Introduction

Spinal cord injury (SCI) alters every aspect of a person's life significantly and usually irreversibly. In addition to the inherent complexity of spinal cord lesions afflicting persons regardless of the age of the afflicted individual, pediatric SCI (pedSCI) is further complicated by issues related to ongoing physical and emotional growth.1, 2

Optimal initial medical and surgical management is of primary importance, but attention must also be given to preventive measures as well as to the injured child's psychosocial development and general quality of life. This may minimize disability and improve community participation in persons with pedSCI.3 PedSCI requires a comprehensive system of medical care tailored to the individual's needs and must furthermore be developmentally based.3

PedSCI is reported to be rare.4, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14 A low incidence and prevalence may result in scattered experiences in treatment and a low priority given to development of research and care programs.15 Thus, there is a need for increased exchange of information, experiences and best practice between countries. The rarity of this disorder would also merit extra European Economic Community (EEC) funding to enable cooperation between professionals in Europe.

An example of a successful international network from a similar field is the Paediatric Rheumatology International Trials Organisation (PRINTO), supported by the EEC, which facilitates and conducts high-quality research, and also provides information to afflicted children and their parents.16

The aim of this survey was primarily to collect information about incidence and systems of care, including prevention, initial care and follow-up for pedSCI in a subset of European countries, and Secondly, to initiate a network of involved professionals for exchange of information and development of research and care programs.

Methods

The member states of the European Union at the time of the study (N=15) were chosen for inclusion, also including Norway and Iceland due to the European Economic Area (EEA) agreement.17 Switzerland and two Baltic countries (Latvia and Lithuania) were also included due to previous contacts and/or a known interest in pedSCI.

The International Spinal Cord Society (ISCoS) membership list was used to identify contact persons in these 19 European countries, that is, Austria, Belgium, Denmark, Finland, France, Germany, Greece, Iceland, Ireland, Italy, Latvia, Lithuania, The Netherlands, Norway, Portugal, Spain, Sweden, Switzerland and The United Kingdom.

The list targeted persons who had a known special interest in pedSCI and/or who had previously published articles on SCI epidemiology. If no such person could be identified, a representative of a major rehabilitation center was contacted.

A semi-structured questionnaire requesting information about established or estimated incidence of pedSCI, management, follow-up and prevention was administrated by mail/e-mail to the contact person (Table 1).

Table 1 Context of the questionnaire
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The contact person was asked to refer the questionnaire to another colleague if such a person was thought to be more knowledgeable with regard to the subject matter of the study.

Several subsequent reminders were made by phone, e-mail and letters in cases of initial nonresponse.

Additional contacts by phone or e-mail were also made if there were any ambiguity concerning the subsequent interpretation of submitted questionnaires.

The questionnaires were sent out in November 2002 and the last questionnaire was analyzed in March 2004, when 18 out of 19 questionnaires had been submitted.

For the countries who could submit the approximate number of pedSCI cases annually and where the corresponding number of children at risk was available, a calculation of estimated incidence was made (Table 2). The midyear population (0–14 years), for each country, was calculated from a publicly accessible database18 (Table 2).

Table 2 Incidence of pedSCI, estimated number of new children with SCI/year, age, midyear population (0–14 years)/million,11 estimated incidence and pedSCI prevention efforts in 18 European countries
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In March 2004, a second survey was performed. The results of the first survey, presented in Tables 2, 3 and 4, were sent to all responders of the first survey (N=18) for comments and subsequent validation. One additional question was also included, regarding cooperation between pediatric and adult SCI rehabilitation facilities. Out of 18 respondents, 17 submitted the second questionnaire.

Table 3 Initial care and rehabilitation in different settings of care for children (0–15 years) with SCI in 18 European countries
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Table 4 Follow-up in different settings of care for children (0–15 years) with SCI in 18 European countries
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Results

In nine countries, the questionnaire was referred from the initial respondent to a colleague. In one country, the questionnaire was referred four times until a qualified respondent was found.

Figure 1 demonstrates the substantial differences in the estimated number of pedSCI/year between the countries. Those with pedSCI who reached the hospital alive were found to vary from four cases in 30 years (0.13 cases/year) to about 60 cases/year.

Figure 1
figure 1

Reported estimated number of pedSCI/year from 18 countries. From Portugal and the UK, children dead prior to hospital admission are included

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Incidence

  • Only Portugal6 and Sweden14 could provide an established incidence of pedSCI, that is 27 children/million children/year and 4.6 children/million children/year, respectively (fatal injuries included).

  • The Public Health Resource Unit in Oxford, UK, provided an incidence estimate for the UK, based on a literature review, data from the national database held by the British Association of Spinal Cord Injury Surgeons (BASCIS) and an analysis of in-patient, outpatient and daycare activity data for pedSCI treated at Stoke Mandeville.19

  • Unpublished data previously presented at the VIII congress of Nordic Medical Society of Paraplegia (NMSOP) in Helsinki, Finland, 2003, by Thomas Glott, Norway provided some additional basis for the Norwegian incidence estimation (Table 2).

  • The above-mentioned data, as well as those national incidence estimates that could be calculated, are presented in Table 2.

  • Submitted data from Greece, Italy and The Netherlands represented only a part of the country with no corresponding clear-cut population-at-risk figure. This precluded calculation of a meaningful incidence estimate for these countries.

Initial care, rehabilitation and follow-up

As can be seen in Tables 3 and 4, management differs between countries, and also within countries, depending on the age of the child and depending on variations in health care organization. Some information deserve special mentioning:

  • The only countries where pedSCI (0–12 years) is treated in a local pediatric hospital without specialized rehabilitation are the UK and Sweden (Table 3).

  • In Scandinavia, with the exception of Sweden, pedSCI is typically treated at a major university hospital, at a neuropediatric specialty ward (Table 3).

  • In Norway, Denmark and Iceland there is a recommendation that SCI specialists should always be consulted and work in cooperation with pediatricians both in the initial treatment and the follow-up of the injured child/teenager.

  • There exists specialized neuropediatric rehabilitation with follow-up programs in Austria, Belgium, France, Germany, Ireland and Italy, Spain and The Netherlands (Table 4).

  • Sometimes children in Italy receive their care and rehabilitation abroad (Table 3).

  • PedSCI in Greece and Portugal is treated at regional pediatric hospitals with no specialized rehabilitation (Table 3), although in Portugal the follow-up is performed by a specialized pediatric hospital (Table 4).

  • PedSCI in Spain is treated in one of three places: Spinal cord injury hospital, General Rehabilitation Departments or Cerebral palsy units. No information was given according to ages in relation to these settings (missing data in Tables 3 and 4).

  • In countries lacking specialized neuropediatric rehabilitation, adolescents (13–15 years) are often admitted into clinics for adults (Table 3).

Prevention

Only Spain reports prevention programs specifically focusing on pedSCI. However, several countries have general programs for pediatric injury prevention and road safety legislation that is, seatbelts, car seats, helmets and speed limits. Prevention programs towards SCI are mainly focusing on adolescents and adults (Table 2).

General comments

  • Most countries lack reliable statistics concerning pedSCI. Many respondents (N=14) expressed a desire to get the opportunity to have more contacts in this field, in order to exchange knowledge in research and the development of care programs.

  • The open comments in the questionnaire revealed some additional relevant information, for example, that in Germany there is a ward dedicated for pedSCI and children with spina bifida, that in France there is a dedicated unit for children with high-level injuries who need ventilators, and the existence in Finland of a fairly recent doctoral thesis on this subject.20

The second survey

Cooperation between pediatric and adult SCI care exists in 13 of the 18 responding countries, mainly at the time when an adolescent is transferred into adult care (having reached the age of ‘adulthood’). In a few countries there is an ongoing cooperation with frequent consultations as soon as a child is injured.

As for the validation, all countries responded that the tables reflected the results of the first survey truthfully and represented the current situation regarding pedSCI in their respective country. Thus, only minor revisions were consequently necessary in the preliminary data that went into Tables 3 and 4.

An incidence estimate was received from the Swiss Paraplegic-Center, which was not previously available in the first survey (Table 2).

Discussion

Incidence

Only four countries have previously attempted to analyze the situation for pedSCI in their country.6, 14, 19, 21

The incidence estimates imply large variations. Countries with a similar socio-economic profile would be expected to have fairly similar incidences, but differences nevertheless are seen. This may of course be due to true incidence variations and also due to scattered care, varying reporting methods, whether fatal accidents are included or not and/or due to differences in operational definitions of the diagnosis. In a multi-lingual region like Europe there are also language barriers that may confound communication.

The chosen method of this study was time-consuming, as the contact person from each country first had to be identified and several reminders subsequently had to be made. The second survey, including data validation, presumably increased the reliability of the survey, but still the incidence estimates must be considered highly preliminary. Nevertheless, this study confirms that pedSCI generally is rare in Europe.

Care

Even though many countries have specialized neuropediatric facilities that can admit pedSCI, the patients are nevertheless often treated in other settings of care (Table 3). During the time of this survey, it was decided that Stoke Mandeville, UK, will open a separate unit for pedSCI (personal communication A Graham 2004-03-30).

Although pedSCI is rare,4, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14 it is not currently included in the list of rare diseases provided by the EURODIS.15 A ‘rare disease’ is defined in this context as a ‘life-threatening or chronically debilitating disease, which is of such low prevalence that special combined efforts are needed to address it. As a guide, low prevalence is taken as prevalence of less than 5 per 10 000 in the Community’.22

It is likely that pedSCI, actually fulfilling this definition, would benefit from special combined efforts directed towards the group. Empirical knowledge, research and preventative efforts need to be coordinated between the European countries.

Exchange of information, research and development of care programs are time-consuming and costly. The low incidence and scattered care would merit extra EEC funding.

Further questions that arise are: How do children afflicted by SCI and their families share best practice and knowledge gained from the adult care when they are treated in scattered care or in a pediatric setting? Or, if a child is treated in an adult setting, how does the child receive developmentally based care, rehabilitation and follow-up?

To be able to integrate all knowledge and experiences that are already available in the field of pedSCI in Europe, the establishment of a European/international network would likely be of benefit to all professionals and, most importantly, to the children with SCI and their families.

This survey hopefully provides a preliminary foundation to be used as an argument for gaining economic support from the EEC and for the ongoing work with a European network of professionals in order to improve the care, rehabilitation, follow-up, prevention and research for the child with a spinal cord.

Conclusions

The survey confirms that pedSCI is rare in Europe and that further dissemination and coordination of knowledge in this field is needed throughout the region. The contacts initiated in the process of the survey may be used to create an international network aiming to become a competent reference body for health professionals, researchers and families, thereby hopefully solving some of the problems arbitrary and/or scattered care identified in this study.