No Abstract.  South African Medical Journal Vol. 96(9) (Part 2) 2006: 914-91

De Decker, R

Kalis, NN

Lawrenson, JB

Pribut, H

Shipton, S

Stirling, J

English

AJOL - African Journals Online

September 2006, Vol. 96, No. 9  SAMJSAMJ FORUM914In all provinces in South Africa specialist medical services are under threat. Many of these services are perceived as being expensive and serving patients with limited outcomes. Resource constraints (in particular nursing shortages and budgetary limitations) have forced specialists to examine their practices and curtail those practices that offer low yield at high financial cost. The recently published ‘head injury’ protocols are a good example of such a relook.1 Denying ventilatory support to premature infants weighing less than 900 g is another example of a policy designed to reassign resources to those who might achieve a better outcome. All cardiac surgical units within the State sector in South Africa are affected by these resource constraints. The long waiting lists for surgery are the most obvious manifestation of these limitations. For children with congenital heart disease, a longer waiting period may mean the difference between low-risk and high-risk (and more expensive) surgery. At worst, the wait may make safe surgery impossible. Prioritising patients within waiting lists is not an easy task. We have some experience with at least three approaches to shortening lists: 1. Increasing resources (even for a short period of time, as happened during 2000 in the Western Cape) helps to shorten the list. ‘Fairness’ can be achieved within a hospital setting by rotation of priorities – cardiac patients this year, tonsillectomy patients next year and so on. 2. The use of more expensive devices may allow non-surgical treatment of simpler lesions in the catheterisation laboratories rather than in the operating theatre. (While the cost of closing an atrial septal defect with a closure device may exceed the cost of the same procedure surgically in the setting of a developing country,2 the opening of an operating space for a child with tetralogy of Fallot may offset that extra cost, in terms of future hospitalisations and morbidity).3. Careful reorganisation of the list, which involves a thorough re-analysis of the indications for surgery and postulated outcome for each patient. It may require the wisdom of Solomon to deny surgery to patients who are perceived as benefiting less from surgery than others. With our profession’s basic commitment to preserve life for all who seek our help, selecting any patients who will not be helped is an ethical minefield. The only criterion that stands even a chance of being a ‘fair’ or ‘just’ basis for selection is the likelihood of long-term medical benefit from the care provided. There is no ethical ground for choosing on the grounds of long-term contribution to society or any other assessment of ‘worth’. But how do we assess the likelihood of long-term medical benefit? Personal moral judgements (of social worth and promise) inevitably cloud assessments of medical benefit. This seems at least in part to be the problem with certain patients being refused cardiac surgery.The ‘Baby Ronnie’ episode brought the (sincere) attempts of one province to shorten the waiting list of children waiting for cardiac surgery into the limelight. Baby Ronnie was flown at great cost to a private facility in the Cape when he was denied cardiac surgery in the State sector in another province.3What made Baby Ronnie different from any other child with the same congenital heart defect? He was born with Down syndrome.Why are some South African children with Down syndrome not being offered cardiac surgery?J B  Lawrenson, N N Kalis, H Pribut, J Hewitson, S Shipton, J Stirling, R De DeckerDivision of Critical Care and Children’s Heart Disease, School of Adolescent and Child Health, University of Cape Town, and Department of Paediatrics, Tygerberg Children’s Hospital, Stellenbosch University, Tygerberg, W CapeJ B  Lawrenson, MB ChB, FCP, MMedN N Kalis, MB ChB, FCP, MMed (current address: Mohammed Bin Khalifa Bin Salman Al Khalifa Cardiac Centre, Bahrain Medical Services Hospital, Bahrain)H Pribut, MB ChB, FCPJ Hewitson, FCS S Shipton, FCP, MRCP, MMedJ Stirling, MB ChB, FCP (current address: Nkosi Albert Luthuli Hospital, University of KwaZulu-Natal, Durban)R De Decker, MSc, FCPCorresponding author: J B Lawrenson (jlawrens@pgwc.gov.za)About 1 in 1 000 children has Down syndrome. Extra chromosomal material results in a myriad of potential problems for the affected individual. About 40% of Down syndrome children will have cardiac abnormalities, ranging from the simple arterial duct to the complex atrioventricular septal defect. Virtually all these defects are amenable to surgical correction and extended survival is possible. In South Africa many of these children do not undergo cardiac surgery. S Afr Med J 2006; 96: 914-919.ETHICSpg914-919.indd   914 8/24/06   10:05:17 PMSeptember 2006, Vol. 96, No. 9  SAMJ916The scope of the problem of children with Down syndrome and congenital heart defectsAbout 1 in 1 000 children has Down syndrome (most commonly due to trisomy 21).4 The extra chromosomal material results in a myriad of potential problems for the affected individual. About 40% of Down syndrome children will have cardiac abnormalities ranging from the simple arterial duct to the atrioventricular septal defect.5 Virtually all defects are amenable to surgical correction and extended survival is possible. The majority of children with Down syndrome and congenital heart defects (60%) will have simpler defects such as ventricular septal defects, tetralogy of Fallot or patent arterial ducts. In our opinion there are no medical reasons that make Down syndrome patients with these particular defects less likely to benefit from surgery than their counterparts with a normal karyotype. The other 40% of patients with Down syndrome and a heart abnormality will have an atrioventricular septal defect, which is a far more complex lesion.  This abnormality in essence involves the presence of a large gap in the walls between both atria and ventricles and abnormal atrioventricular valves. (These patients have a common atrioventricular valve instead of true ‘mitral’ and ‘tricuspid’ valves.6) Repair of the atrioventricular defect is a significant challenge for the surgeon. If repair of atrioventricular septal defects is delayed beyond the first year of life, irreversible pulmonary vascular changes may ensue as a consequence of the high-pressure left to right shunt. Surgery may then no longer be possible.7 After the first year of life, if they survive congestive cardiac failure and repeated chest infections, unoperated children enter a ‘honeymoon period’ as they develop irreversible pulmonary hypertension. They then succumb in their third decade from right heart failure or the consequences of persistent cyanosis.Patients with Down syndrome also have other associated abnormalities such as mid-face hypoplasia and airway obstruction that increase their propensity to develop accelerated pulmonary hypertension.8 For these reasons, there is a perception that cardiac surgery in these patients is more hazardous and associated with a higher morbidity and mortality. This perception is incorrect.Reasons given for not operating on Down syndrome children with an atrioventricular septal defectPoor surgical resultsIn an article published in the early 1980s Bull and colleagues9 argued that not offering surgery to Down syndrome patients with an atrioventricular septal defect resulted in equivalent survival to the surgical option. Despite cogent arguments, their ideas were highly controversial at the time. The article was published in The Lancet after having been rejected by the New England Journal of Medicine.10Improvements in surgical outcome have resulted in their conclusions becoming obsolete. The life expectancy of patients with Down syndrome has doubled since the 1970s largely as a consequence of treatment of these heart lesions.11 Most USA and UK centres would claim 30-day mortality figures of less than 5% for correction of an atrioventricular septal defect in a patient with Down syndrome.12There is no reason why South African surgical centres cannot achieve equivalent surgical results (Table I).  At Red Cross Children’s Hospital, the mortality for isolated complete atrioventricular canal repair was 6.9% (4 of 58, of whom 44 had Down syndrome).13 Limited lifespan and early dementia of patients with Down syndromeThe lifespan of a person with Down syndrome is less than that of a person with a normal karyotype.11 Nevertheless, the patient may reach an advanced age. In societies with resources, 13% of patients with Down syndrome reach the age of 60 years.14 Intellectual handicap is universal in individuals with Down syndrome and most children will face institutionalisation as adults. Up to 50% of patients with Down syndrome will show SAMJ FORUMTable I. Five years’ experience at Red Cross Children’s Hospital, comparing selected procedures with Stark et al.*              Red Cross: 1 unit, 5 years    UK: 5 units, 1 yearOperation  Number   Mortality (%)  Number   Mortality (%)All operations  1 334   5.1   1 378   4.0Isolated VSD     162   1.2      168   0.6Fallot’s tetralogy     115   3.5        88   2.3Switch (simple TGA)     15   6.7        67   0Complete AVSD      58   7        55   3.6*Reproduced from: Hewitson et al.13  VSD = ventricular septial defect; TGA = transposition of the great arteries; AVSD = atrioventricular septal defect.pg914-919.indd   916 8/24/06   10:05:18 PMSeptember 2006, Vol. 96, No. 9  SAMJ918signs of Alzheimer’s disease by the age of 50.15 Recent data suggest that the diagnosis of dementia in these individuals is difficult and late-onset hypothyroidism as well as depression as a consequence of the death of a caregiver may cause a ‘pseudodementia’.16For some, sheltered employment will be possible. For all affected individuals, however, the intangible elements that make up ‘quality of life’ will nevertheless be present in abundance.17Does not fixing a cardiac defect save money?The patient with a significant defect is likely to suffer from cardiac failure and repeated chest infections. The ‘honeymoon’ period alluded to previously may take a year to develop with symptoms and signs of congestion gradually decreasing unless the patient succumbs prior to this time. Each episode of pneumonia might result in a hospital admission. Each illness may be associated with time away from work for the child’s parents. The cost of an admission to hospital may rapidly start to offset money saved by not repairing a defect (see article by Roussot et al.18 in this edition of the journal). The patient with severe pulmonary hypertension is said to be relatively free from symptoms until the 2nd or 3rd decade.9 The end of their lives is associated with worsening dyspnoea and cyanosis and life-threatening dysrhythmias; in our experience, the last few years of these adolescent children’s lives are often miserable.  How many Down syndrome patients are there?Only 8% of patients with congenital heart disease have Down syndrome (i.e. on the average surgical waiting list only 8 of every 100 patients should have Down syndrome).  Therefore not performing surgery on these patients will represent only an 8% decrease in waiting lists. (If simpler lesions are corrected and atrioventricular septal defects are not, a ‘saving’ of 3 - 4% on a list will be achieved.) What are the rights of patients with Down syndrome?The rights to basic health care are guaranteed to all South Africans as a constitutional right.19 The definition of what constitutes ‘basic health care’ is open to interpretation. In South African law, the Soobramoney case is cited as a legal definition of basic health care and has been used to justify withholding super specialist services.20 It could be argued that an expensive operation that ‘guarantees’ a long survival is also effective and basic health care when the costs are defrayed over a long time period. For example, the chances of surviving for more than 30 years after repair of tetralogy of Fallot are excellent.21The challenge to health care planners is not only to make sure that no one succumbs from a simple pneumonia but also to ensure that the patient with congenital heart disease from Mafikeng has a similar chance to the patient from Cape Town to receive the surgery that will extend his/her life. The patients who benefit from so-called ‘ivory tower’ medicine do not come from a different country to the patient who dies when gastroenteritis is not treated properly.  Is it unethical not to offer surgery to South African patients with Down syndrome?Distribution of medical care on the basis of lifespan, quality of life, or contribution to society is a slippery slope towards denying care to many other patients, such as severe burns or oncology patients to mention only two. Why should Down syndrome be singled out?In an elegant article in the British Medical Journal, Savulescu22 argues that medical practitioners are used to rationing resources. Furthermore, limiting available treatments with some benefit in favour of treatments benefiting more people to a greater degree may not be illegal despite being unethical, provided decisions taken to limit medical care are taken in consensus and that the process is transparent. In addition, the decision to limit access to a particular resource should be considered for reversal should circumstances change. The reasons for not offering surgery to children with Down syndrome and congenital heart defects – never working, intellectual impairment, and the ‘stealing’ of surgery from potentially productive individuals – need to be carefully examined. In a country with 30% unemployment, ‘not being employable in the open market’ is a somewhat soft reason to deny surgery. In addition, the right of mentally handicapped individuals to work is protected under basic labour laws!23When we reject a child’s candidacy for surgery because he has a low IQ, then we have allowed judgements of social worth and promise to masquerade as questions of the likelihood of long-term medical benefits. It is disconcerting that within major urban centres there are units in the public sector hospitals which feel that they are not able to offer surgery to patients with atrioventricular septal defects and Down syndrome existing cheek-by-jowl with centres in the private sector where such children are offered surgery because they have parents with sufficient financial resources. ConclusionThe history of the South African medical establishment is tainted by less than perfect decisions taken for political and/or financial expediency. The decision to restrict a service or withhold a particular service must be taken against a backdrop SAMJ FORUMpg914-919.indd   918 8/24/06   10:05:18 PMSAMJ FORUM919of idealism and striving to obtain the maximum good rather than as a reaction to political or economic pressure. In the ‘supermarket’ of medicine, super-specialists are the ‘loss leaders’. They enhance the quality of the entire system and this benefits not the health economist but the patient.  Given scarcity of resources and in the face of the need to make tragic choices about how to deal with long waiting lists, what can we do? The first thing is to be truthful: acknowledge the limits and scarcities we face to both our patients and the public. There is a reticence to speak openly in the media about the health services in crisis because it puts health politicians in a bad light. But public money is being spent on a public service about which we must be transparent. Such transparency implies a readiness to admit that there are limits to what we can do, but also that we will not turn our backs on basic patient rights. The problems that beset South African cardiac surgery (which can be summarised as ‘too few operations for too many patients with too few nurses’) may in our opinion be better dealt with by reviewing our practices as a whole rather than denying surgery to one group of children with an easily identifiable genetic abnormality. References  1.  Benatar SR, Fleischer TE, Peter JC, Pope A, Taylor A. Treatment of head injuries in the public sector in South Africa. S Afr Med J  2000; 90: 790-793.  2.  Vida VL, Barnoya J, O’Connell M, Leon-Wyss J, Larrazabal LA, Castaneda AR. Surgical versus percutaneous occlusion of ostium secundum atrial septal defects: results and cost-effective considerations in a low-income country. J Am Coll Cardiol 2006; 17; 47: 326-331.  3.  Smetherham J. Down’s syndrome baby on the mend after heart op. The Star (Johannesburg) 5 February 2002.  4.  Boughman JA, Neill CA,  Ferencz C, Loffredo CA. The genetics of congenital heart disease. In: Ferencz C, Rubin JD, Loffredo CA, Magee CA, eds. Epidemiology of Congenital Heart Disease. The Baltimore-Washington Infant Study 1981-1989. Mount Kisco, New York: Futura, 1993: 123-167.  5.  Freeman SB, Taft LF, Dooley KJ, et al. Population-based study of congenital heart defects in Down syndrome. Am J Med Genet 1998; 80: 213-217.  6.  Anderson R, Ho SY, Falcao S, Daliento L, Rigby ML. The diagnostic features of atrioventricular septal defect with common atrioventricular junction. Cardiol Young 1998; 8: 33-49.  7.  Clapp S, Perry BL, Farooki ZO, et al. Down’s syndrome, complete atrioventricular canal, and pulmonary vascular obstructive disease. J Thorac Cardiovasc Surg 1990; 100: 115-121.  8.  Cooney TP, Thurlbeck WM. Pulmonary hypoplasia in Down’s syndrome. N Engl J Med 1982; 307: 1170-1173.  9.  Bull C, Rigby ML, Shinebourne EA. Should management of complete atrioventricular canal defect be influenced by coexistent Down syndrome? Lancet 1985; i: 1147-1149.10.  Shinebourne E, Carvalho J. Atrioventricular septal defect and Down’s syndrome. British Council Paediatric Cardiology Course, London. 9-16 March 1994. 11.  Yang Q, Rasmussen S, Friedman J. Mortality associated with Down’s syndrome in the USA from 1983 to 1997: a population-based study. Lancet 2002; 359: 1019-1025.12.  Hanley FL, Fenton KN, Jonas RA, et al. Surgical repair of complete atrioventricular canal defects in infancy: twenty year trends. J Thorac Cardiovasc Surg 1993; 106: 387-394.13.  Hewitson J, Brink J, Zilla P. The challenge of pediatric cardiac services in the developing world. Semin Thorac Cardiovasc Surg 2002; 14: 340-345. 14.  Baird A, Sadovnick A. Life expectancy in Down syndrome adults. Lancet 1988; ii: 1354-1356.15.  Menendez M. Down syndrome, Alzheimer’s disease and seizures. Brain Dev 2005; 27: 246-252.16. Roizen N, Patterson D. Down’s syndrome. Lancet 2003; 361: 1281-1289.17.  Alderson P. Down’s syndrome: cost, quality and value of life. Soc Sci Med 2001; 53: 627-638.18.  Roussot MA,  Lawrenson JB, Hewitson J, Smart R, de Decker HP. Is cardiac surgery warranted in children with Down syndrome? A case-controlled review. S Afr Med J 2006; 96: 924-930 (this issue).19.  Constitution of the Republic of South Africa 1996  Chapter 2 Section 27.20.  Soobramoney v. Minister of Health, KwaZulu-Natal CCT32/97.21.  Murphy JG, Gersh BJ, Mair DD, et al. Long-term outcome in patients undergoing surgical repair of tetralogy of Fallot. N Engl J Med. 1993; 329: 593-599. 22.  Savulescu J Resources, Down’s syndrome, and cardiac surgery. BMJ 2001; 322: 875-876.23.  South African Parliament. Employment Equity Act No. 55 of 1998.Accepted 4 July 2006.September 2006, Vol. 96, No. 9  SAMJpg914-919.indd   919 8/24/06   10:05:21 PM

Why are some South African children with Down syndrome not being offered cardiac surgery?

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Why are some South African children with Down syndrome not being offered cardiac surgery?

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