Roberts I and Montalembert M de – Department of Haematology, Imperial College London, UK (IR), Service de Pédiatrie Général, Hôpital Necker, Paris, France (MdM). Haematologica, the Haematological journal, 2007; 92(07): 865-871.

Telfer P, Coen P, Chakravorty S, Wilkey O, Evans J, Newell H, Smalling B, Amos R, Stephens A, Rogers D and Kirkham F – Haematologica, the Haematological Journal, 2007, 92 (07): 905-912.

COMMENTARY

By Dr Michael Angastiniotis, TIF Medical Advisor

European health services are becoming increasingly aware of the challenges posed by “new” chronic disorders – ones which are not prevalent in the indigenous populations, but are “imported” by immigrant groups. The most important of these are the haemoglobin disorders, Thalassaemia and Sickle Cell Disease (SCD). These conditions require expert multidisciplinary care, which in turn requires organised services for patients, but also programmes for carrier detection, early diagnosis, prevention, psychosocial care and community outreach.

In high prevalence areas such as Southern Europe, services have developed which include state-of-the art patient care coupled with prevention. These services have resulted in patient survival, good quality of life and a significant reduction in new affected births. The challenge facing Europe is to reach the same level of success in a situation where immigrant groups are scattered and living at some distance from specialised services, often not using the services available because of language, education, culture and factors.

As Irene Roberts and Mariane de Montelembert state in their excellent editorial, “the implications are much wider than the direct challenge of adapting services to patients’ needs”. As a first step, they point to the need to educate haematologists, physicians, paramedical staff and a wide variety of non-medical professional organisations involved in patient support. Community-based professionals who see few such cases will have little knowledge and experience in managing the disorders.

The planning of services in non-endemic countries is difficult, but is made more so by a lack of accurate epidemiological data. Many immigrant populations tend to settle in localised groups, leading to foci of high prevalence in their adopted countries, a fact which allows better planning of services. Clearly, these foci do not include all patients across Europe. A more accurate picture could be provided by national registers. However, attempts to establish comprehensive national registries have so far largely failed, because of insufficient funding. An alternative starting-point is to estimate carrier frequencies in the host countries, calculated from the frequency in the country of origin of the immigrant group. This was used in B Model et al (Epidemiology of haemoglobin disorders in Europe, Scand J Clin Lab Invest, 2007, 67:39-70). Such data give general information which can alert health authorities to the need for services. Another factor to keep in mind is the often rapid epidemiological change observed even in low prevalence countries; for example, the number of sickle cell carriers in Ireland increased from 60 known cases in 2001 to more than 3000 in 2006.

With improved life expectancy of patients and the young immigrant population, the prevalence of haemoglobin disorders can be expected to increase in the European setting. This was demonstrated by P Telfer et al in an article in the same journal (Clinical Outcomes in children with Sickle Cell disease, living in England: 2007, 92(7):905-912) in which they conclude that mortality in childhood SCD “can virtually be eliminated in a well-resourced health service setting, linking community-based care with a specialised hospital-based centre”.

It is this kind of comprehensive service that needs to be extended throughout Europe, with reference centres located at “foci of high prevalence”, but also, as the authors point out, “the establishment of clinical networks linking specialized centres with smaller clinics in areas, where the prevalence of SCD is low”. Another recommendation is the implementation of national standards of care.

In SCD, where neonatal screening is possible (which is not the case with Β-thalassaemia), early identification of affected individuals leads to their receiving specialised care early in life, as has been demonstrated by the good clinical outcomes reported by P. Telfer et al, and the linked ante-natal and newborn haemoglobinopathy screening programme established in England and Wales (see www.screening.nhs.uk). Such programmes are developing in some endemic countries such as Ghana, but these need to be expanded and adopted by health authorities as national programmes. In the well-resourced countries of Europe, Roberts and de Montelembert also recommend the formation of clinical care networks. They point out that motivated professionals and political will help to establish these policies. We would like to add that, as our own experience has shown, patient advocacy groups can also do a lot to promote political will.

Concerning patient care, the authors argue that “the aim has to be to provide comprehensive, but also seamless and holistic, care to address all of the patients’ needs, rather than fragmented care”. To achieve this high standard, both leadership and a team approach are required.

Modern management of chronic disorders includes the patient as an informed decision-maker alongside the care-team, giving him/her more control over the disease. Survival into adulthood requires the setting and fulfilment of goals, such as education, employment, marriage and social integration. These aspects of comprehensive care cannot be satisfied by specialised hospital services alone; they also require community-based care and support.

Finally, the importance of research networks to increase knowledge and understanding is highlighted. In particular, research collaborations between developing countries of high prevalence and well-resourced, non-endemic countries will be mutually beneficial, and will especially benefit the patients.

These two excellent papers refer to sickle cell disease, but the principles apply equally to thalassaemia.

In summary, several recommendations emerge which are useful for planning a comprehensive programme, not only Europe but also in the high prevalence areas of the world. These include:

  • Raising awareness
  • The need for accurate data to support health planning and the location of services
  • Screening programmes to identify carriers and affected children
  • Integrated clinical and laboratory services
  • Specialised hospital-based clinics offering comprehensive care and using audited, agreed guidelines of care
  • Clinical networks
  • Community-based services involving informed primary care doctors, nurses, social workers and patient support groups
  • Research collaborations within countries and beyond, especially with endemic low-resourced countries

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