DR LITTLEWOOD’S DIARY – January to July 199
Including lecture tours of South Africa and Australia
I HAD RETIRED FROM THE NATIONAL HEALTH SERVICE IN 1997 BUT CONTINUED AS CHAIRMAN OF THE RESEARCH AND MEDICAL ADVISORY COMMITTEE OF THE CYSTIC FIBROSIS TRUST UNTIL 2003 WHEN I BECAME CHAIRMAN OF THE TRUST
Jan. 8th Meet Martin Scott, Medical Director of the CF Trust and Anil Mehta paediatrician from Dundee who, with his wife Gita, is responsible
for the UK CF Database to discuss how the Databaseproject is progressing. Some clinics are unwilling to join at present due to lack of staff to enter the data. Getting the database going is a slow job but it’s an excellent database which will undoubtedly be up and running within two years. We stay overnight at the Royal Society of Medicine (RSM) in Wimpole Street.
We used the RSM as our London base for overnight stays until I retired from the Chair of the CF Trust in 2011.
Jan. 9th Membership Exam Scoring session at the Royal College of Physicians in London I sometimes combine Royal College work and CF
Trust visits to London to spare the CF Trust my travel costs. However, I have resigned as an examiner at the RCP as the CF Trust voluntary work is proving increasingly demanding and adversely affecting my golf handicap.
The current headquarters of the Royal College of Physicians is a Grade I listed building designed by renowned architect Sir Denys Lasdun (1914–2001). Opened in 1964, next to Regent’s Park, it is widely considered a modernist masterpiece and one of London’s most important post-war buildings.
Jan. 15th Much telephoning to Bristol. The CF Trust has been asked to explore the possibility of funding a CF Centre in the new Children’s Hospital using Blue Peter money. There was an astonishing lack of interest from the senior hospital administrators. We know there are problems on the medical side there and the need for a major CF specialist service in the Southwest – fortunately Martin Scott has taken over and sorted out the matter so the development will go ahead. There have been problems and the present CF consultant, Dr Flemming Carswell (1937-2019), no longer sees CF children and a new consultant will be appointed soon and will hopefully provide a regional service for the South West.
Jan 22nd. Spoke to Dr Keith Dodd, secretary of the Royal College of Paediatrics and Child Health.
I asked him if the RCPCH would sign a letter to the DHSS pressing for neonatal CF screening. Keith implied that most paediatricians were in favour and there should be no problem. However, when asked to sign such a letter the RCPCH declined as they considered the matter was being handled (or more correctly mishandled) by the UK National Screening Committee. This gave me an unpleasant deja vu feeling as I recalled how the College (British Paediatric Association as it was then) had failed to approve the first CF Working Party report in the early Eighties because their Council considered that specialist CF centres would compromise the position of the general paediatric consultants. I was afraid the RCPCH had an unsatisfactory record with regard to cystic fibrosis and they needed watching!
With regard to neonatal CF screening, the National Screening Committee (NSC) agreed to report on this in late 1998. I believed, at the time, they would lean toward supporting neonatal screening (how wrong I was!). Certainly no health authority, not already screening, would move on this until the NSC reports.
Jan. 23rd-25th. Flew to Brussels.
To give a lecture to the Brussels CF on “The lower bowel in cystic fibrosis”. It was very cold in Brussels but the roast beef there was fabulous. I visited Brussels a number of occasions and I was impressed by their friendliness and lack of punctuality! We had some time for sightseeing on this visit including a visit to the Arts Museum. The Grand Place was very impressive.
Jan. 26th -28th.
Two day’s intense work to finish an article about my recent visit to Copenhagen to make a video of the work at the Rigshospitalet CF Unit.
Also I had to complete a chapter on Nutrition and Growth with Sue Wolfe for next addition of Margaret Hodson and Duncan Geddes’s text book on Cystic Fibrosis.
Jan 28th. We start our journey to South Africa.
In view of the disaster some years ago when snow at Leeds-Bradford Airport caused us to miss our connecting flight to Australia, where I had been invited to speak at the Australian CF Association meeting, we stay at the Heathrow Hilton in Terminal 4 Heathrow the night before our flight to be certain not to miss the South African flight.
Jan 29th-30th. Travel to South Africa
We were greeted at Johannesburg airport by members of the South African CF Association (SACFA). Alan Dunn (President), George Pearson (local CF group), Tracy Mulden (Solvay Local manager) – Solvay were sponsoring my travel.
They welcomed us and took a photograph. We then flew on to Capetown where the main CF conference was to be held.
Feb 1st & 2nd. CF Conference at Capetown University.
Duncan Geddes, the other invited UKspeaker, gave his usual excellent state of the art lectures on the chest and another talk on nebulised drugs.
I gave three lectures over the two days – one on the state of the art on nutrition, one on pancreatic enzymes and the other on the cost of care in the UK. The conference appeared to be a success according the conversation Duncan overheard in the gents!
This was only a two day conference but we stayed on in South Africa until the 16th to see some more of the country. One of the obvious advantages of being fully retired from the NHS! It was interesting that some black families were now using the hotel which was not the case on our previous trip in 1991 when there was strict racial segregation.
February 3rd – 6th. We did the Garden Route tour.
This included the Vergelegan Wine Tour – an historic wine estate in Somerset West, in the Western Cape province and the Vergelegen library in the old wine cellar. Founded in 1700, in 1798 the estate was sold to the Theunissen family, who planted extensive vineyards and concentrated on the production of grapes until an infestation by the phylloxera louse in the late nineteenth century wiped out most of Vergelegen’s grape production. Since 1992 Anglo American have produced high quality wines again.
Feb 7th. Visited Stellenbosch town and museum
Stellenbosch is a town in the Western Cape province of South Africa, situated about 31 miles east of Cape Town. It is the second oldest European settlement in the province, after Cape Town. The Stellenbosch wine route, established in 1971 by Frans Malan from Simonsig, Spatz Sperling from Delheim and Neil Joubert from Spier is a world renowned and popular tourist destination.
Feb 8th. The Cape Town Gardens and Bird Sanctuary.
Very impressive. Situated on the eastern slopes of Table Mountain, Kirstenbosch is internationally acclaimed as one of the seven most magnificent botanical gardens in the world. The 528 hectares estate includes a cultivated garden and a nature reserve. The developed garden displays collections of Southern African plants.
Feb 9th. Flew to Durban.
We stayed at the Crown Plaza Hotel in Durban. A large modern hotel on the seafront. Also bought a new pair of binoculars as mine were stolen having left them out on the bed in the previous hotel bedroom! My impression of Durban during the short time we were there was it was rather bleak. The photo gives the correct impression.
Feb 10th Clinic, Meetings and Durban City Tour
We bought a small green hippo’ and arranged for it to be shipped back to the UK.
I did a CF clinic in the morning with the local paediatrician, Jonathan Edger, then a lecture to the CF staff at the hospital.
Ann the meanwhile did the City Tour with a taxi driver as a personal guide as there were not many tourists in the city at that time.
That evening I gave a lecture to the local CF Society and there was a very good turn out of both parents and professionals. The talk went well and the questions seemed to go on for hours! There was buffet afterwards but I was so busy answering questions I missed the food. I had not eaten all day! It was now 10pm. We then went to a posh looking hotel on the front to get a meal but all we could obtain was peanuts – the place really seemed dead! However, the important thing was the meeting seemed to go very well.
Feb 11th Fly to Johannesburg.
We stay at the Holiday Inn Garden Court. I did a clinic at the very large Charlotte Maxeke Johannesburg hospital between 11.00 and 1.30. Ann discussed the use of the Leeds CF database with Alan Wolfe of the local CF association as they wanted to use our Leeds database and were very interested in our system.
I took part in a long CF clinic at the General Hospital – I think they produced all their most difficult cases! The Charlotte Maxeke Johannesburg Academic Hospital, nicknamed “Joburg Gen” is an accredited general hospital in Parktown, Johannesburg, Gauteng, South Africa. It has 1088 beds. The hospital’s professional and support staff exceeds 4000 people.
In the evening I did a parents lecture and well over 50 people turned up. There were many questions and it seemed to go well.
We had a tour round parts of Johannesburg including Soweto where there were flash floods. Also went round Nelson Mandela’s bungalow and saw many of the cheerful young residents.
This, was our second lecture tour of South Africa, and it has been very interesting. In the various meetings I seem to have provided what they were looking for. Many of the families were hungry for knowledge and the basic details of the latest care and treatment. I had many subsequent emails and communications from people there asking about various aspects of CF treatment. So now the work part of the visit was finished.
February 13th and 14th. To Victoria Falls.
Now work was finished and we flew to Victoria Falls where we stayed at the Victoria Falls Hotel.
To quote – “Built in 1904, The Victoria Falls Hotel was the very first hotel to be built in Victoria Falls. The hotel exudes original colonial Edwardian charm but recent refurbishments offer guests the modern comforts one would expect from a luxury hotel” It certainly is a very impressive hotel.
We had a walk along Victoria Falls and a river trip on the Zambezi. The following day we had a helicopter ride over the falls. We also did an early morning safari ride in a jeep through a game park and another in the evening. Still have not seen a lion!
The hotel was impressive inside and out with wildlife in the grounds and good views of the falls. Ann had a sharp attack of gastroenteritis due to tasting too many of the local meats – crocodile, zebra etc etc!!
We went on an escorted day trip to the bush in a 4-wheel-drive and saw various local people and activities including elephants at close quarters.
The Blue Train. Origins date back to the Union Limited and Union Express trains which began in 1923, taking passengers from Johannesburg to the ships departing from Cape Town to England. Prior to 2002, the Blue Train operated on four distinct routes including this one to Zimbabwe‘s Victoria Falls but this was suspended due to erratic rail rates being charged for access to the network of financially strapped Zimbabwe. As of 2007, the only regular route in operation was Pretoria-Cape Town and some special package tours.
Finally we had a memorable cruise on the Zambezi River and an impressive sunset.
Evening cruise on the Zambe
This was a very good 48 hours – the usually recommended time to spend at Victoria Falls to see most of the sights.
Later we return to Johannesburg airport. Travelling up the airport escalator I had a phone call from Australia asking if I would do a lecture tour there! Yes I would be interested to discuss.
This was the end of this South African trip and we returned to UK via Johannesburg on February 16th.
Feb 25th. Attended a Royal Society of Medicine lecture at Dean Clough Mills, Halifax.
Dean Clough in Halifax is a group of large factory buildings built in the 1840-60s for Crossley Carpets. Closed in 1983 it was developed into a Grade II listed site for various commercial and cultural uses.
The lecture was by Sir Alan Langlands, Chief Executive of the NHS among many other important jobs including one time Vice Chancellor of Leeds University. He gave an interesting talk on the first 50 years of the NHS.
I managed a brief word with Sir Alan afterwards to emphasise the inequalities of CF funding and the importance of central decisions for serious expensive diseases rather than allowing funding to be decided locally. He seemed sympathetic and seemed well versed in CF matters. I note that the NHS Commissioning for Specialised Services is to include cystic fibrosis.
March 3rd. CF Trust Clinical Trial Advisory Committee (CTAG) London.
This was the first meeting of this committee.
Our new advisory group consists of Duncan Geddes (chest physician at the Royal Brompton), Ros Smyth (paediatrician Liverpool and coordinator of the UK CF Cochrane Group), Prof Deborah Ashby (professor of Statistic at the Wolfson Institute of Preventive Medicine, London), David Spencer (Paediatrician, Newcastle), Martin Scott and myself. The function of CTAG is to provide advice to those contemplating research into CF. Methods of working were discussed and a further meeting arranged for early July.
March 4th. CF Trust branch meeting at Goodwood Country Club, Chichester
There was a very big audience with many questions and much discussion. I always enjoy such meetings. They are mostly attended by parents and relatives who are interested in the practical details of treatment and how research is progressing. I don’t hold back now and just tell them what they should be getting by way of treatment, even if it occasionally upsets the local medical establishment. This is childrens’ lives we’re talking about.
March 9th. CF Trust’s Working Group on CF Adults. London.
This was a very useful meeting chaired by Rosie Barnes, the CEO of the CF Trust, to discuss the problems of the expanding adult CF services. A very useful and productive meeting.
The following points were agreed – that the CF Trust should lobby the government on the needs of CF adults; the CF Trust should help with starting new adult clinics, initially in partnership and eventually handing over to the NHS; CF clinics required a second consultant if there were over 200 to 250 patients; shared care was not appropriate for adults at present; accreditation should be implemented; the CF Trust guidelines should be revised – there should be wider representation on the group including a Department of Health representative.
10th March. Attended Leeds University where Kevin Southern has PhD viva
Kevin Southern, our previous CF Research Fellow, was having his viva with the local professor of pathology and Dr Eric Alton from the Brompton and Imperial College in London. Eric was, and still is in 2021, a great expert on gene therapy and leader of the UK Gene Therapy Consortium.
The subject of Kevin’s PhD thesis was “Liposome-mediated gene transfer as a potential therapy for cystic fibrosis”. The degree was duly awarded. Well done Kevin! I believe Kevin is the first of our CF Research fellows to be awarded a PhD, although admittedly for this excellent research study he had an MRC grant and had moved to Oxford where the work was conceived.
Steve Hyde and his wife Deborah Gill (both now professors by 2020 in Oxford) were the main scientists involved. Kevin was subsequently appointed consultant paediatrician at Liverpool where he eventually became Professor Kevin Southern.
11th March. CF Trust lecture at Papworth in Cambridge the Verrier Jones lecture theatre. “Cystic Fibrosis in the Millenium”
We stayed overnight at the very pleasant Old Bridge Hotel in Huntington for the lecture the next day. It was good to see Di Bilton who is now the director of the Papworth Adult CF Centre; she is having a major favourable impact on CF care in East Anglia; she subsequently moved to the Brompton in London where she succeeded Margaret Hodson as Director of the Adult CF Unit.
12th March. Dr Bruce Montgomery, medical director of Pathogenesis the makers of TOBI, their new inhaled tobramycin, and Dr Patrick Rafferty, their European manager, came to Cambridge to show me the latest follow-up results from the US TOBI trial.
The results are very impressive and the trial is one of the biggest and best in the CF literature . However, this new preparation of tobramycin in this preservative free form is going to be expensive, perhaps similar in cost to Pulmozyme. The DHSS have already been alerted to this problem, fortunately the excellence of the trial means that the efficacy of the product is not in doubt – but neither was the efficacy of Pulmozyme yet there were funding problems. Many patients are already taking regular inhaled tobramycin, the same as is used for intravenous treatment, with good effect but without any problems. The present cost is £3419 per annum for an adult taking 180 mg twice daily – TOBI may cost over £8000 a year so it may be difficult to convince purchasers that the change to the new preparation should be made.
Addendum. Dr Bruce Montgomery subsequently has had a quite outstanding career. He is now (2020) the CEO of Avalyn Pharma and has 30 years of pharmaceutical company experience in research, drug development, operations and financing. He has raised over $370 million in venture or public financings. He has authored 26 US Patents and more than 45 peer reviewed publications. He has been instrumental for three complete drug approvals, aerosolised pentamidine, tobramycin solution for inhalation, and aztreonam lysine for inhalation (the only three FDA approved inhaled antimicrobials); from invention to advisory board presentations. He also received a special award from the CF Foundation.
Dr Patrick Rafferty was a wonderful larger-than-life character who bridged the gap between the CF community and the pharmaceutical industry. Patrick was a great host at conferences and had a fund of dubious stories!
17th March. R&D Antenatal and Neonatal Screening Report.
Spent most of the day reading the final draft of this report which I am one of the co-authors. I had a meeting with Prof. Howard Cuckle and the main writer Dr Jenni Murray at the Leeds University to discuss the various points relating to the neonatal section.
Addendum: The report was subsequently published and well received. We recommended antenatal screening should be introduced – but this has not happened by 2020. We recommend that neonatal screening should be considered and it was eventually approved by the UK government in 2001, (surprisingly but wisely against the advice of the resistant National Screening Committee), and it eventually became nation-wide by 2007 – FORTY years after I had introduced neonatal CF screening at St Mary’s Maternity Hospital in Leeds in 1975 !!
18th March. CF Trust Lecture at the Blossoms Hotel. Chester. There was a reasonable attendance but I suspect an important Liverpool football match had thinned out the audience. I tried to reduce the number of slides and removed one referring to the CF Cochrane group which is based in Liverpool. Just my Luck! The excellent coordinator of the Cochrane Group – Jill Motely, who is also a CF parent, was at the lecture and wrote to me afterwards pointing out I should have mentioned the Group. She was of course correct and they are indeed doing a very valuable job and continued to go from strength to strength. The group was started by Ros Smyth, the consultant at Liverpool and they do receive some regular CF Trust funding.
20th March. CF Ball at the Banqueting House in London.
Duncan Bluck, the Chairman, and his wife Stella invited Ann and myself to be guests in their party. It was an excellent evening including a most impressive charity auction by Lord Archer. He was on our table and seemed rather withdrawn – also he had to have special food. Perhaps he was nervous!
24th March. CF Trust Lecture. NW London Branch. Meeting at the Royal National Orthopaedic Hospital, Elstree. There was a reasonable attendance. It was good to see two experienced and respected colleagues in the audience and to have a pleasant chat with them afterwards.
Dr Tony Jackson (1918-2005) who was chair of Research and Medical Advisory Committee before I took over from him in 1996. He one of the pioneers of CF care in the UK and was a consultant at the London Hospital and the Queen Elizabeth Hospital for Children, London.
Another attendee was Dr Ty Pitt who has been a central figure in understanding the microbiological aspects of cystic fibrosis collaborating closely with clinicians both at the Brompton Hospital in London and also with national studies. Of particular importance was the national survey of CF centres revealing widespread evidence of cross infection both within and between CF Centres in the UK.
25th March. First viewing of the Copenhagen video filmed last autumn.
Before leaving Elstree Gaurang Majmudar, Senior Product Manager from Pharmax (makers of colistin) came to the hotel to show us the final version of the Copenhagen video. It faithfully depicts the excellent treatment at the Copenhagen clinic – undoubtedly one of the best in the world.
Dr Christian Koch the clinic Director comes over particularly well. – “if you want to pick out any single thing which we’ve done over the last 20 years or more it would be the introduction of the in the mid 70s of the regular three monthly intravenous antibiotic treatment for chronic pseudomonas infection another thing that I would emphasise would be the very early treatment of pseudomonas aeruginosa this is probably the best thing we have done for the patients”
The firm’s representatives (Pharmax) are now (1998) showing the video to doctors and other professionals at CF Units throughout the United Kingdom.
The detailed article I wrote about the visit is now available as a booklet, “A Visit to Copenhagen CF Centre”; a copy of this is given to the CF clinic staff when they see the video.
26th March. Annual Meeting of the Staffordshire Women’s Institute.
The meeting was at the Staffordshire County Show ground. I agreed to speak for 10 minutes on neonatal CF screening in support of a proposal of Mrs Maggie Smith whose son Hamish, 25 years old, has cystic fibrosis. Maggie Smith rings me up quite often about CF matters!
After my short talk the 700 delegates were unanimous in supporting the introduction of neonatal CF screening, which may now be chosen as a national subject for debate by the Women’s Institutes. Generally the neonatal screening issue is looking hopeful. My detailed report for the CF Trust on Screening for Cystic Fibrosis has been completed and will soon be sent to the National Screening Committee.
Addendum. Despite a vigorous campaign by the CF Trust, national neonatal CF screening in the UK was not finally agreed by the government until 2001 (and then against the advice of the UK National Screening Committee!) and not generally implemented throughout the whole UK until 2007.
28th March. AUSTRALIAN TOUR STARTS.
I was invited to give lectures mainly concerning malabsorption and pancreatic enzymes at five major CF centres in Australia – Brisbane, Melbourne, Sydney, Adelaide and Perth. Solvay, the makers of a widely used excellent pancreatic enzyme preparation (Creon) funded the trip. Ann and I left Leeds Bradford airport the day before the main flight to Australia to avoid missing the connection, as we did last autumn. Leeds Bradford to Heathrow and then to Vienna where we planned to join the Air Lauder Vienna to Sydney flight the next morning. So we stayed at a Vienna airport hotel which was almost in the departure lounge. Nothing could go wrong from now – but read on!!
29th March. Vienna Airport. At 11 am, three hours before the Sydney flight, we walked across the road from the hotel to departures to be told at Air Lauder check-in desk that their Sydney flight had been cancelled due to “technical difficulties”, We seem fated never to reach Australia after the fiasco we had with the fog last autumn. However, after many tortuous re-routings we eventually arrived in Sydney two days later.
We were greeted at the airport by Pam Noble, the Senior Product manager from Solvay who took us to the Hotel Nikko overlooking Darling Harbour. Pam looked after us magnificently throughout our tour.
31st March. Sydney to Brisbane. The next morning with Pam we took
the 11:05 am plane to Brisbane where a meeting for CF specialists and their teams had been arranged for that evening. We stayed in a pleasant hotel overlooking the race track which the Albion Park Racing Club used for trotting races. On the evening of our arrival we went to a very pleasant seafood restaurant. My talk was “Current issues in the use of pancreatic enzymes” delivered to a candlelit audience consuming enormous quantities of shelled and wobbly things from the ocean, between the first and second courses! It was an excellent format. The guests, many of whom had come straight from work, had a drink and the first course and were then happy to listen to a 30 minute lecture – rather than wondering when would the food arrive!
The evening went very well. I was particularly fortunate to have dinner next to Dr Ros Shepherd, the senior CF man of the area and an internationally known paediatric gastroenterologist. Ros had worked with a wonderful Canadian doctor named Douglas Crozier in Toronto, a man I had always admired but never met. Dr Crozier started the Toronto CF clinic in the 1970s and was distinguished for his very positive attitude to treatment described in his 1975 article (Crozier DN. Cystic fibrosis: a not so fatal disease. Pediatr Clin North Am 1974; 21:935-948.[PubMed]
On local matters Ros Shepherd described the problems they had with the great distances involved with shared care. He described the use of video consultation for distant patients and their doctors.
This was a really good evening which everyone seems to enjoy and was a good start to our lecture tour.
1st April. Brisbane to Melbourne. The following day we flew to Melbourne and landed around lunchtime and after lunch at our hotel – The Novotel on Collins
I went to visit Prof. Bob Williamson at the Melbourne
Children’s Hospital where he is now director of medical genetics unit and responsible for some 130 staff, He seemed well and in good spirits. He and his wife Robin, a chest physician, now have two children. He is not working directly on CF now but he is an advisor to the Australian CF Association. Bob sent his best wishes to all his friends in the UK – and he had many friends there when he was one of the leading, CF researchers prior to the identification of the gene. In fact, he was very close to identifying the gene and he was very disappointed his team at St Mary’s in London were not successful.
In the evening I spoke to the Melbourne CF doctors on “Cystic fibrosis in the UK” and “Current issues in the use of pancreatic enzymes” between first and second courses of an excellent Greek style dinner. I was again delighted that the evening went so well and the presentation was followed by a long and lively discussion chaired by Phil Robinson one of the paediatricians from the Royal Melbourne Children’s Hospital. Why did Australians not start their screened infants on long-term flucloxacillin? I asked when 30% of their infants had Staph. aureus in their bronchi by six months of age. No satisfactory explanation was given. Also home IV antibiotic treatment was not used largely due to funding problems so IV antibiotics can only be given in hospital.
2nd April. Return to Sydney and the Nikko Hotel.
To prepare for meeting at the meeting the next day. The people from Solvay regarded the Sydney meeting as the “Beecher’s Brook” of our race around Australia! The senior paediatric gastroenterologist Kevin Gaskin stolidly maintained that the intestinal malabsorption in all CF patients could be controlled with one standard pancreas capsule (5,000 international lipase units per capsule per kilogram per day) and furthermore that the nutritional state of his clinic patients was quite normal. Our own Leeds clinic and the Toronto clinic find that malabsorption is controlled and nutrition is good but on slightly larger doses of enzymes (10,000 to 15,000IU lipase’/kg/day). Also it was Kevin Gaskin who, some years ago, suggested that CF liver disease was caused by narrowing of the lower end of the bile duct – a finding disproved by subsequent studies including those from the Brompton and our own clinic in Leeds. I was warned the Kevin could be ‘difficult’ but he was very well-behaved at the meeting and subsequently greeted me as an old friend whenever I saw him on future occasions.
Meeting at the Royal Alexandra Children’s Hospital Westmead, Sydney.
This is a large Children’s Hospital on the west side of Sydney which has replaced the old more central paediatric unit – the Camperdown Children’s Hospital. The east side of the of the city (of 8 million people) is served by a second paediatric unit which is now called the Children’s Hospital. Adult care in Sydney is at the Prince Alfred Hospital where Dr Peter Bye is the Director – he recently reported the value of inhaled hypertonic saline in CF patients. There is also a smaller Adult clinic adjoining Westmead Children’s Hospital.
I gave two talks at the afternoon meeting – “CF in the UK” and “Current issues in the use of pancreatic enzymes”. There was considerable interest in the early treatment of Pseudomonas that we use and also the low prevalence of both Staph. aureus and Pseudomonas aeruginosa in the Leeds clinic. Among those present were Kevin Gaskin (senior paedaitric gastroenterologist), Peter Bye (adult physician from the Prince Albert Adult unit), Peter Cooper (Director of the CF Clinic and paediatric pulmonologist at the Westmead).
I also met Bridget Wilcken (very famous for early neonatal CF screening and Director of the Neonatal Screening Programme).
Dr Craig Mellis, respiratory paediatrician, was particularly interested on the microbiological side and we promised to correspond.
So despite the Solvay people’s anxiety, the meeting was very friendly and went very well. I was particularly pleased to talk to Bridget Wilcken one of the real pioneers of neonatal CF screening from the early Eighties.
5th April. Fly to New Zealand. As we were already in Australia and had a few days free, we were happy to accept an invitation from the New Zealand CF Association and Julie McClelland of the pharmaceutical company Boehringer Ingelheim, for Ann and myself, with business class travel, to visit New Zealand for two days for me give two lectures in Auckland. I’d been there before in 1990 on a long lecture tour when we visited most of the cities in both islands talking about cystic fibrosis and taking part in clinics.
6th April. Visit the Starship Children’s Hospital in Auckland.
I have the opportunity to have a discussion with Alison Wesley who has run the CF service in Auckland since I was last there in 1990. She has a new colleague, Cass Byrnes a paediatric pulmonologist recently returned to New Zealand after six years at the Brompton Hospital in London. Dr Phil Pattermore the CF paediatrician from Christchurch had flown up for the meeting and John Fleming, previously paediatrician in Huddersfield, had travelled with his team from the other side of the North Island.
New Zealand infants are all screened for CF using the immunoreactive trypsin test (IRT) was first developed and introduced there by Janet Crossley and Bob Elliot in the 1970s. It’s not surprising that the distance is a problem in a country that is the same size as the United Kingdom but contains only 3.5 million people – the same as Yorkshire . Alison Wesley visits clinics in other parts of the country to provide a shared care service.
It was good to visit New Zealand again although it was not on my original schedule. Apparently the parents had heard I was in Australia and organised the visit. Many remembered my previous tour of New Zealand almost 10 years ago.
7th April. Lecture to Auckland branch of the New Zealand CF Association. In the afternoon Jill Thorrat and the parents gave us a trip round the Auckland bay in their launch and a picnic lunch.
In the evening the lecture was in one of the function rooms at the famous Eden Park rugby ground. It was easter holidays so there was a rather modest turnout but the 35-40 people they had numerous questions. The talk was recorded on video and I left them copies of our Leeds management booklet which they intended to reproduce as they had done in Australia. Subsequently, I heard that two children had received courses of intravenous antibiotics following my talk on the need for more aggressive treatment and they were much improved.
8th April. Fly from Auckland to Sydney. No more meetings for a week.
We had a pleasant few days sightseeing. Visited the impressive Victoria shopping centre – narrowly avoided purchase of a ridiculous £700 coloured jacket at Joe Bananas.
On the 11th we attended a concert at the Opera House called the “Last Night of the Poms”; it was very good.
On the 12th we went on a day tour to Canberra. The site was selected as the capitol in 1908 as midway between the two rival cities Sydney and Melbourne. As the seat of government of Australia Canberra is the site of Parliament House, the High Court and numerous government departments and agencies. It is also the location of many social and cultural institutions of national significance such as the The Australian War Memorial, Australian National University, Australian Institute of sport, National Gallery, National Museum, and the National library
On the 13th. We had a Tour of Tyrrell’s Winery.
Very interesting history, on the internet, the family date from 1000 when Walter Tyrrell arrived in England with William the Conqueror
14th April. Discussion with Helen Griffiths, CEO of both New South Wales CF Association and the Australian CF Association.(ACFA)
The six Australian state CF Association are strong and seem to be virtually autonomous. The Queensland Association opted out of the National ACFA some years ago and Melbourne South Australia has also left the a ACFA last year. Obviously the great distances involved are a problem. The executive committee of the ACFA has one representative from each state CF Association; each association pays an annual fee. Infants newly diagnosed by screening usually are usually notified to the New South Wales CF Association and are visited by one their social workers. The parents may have been informed of the diagnosis already by a paediatrician or even an obstetrician. It didn’t sound a very satisfactory arrangement.
The ACFA has the following functions – administration of national research fund, the national data registry, organisation of the national Australian CF conference every two years and production of educational literature.
The state CF associations. There are seven state CF Associations In New South Wales there approximately 700 patients. Parents, patients, researchers and one clinical Dr (Dr. Peter Cooper) are on the New South Wales committee. They have an annual income of $0.5 million (£250,000) most of which is spent on services rather than research. Their 11 staff include social workers, home therapists who provide a variety of help with treatment in the home including physiotherapy. A financial consultant advises on the fundraising on an ad hoc basis.
My impression was that associations were less influential and involved than the CF Trust and the state organisation did their own thing, making only a small contribution and input into the ACFA national organisation. Obviously the great distances involved between centres and states was a problem.
15th April. From Sydney to Adelaide. Lecture at Adelaide Hospital for Women and Children’s lunchtime Grand Round.
An impressive new Children’s Hospital with an excellent lecture theatre attendance was good and the talk went well. Again the questions were mainly on our low prevalence of Staph aureus and Pseudomonas aeruginosa in Leeds. Mark Holmes from the Adult CF clinic at the Royal Adelaide Hospital also had noted the low prevalence of Staph. aureus in patients transferred to him from the paediatric clinic where long-term flucloxacillin was routine. Ross Butler, paediatric gastroenterologist, was currently organising a trial to compare Creon and Pancrease. Geoff Davidson, the senior paediatrician interested in gastroenterology, was out of the country but I recently met him in Berlin and we had a good discussion.
Kingsley Coultard, the chief Adelaide pharmacist, visited us in Leeds and it was good to see him on his home territory. After the meeting we went out to lunch with Pam and the local Solvay representative. Unfortunately she spilt a glass of red wine over my trousers; my other pair were in Sydney! Fortunately she arranged express cleaning as we had to leave for Perth the next day.
In the evening we went out for dinner at a restaurant on a hill, Mount Lofty, overlooking the city and observed twinkling lights – said to be particularly good in Adelaide.
16th April. From Adelaide to Perth. We did the Perth city tour in the afternoon. The lecture in the evening was a dinner style meeting in a very pleasant restaurant over looking the Murray river which flows through Perth.
There was much discussion and a really excellent dinner (the Australian food is really excellent). had a discussion with Dr Kathy Mews paediatric gastroenterolgist who was in charge of the CF unit in Perth. CF treatment there was much the same as tin the UK.
This was my last “official performance”. It was great relief that the tour had gone so well. Also Ann and I seemed to hit it off with our “minder” Pam Noble of Solvay who organised everything so brilliantly. We had lots of laughs despite the quite heavy schedule.
15th April. From Perth to Sydney. The 6.15 a.m. flight from Perth necessitated at 4:30 alarm call. The distances are enormous in Australia for example the flight from Perth to Sydney is 4,500 miles and takes four hours making Perth the most isolated major city in the world. No wonder there are problems coordinating the various CF Associations within the Australian CF Association. Indeed the major CF centres do seem to stand alone and have less contact with each other than occurs in the UK.
However, on leaving Australia I reflected it was Professor Peter Phelan of Melbourne and Dr Edmund Hey who was visiting him from the Newcastle UK, who pointed out that the better survival of CF patients in New South Wales than in England and Wales. This was in a classic article published in 1984 which prompted the formation of the first UKCF Working Group and also was the start of the UK Survey to see how we could improve the situation. Phelan and Hey attributed the better Australian survival to CF centre treatment rather than at local hospitals and I’m sure they were correct.
17th April. Drive to Sydney North Beaches. Pam Noble and her partner Doug called for us to visit the Sydney North beaches. They were absolutely fabulous. We had lunch at a restaurant high on the cliffs overlooking the bay where Kerry Packer lives. We checked in at Sydney airport at 3:30 pm on the start of a long journey home – and my goodness isn’t it a long journey. A really wonderful conclusion to what has been our most enjoyable overseas tour yet’ We do like Australia and the Australians. Virtually all the people we met were cheerful, friendly and proud of their country.
BACK IN THE UK
27th April. CF Trust Lecture. Somerset Branch. North Peverton near Taunton.
A small but keen audience at the Walnut Tree Hotel. I wrote a separate report on this visit to the South West for the CF Trust. The local paediatricians were Drs French and David Challacombe. David C published a letter in the Lancet in the same week as I published a letter reporting the falling incidence of coeliac disease (Littlewood et al. Childhood coeliac disease is disappearing. Lancet 1980;ii:1359). We had discussed the matter some weeks previously and it was obviously quite new to him before the discussion!
28th April. CF Trust Branch Lecture. Burcherell Hotel, Exeter.
The lecture went very well with a good response. In the afternoon visited Patrick Dr Oades the paediatrician who looks after the CF children at the Royal Exeter and Devon Hospital. Chris Sheldon the physician who looks after the adults, was recovering from a neck operation; I phoned his wife and he was doing well. Patrick hades does some shared care with Dr French in Taunton. Attempts at shared care between Exeter and Bristol failed, however, Exeter seems to provide a reasonable service.
29th April. CF Trust Branch Lecture. Duke of Cornwall Hotel, Plymouth
In the afternoon I had an opportunity to visit Dr Robert Jones the local paediatrician at Derriford Hospital. He provides a very good service. Attempts to develop a shared care service with Bristol also failed here.
The evening lecture at the hotel was very well attended by families “from all points west” and there were numerous questions.
30th April. Visit Dr Jenny Tyrell in Bath.
Jenny runs an excellent CF clinic in Bath. She was a CF Research Fellow in Nottingham some years ago and is now the main driving force behind the SW CF Database. Subsequently Jenny had a distinguished career in many areas of paediatrics particularly psychological areas. She died of breast cancer in 2013. Jenny and was described as an inspiration to her patients, consultant colleagues, ward nurses, and trainees, as demonstrated by the 500 or so people who attended her memorial service.
The South West has always been a problem regarding the provision of specialist CF centre care for people with CF. I summarised my thoughts for the CF Trust on the situation at the time.
Over the years Bristol has failed to provide a satisfactory to Tertiary Referral Service for the SW region which was acceptable to families and referring doctors. However, the recent appointment of a new consultant paediatrician, Dr Langton-Hewer as director of the CF Unit in Bristol to replace Dr Fleming Carswell (who would not do shared care) and more involvement of Dr Hugh Thomas a paediatric respiratory consultant in Bristol will we hope solve the problem.
I considered the CF Trust could help the situation by ensuring –
- That all level III units have access to CF Nurse Specialist whenever required, particularly for home IV treatments
- The staff are kept up-to-date with the latest advances in the form of hardcopies of Medline CF references
- That some members of staff have the opportunity to attend occasional international CF meetings perhaps with involvement in some clinical research
- Nurses, physiotherapist and dietitians should be members of their respective special CF interest groups; in this respect it is unfortunate that UK CF nurses group appears to be rather elitist only offering full membership to full-time nurses! Unfortunately, this excludes the very people who would benefit i.e. paediatric community nurses do spend a significant, but not all, their time with CF patients. We need to look into this problem
- All CF patients should be registered on the new central UK CF database
- Protocols of treatment should be provided. These are increasingly needed when units are smaller and local experience, of necessity, not so extensive. The CF Trust consensus groups reports will fill this need
In summary, there have been problems with CF care in the South-West but the region is a very large in geographically difficult area. However, I was reassured by what I saw and I would expect great improvements, including the provision of a suitable specialist CF service when Dr Simon Langton-Hewer, the new Bristol CF consultant, is in post. I believe the previous Bristol consultant was unwilling to undertake shared care. It is timely and good that Bristol will receive some Blue Peter support. All the paediatricians and I met were quite prepared to share care with the major CF Unit if the service was available and acceptable to the patients, which it clearly has not been up to now. I promised to keep in touch with the clinicians and also will visit Dr Prendeville in Truro in October after the Torquay meeting.
13th May. Visit Dr Iolo Doull, paediatrician appointed recently to replace Mary Goodchild who was in charge of the Cardiff CF CF unit. Iolo was at one time an SHO on my ward at St James’s. We had a good discussion about the local situation and the recently re-introduced Welsh neonatal screening programme. There are geographical difficulties in both north and south Wales. The presence to two substantial CF units in Newport (30-40 patients) and Swansea`(30-40patients) increase the importance of the CF Trust clearly recognising Cardiff as the regional CF Centre. I’m sure Iolo Doull’s personality and abilities will facilitate this; he has already been invited to see the Swansea children on a shared Care basis. It is good that he has recently been awarded a research grant by the CF Trust. He has the enthusiasm and skills to make a major contribution to CF care.
14th May. Northern CF Club. Monk Fryston Hall near Selby Yorkshire.
This is a medical social club which I started some 13 years ago for senior doctors in the North who spend a substantial part of their time dealing with cystic fibrosis. I recently handed over the chairmanship of the club to Dr Kevin Webb.
The purpose of the club wa ,and still is, to provide a forum for the senior clinicians who are ultimately responsible for the care of CF patients so they can discuss difficult problems with their colleagues facing similar problems. Full time CF research fellows are also invited. These meetings have been particularly useful as the treatment has changed quite significantly over the past 10 years. We are grateful to the pharmaceutical firms Pharmax and Solvay for sponsoring the meetings twice a year – one on either side of the Pennines.
26th May. CF Trust’s Antibiotic Consensus Group. 2nd meeting London.
This was an excellent meeting where we made a great deal of progress. The Group consists of Kevin Webb , Steve Conway, and Margaret Hodson (physicians), Ros Smyth (paediatrician and CF Cochrane Group)), John Govan and David Livermore (microbiologists), Gary Connett (paediatrician), martin Scott and myself (CF Trust). We hope to have a provisional draft ready for the Clinical Directors meeting in September. WE have also contacted the CF Nurses group who have agreed to help with the sections Home IV Treatment and the management of totally implantable venous access devices (Port-A-Caths).
27th May. Research and Medical Advisory Committee (RAMAC), London.
To see Professor Alan Cuthbert back on the committee and also to welcome Dr Stuart Elborn director of the Belfast adult CF Unit. I enjoy the RAMAC meetings but wish there were more relevant applications for clinical studies to answer questions which are fundamental to optimal clinical management e.g. the value of continuous anti-staphylococcal antibiotics, the routine use of three monthly intravenous antibiotics for chronic pseudomonas affection etc.
However, I’m optimistic that our new Clinical Trials Advisory Group, our various Consensus Groups and the CF Cochrane group will have a favourable influence. Dr Brian Lask attended the meeting to discuss the failure of the CF Trust to fund psychological research . We had a good discussion but I had to point out that there had only been three applications of a psychosocial nature in the past six years and all were regarded by the RAMAC as quite unsuitable for funding. It is encouraging that the Psychologists and psychiatrists have now formed a new Group and would like the CF Trust to become involved.
3rd June. Visit Dr Ron Knight’s CF Unit at Frimley Hospital, Surrey
I accompanied Rosie Barnes and Martin Scott to Frimley Hospital and we spent the afternoon seeing the facilities there and talking with Dr Knight and his team. There is a separate report of this visit but, in summary, we were all impressed by the facilities and the staff who cared for over 100 CF patients. Ron Knight has subsequently asked me to speak to the South Thames Respiratory Registrars training day in Guildford and Dr Knight has been invited to the Clinic Directors meeting in September. The potential for caring for more adults in this unit is reassuring in view of the general shortage of adult facilities.
15th June. Travel to Berlin for the 22nd European Cystic Fibrosis Society meeting
16th June . Chaired the Solvay sponsored meeting on Pancreatic Enzymes.
A well attended meeting which went well. The only new information was that the new Creon 10,000 will replace Creon 8000. In a study from Normandy it proved effective and acceptable to patients; we are assured that there is no question of it containing any new substance that could be harmful. It is my firm opinion, but some would not agree, the most important factor in the causation of fibrosing colonopathy is the ingestion of a large quantities of the copolymer eudragit which is in the coating of the strength pancreatic enzymes Pancreas HL, Nutrizym 22 and also the standard preparation Nutrizym GR. It is not in standard Pancrease, Creon 8000. Creon 10,000 or high-strength Creon 25,005 and fibrosing colonopathy has never been reported with these preparations even when given in high doses. I would not prescribe describe Pancrease HL, Nutrizym 22 or standard Nutrizym GR to any patient of any age.
17th June. International CF Association Symposium CF Care. My lecture “Clinical strategies and organisation of a clinical CF service”
This meeting was organised by Dr George Davidson of Vancouver who invited me to speak but unfortunately very few delegates attended – concurrent meetings were obviously of more interest!
George Davidson is a very pleasant man and a senior member of the ICFA organisation. He is active in research and is director of an excellent CF centre in Vancouver.
18th June. Useful informal meeting with Rosie Barnes, Iolo Doull, David Heaf to discuss how we should proceed with Service Standards fro CCF and Accreditation of CF clinics.
There is a full report of the meeting. In summary, and we made good progress. We have written to CF clinics asking if they have protocols and guidelines which we may see and we hope to have made considerable progress by the the time of the Clinic Directors meeting in September. We also decided to classify CF clinics either into large CF Centre (previously level I & II clinics) or CF Clinics (previously level III & IV) irrespective of size but depending on the service offered. Mr Tony Jenkins, at Health Services Accreditation offered to help with the process.
19th June. Berlin. Appeared on a panel to discuss “What is good care for CF?”
This was an interesting session. Three case histories were described and the panel of five senior CF clinicians were asked how they would investigate and treat the patients. Members of the audience took part in the discussion. There were some quite astonishing differences in approach. One senior US paediatrician suggested using Intal for an early exacerbation of the chest infection! I was reassured that the Dr Christian Koch of Copenhagen and myself (both “early aggressive treaters”) seemed to be on the same wavelength!
The Berlin meeting was good and an opportunity to have discussions with colleagues – one of the most import functions of these meetings. There was little new by way of major advances in treatment.
30th June. Leeds Annual CF Workshop of the Adult (Seacroft) and Paedaitric Units held in the Clinical Sciences Building at St James’s University Hospital, Leeds.
For professionals from Yorkshire to update them with CF care. Football enthusiast cut the start of the viewing of the England Argentina match, which is on the same evening, rather fine as the meeting finished and the football started at 8 pm! However, around 100 people did attend but, as always, there was a disappointing response from general practitioners and even worse from some paediatricians responsible for CF patients and taking part in shared care with the Leeds units – perhaps they really couldn’t miss the football!
1st July. CF Trust’s Clinical Trials Advisory Group (CTAG)/ 2nd Meeting in London.
There was much encouraging progress. At this meeting the group considered a number of topics which would be suggested to the clinical directors in September. Topics that would be suitable for a multi centre CF trial. Also a number of new proposals were considered and a method of response agreed, The members of the group left in good spirits, now convinced that CTAG was going to fulfil an increasingly useful role. The concept of a quickly responding expert advisory group was further developed.
In conclusion, I apologise for increasing the length of these so-called diaries but I hope you may find in them some points of interest. They are an as yet uncensored method of expressing my views on the general CF situation. Please contact me if you require further details on any of the items but just read the headings if you find the document too wordy!
Jim Littlewood July 6th 1998. Revised and expanded 2021.