Cystic fibrosis (CF) is a serious inherited condition. One in 2,500 babies born in the UK have CF, with over 10,000 people living with CF in the UK today. CF is caused by variants of the CF transmembrane conductance regulator (CFTR) gene, resulting in the build-up of thick, sticky mucus in various organs, especially those of the respiratory and digestive systems. CF is therefore characterized by pulmonary infections and progressively declining lung function leading to most people with CF dying prematurely from their disease through respiratory failure. However, over the past decades, due to improvements in treatments and care, people with CF are living longer, healthier lives [1]. Half of the babies born with CF in the UK today are expected to live at least into their late forties [1, 2]. Employment prospects and financial stability are therefore becoming increasingly important issues. In 2019 13.5% of people with CF over the age of 16 were unemployed in the UK [2] compared to approximately 4% in the general population [3]. Evidence suggests that the likelihood of having full-time employment is more strongly linked to educational attainment than to disease severity [4]. Ensuring that children with CF achieve their academic potential is thus of utmost importance. However, few studies have investigated educational outcomes of children with CF, with most conducted in the US more than a decade ago. These studies suggest that children with CF show academic achievement levels comparable to the general population [5, 6].

To our knowledge, no study has looked at educational outcomes in the UK CF population. We utilised our recent population-scale data linkage in Wales [7, 8] to assess educational outcomes and the provision of extra learning support in the Welsh CF population, using comparable national data to contrast outcomes for children with CF to the general population of Welsh children. Due to data availability, we focus on educational achievements at ages 10-11 years, which have been shown to be strongly correlated with educational attainment at the end of high school [9, 10].

We identified 312 children with a CF code in the linked data sources. 150 of these children were born before 2006 and could therefore have KS2 results by 2016; out of these 15 had moved out of Wales before age 11. 119 children had KS2 outcomes recorded. Table 1 shows the demographics of this population and hospital attendances as a marker for disease severity and potential school absence.

77% (95% CI: 69%–84%) of children achieved the expected levels in English, 81% (95% CI: 73%–87%) in Mathematics and 82% (95% CI: 75%–88%) in Science. There were no statistically significant differences in attainment in the full sample compared to the subpopulation of children that had no CF-related hospital admissions in the year of KS2 assessment (Table 1, Figure 1). The general Welsh population of children who sat KS2 assessment between 2011 and 2016 achieved higher average success in all three areas: 83.4% to 91.1% achieved the expected level in English in each of the years with a crude average across years of 87.3%; 84.9% to 91.6% achieved the expected level in Maths in each of the years with a crude average across years of 88.2%; and 87.1% to 92.2% achieved the expected level in Science in each of the years with a crude average across years of 89.8% [18] (Table 1, Figure 1).

Almost 70% of the children with CF were recorded as receiving extra learning support (Table 1).

We assessed the educational outcomes at age 10-11 of children with CF in Wales. Our results suggest that children with CF may have lower educational achievements than the general Welsh population of children. Almost 70% of the children with CF received additional learning support which compares to 22.5% of all Welsh pupils in 2014/2015 [16].

Our analysis provides proof of principle demonstrating the feasibility of linkage of data on children with CF to educational data on educational progression and attainment, but there are a number of limitations. First, relying on ICD-10 codes can result in misclassification of patients with CF [19]. In a previous study on the same population we used both ICD-10 and Read codes to identify patients with CF across a range of linked data sources and found that 27% of individuals were wrongly classified as having CF when identification was based only on routinely-collected data [8]. Misclassification is therefore likely to be present in this study, and consequently the results have to be interpreted with caution. This is challenging to address because educational attainment data are incomplete in the UK CF Registry, the national disease registry which captures data on 99% of people with CF [20]. Linkage of the Registry to non-health data sources is currently not possible since this is not covered within the individual consent for linkage in the UK CF registry. Second, we did not have access to individual-level data on health status, such as lung function measures, or co-morbidities such as CF-related diabetes. We anticipate that we will be able to overcome these limitations in the context of a UK wide linkage of CF Registry data to the type of routine data sources available in SAIL. In the absence of this, the routine data linkage implemented in our study may offer the only possibility to gain insights into the impact of CF on educational attainment in a whole population of children. Third, we did not have individual-level data linked to educational outcomes for the non-CF population. Therefore, we were not able to adjust for potential confounders. However, our previous study on Welsh children showed that the distribution of many demographic variables, such as socio-economic conditions and sex, were comparable between children with and without CF [7]. Fourth, due to the small sample size, we were not able to explore whether there may be a differential impact of CF on educational outcomes by socioeconomic status or sex.

Our study suggests that children with CF may have worse educational attainment, and the reasons for this need further investigation. Children with CF are a heterogeneous group, and more detailed data on health status and co-morbidity is needed to assess health related pathways affecting educational attainment. Long-term school absence may be one of the main mechanisms through which chronic physical health conditions impact educational achievements [17]. Only 19% of children in this study spent any time in hospital during the year of assessment, and we found similar results when we excluded these children from the analysis. However, we did not capture absences in the years prior to the assessment, or absences from school due to factors other than hospitalization. A recent study highlighted the potential impact CF treatment regimens have on children’s home learning and their ability to participate in school activities such as PE and school trips [21]. The same study demonstrated that complex treatment regimens additionally restrict the time children have to develop and maintain friendships. This social disconnectedness has also been suggested as a mechanism through which physical illness can impact educational attainment [17]. With the advent of disease modifying treatments (CFTR modulators) for much of the CF population [22], it can be anticipated that children will spend less time in hospital and have less complex treatment regimens which may positively impact their education. This will increase the importance of contemporaneous research investigating the factors that influence the relationship between CF and educational achievement and the potential barriers that children with CF face.

Previous work has shown that people with CF are more likely to be unemployed if they are from more disadvantaged backgrounds and that disease severity has a larger impact on employment chances in these individuals compared to their less disadvantaged peers [23]. In future work, there is a need to explore the extent to which this can be explained by a differential impact of CF on children’s educational attainment. Understanding social consequences of ill health is a key step in elucidating the pathways to health inequalities, since any adverse social outcomes that occur as a result of ill health can create a feedback loop and further damage health status.

In conclusion, we demonstrate that having CF may impact children’s educational achievement. More research and better linked data in this area is needed to be able to develop policies and interventions to better support children to reach their full potential.