Cystic Fibrosis Worldwide (CFW) launched a new educational programme in Muscat, Oman by participating in the Gulf Respiratory Society’s 3rd Annual Meeting on February 28, 2007. Dr. Harry Heijerman, CFW Medical Advisor, Louise Lannefors, Physiotherapist and member of the International Physiotherapist Group for CF (IPG/CF) and Alison Morton, Dietitian at Leeds Regional Adult CF Unit, UK, member of the European Cystic Fibrosis Nutrition Group (ECFNG) and UK Dietitians CF Group presented a full day educational programme aimed at improving care for persons living with CF in the Gulf States.

Dr. Harry Heijerman, CFW Medical Advisor at Oman CF Conference

Plenary Session

The first part of the plenary session was dedicated to the link between the genetic defect and the clinical manifestations of Cystic Fibrosis. Depending on the class of mutation the clinical manifestations leading to the diagnosis of CF may differ. The DeltaF508 is the most common mutation (class II mutation) in Caucasian countries, while in the Middle East, there is a recognized difference. Studies show that approximately 25% of all mutations are the deltaF508. This may very well lead to a higher prevalence of so called mild mutations. Persons with a mild mutation present themselves with pulmonary symptoms at a later stage. Most of these people have no gastrointestinal involvement and therefore may not be recognized as having CF. Additionally, as persons with mild mutations have residual chloride secretion, sweat tests may be normal. Therefore persons that suffer only from bronchiectasis may very well have CF despite a normal sweat test. In these situations extensive mutation testing may lead to the correct diagnosis.

The second part of the plenary session emphasized the role of Pseudomonas aeruginosa (PA) infection in the lungs. Chronic PA infection may lead to a more rapid decline in lung function as the bacteria may adapt themselves to the intrabronchial environment. After a planktonic state they may form micro colonies and consequently start to produce a biofilm. This biofilm protects the bacteria (PA) from neutrophils and administered antibiotics. With the formation of biofilm the infection will start to be chronic and virtually impossible to eradicate.

The result of such a chronic infection is an ongoing inflammatory response in the airways leading to progressive lung tissue destruction.

The third session focused on the prevention and treatment of infections with PA. As PA is easily transmitted from one CF person to another, most clinics have a strict segregation policy. This policy has proven to be successful and will lead to a decrease in the incidence and prevalence of patients with a chronic infection with PA. When patients have the first infection with PA it is still possible to eradicate it for a longer period of time, using a combination of systemic and inhaled antibiotics. It has been shown that a three week oral administration of ciprofloxacin in combination with inhaled Colistin for six weeks is effective in the majority of patients. When patients have a chronic infection with PA continuous treatment with inhaled antibiotics (Tobramycin or Colistin) will reduce the number of (infectious) exacerbations and inhibit the decline in lung function. Additionally oral Azithromycin may also lead to an improvement in lung function and a decrease in infectious exacerbations. The exact mechanism of action of Azithromycin is not completely clear but Azithromycin probably inhibits the formation of biofilm in chronic PA infections and may have some anti-inflammatory action.

Physiotherapy in Cystic Fibrosis

The physiotherapy aspects of the meeting consisted of a plenary session and two workshops. The plenary session entitled “Physiotherapy in CF – state of the art” emphasized physiotherapy as an essential part of the CF care package, highly interacting with the other basic parts of the therapy. It demonstrated the impressive development of physiotherapy through-out the world and how many factors influence the way we are working, what techniques are chosen and to what extent the priorities in different cultures vary. That modern physiotherapy in CF includes inhalation therapy, airway clearance therapy, physical exercise and a continuous ongoing education of patients, parents, other team members, hospital staff and more, was emphasized.

The presentation stressed the importance of defining the aims of physiotherapy in CF and the role of the physiotherapist in modern CF care. Is it 1) trying to prevent the disease from progressing by early intervention, reduce peripheral airway obstruction and maintain all parts of the lungs ventilated, or 2) trying to regain what has been lost by intense physiotherapy during admittances to hospital, and provide physical therapy in connection to musculo skeletal pain? Where to put the physiotherapy focus was presented as an active essential decision to be made, since it is of great importance for the organisation and for the amount of physiotherapy resources available at the CF centre. The role of the physiotherapist to tailor an individual physiotherapy regimen was also discussed, knowing that maximal treatment not necessarily is the same as optimal treatment. The number of factors influencing what is the best regimen for the single individual was listed. But, as factors change over time, the therapy needs to be evaluated and optimized regularly or changed in order to maintain adherence. The importance of evaluating the regimen together with each patient and parents was brought up, where parameters of immediate interest are chosen as a complement to the more traditional ones. Making it possible for patient and physiotherapist to have frequent contacts was emphasized and the fact that physiotherapy always must be carried out in a way that makes future co-operation possible.

The workshops gave opportunities for more detailed and interactive discussions. Physiotherapy in infants and small children was one topic actively discussed. When to start in small children who apparently have no lung symptoms was thoroughly addressed. Referring to the plenary session, aims with the treatment were discussed in detail, based on the knowledge about mucus plugs in young children with CF and that non-ventilated airways constitute an environment that makes certain bacteria thrive. To what extent individually tailored physiotherapy as an early intervention can influence these pulmonary changes in the very peripheral airways was also discussed. Questions raised were what techniques to include, duration of each session and frequency, different techniques possible to use, known positive and negative side-effects from the techniques available, cultural influences and practical problems affecting the choice. Many questions and experiences were explored. The participants were actively engaged, making the discussions both practical and useful.

The second workshop focused on physical exercise in different ages, but especially in small children. The workshop began with a review of how an obstructive breathing pattern and shortened chest muscles affects the spine and posture, how it allows a thoracic kyphosis (stooped position) to develop. This is probably a reason to why musculo skeletal problems and back pain are common among CF adults. All participants agreed that maintaining mobility is so much easier than trying to regain what has been lost and that physical exercise in CF should be introduced early. This should include chest wall mobility, postural muscle strengthening exercises as well as working on capacity training, all being relevant to patients´ age. Why patients with poor arterial oxygen saturation need oxygen supplementation during physical exercise was discussed, as well as the fact that there may be poor lung function, CF individuals can have a relatively good physical condition and look healthy!

Alison Morton, CF Nutritionist from UK, delivers nutritional seminar

Nutrition in Cystic Fibrosis

The nutritional aspects of the meeting consisted of a Plenary and two workshops. The plenary Session entitled “Nutrition and Enzymes: That’s Where It Starts!” highlighted the multi factorial pathogenesis of malnutrition in Cystic Fibrosis. It demonstrated how the impact of nutritional status on morbidity and mortality has been recognised in the literature for over 20 years and emphasised the increasing evidence of the positive effects of nutritional status, weight and body mass index on respiratory function.

The presentation reviewed the importance of optimising nutrition and pancreatic enzyme replacement therapy as early as possible following diagnosis and showed the long term impact of optimising nutrition in the early years. As CF is a complex multi system disorder it is essential that a proactive multi disciplinary team is involved in the care and management of the patients and interdisciplinary work helps to facilitate this. This was highlighted in the Plenary Session but also by the presence of the multidisciplinary audience.

Christine Noke, CFW Executive Director poses with Oman CF family, participants in the Needs Analysis.

The plenary provided background for more detailed and interactive discussions in the two workshops. The first workshop “Management of the Newborn with CF” (though this was really the newly diagnosed patient as CF rarely presents as meconium ileus in the UAE) highlighted the advantages of breast feeding, the importance and practicalities of the early introduction and monitoring of pancreatic enzyme replacement therapy, fat soluble vitamin supplementation and the management of complications such as meconium ileus, gastrooesophageal reflux, failure to thrive and feeding behavior problems.

The second workshop “Treatment of Malnutrition” focused on the factors contributing to malnutrition in CF. There were discussion about methods of nutritional assessment, criteria for a staged approach to nutritional intervention, and practical aspects of providing nutritional support, including supplementing dietary intake to more invasive forms of support such as nasogastric and gastrostomy feeding.

Needs Analysis for Future Programs in the Gulf Region

During the day long seminar, parents, patients and medical professionals were interviewed to determine how CF Worldwide can further support patients in Oman and the surrounding Gulf States. An analysis was carried out through questionnaires that asked a series of detailed questions aimed at determining the specific needs of the region.

Oman, Bahrain, Saudi Arabia, Dubai and other Gulf States supply necessary medication and treatment for patients through their national health programs. Most medications are readily available in all regions but there is little knowledge of appropriate treatment among the medical community. The Gulf Respiratory Society is actively increasing awareness of CF and will continue to work with CFW to host future CF Seminars.

Parents and persons living with cystic fibrosis in the region do not have access to appropriate information.

There are few CF sources on the web or in print in Arabic, the predominant language of the region. This has made it difficult for families to understand the daily needs of CF persons and how to cope with the demands on all family members. There are few resources available to train parents and patients on daily Nutritional and Physiotherapy needs. Home treatments are not available making patient hospital stays more frequent and prolonged. One family interviewed said their child with CF spends up to 2 weeks every 2 to 3 months in the local pediatric clinic for antibiotic treatments and physiotherapy.

Many parents expressed the desire to have a CF newsletter in Arabic and to form support groups for parents and patients. CFW is planning to host a Parent/Patient educational workshop in Oman in 2007 addressing home Physiotherapy and Nutritional needs. During this workshop a concerted effort will be made to form a local Parent/Patient organization to help support the initiatives of the Gulf Respiratory Society. The long term goal will be the development of several support groups throughout the region.

CFW looks forward to a positive evaluation of the Oman Conference and new Educational Programs aimed at spreading knowledge about cystic fibrosis. The Oman conference offered us a chance to better understand the needs of persons in a region where we are slowly uncovering a CF population. We look forward to returning to Oman in the near future to help improve the lives of people living with CF in the Gulf Region.