Seventies – First decade as a consultant paediatrician


My first NHS paediatric consultant post in Leeds – 1968

In 1968 I had moved from a Senior Lecturer Consultant post in Leeds University to that of a National Health Service Consultant in the city employed by the Yorkshire Regional Health Authority. I made this move for two reasons. First, I wished to see more patients and gain more practical experience rather than organising teaching as major part of my job. Second, I did not wish to be always ultimately responsible to the head of the department i.e. the Professor of Paediatrics at the time. I preferred to be my own boss!

A few reflections on consultant practice

“Experiential learning is the process of making meaning from direct experience i.e. learning from experience”

William_Osler_c1912

Sir William Osler, the famous Canadian physician and one of the four founders of the Johns Hopkins Hospital in Baltimore, observed “Medicine is to be learned by experience; and is not an inheritance; it cannot be revealed. Learn to see, learn to hear, learn to feel, learn to smell, and know that by practice alone can you become expert”

This is one of my favourite quotations!

It seems unfortunate that my more than 40 years of intense clinical experience and lessons learned as a busy general paediatrician in a large northern city should be wasted – particularly as in the present NHS the numerous, often specialised, excellent hospital doctors are unlikely to be subjected to so many and so varied problems as was usual for the relatively few paediatricians in 1968 when I first became a general paediatric consultant in Leeds. So I decided to record a few observations that I hope may prove of interest.

Detailed clinical evaluation is certainly essential in paediatrics to identify clinical problems and also to recognise areas where answers are required.  As Dr. William Waring of New Orleans observed in relation to cystic fibrosis, “clinical research comes naturally as a result of the questions that arise from patient observations”.  I recall, as a young tutor in paediatrics, sitting in my office in Professor Craig’s Department of Paediatrics and Child health wondering just what I should research on. However, as clinical experience increases, they are a number of areas where management is identified as unsatisfactory and prompt a search for improvement. This is precisely what later happened in my own clinic in relation to a number of conditions affecting children particularly those with cystic fibrosis..

IMG_1834


Map of the Leeds area showing the hospitals where I  provided paediatric cover        

In those early days my job as a consultant in general paediatrics was varied and very busy.There were 3-hourly weekly outpatient sessions in Leeds at the General Infirmary, Seacroft Hospital, St James’s Hospital (not yet a “University Hospital”) and at Wharfedale Hospital in Otley and also a follow up clinic at at St Mary’s Maternity Hospital, Bramley.  I also had responsibility for a ward of paediatric beds at Seacroft, two paediatric beds at St James’ and half the small paediatric/surgical ward at Wharfedale Hospital Otley. Neonatal work was a significant part of my job including paediatric cover for the 3000 infants born annually at St Mary’s Maternity Hospital in Bramley, also occasional requests to see babies at Four Gables Maternity Home, at Horsforth in Leeds and at a small Maternity Home in Ilkley. The neonatal work could be very demanding; I recall once personally performing 5 exchange transfusions (taking 3 hours each) for haemolytic disease of the newborn at St Mary’s during the course of one weekend! On the Monday morning, after this personal tour de force, a notoriously irascible and senior pathologist phoned to ask if I would tell my junior staff to avoid sending so many blood specimens to the laboratory over the weekend – I put him in the picture!  In addition there were domiciliary visits to see ill children in their own homes at the request of their family doctor

Screen Shot 2018-04-06 at 11.24.14 2

Also there was one session each week of private practice on Friday afternoons in “rooms” at a house in 20 Clarendon Road, Leeds used by many of the established Leeds consultants.  Clarendon Road is near to the Infirmary and various houses used for consulting rooms and nursing homes for many years by the local consultants.   My modest private practice was managed by my wife Ann for the next 26 years. In 1970 for the privilege of doing private work the NHS consultant was termed “part time” and surrendered 2/11 of his relatively modest salary of £3,200pa.

But believe it or not, despite this ridiculous workload by present day standards, I was happy and really enjoyed my work – also I was so grateful to have obtained a consultant paediatric appointment in my home city, Leeds, even if not at the Leeds General Infirmary, the main teaching hospital. My appointment was a close run contest with Dr Charles Livingstone, an already established consultant in the nearby city of Wakefield, to the extent that the decision of the appointments committee in Leeds was not unanimous so the decision went to the Yorkshire Regional Health Authority in Harrogate. Charles Livingstone was supported by Dr Eric Allibone who was reluctant to see me, a “Craig man”, appointed. Medical politics can be a real pain, believe me!

So I was appointed and much relieved. Undoubtedly Ann and our young family adapted to this lifestyle and seemed to be happy, perhaps as that was all they knew! However, Ann and the family were a great support which I much appreciated.  In those days it was accepted that a doctor’s life was like that. It was to be expected when one entered the profession that one’s work would take high priority over most things other than one’s immediate family’s wellbeing. Most doctors worked full time and there was limited off duty – none for hospital consultants.  As one older doctor put it “medicine is a life not a job” which was certainly true in those days.    

Many lessons I learned during this period through the Seventies were those acquired by practical experience of dealing with numerous patients with a wide variety of acute and chronic conditions and with their parents. Also I learned a great deal from colleagues expert in many other disciplines. When more paediatricians were appointed in the early years of the National Health Service, it was tempting for people to consider they could deal with any medical problem affecting children which, of course, was not the case. Undoubtedly the gradual emergence of paediatric subspecialties led to a major improvement in health and survival of many children with more uncommon and serious conditions such a leukaemia, rare metabolic disorders, extreme prematurity and eventually cystic fibrosis. Undoubtedly the Seventies and Eighties were a time of major changes and progress in the treatment of childrens’ disorders.

Advice from a respected family doctor

Soon after I became an NHS consultant in 1968 a wise senior family doctor, Henry Shapiro, gave me some advice. He recommended I should be contactable at all times so the family doctors would realise that I was always available and were aware that I did not object to being contacted. He suggested  I should to treat all categories of patients in similar manner be they NHS or private. He suggested that I see private patients, not in my own home (as originally I had intended to do), but to hire a consulting room in the same building where other successful Leeds consultants had their “rooms”.  I took Henry Shapiro’s advice and was fortunate that Mr Reg Broomhead, the senior orthopaedic consultant at the Leeds General Infirmary, rented me the Friday afternoon session in his room at 20 Clarendon Road in the centre of Leeds as I have described.  So my plate was screwed at the bottom of a long list of successful consultants to right of the gate – just within range of the larger dogs seeking to relieve themselves. I added one further piece of advice myself – to treat doctors’ and colleagues’ children in the same way as any other patient i.e. not to spare investigations or hospital admission if I considered them to be necessary.

The many lessons learned during this period were from practical experience of dealing with numerous young patients and their parents and also learning from one’s colleagues in many other disciplines – hence the title “experiential learning” is relevant. I stress “the learning from and collobarating with colleagues” as of central importance in an era of increasing technology.  Much of my modest success has been due to collaboration with many expert colleagues such as Prof. Monty Losowsky and members of his Department of Medicine and many other colleagues at St James’s University Hospital.

Traditional consultants: “a consultant – a person who provides expert advice professionally”

When younger I was fortunate to meet or observe many of the older well-known paediatricians who had worked in the Thirties. One such consultant of the old school was

pasted-image-small-23

Dr. Bernard Schlesinger (1896-1984) a leading London paediatrician at The Hospital for Sick Children, Great Ormond Street London up to the early 1960s. During the Fifties he delivered a lecture to the Portsmouth Medical Society describing common mistakes that he had observed others make in his extensive paediatric consulting practice. The article was full of wisdom and good advice. He dryly observed “One of the few benefits which a physician gains from advancing years is experience through his mistakes”

I remember Dr Schlesinger vividly for he retired from the staff of the Hospital for Sick Children, Great Ormond Street, London in 1962 on the same day that I started work there as a Senior House Officer (SHO) working for Dr Phillip Evans. On my first day I was privileged to attend Dr Schlesinger’s very last ward round, also attended by many of the staff who had worked with him in the past. As a new boy from the North I was somewhat apprehensive when, during the course of the ward round, one of the children was noted to be in obvious respiratory distress with an acute attack of asthma. As the new resident doctor for the ward I was called upon to give some intravenous aminophylline.
“Had I used the drug before” asked Dr Schlesinger.   I confess “Yes Sir – many times” I said. So I administered the aminophylline as an intravenous bolus, as we did in those days, the child improved and the ward round moved on to my great relief!

The few leading paediatricians of that time, and there were still only a few, did a great deal of consulting work without the backing of the usual paediatric team of to-day. They saw numerous ill children and gained a vast personal experience. When they discussed a problem it was very much “my” opinion rather than “our” opinion and “I” did this rather than “we” did this as is customary today.

I confess on occasion I found the job to be a very lonely and  stressful, perhaps less so than experienced by many young consultants today! The fact consultant paediatricians were asked by family doctors to see ill children who were presenting significant diagnostic or management problems, in their own homes “out-of-hours”, considerably concentrated and enhanced their clinical experience and diagnostic skills. In the early days in the Sixties the family doctor would often be at the patient’s house to meet the consultant and would be present at the consultation. After seeing the child we would retire to another room and decide how to proceed; we then returned and informed the parents our opinion and what we suggested was the best course of action. Over the years I admitted to hospital approximately 50% of the children seen on such domiciliary visits – or conversely prevented 50% of such children being admitted to hospital. Good value for the NHS as I was only paid £6 per visit and mileage! However, there was no doubt that my willingness to see patients any time, anywhere, NHS or private, brought many children with difficult diagnostic and medical treatment problems under my care.

Children with type 1 diabetes usually presented as an acute problem that required immediate action when a nurse or doctor checked the urine of an ill child for sugar and ketones. Some years prior to my appointment as a consultant these children had been treated by the adult endocrinologists – not really very approriate. Some years previously, when a junior doctor, I observed an ill child admitted to a paediatric ward under the care the adult physicians, but dealt with by the registrar; the child died in a diabetic coma. I vowed that if ever I became a consultant I would treat children with diabetes – which I did from the time of my appointment. I went up a steep learning curve but Meventually had a regular paediatric diabetic clinic of over 40 patients. I eventually persuaded an absolutely brilliant diabetic nurse specialist, who worked in the the adult clinic at St James’s, Mrs Margaret Jackson, to help with the management of our children- her input was quite invaluable. There were relatively few consultant paediatricians in the UK even in the 1960s and they were expected to be experts on almost any condition affecting children – which of course they were not. It was inevitable that they would subspecialise as medicine became more complex.

One of the first paediatric appointments in the UK was in Leeds where, in 1919, a medical children’s department at the Leeds General Infirmary (LGI) was formed.

One of the physicians there, Dr. Wilfred Vining (1883-1967) (apologies for the quality of the photo) gave up his adult beds to take charge of this new unit. In 1921 a lectureship in the University of Leeds was established to which Dr. Vining was appointed; in 1927 he became the first Professor of Children’s Diseases in the UK, which carried with it an honorarium of £50 per annum. The income of doctors at that time before the NHS was derived from private practice.
At that time there were only three clinicians engaged full-time paediatric practice outside London; before the start of the NHS in 1948 other paediatricians had to combine their study of children with adult work in order to make ends meet. “Daddy” Vining, as he was affectionately known, eventually retired from the LGI in 1946 when he was succeeded by Professor Stuart Craig (1903-1975). In 1948 Dr Eric Allibone was appointed as the first NHS consultant paediatrician in Leeds with responsibility for half the city’s paediatrics, most of the domiciliary and private practice and substantial teaching commitments.

Throughout his career Prof. Vining was closely involved in the development of paediatrics in the UK. I well recall he was still doing consultant locums at the Leeds General Infirmary (LGI) when I was a paediatric house officer there in 1956/57. Famous quotes of his included “The children of the rich do not ail”. In an address to the West Riding Medico-Chirurgical Society in 1941 he said – “I will begin with what I believe to the keystone of the whole of children’s medicine, namely nutrition”. It is relevant that for much of his career many families in the UK were too poor to feed their children an adequate diet.

The British Paediatric Association

RCPCH 2″ width=”250″>From an Association to a Royal College: The History of the British Paediatric Association and Royal College of Paediatrics and Child health 1988-2016. Alan Craft and Keith Dodd. Springer May 2017.

The advertisement for this book states – “This recent book describes the history of the Royal College of Paediatrics and Child Health (RCPCH) covering the period from 1988 to 2014, which includes the transition from the British Paediatric Association (BPA) to the formation of the now established RCPCH. The book contains a collection of viewpoints from paediatricians who held officer posts with the association and College through this time. The authors offer insights and reflections to help the reader understand where the College is at the moment on a variety of core paediatric issues, and significantly where it has come from. The formation of the RCPCH from the BPA was very controversial, with some members resigning from the RCP. Since then, the College has come a long way and is now recognised as the definitive body to speak for children’s health in the UK and has forged relationships with many overseas countries. It has produced influential documents, and lobbied for advances in the practice of paediatrics. It has reported on standards for how children should be cared for in emergency and urgent care settings, launched policy to improve how children are looked after in the secure estate, and produced teaching and training materials for child protection among many other contributions to child health”.

In the Sixties most paediatricians were “general paediatricians”

Only a minority of paediatricians in the major hospitals, such as Great Ormond Street, and Birmingham Children’s Hospital had special interests – most paediatric physicians in those days specialised in children rather than organs! Paediatric cardiologists were the exception and had already established themselves as specialists in the Fifties with their skills at performing and interpreting the results of cardiac catheterisations and angiograms of children with congenital heart disease.

In Leeds we were fortunate to have one of the early paediatric cardiologists, Dr Olive Scott, when her husband James Scott was appointed to the Chair of Obstetric and Gynaecology. Olive Scott was the first dedicated consultant paediatric cardiologist in Britain, appointed in 1966. Her original, tiny unit at Killingbeck Hospital, Leeds, grew to become one of the world’s leading centres in this small but important and fast-growing speciality, involving so-called “blue babies” and “hole-in-the heart” children. In the Sixties and early Seventies there were relatively few of the other specialised investigative procedures and treatments that are routine to-day. Paediatrics was itself a young speciality, which had only really became widely established following the start of the National Health Service in 1948 when, for the first time, there was a guaranteed income for those specialising in the subject.

Much of my early experience as a consultant paediatrician from 1968 was working in the old style as a medical paediatric consultant, professing expertise in disorders of children, prepared to see any child, at any time, anywhere if requested by the family doctor or a hospital colleague. If one was prepared to be available at any time of the day or night to see patients either as a NHS patient or a private patient, and gave a reasonable service to all wherever they were seen, gradually one built up a large practice. In the process I was fortunate to accumulate an increasingly wide experience in a variety of children’s disorders. Obviously this type of practice was not at all straight forward and as one gained more experience one learned of situations which would cause increasing anxiety and which should be approached with extreme caution.

Every consultation was a learning experience

Although Henry Ford, the great industrialist, maintained “History is more or less bunk” he knew nothing of medicine and a more appropriate quote for the clinician would be – “Intelligence is learning by experience”. This is now a vast subject in it’s own right and referred to as “experiential learning”. However, the concept is not original for even in 450 BC Confucius observed “Tell me, and I will forget. Show me, and I may remember. Involve me, and I will understand.”

One of the major problems particularly with modern medicine, both in hospital and the community, is the lack of continuity between doctor and patient which significantly reduces the learning experience of the doctor, which is already compromised by the mandatory generous off duty. So the problem in organisations such as the NHS is that the doctor may not observe the outcomes of his/her actions and, thus, cannot reflect on them to learn – an essential part of experiential learning. Obviously Einstein’s contention that experience is the only source of knowledge is not true although he obviously considers it important.

Peter Senge (1990) observed that we each have a learning horizon, a breadth of vision in time and space within which we assess our effectiveness. When our actions have consequences beyond our learning horizon, it becomes impossible to learn from direct experience. Herein lies the core learning dilemma that confronts organisations [and thus individuals]. We learn best from experience, but, unfortunately working in the modern NHS, the doctor may never directly experience the consequences of many of his/her most important decisions.

The paediatric consultation is a major learning experience for both the child’s parents and the doctor

clinic-16

Every child and their relatives seen for whatever problem by the doctor, however apparently trivial, represents a learning experience for the clinician and is usually a significant event for the child and the parent/s.
Whatever the evidenced-based medicine (EBM) experts say, it is the experience gained from studying many patients and following them up to observe the outcomes, that provides the clinician and his team with an ever-increasing personal database of experience on which to draw in the future. In medical schools there is great emphasis on anatomy and physiology and working problems out from first principles. However, in practice, usually clinical diagnoses are made by recognising patterns of symptoms and/or signs that are well known or that the doctor has seen previously, knows the significance of, is aware of the treatment of and likely eventual outcome.

Follow-up or a parental report on progress very important

The importance of the doctor following through a particular episode of illness cannot be over emphasised as one cannot know the importance of a particular sign or clinical situation if one does not have an idea of the eventual outcome. Mistakes occur due to patients having been seen by many different doctors not one of whom acquires the whole picture or grasps the seriousness of the condition – sometimes, sadly, until the situation is irretrievable.

During my first decade an NHS consultant paediatrician I gained a vast amount of experience.

Following my appointment as an NHS consultant in 1968, I became a very hard working, but very happy, general paediatric consultant with an increasingly busy consulting practice in Leeds and the surrounding area. I was prepared to see any child with a problem, anywhere, any time, privately or as an NHS patient; respiratory or gastrointestinal problems were frequent referrals. The many children referred presented many interesting and difficult clinical problems; I had a friendly fruitful collaboration with many of the paedaitric and non-paedaitric colleagues at Seacroft and St James’s University Hospital; sadly less so with the two paediatric colleagues at the Leeds General Infirmary!

This experience gained during the Sixties and Seventies formed the basis of a number of varied publications and provided a broad experience of paediatric medicine, which proved very valuable when dealing with children who had cystic fibrosis e.g. newborn screening, respiratory problems, nutritional and gastroenterological disorders, and later even diabetes.

Neonatal care and the introduction of cystic fibrosis screening

Through the Seventies I also still had a major commitment to neonatal care and was responsible for the supervision of 3000 newborns at St Mary’s Maternity Hospital in Bramley, Leeds. I also ran a large asthma clinic, a gastroenterology clinic, provided a jejunal biopsy service, a urinary infection clinic and even a paediatric diabetic clinic – in fact, I became an experienced and very general paediatrician – or perhaps others would describe me as a “jack of all trades” and master of none!

Despite theses wide interests, I always favoured the idea of specialised clinics where one could collaborate with expert colleagues such as other clinicians, laboratory workers, physiotherapists, dietitians, experienced nurses and where one could work as part of a team – a technique which eventually formed the basis of our Regional Cystic Fibrosis Service. From an early stage I always enlisted the help and expertise of colleagues be they medical, surgical, nursing, allied professionals, laboratory workers 0r administrators  – this was undoubtedly an important factor when building up our CF team through the Eighties. Also I encouraged those colleagues with whom we cooperated to become involved in research relating to the clinical problems that we were encountering. The publications from colleagues and myself during this “pre-CF” phase up to 1980 reflect this tendency. The publications detailed below include one or more chemical pathologists, biochemists, laboratory technicians, neurologists, radiologists, nurses, adult physicians, general practitioners in addition to my immediate junior paediatric staff and colleagues.

– Littlewood JM. Candida infection of the urinary tract. Case report, with a review of the literature and a study of frequency of yeast isolations from the urine of children with bacterial urinary infections. Br J Urol. 1968 Jun;40(3):293-305.
– Dickinson JP, Holton JB, Lewis GM, Littlewood JM, Steel AE. Maple syrup urine disease. Four years’ experience with dietary treatment of a case. Acta Paediatr Scand. 1969 Jul;58(4):341-51.
– Littlewood JM. Asymptomatic bacteriuria. Br Med J. 1969 Aug 16;3(5667):416.
PMID: 5797794
– Littlewood JM, Kite P, Kite BA. Incidence of neonatal urinary tract infection. Arch Dis Child. 1969 Oct; 44(237):617-20. PMID: 4981204
– Littlewood JM. Spina bifida. Nurs Times 1970; 66(1):5-8
– Littlewood JM, Hunter I, Payne RB, Miles DW. Placental transfer of an IgG paraprotein associated with prolonged immunosuppression. Br Med J. 1970 Oct 10;4(5727):94-5. PMID: 5471777
– Littlewood JM. White cells and bacteria in voided urine of healthy newborns.
Arch Dis Child. 1971 Apr;46(246):167-72. PMID: 5576025
– Littlewood JM. 66 infants with urinary tract infection in first month of life.
Arch Dis Child. 1972 Apr;47(252):218-26. PMID: 5023470
– Littlewood JM. Letter: The prevalence of bacteriuria in full-term and premature newborn infants. J Pediatr. 1973 Nov; 83(5):888-9. pMID: 4742588
– Davies JM, Gibson GL, Littlewood JM, Meadow SR. Prevalence of bacteriuria in infants and preschool children. Lancet. 1974 Jul 6;2(7871):7-10. PMID: 4134405
– Kissach AW, Currie S, Harriman DG, Littlewood JM, Payne RB, Walker BE. Letter: Leigh’s disease and failure of automatic respiration. Lancet. 1974 Sep 14;2(7881):662. PMID: 4137785
– Arrowsmith WA, Payne RB, Littlewood JM. Comparison of treatments for congenital non-obstructive non-haemolytic hyperbilirubinaemia. Arch Dis Child. 1975 Mar;50(3):197-201. PMID: 1147651
– Meadow SR, Davies JM, Gibson GL, Littlewood JM. Letter: Urinary tract infection in children of preschool age. Arch Dis Child. 1975 Jul; 50 (7):578. No abstract available. PMID: 1167077
– Eastham DG, Littlewood JM, Burton D. Letter: Monoparesis following CPAP.
Arch Dis Child. 1975 Aug; 50(8):668-9. No abstract available. PMID: 1106335
– Ghosh SK, Littlewood JM. The clinical picture of coexisting toxoplasma and toxocara infection and its management. A small child with a rare double infection. Clin Pediatr (Phila). 1976 Jan;15(1):31-3. No abstract available. PMID: 1245080
– Littlewood JM, Lee MR, Meadow SR. Treatment of childhood Bartter’s syndrome with indomethacin. Lancet. 1976 Oct 9;2(7989):795. No abstract available. PMID: 61458
– Littlewood JM, Payne RB. Placental transfer of IgG paraprotein with prolonged immunosuppression. Br Med J. 1977 Jan 29; 1(6056): 291. No abstract available.
PMID: 837084
– Littlewood JM. Neonatal screening: the present position. Midwives Chron. 1977 May;90(1072):91-3. No abstract available. PMID: 586487
– Ghosh SK, Littlewood JM, Goddard D, Steel AE. Stool microscopy in screening for steatorrhoea. J Clin Pathol 1977 Aug;30(8):749-53. PMID: 599187
– Littlewood JM, Jacobs SI, Ramsden CH. Comparison between microscopical examination of unstained deposits of urine and quantitative culture. Arch Dis Child. 1977 Nov; 52 (11):894-6. PMID: 339849
– Treatment of Bartter’s syndrome in early childhood with prostaglandin synthetase inhibitors. Littlewood JM, Lee MR, Meadow SR. Arch Dis Child. 1978 Jan; 53(1):43-8. PMID: 415668
– Smith SE, Littlewood JM. The two-film barium meal in the exclusion of coeliac disease. Clin Radiol. 1977 Nov;28(6):629-34. PMID: 589918
– Littlewood JM. The diagnosis of cystic fibrosis. Practitioner. 1980 Mar; 224(1341): 305-7. PMID: 7403012

This last article was the first on cystic fibrosis I wrote at the invitation of the late Dr. Archie Norman of The Hospital for Sick Children, Great Ormond Street, London who, at the time, was Chair of the Cystic Fibrosis Trust’s Research and Medical Advisory Committee.

ARCHIE NORMAN

During the Seventies consultants in the Yorkshire Region increasingly referred children to Seacroft for Mr Alan Steele’s reliable sweat tests and my jejunal biopsy  service which I had started in the late Sixties. The sweat tests performed by Alan Steele from the early Sixties became a well-established service and most paediatricians from Leeds and surrounding general hospitals used the service for their patients.

In 1968 I obtained a paediatric sized Crosby intestinal biopsy capsule (Watson capsule) and started a paediatric jejunal biopsy service; this facility soon attracted referrals from paediatricians in the region to confirm or exclude coeliac disease in their children. We eventually changed to the twin port Kilby modification of the Crosby capsule to use one specimen for histology and the other for disaccharide estimation (Kilby, 1976). In this venture I had the invaluable help of my friend

and colleague Dr. Sidney Smith, our paediatric radiologist, for rapid X-ray screening of the capsule into the duodenum, and Dr. Mike Mason the pathologist for histology reports.

Later Dr. Avril Crollick was appointed as my Clinical Assistant and eventually performed many of the biopsies until I started using the paediatric endoscope for the purpose during the Eighties. The ability to obtain jejunal mucosa for histological and biochemical examination from children was a relatively recent but major advance in the Seventies.

Avril Crollick

The characteristic histological small bowel appearance of subtotal villous atrophy of gluten induced coeliac disease was first identified in 1954 by Paulley, a physician in Ipswich, in laparotomy specimens from four adults with idiopathic steatorrhoea (coeliac disease). In 1957 Margot Shiner was the first to obtain specimens by per oral small bowel biopsy from an 8-year old child; soon a series in children was reported by Charlotte Anderson.
In contrast to the subtotal villous atrophy in coeliac disease, in people with CF the intestinal villi are of normal height – in some children with CF they appeared to be even taller than normal.

Normal intestinal mucosa -tall villi
Subtotal villus atrophy in coeliac
Paulley J W. Observations on the aetiology of idiopathic steatorrhoea. Jejunal and lymph node biopsies. BMJ 1954; 173:1318-1321. [PubMed]
– Sakula J, Shiner M. Coeliac disease with atrophy of the small intestine mucosa. Lancet 1957; ii: 876-877. [PubMed]
– Anderson CM. Histological changes in the duodenal mucosa in coeliac disease. Reversibility during treatment with a wheat gluten free diet. Arch Dis Child 1960; 35:419-427. [PubMed]
– Kilby A. Pediatric small intestinal biopsy capsule with two ports. Gut 1976; 17:158-15

Jejunal biopsy with a paediatric sized Crosby capsule was a relatively new technique in the Sixties. Initially Sir Wilfred Sheldon (the senior consultant paediatrician at Great

Tempany Eddie

Eddie Tempany 

Ormond Street) and a senior registrar at GOS at the time, Dr. Eddie Tempany, who was subsequently Professor of Paediatrics in Dublin, considered the investigation to be “outside the scope of routine investigations in children” due to the increased difficulty and risk of perforation reported in small children.
It is true there were a number of reports of intestinal perforation. On one occasion, when I was working at GOS and observing Eddie performing a biopsy, the capsule could not be retrieved until the next day.

However, my delay in starting jejunal biopsies in Leeds was mainly related to the fact that Sir Wilfred Sheldon and our Professor Stuart Craig were old friends and, as I was then a lecturer in Craig’s department, he did not approve of my performing jejunal biopsies. So I had to wait until 1968 and I became an NHS consultant.  Over the next 25 years in my unit we had no serious complications from jejunal biopsies in children aged down to 3 months.

Subsequently elsewhere I have described in detail our experience with over 1000 jejunal biopsies and 108 children with coeliac disease in my friend Professor Peter Howdle’s book (Howdle PD, 1997). Peter, a major friend of paediatrics, eventually succeeded Monty Losowsky as professor  of medicine at St James’s. He was an excellent and helpful colleague in many areas of gastroenterology where his expertise benefited many of our young patients including those with cystic fibrosis.

Littlewood JM. Coeliac disease in childhood. In: Howdle PD (ed) Coeliac Disease. Clinical Jgastroenterology. Vol9/No2. 295-328. Bailliere Tindall, 1995

Peter Howdle was a brilliant colonoscopist and his skill in this area was central to the development of our paediatric colonoscopy service – one of the first in the UK (Howdle et al, 1984). Peter taught me how to perform fibreoptic sigmoidoscopies but I never mastered full colonoscopies which Peter performed for us for many years. His experience with colonoscopies in children was valuable when fibrosing colonopathy was described in the Nineties as a new and worrying complication in some children with cystic fibrosis

Howdle PD, Littlewood JM, Firth JM, Losowsky MS. Routine colonoscopy service. Arch Dis Cild 1984; 59(8):790-3. PMD 6476884

Incidentally, from our experience with performing jejunal biopsies for many paediatric colleagues around the Yorkshire region, over 10 years we observed that coeliac disease was becoming less common in young children presumably due to changes in diet (Littlewood et al, 1980). (see also pages below on original observations for details)

Professor John Walker-Smith believes that the development of the technique to perform oral small intestinal biopsy in childhood was the real beginning of paediatric gastroenterology.  I would support this view (Walker-Smith, 1997). Certainly to provide a specialist CF service the jejunal biopsy was an essential investigation at the time although immunological tests have become more reliable in recent years.

– Sheldon W, Tempany E. Small intestine per oral biopsy in coeliac children. Gut 1966; 7:481-489.
– Howdle PD, Littlewood JM, Firth J, Losowsky MS. Routine colonoscopy service. Arch Dis Child 1984; 59:790-3
– Walker-Smith JA. Historic notes in pediatric gastroenterology. J Pediatr Gastroenterol Nutr 1997; 25: 316
– Littlewood JM. Coeliac disease in childhood. In: Howdle PD (Ed.). Clinical Gastroenterology. International Practice and Research. Bailliere l, London. 1995; 9: 295-328.
– Littlewood JM, Crollick AJ, Richards IDG. Childhood coeliac disease is disappearing. Lancet 1980; ii: 1359.

Paediatric sub-specialities usually followed the introduction of new techniques in adults

In the Sixties and Seventies paediatric sub-specialities often developed following the introduction of a specialised investigation that paediatricians learned from adult physicians; most were introduced during the Fifties and Sixties The first was obviously cardiac catheterisation by paediatric cardiologists in the late Fifties and then came various needle biopsy procedures. These included renal biopsy, percutaneous liver biopsy using the fine Menghini needle and small intestinal biopsy during the Sixties.

During the second half of the Seventies paediatric fibreoptic bronchoscopy was developed by Dr. Robert Wood of Cincinnati  when a prototype paediatric instrument became available leading to the first paediatric flexible bronchoscope in 1978. In 1980 Wood writes “We have utilized a prototype pediatric flexible bronchoscope to perform diagnostic and therapeutic procedures on pediatric patients ranging from 840 gm to 14 years of age. Flexible bronchoscopy, with appropriate instrumentation and careful attention to physiological requirements of the patient, is safe and effective in pediatric patients. The availability of new instrumentation promises to have as great an impact on pediatric pulmonology as the standard flexible bronchoscopes did on adult pulmonology. With this instrument, the indications for bronchoscopy may be considerably expanded”

The indications and availability of paediatric flexible bronchoscopy continued to expand and more recently it has even been suggested that every infant with CF identified by newborn screening should be bronchoscoped to obtain cultures from the lower airways (Hilliard TN et al. Arch Dis Child 2007; 92:898-899). However, provisional data from a more recent major study from Australia by Claire Wainwright would not support this suggestion, as the information from regular bronchial lavages resulted in no better outcome than from routine therapy (Wainwright CE et al 2011).

– Wood RE, Sherman JM. Pediatric flexible bronchoscopy. Ann Otol Rhinol Laryngol 1980; 89(5Pt1):414-416
– Wood RE. “Pediatric flexible bronchoscopy: The inside story”. In: Doershuk CF, (Ed.). Cystic Fibrosis in the Twentieth Century. Cleveland: AM Publishing Ltd, 2001:112-119.).
– Wainwright CE et al. Effect of bronchoalveolar lavage-directed therapy on Pseudomonas aeruginosa infection and structural lung injury in children with cystic fibrosis: a randomized trial. JAMA 2011; 306:163-171). [PubMed]

Many of these developments occurred in a limited number of centres pduring the Seventies and Eighties so our local combination of a reliable sweat test and a jejunal biopsy proved to be a powerful, and, as yet, not widely available, combination for investigating children with significant malabsorption problems and possible cystic fibrosis. As more children were referred to me for investigation by our team, there was an increasing interest by colleagues in paediatric gastrointestinal problems at Seacroft and St James’s. Subsequently this led to increasing cooperation with Professor Losowsky’s University Department of Medicine at St James’s University Hospital; in that unit the main research interests were gastroenterology and nutrition.

In 1975 – the first publication on CF from Seacroft

In the late Sixties Dr. Beat Hadorn, a paediatrician from Bern, had published a method for performing pancreozymin-secretin stimulated pancreatic function tests using a triple lumen tube (Hadorn et al, 1968). I obtained a supply of these tubes and our senior registrar at the time, Dr. John Davies, developed pancreatic function tests at Seacroft

Pancreatic function test in progress

Pancreozymin secretin pancreatic function test is involved and upleasant 
Pancreatic function test Anderson & Burke 1973

with the help of our consultant radiologist Dr. Sydney Smith. For the pancreatic function test the patient is fasted, heavily sedated has an intravenous line inserted and the triple lumen tube sited under x-ray guidance. After an injection of pancreozymin (P) two 10-minute periods of juice rich in enzymes and bile are obtained. After secretin (S) four 10-minute periods of juice rich in bicarbonate is obtained. In CF juice is very scanty and low in enzymes and particularly in bicarbonate.
The tests proved useful and interesting but were too involved and invasive for routine use; but they did contribute significantly to the first ever publication on CF from Seacroft

Jim Sarsfield
Jim Sarsfield

in 1975 by the Drs. Jim Sarsfield and John Davies. The tests provided supporting evidence that two children with negative sweat tests, but the typical clinical features of CF did have cystic fibrosis (Sarsfield & Davies, 1975). The rare occurrence of a normal sweat test in an individual with clinical features of CF has been confirmed in subsequent publications. Subsequently we had another child who had gross malabsorption and the typical clinical features of CF but a repeatedly normal sweat and a normal jejunal biopsy. The sweat test in this child eventually became positive and she died of CF at the age of 25 years.

Sarsfield JK, Davies JM. Negative sweat tests and cystic fibrosis. Arch Dis Child 1975; 50:463-466.

1975 Neonatal cystic fibrosis screening begins at St Mary’s Maternity Hospital, Bramley

pasted-image-small-51

St Mary’s Maternity Hospital in Leeds was initially the Bramley Union Workhouse and which opened in 1871. By the time I was appointed in 1968 to provide paediatric cover there it had become the largest maternity unit in the city but with slightly less privileged clientele than the other two units at the city’s main teaching hospitals. A significant proportion of our 3000 annual admissions were admitted for “social” reasons. It was slightly “out on a limb” and not really ideal for acute obstetrics but the staff were very dedicated and there were plenty of babies and a really excellent special care baby unit sister, Doreen Burton.

My personal interest in newborn screening had developed some years previously during the mid-Sixties when I was lecturer in paediatrics with Professor Craig at the Leeds Maternity Hospital; this eventually led to an MD thesis in 1968 on the incidence of neonatal urinary tract infection and various other related publications from the thesis.

– Littlewood JM. Asymptomatic bacteriuria Br Med J. 1969 Aug 16;3(5667):416. PMID: 5797794
– Littlewood JM, Kite P, Kite BA. Incidence of neonatal urinary tract infection. Arch Dis Child. 1969 Oct; 44(237):617-20. PMID: 4981204
– Littlewood JM, Hunter I, Payne RB, Miles DW. Placental transfer of an IgG paraprotein associated with prolonged immunosuppression. Br Med J. 1970 Oct 10;4(5727):94-5. PMID: 5471777
– Littlewood JM. White cells and bacteria in voided urine of healthy newborns. Arch Dis Child. 1971 Apr;46(246):167-72. PMID: 5576025
– Littlewood JM. 66 infants with urinary tract infection in first month of life. Arch Dis Child. 1972 Apr;47(252):218-26. PMID: 5023470
– Littlewood JM. Letter: The prevalence of bacteriuria in full-term and premature newborn infants. J Pediatr. 1973 Nov; 83(5):888-9. pMID: 4742588
– Davies JM, Gibson GL, Littlewood JM, Meadow SR. Prevalence of bacteriuria in infants and preschool children. Lancet. 1974 Jul 6;2(7871):7-10. PMID: 4134405
– Meadow SR, Davies JM, Gibson GL, Littlewood JM. Letter: Urinary tract infection in children of preschool age. Arch Dis Child. 1975 Jul; 50 (7):578. No abstract available. PMID: 116707
– Littlewood JM. Neonatal screening: the present position. Midwives Chron. 1977 May;90(1072):91-3. No abstract available. PMID: 586487
– Ghosh SK, Littlewood JM, Goddard D, Steel AE. Stool microscopy in screening for steatorrhoea. J Clin Pathol 1977 Aug;30(8):749-53. PMID: 599187
– Littlewood JM, Jacobs SI, Ramsden CH. Comparison between microscopical examination of unstained deposits of urine and quantitative culture. Arch Dis Child. 1977 Nov;52 (11):894-6. PMID: 339849

Although from 1968 my role as a general paediatrician included the paediatric supervision of the 3000 infants born each year at St Mary’s, neonatology in the days before ventilation of neonates was quite different and much less demanding than today. In the mid-Seventies, after discussion with Miss Little, the very supportive Matron, it was agreed we introduce the recently available BM Meconium screening test for cystic fibrosis.  The BM meconium test depends on the abnormally high protein content of the meconium (first bowel action) of infants with cystic fibrosis.    In Wiser’s study (figure) using immunoelectrophoresis, the meconium from five infants with a history of CF was examined for increased protein (figure). Three infants with CF (1,2,4) had obviously raised albumin levels, which did not occur in the two unaffected at risk cystic fibrosis infants (3,5) or in two healthy controls (6,7)

Wiser’s study

Later Schutt & Isles (1968) from Bristol showed that the increased meconium albumin could be recognised very simply by using the Albustix dipstick test designed for testing urine for albumin. A few drops of water mixed with a small amount of meconium was placed on a tile and an Albustix applied to the edge of the drop when it would turn blue if there was excess albumin (figure of urine Albustix). This method eventually formed the basis of the commercially available Boehringer Mannheim test (BM test) used with variable success in a number of neonatal CF screening studies in the Seventies (Stephan et al 1975; Ryley et al 1979; Evans et al, 1983).

– Schutt WH, Isles TE. Protein in meconium from meconium ileus. Arch Dis Child. 1968 Apr;43(228):178–181.
– Stephan U, Busch EW, Kollberg H, Hellsing K. Cystic fibrosis detection by means of a test-strip. Pediatrics. 1975 Jan; 55(1):35–38
– Ryley HC, Neale LM, Brogan TD, Bray PT. Screening for cystic fibrosis by analysis of meconium for albumin and protease inhibitors. Clin Chim Acta. 1975 Oct 15;64(2):117–125.

Undeterred by the lack of enthusiasm displayed by my two senior paediatric consultant colleagues at the other two maternity units, in 1975, after discussions with Miss Little the very supportive Matron, we introduced the BM Test at St Mary’s Maternity Hospital. However, after a few weeks, on my visits to the maternity wards, I frequently noted little plastic pots containing small quantities of meconium on the window sills, some even growing mould! It soon became obvious that even this simple strip test was asking too much of the overworked but always willing midwives! Fortunately Dr. Robert Evans, at the time the consultant biochemist at St James University Hospital, across the city, agreed to perform our BM tests in his laboratory on specimens of meconium collected into tiny pots by the midwives from all infants. This system worked very well and in 1981 we reported favourable results since we started BM screening in 1975 (Evans et al, 1981). Although by 1981 most paediatricians had either never started or by this time rejected the BM test for neonatal CF screening, at St Mary’s Maternity Hospital in East Leeds the test had become an established routine.
We had more success than in some other places and our false negative rate of only 12% – but as described above I found, as had the people in Veneto, Italy that the test must to be carried out in the laboratory with proper quality control and not on the wards by the midwives who, we soon realised, were far too busy. This was undoubtedly the reason other studies had failed.

Neonatal screening is established routine at St Mary’s, Leeds

Although others were reporting the BM test was unsatisfactory (Stephan et al 1975; Ryley et al 1979; Prosser et al 1974), it slowly became apparent that we were obtaining reasonable results, but only because the test was performed in the laboratory with proper quality control which was not the case elsewhere. The other important reason many paediatricians were unenthusiastic about neonatal CF screening was they did not believe early diagnosis affected the long-term outlook; of course this was true if early diagnosis was not accompanied by early effective treatment. It is true that in the Seventies and Eighties there was no published evidence that early diagnosis through neonatal screening and the start of treatment available at the time, improved the eventual appalling outlook for infants with CF. However, common sense suggested otherwise! Indeed it was not until 2001 that definite generally accepted evidence of long term benefit on growth was published from Phillip Farrell’s unit in Wisconsin (Farrell et al, 2001). In fact, a large study from Wales and West Midlands in the Eighties, funded by the Cystic Fibrosis Trust had failed to show an advantage for the screened infants (Chatfield et al 1991). This was almost certainly due to the fact that many of the CF infants identified did not receive specialist CF centre care but were treated at a number of local hospitals.

– Prosser R, Owen H, Bull F, Parry B, Smerkinich J, Goodwin HA, Dathan J. Screening for cystic fibrosis by examination of meconium. Arch Dis Child. 1974 Aug;49(8):597–601.
– Chatfield S, Owen G, Ryley HC, Williams J, Alfaham M, Goodchild MC, Weller P. Neonatal screening for cystic fibrosis in Wales and the West Midlands: clinical assessment after five years of screening. Arch Dis Child. 1991 Jan;66(1 Spec No):29-33.
– Farrell PM, Kosorok MR, Rock MJ, Laxova A, Zeng L, Lai HC, Hoffman G, Laessig RH, Splaingard ML. Early diagnosis of cystic fibrosis through neonatal screening prevents severe malnutrition and improves long-term growth. Wisconsin Cystic Fibrosis Neonatal Screening Study Group. Pediatrics. 2001 Jan; 107(1):1-13.

So despite the lack of national and local enthusiasm (including both consultant paediatricians at the other two maternity units in Leeds) neonatal CF screening became an established routine in our own neonatal unit at St Mary’s, Bramley. In the first 5 years from 1975, 15,734 newborns were screened; a positive BM Test was obtained in 130 of whom 7 had CF – this was the expected incidence of 1/2243 live births. Also the false positive rate was an acceptable 0.83% (Evans et al, 1981).

– Evans RT, Little AJ, Steel AE, Littlewood JM. Satisfactory screening for cystic fibrosis with the BM meconium procedure. J Clin Pathol 1981;34(8):911-3.

Periodically during this first five years from 1975 we would identify a newborn with CF and it would seem sensible to start treatment with a regular anti-staphylococcal antibiotic (cloxacillin) as recommended by Dr. David Lawson (figure) one of the UK.’s few leading experts at the time, and also to start physiotherapy, pancreatic enzymes and vitamin supplements.

David Lawson

We used the BM test for the next 20 years in the eastern half of Leeds both at St Mary’s and later also at the smaller St James’s University Hospital maternity unit when the two units merged and moved into new accommodation at St James’s in 1979. Dr. Ian Forsythe, the consultant paediatrician in charge of the St James’s neonatal unit before we merged, reluctantly agreed to introduce the test; he usually voiced his opposition to CF screening at every opportunity! We eventually replaced the BM test with the immunoreactive trypsin heel prick test (IRT) in 1995 at which time the neonatal unit at the Leeds General Infirmary eventually and reluctantly agreed to introduce neonatal CF screening. So there has been continuous neonatal CF screening in East Leeds first at St Mary’s and later at St James’s since 1975. Only one area of Italy had been screening continuously for longer having started in 1974 (Mastella et al, 1981).

– Mastella G1, Zanolla L, Castellani C, Altieri S, Furnari M, Giglio L, et al. Neonatal screening for cystic fibrosis: long-term clinical balance. Pancreatology. 2001;1(5):531-7.

1976 A monthly clinic at Seacroft was reserved for children with CF – the start of the Leeds CF Service

In 1975/6, soon after neonatal CF screening started at St Mary’s, I started to follow-up the first few screened CF infants, and with the agreement of the nursing staff, we set aside a monthly Monday afternoon paediatric clinic session for our few CF children at Seacroft. The majority of the children’s medical beds were situated there and it was my base where I had a major clinical commitment. I had an office and my first full time secretary, Christine Silburn, who came straight from college, worked with me until I retired in 1997 and is still in 2017 working as senior manager of the Regional Paediatric CF Unit in Leeds! In this CF clinic venture I had the enthusiastic support of the nurse in charge of Seacroft outpatients (Sister Moriarty), the paediatric physiotherapist (Miss Jenkins) and the paediatric dietitian. June Wilson, was Sister on one of the paediatric wards where all my beds were situated at that time.

pasted-image-small-89
The modest outpatient clinic at Seacroft 

In the photos is the small outpatient building where first CF clinic was held. The outpatient Sister Moriarty and ward sisters were also becoming increasingly interested and knowledgeable about CF as more children with condition came to the ward either for investigations such as sweat tests or for admission. The importance of the support and interest of staff with special expertise cannot be over emphasised.
Paediatric colleagues gradually became aware of our new Monday afternoon “CF clinic” and of my increasing interest in the condition. Also when the Professor of Paediatrics, Dick Smithells, informed all paediatricians in the Leeds Region of the special clinics in his department at the General Infirmary, I took the opportunity to inform our regional paedaitric colleagues of my CF clinic at Seacroft!

By the late Seventies I was also running special clinics sessions designated for gastroenterology, asthma, diabetes and urinary tract infections (this with Professor Roy Meadow and Mr. Bob Williams, a distinguished local urologist). This may seem rather excessive but I was now an established Leeds consultant and receiving many referrals; it seemed reasonable to assemble children with certain chronic problems into special clinics where one could concentrate the mind on their problems and they could be seen and discussed with various expert colleagues.

A broad experience in paediatrics as a base for a CF service

An undoubted highly relevant factor in the development of our Leeds CF service was my broad experience of general paediatrics – so important when dealing with children with CF, a condition that seems to affect every system in the body.

In this context, in the early 20th Century Sir William Osler (used to teach “Know syphilis…and all other things clinical will be added unto you”.In the Sixties Sir Stanley Davidson suggested that, as we passed from the era of infection to metabolic disorders, Osler’s dictum might well be changed to “Know diabetes……..”. Now in the era of genetic diseases, in a lecture in 1983 I suggested – “Know cystic fibrosis …and all other things clinical will be added unto you” may be even more appropriate, so varied and complex are the problems encountered in people with the condition?

(from Advanced Medicine. 1983 Eds. Losowsky MS, Bolton RP. Royal College of Physicians, London 1983).

1978. The first cystic fibrosis research study from Leeds

At that time, when we had some 25 children attending our monthly CF clinic at Seacroft, research collaboration began with my friend and colleague Professor Monty Losowsky, Professor of Medicine at St James’s University Hospital. His academic department had a distinguished record of research  particularly into nutrition and various aspects of gastroenterology. I asked Monty if he and his colleagues would help us investigate the nutritional state of our CF children, as maintaining a reasonable nutritional state was a major problem, in fact virtually impossible.

As the children’s  respiratory infection progressed relentlessly it was virtually impossible to maintain a reasonable nutritional state as evidenced in the figure published around that time (Berry et al, 1975).

Monty agreed to join forces and with Dr. Jerry Kelleher, the senior biochemist in his department, we planned and started the first of many collaborative research projects with members of the Department of Medicine at St James’s after we moved there in 1980.

  Berry HK, Kellog FW, Hunt MM, Ingberg RL, Richter L, Gutjahr C. Dietary supplement and nutrition in children with cystic fibrosis. Am J Dis Child 1975; 129:165-171. 

In this first study, in the two years before the move to St James’s from Seacroft, we performed an evaluation of the present nutritional state of our patients with particular reference to the vitamin status – a particular interest of Dr. Jerry Kelleher’s, the senior biochemist in Monty’s department at St James’s. Also, we enlisted a few additional children with CF attending the clinics of the more cooperative and interested paediatric colleagues, to reach a total of 36 children with CF and 21 age-matched non-CF children as controls. When the children attended the  monthly CF clinic at Seacroft I would take a venous blood specimen and Jerry Kelleher’s wife would deliver it to his laboratory at St James’s some 2 miles down the road.

Jerry Kelleher

The parents of the children were enthusiastic, relieved that at last some research was underway and they were very supportive – as we always found them to be subsequently with all our research studies.  I was very pleased and grateful to be cooperating with such a very professional team of experts who were more experienced than myself or any of our team in research of this type. The study was supported by a grant from the West Riding Medical  Research Trust.

Cooperation with members of Monty Losowsky’s Department of Medicine at St James’s over nearly 20 years after this first and numerous subsequent combined projects, was undoubtedly one of the major factors responsible for the success and growth of our Leeds CF service.

This study was published in 1981 and I presented the provisional results at the North American CF Conference in Toronto in 1980.

Congdon PJ, Bruce G, Rothburn MM, Clarke PC, Littlewood JM, Kelleher J, Losowsky MS. Vitamin status in treated patients with cystic fibrosis. Arch Dis Child 1981; 56(9):708-714. (PMID 7294874)

In a future section I will describe in more detail the development of the Leeds Regional Cystic Fibrosis Service from its origin in the small monthly CF clinic at Seacroft to an internationally recognised clinic (www.cysticfibrosis.online) headed by Professor Daniel Peckham at St James’s University Hospital and Dr.Tim Lee at the Leeds General Infirmary.