Emma Wicks, Patient Advocate CF Trust
The Cystic Fibrosis Trust welcomed this year's delegates to the international Conference Centre in a rather sunny Birmingham. 2004 is the CF Trust’s 40th Anniversary and aside from hosting the conference, and throwing a wild party, managed to squeeze in their Biannual Conference too. While I’m sure you’re all dying to know what happened when all those highly regarded medical staff and scientists headed out on the dance floor, I can’t tell you as I have been sworn to secrecy. However I can tell you a bit about the conferences. Of course, I was not able to get to every session so here are the highlights.
What People with CF hate
Professor John Price from Kings College Hospital in London, UK pointed out that if on the 1st of January, a person with CF carried out their entire years worth of treatment with no breaks, for 12 hours each day it would take until 9 March to complete. (Phew!) Young people with CF, understandably see this routine as a burden, and as that burden increases, as a signpost of the progression of their disease. Hospital stays and trips are seen as simply part of life with hospitals classed as an unpopular friend.
Of course it is not just the person with CF who lives with the disease but parents, siblings and other family members.
Parents have a number of hopes and fears for their child. They hope that their child will remain well or improve, they hope for a cure and for a miracle. Childhood illnesses and colds are a common worry, while setbacks, the Future and the possibility of death are more pressing. But generally these concerns stay in the background of daily life. Women are more accepting of reality while for men the pain of actuality can be too great. This means CF Mothers – wanting to be the perfect mum – often try to cope alone with the burden.
According to Dr Keith Brownlee of St James University Hospital in the UK the reason people hate having IV’s is not due to a fear of needles, rather the thought of having them. In other words, time spent in the waiting room is far scarier than actually having the IV inserted and it is important to help children overcome these fears as soon as possible. If this is not done having IV's will cause the child distress and fear. The emotional impact on the child is not the only concern. A fear of needles can result in cutting short IV’s or not getting them done at all, likewise with blood tests. This fear can also destroy the child’s relationship with their CF Team. Luckily much can be done to prevent these problems and reduce worry.
• Avoiding unnecessary blood tests
• Insertion of IV’s and blood tests carried out by experienced staff well known to the child
• Distraction in forms of music and books
• Anaesthetic Creams
• Less time spent in Waiting room
Bugs and segregation
Professor John Govan, who is based at the University of Edinburgh in the UK, discussed the issue of bugs and segregation, something which evokes lively debate within the CF Community. But as Prof. Govan pointed out; bugs are everywhere, whether on other people or in the environment. So if we can’t prevent bugs what can we do to limit the damage? Segregation is not a popular choice and cause extra anxiety as well as increased pressure on facilities at clinics. Segregation in hospitals was occurring as early as the 1800’s mainly due to the work of Joseph Lister. Lister did not discover a new drug but he did make the link between lack of cleanliness in hospitals and the occurrence of illness and deaths after operations.
“prolonged social contact with other CF People is a much higher risk than diving headfirst into a lake”
So why bother segregating patients if bacteria are everywhere anyway? Bacteria spread from humans are more resistant to antibiotics than bacteria spread by the environment. This means prolonged social contact with other CF People is a much higher risk than diving headfirst into a lake. So while cross infection can not be avoided entirely, segregation does reduce exposure. Prof. Govan noted that some environments are more dangerous than others but generally the benefits of participating outweigh the infectious hazards.
“prevalence of the strain has not increased since the implementation of the segregation policy”.
The consequences of segregation was also examined in light of the epidemic strain of Pseudomonas Aeruginosa that occurred in Liverpool UK. In 1995, fifty-five of the 120 Children who attended the Royal Liverpool Children’s Hospital were diagnosed with having a genetically identical strain of pseudomonas. This resulted in a new Segregation policy. Eight years later the team at the Royal Liverpool developed a way to identify the prevalence of this particular strain in their Clinic population. Only 37 of the 55 were available and all were still infected by the strain. Although it appears that once a patient is infected with the strain it cannot be eradicated, prevalence of the strain has not increased since the implementation of the segregation policy.
Gerald Ulrich reflected upon both the expected and unexpected consequences on segregation at the Hanover Medical School in Germany, where segregation policies have been in place for the last 15 years. Despite worries that the CF community would fall apart, technological advances such as Mobile phones and the Internet has gone some way to compensate for personal contact.
Tom McLoughlin and Sophie Knight two of the CF Trust’s advocates gave an overview of the Trust’s Advocacy project. Due to the conference cross infection policy only Tom was present on the stage, while Sophie’s contribution was done by video.
As the CF Population grow older we are seeing more complications arise. People with CF have many risk factors for reduced bone mineral density or osteoporosis. Dr Steve Conway at St James’s and Seacroft Hospital in Leeds gave his views on what he described as a silent illness. Not only can bone disease be painful but difficult to detect until fractures actually occur. People with CF have many risk factors for reduced bone mineral density. It is extremely important to monitor bone density as vertebral and rib fractures can result in reduced lung function.
Osteoporosis does not tend to be seen in childhood as the problems do not begin until bone growth accelerates during puberty. When the new bone grows, it does not achieve normal peak bone mass making the bones weak. Dr Conway recommends that from the age of ten, DXA scans are preformed every two years.
Existing bone is worn away we get older, but in CF there is no increase in new bone growth. People with CF have many risk factors for reduced bone mineral density.
• Disease severity
• Delayed Puberty
• Lack of vitamins
• Lack of exercise
• Corticosteroid therapy
Dr Conway also outlined a number of ways to prevent and treat Osteoporosis
• Vitamin D, as well as supplements this also includes getting out in the sunshine. For those of us who live in places like the UK this can be somewhat of a challenge and results in a huge seasonal variation
• Weight bearing exercises
• Better Nutrition
• Use of Bisphosphonate
A study carried out in France supported the notion that CF related osteoporosis is directly related to the genetic defect in the CFTR. The Adult CF Unit at Southampton, UK looked into the factors influencing the development and progression of the disease in adults. This study reinforced the importance of both nutrition and weight bearing exercise in addition to standard pharmacological measures in order to minimise occurrence and implications of bone disease.
Dr Martin Walshaw of the UK Liverpool Adult CF Clinic, presented the delegates with information regarding abnormalities in Kidney Function in CF. A subject which would have not be considered worth talking about even a few years ago, the complication is now starting to be recognised as a feature of CF. While kidney function is not greatly altered in CF, kidney stones are an increasingly common complication as life expectancy increases. The kidneys stones result from a build-up of calcium and oxalate. Oxalate is a by-product of digestion. It appears that prolonged antibiotic use and other high-dose drug regimens may preclude natural colonisation of the bowel with the organism.
New Therapies and Medication
This session included an overview of new therapies and a look at the amount of progress which has been made over the last year. Severity of lung disease differs depending on the mutation and therefore a better understanding of how the genes interact with each other is needed. Disease modifying drugs and the prevention of infection is seen as the main goal in lung therapy. However not without some concern about adherence if too many new drugs are introduced without replacing the existing ones.
During this session the effect of receiving a CF Diagnosis was considered. It is thought that an early diagnosis can increase the likelihood of depression in both parents. This increased effect is a cause of concern for many clinicians. How siblings cope with CF was also highlighted. Younger children just want the CF to go away maturing into feelings of acceptance as they reached their teens.
Gerard Ullrich from Hanover Medical School, Germany looked at the effects of Pseudomonas Aeruginosa (PA) on parents. In a study carried out at the school 28% of parents feared their child would only live a few more years if they acquired PA infection. This and similar concerns leads to exaggerated hygiene methods. Lack of knowledge about acquisition and infection can trigger unnecessary actions undermining the child’s quality of life. Counselling and education about PA is paramount.
There were sessions on both Male and Female reproduction issues.
The first session focused on four women from Sweden, Denmark and the UK. Issues covered during this session included problems which occur to mothers during pregnancy and what happens when this results in a decline in the mother’s health. Women intending to get pregnant worry about whether their CF will affect the baby and in turn if having the baby will affect their own health. Despite these concerns it seems that women with CF who want children will attempt to conceive regardless of their existing state of health.
Early studies report poor outcomes for some mothers – even in healthy women, pregnancy can result in an unexpected decline. Christiane Knoop from Brussels Erasme Hospital in Germany presented the group with the dilemma of a patient desperate to become pregnant despite needing a lung transplant. This opened the floor to some lively debate. Frank Edenborough from Sheffield’s Northern General Hospital and Marie Johannesson, a Professor in Paediatrics from Sweden, who chaired the session, presented a draft for a prospective European study of pregnant CF women and their outcomes. The suggestion of assembling a working group to take this idea forward was well received.
Reproductive issues in CF males and infertility was also looked at. Su Madge from the Royal Brompton Hospital in London pointed out that young men are in danger of being exposed to mixed messages, and as boys will find out one way of another, the issue needs to be addressed as early as possible. In a study of CF males 71% were aware that they could not father children. The same amount wanted to have children at some point but many were unaware of the options available to them. Parents at Great Ormond Street hospital felt that 13- 14years old was the ideal age to tell their sons and that they wanted to share the responsibility of informing them with the CF team. Most men felt that the later in life they were told the facts, the harder it was to accept them, while some would rather remain in ignorance of the whole thing.
Iwan Lewis Jones discussed what to tell and presented some of the options available to men wanting to become fathers. Susan Sawyer based at Royal Children’s Hospital, Melbourne, Australia, asked how the process could be managed. Patients want information from lots of different sources but most patients find that embarrassment is a barrier to asking for that kind of information and many staff lack the training to deal with the subject sensitively.
Sean Bell, a business advisor from Manchester, UK recounted his experiences of becoming a father. His Journey to parenthood was not an easy one and Sean admits taking the decision to embark on fatherhood was a big challenge. Despite having some reservations, mainly the prospect of having sharp needles stabbed in private places, two and a half years and over three cycles of IVF later, his daughter India was born. 7 Months later, using the same embryos, Scarlett was conceived. Sean joked that he now has a new career as a full time children’s entertainer!
Editor’s Note: Part 2 of the Highlights by Emma Wicks will be in the next issue of the CFW Newsletter