CFW Newsletter - Highlights Conference

Emma Wicks, Patient Advocate CF Trust

The Cystic Fibrosis Trust welcomed this year's delegates to the international Conference Centre in Birmingham, UK. This is the second part of my report of the Birmingham Conference which was held in 2004, the 40th Anniversary of the establishment of the CF Trust. For those of you who missed the first part of my report, please refer to the previous issue of CF World Newsletter. Of course, I was not able to get to every session; here are the highlights, and some personal observations of the ones I did attend

Independence

Dr Diana Bilton, from Papworth Hospital, and Professor Duncan Empey, of the London Chest Hospital (both in the UK) gave the clinician’s view of parents’ roles regarding their adult children. Margaret Wotton, a CF mum, and myself provided some insight into the relationship between CF adults and their parents. All agreed that there are no hard and fast rules and that flexibility and good communication were essential.

Trudy Havermans from the University Hospital Gasthuisberg in Belgium, had investigated the levels of agreement between parents and their adult children using a health related quality of life questionnaire. Agreement was found with symptom related aspects, including Nutrition while children and parents disagreed most about treatment with Physiotherapy being argued about the most.

A study carried out in Vancouver showed that with increasing age and disease progression, treatment adherence issues arise due to conflicts between health care and lifestyle issues. Steven Wright pointed out that while scientific and clinical expertise exists, we also have to appreciate its limits.

Genetics

“Studying twins provides a method of determining the contribution of genetics to a disease trait”

Michael Knowles from the USA talked about the symptoms of atypical CF while Garry Cutting from the institute of Genetic medicine asked whether phenotype was influenced by genes as well as environment. Currently one gene (CFTR) has been linked to CF. However; patients with the same CF gene mutation can have different symptoms and severity of disease. The institute has carried out a twin and sibling study. Studying these groups provides a method of determining the contribution of genetics to a disease trait. Direct questions about genetic control over symptoms of CF about the genes controlling them can be asked. Siblings share 50% of their genes as well as their environment. Twins share genes and environment before and after birth. The study of identical twins provides insight into environmental effects on disease and the effects of the interaction of genes with the environment. Determining genetic modifiers of CF will increase our understanding of the mechanisms of the disease and its symptoms. Analysis of similarities and discrepancies in symptoms in twins and siblings will help us to target potential genetic or environmental modifiers of these symptoms and suggest potential targets for medical intervention.

Coping

“CF diagnosis has an effect on all members of the family”

The session looking at how parents and close relatives survive the effects of receiving a CF diagnosis and a Swedish study evaluated the impact this has on attendance at future neonatal screening programs. CF diagnosis has an effect on all members of the family and it is felt that more effort should be extended into helping relatives become as good a support to parents as possible.

Adolescent Clinics

“Teenagers would rather talk to their parents, while parents would rather their child talked to the CF nurse”

Parents and patients at Birmingham Children’s Hospital were asked for their views on proposals for a young person’s clinic. Children wanted to start attending the adolescent clinic between 13 and 14 years of age, whereas parents wanted to wait another year. Teenagers would rather talk to their parents, while parents would rather their child talked to the CF nurse. Although the young patient is invited to be seen on their own, the majority of young people felt they did not know enough about their CF or that they liked having a family member with them. In conclusion the centre found that the CF Team and parents need to work as a team and the program followed has to be adapted to suit individual needs.

Adult issues

“One common theme from the study was that most people with CF expected to stay at home all their life or return home after living independently.”

Annette Landy from Papworth Hospital looked at the challenges of CF due to longer life. Can people with CF lead normal lives, and are dreams realistic? A number of issues arise such as physical maturity and psychosocial such as relationships, living away from home and reproduction and these need to be handled sensitively. Changing attitudes to CF in terms of adherence to treatment, progression of disease, transplantation, and death need to be taken into consideration. The way people feel about their CF changes with their level of lung function. One common theme from the study was that most people with CF expected to stay at home all their life or return home after living independently.
Future investigation and monitoring is required but Annette viewed her current role as offering support and to assist resolution.

Women’s Health

Margaret Sherburn, from the University of Melbourne in Australia, discussed the prevalence and severity of symptoms of urinary incontinence (UI) in women with CF. This is only just beginning to be recognised as a widespread problem and varies from between 36% and 64% of women, increasing in older women and those with poor lung function. The study Margaret carried out showed there is a higher prevalence of lower urinary tract and bowel symptoms in women with chronic lung disease than the healthy female population and that women who suffer with UI generally accept and manage their symptoms. The surprisingly high prevalence of night-time enuresis in the CF group needs further investigation. Additional research is under way to clarify these findings

Working with Minorities

Analyses of the UK CF database by Jonathan McCormick, based at the Tayside Institute of Child Health in the UK, suggested that Asian CF phenotype results in a more severe lung function deficit than in the Caucasian ?F508 phenotype. However it is worth remembering that social and cultural factors may be contributing to the severity of CF in this group.

“Albanian parents were defensive about increasing their knowledge of CF with some refusing to receive any information whatsoever”

Due to an increased influx of Albanians into Italy over the last decade, Paola Catastini from the CF Centre of Tuscany studied groups of Italian and Albanian parents of CF children to examine how the different cultural backgrounds affect the parents’ experiences and ways of coping with the disease. Psychological interviews with the parents showed that Albanian parents were defensive about increasing their knowledge of CF with some refusing to receive any information whatsoever. These results show the relationship and amount of communication the CF team should adopt with parents from minority groups needs to be questioned in more detail in order to achieve the best possible care.

Screening and diagnosis

Filippo Festini, of the Cystic Fibrosis Centre in Tuscany, studied the differences in clinical conditions between those who were diagnosed via neonatal screening and those diagnosed by symptoms. All the participants were 15 years of age and those who had been diagnosed due to Meconium Ileus were not included. Those diagnosed by neonatal screening had a better nutritional status and less of them were colonised with Pseudomonas. Lung Function was similar in both groups.

Maurice Super looked at the complexities involved with genetic screening, focusing on what should be told during a counselling session. In this situation the client must be allowed to control and to set the agenda, rather than the doctor telling them what to do. The doctor has to be a good listener and be careful not to press their own moral beliefs onto the client. Dr Super stressed that more education for those requiring genetic counselling is needed. Doctors must ensure that those with a family history are advised appropriately

Physiotherapy

“Physiotherapists taking on other roles in the future with relatively new concerns over urinary incontinence and bone disease”
Presenting the case for Physiotherapy was Mary Dodd from Wythenshawe Hospital, in Manchester, UK, who showed delegates that physiotherapy is about more than just airway clearance. Physiotherapists also play the role of teacher, whether instructing patients on nebuliser use and care, or helping with exercise and breathing techniques. Mary sees physiotherapists taking on other roles in the future with relatively new concerns over urinary incontinence and bone disease. Most patients consider the burden of therapy worse than the CF and physios should help with goal setting and plans to balance the burden of care.

Nutrition

Alison Morton and the CF team from Seacroft Hospital in the UK studied whether nocturnal Enteral Tube Feeding (ETF) in malnourished patients improves dietary status and slows clinical decline. The results showed that ETF does contribute to the stabilisation of lung infection even in the presence of greater disease severity and significantly improves the patient’s nutritional status.

“Children attach little meaning to weight gain as a goal in their diet management”

Another Study held at University College Cork in Ireland by Elieen Savage showed that children with CF (aged 6-14) and their parents had rather a different perspective regarding their diet. The main priority for parents is to protect their child’s long term health and survival by keeping their weight up. The challenge most parents face is getting their child to cooperate as children attach little meaning to weight gain as a goal in their diet management. Children are far more concerned with having the energy for activities. This, rather than weight is of more relevance to their daily lives. These findings highlighted the need for health care professionals to understand these differences and that the meaning that different family members attach to food and eating can influence the decisions made regarding dietary advice given by their CF team.

Patient Care

“Infection risk can be over emphasised in parents’ minds so clinicians must help keep infection issues in perspective”
Professor Duncan Geddes from the Royal Brompton Hospital, London posed a number of questions to the people who are responsible for the clinical decisions in Cystic Fibrosis. While survival is increasing due to good standards of paediatric care and new aggressive treatments, it presents many new challenges.

• Eating disorders are becoming more apparent with the increased focus on nutrition.
• How much physiotherapy to do and for whom? We know physiotherapy works but what works best for the individual.
• Readdress infection measures to prevent an outbreak of “Pseudo-terror” Infection risk can be over emphasised in parents’ minds so clinicians must help keep infection issues in perspective without down playing the risks.
• In later stages of the disease, the issues of oxygen and transplantation need to be studied. Transplantation is the single most life saving treatment but due to lack of donors and an unlikely increase in their numbers, living lobar treatments should be investigated.
• Patients will not be able to relate to every member of the team, and clinicians must remember that the patient is an important part of that team.
• The usefulness of new therapies must be validated or clinicians are in danger of just adding more burden and side effects for patients.

Prof Geddes finished by stating that while looking to the future, the aims of clinicians should be to achieve less treatment and more life for their patient.

Here’s one patient who’s not going to argue with that.