CF. The VP shunt your child has helps to decrease the pressure in the brain by draining some of the fluid into his abdomen. Unfortunately, these shunts can block and this is unrelated to him also having CF. When the shunt blocks the pressure can build up again and so it is important to seek medical help whenever you suspect that this is happening.'
Question: I had a friend pass at the start of this year that suffered CF, and I was perplexed about the nutrition advice that she got when she was in hospital. I am half way through my Bachelor of Health Science (Nutritional Medicine) and would like to know what information you have been given as far as foods to focus on as perhaps in the future I may help to support the health of someone suffering from this horrible disease (I know 2 other people that have it) and may even find some improvements.
Thanks to Tamarah Katz Answer:
Thankyou for your question.
When talking about nutrition it is really a case of one size fits most but not all. Nutrition advice should be tailored to the individual’s health, activity levels as well as the particular CF genes they have. However for the most part those with CF need a high energy, high fat, high protein diet.
An individual with CF needs anywhere from 120-150% of usual energy requirements. This is because they burn more energy breathing and fighting infections and they don’t absorb all the energy from the food they eat, even when compliant with enzyme therapy. The best way to achieve their high energy needs is to eat nutritious foods high in fat such as dairy products as well as adding fats to foods such as margarine to vegetables, this is because fat is much more energy dense than protein and carbohydrate, with 1g of fat equal to 9 calories and 1g of carbohydrate and protein equal to 4 calories, less than half that of fat. Because fat is so energy dense the volume of food you need to eat to meet your requirements is much less. For example to eat 1000 calories a day you would need to eat 18 pieces of bread or 9 pieces of bread spread with margarine, which is much more achievable! It is important to not mistake a high fat diet for a fast food diet but rather use healthy foods as a base on which to add extra fats. Sometimes conventional food is not enough to meet energy requirements in someone with CF and they are prescribed a high energy supplement drink and occasionally they need to be fed via a tube into their stomach overnight. It is often a real challenge for someone with CF to maintain their weight and it is fantastic that you have taken an interest in the area in order to help your friends. I am sorry for your loss and hope this information may be of some use. There are Australasian Clinical Nutrition Guidelines which were produced by the dietitians that work in this area across Australia and New Zealand, they are very comprehensive and contain a lot more information, you can find them on the CF Australia website.
Question: My daughter is 9 and has trouble with compliance i also have trouble with compliance as i want her to be "normal" we will often miss weekends of medications or physio but will pick it up again during the week at most we will miss 4 days in a row how detrimental is this for a cf person and would this cause super bugs that are not treatable.
Thanks to Dr. Claire Wainwright Answer:
Compliance or adherence as we now call it is an issue for all patients and families with chronic health problems that need long term treatment. For most long term therapies there is on average 50 percent adherence. What this tells us is that your problem is very common. There is an ever increasing burden of treatments needed for patients with CF and their families and while we would like to have 100 percent adherence with all the treatments we use we know that this is unlikely to happen. We would love to have a magic answer however there isn’t one! It is hard to predict what the risks are for any individual from lack of adherence without knowing the full health care story and treatments being used. The best solution is to be very honest with your treating team and discuss all the options and strategies with them. It may be that there are ways to improve your adherence and your child’s adherence. Your team may feel that some therapies are more important than others for your child so that you might be able to concentrate on specific therapies or there may be some factors like difficulty managing the cleaning of respiratory equipment like nebulisers or airway clearance equipment that do increase the risk of difficult infections that they can specifically talk to you about. If you can’t talk to your doctor about this then try and talk to your CF nurse, clinic coordinator or allied health staff. No one will think you are a “bad” parent because you admit to problems with adherence, they will understand and be very pleased to help you.
