Direct healthcare costs in the first 2 years of life: A comparison of screened and clinically diagnosed children with cystic fibrosis - The Irish comparative outcomes study of CF (ICOS).

BACKGROUND: In July 2011, Cystic Fibrosis (CF) was added to the Newborn Bloodspot Screening Programme in Ireland. The Irish Comparative Outcomes Study (ICOS) is a historical cohort study established to compare outcomes between clinically-detected and screen-detected children with CF. Here we present the results of economic analysis comparing direct healthcare costs in the first 2 years of life of children born between mid-2008 and mid-2016, in the pre-CF transmembrane conductance regulator modulator era. METHODS: Healthcare resource use information was obtained from Cystic Fibrosis Registry of Ireland (CFRI), medical records and parental questionnaire. Hospital admissions, emergency department visits, outpatient appointments, antibiotics and maintenance medications were included. Costs were estimated using the Health Service Executive Casemix, Irish Medicines Formulary and hospital pharmacy data, adjusted for inflation using Consumer Price Index data from the Central Statistics Office. A Negative Binomial regression was used, with time in the study as an offset. RESULTS: Overall participation was 93 %. After exclusion of those with meconium ileus, data from 139 patients, with follow-up to 2 years of age, were available. 72 (51.8 %) were from the clinically diagnosed cohort. In the final model (n=105), clinically diagnosed children had 2.62-fold higher costs per annum (p<0.0001), when adjusted for confounders, including homozygous ΔF508 or G511D mutation, socio-demographic factors and time between diagnosis and first CFRI interaction. CONCLUSIONS: There are few studies evaluating economic aspects of newborn screening for CF using routine care data. These results imply that the benefits of newborn screening extend to direct healthcare costs borne by the State.

Parental awareness of newborn bloodspot screening in Ireland.

BACKGROUND: There is little known regarding how familiar parents are with the newborn bloodspot screening (NBS) test or how well parents of a child with a screen-detected condition understand that condition initially. AIM: The study aim was to examine parental NBS awareness and conditions screened. METHODS: Two studies were conducted: [1] Parents of children with cystic fibrosis (CF) detected via NBS and subsequently, diagnosed (n = 124) completed a telephone questionnaire regarding information they received at the time of NBS. [2] A cross-sectional study of women (n = 662 (58%) antenatal; n = 480 (42%) postnatal) attending three large maternity hospitals completed a questionnaire addressing NBS awareness. RESULTS: Mothers incorrectly identified diabetes/asthma (35% postnatal; 70% antenatal) and sickle cell disease (26%) as conditions on NBS in Ireland. Phenylketonuria was correctly identified by 48/26%, CF by 82/64%, and congenital hypothyroidism by 35/13% postnatal and antenatal women respectively. Of parents of children screen-detected and subsequently, diagnosed with CF, only half (n = 63; 51%) reported awareness at the time of NBS that CF was included. These results should be used to improve the information provided to expectant mothers and to inform health professionals' initial discussions with parents about their child's diagnosis, building on parents' pre-existing knowledge.

Carotenemia and hepatomegaly in an atopic child on an exclusion diet for a food allergy.

Eczema is a frequent childhood manifestation and a few atopic children are allergic to certain foods or aeroallergens. Anxious parents of atopic children often have a fear of topical steroid-related side-effects, and some may try a range of elimination diets to avoid allergies. Elimination diets increase the risk of anaphylaxis on re-exposure to previously tolerated foods from the loss of oral tolerance. Unbalanced diets together with an inadvertent excessive consumption of fruits and vegetables may lead to carotenemia from the carotenoids in the plant foods. Carotenemia is benign but unusual diets and the consumption of preformed vitamin A in health supplements can lead to vitamin A toxicity. We discuss a child with eczema on an exclusion diet presenting with anaphylaxis to dairy food. He had carotenemia with hepatomegaly, which resolved on dietary management.

Longitudinal follow-up of the relationship between dietary intake and growth and development in the Lifeways cross-generation cohort study 2001-2013.

In this paper we will review evidence on the early life and familial influences on childhood growth and development, with particular reference to the Lifeways cross-generation cohort study in the Republic of Ireland. The Lifeways cross-generation cohort study was established in 2001-2013 through two maternity hospitals in the Republic of Ireland and was one of many new cohort studies established worldwide in the millennium period. Mothers were recruited at first booking visit, completing a self-administered questionnaire, which included a 147 item semi-quantitative FFQ. Longitudinal follow-up is ongoing in 2013, with linkage data to hospital and general practice records and examination of children when aged 5 and 9 years. The study is one of very few containing data on grandparents of both lineages with at least one grandparent recruited at baseline. There have been consistent associations between parental and grandparental health status characteristics and children's outcomes, including infant birth-weight, BMI when child was aged 5 years and childhood wheeze or asthma when child was aged 3 and aged 5 years. In conclusion, empirical evidence to date shows consistent familial and cross-generational patterns, particularly in the maternal line.

Infants hospitalised with pertussis: estimating the true disease burden.

AIM: New Zealand (NZ) has a large pertussis disease burden compared with other developed countries. Accurate ascertainment of disease burden is fundamental to controlling pertussis and informing immunisation policy. Disease burden estimates are primarily from passive surveillance, which underestimates disease incidence. The aim of this study is to use active surveillance to determine pertussis disease burden in infants hospitalised in NZ. METHODS: Using the NZ Paediatric Surveillance Unit, active surveillance from 08/2004 to 07/2005 for infants <12 months old, hospitalised with pertussis. RESULTS: 110 infants identified (196 per 100,000), including six with complications, eight intensive care admissions and one death. The hospitalisation rate (per 100,000) varied with ethnicity, being higher for Maori (296) and Pacific (358) compared with European/other (117). Twenty-four per cent were too young to be immunised. Of infants 6 weeks and older 46% had received no immunisations. Despite being more likely to be immunised Pacific infants had a higher hospitalisation rate owing to a larger proportion acquiring pertussis prior to age 6 weeks. Cyanosis and apnoea were frequent symptoms in young infants. Under-identification, estimated using capture-recapture analysis, was modest for both active surveillance (16%) and passive notification (19%). CONCLUSIONS: Infant pertussis hospitalisation rates are three to six times greater than rates in the USA, England and Australia. Underestimation of disease burden by passive notification in hospitalised infants is modest, suggesting a high degree of clinical awareness by paediatricians in NZ. New immunisation strategies are needed to protect infants from a younger age.

Hospitalisations due to pertussis in New Zealand in the pre-immunisation and mass immunisation eras.

AIM: Pertussis disease burden in New Zealand in recent decades has been large compared with other developed countries. However, these comparisons use data from relatively short time periods given the long epidemic cycle of pertussis. To better understand the current disease burden, this study examined pertussis hospitalisation data in New Zealand in both the pre-immunisation and mass immunisation eras. METHODS: Hospital discharge data and population data from 1873 to 2004 were used to estimate average pertussis hospital discharge rates per decade. Rates were compared using relative risks and 95% confidence intervals (CI). RESULTS: Average annual pertussis hospitalisation rates per 100000 were less than two from 1873 to 1919, increased to 12 in the 1940s, decreased to less than four in the 1960s and have increased since then with the rate in the current decade being 5.8. Compared with the 1960s (3.8 per 100000) the average annual rate has been significantly greater in the 1980s (RR=1.11, 95% CI 1.03, 1.21), 1990 s (RR=1.33, 95% CI 1.23, 1.44) and 2000s (RR=1.55, 95% CI 1.42, 1.68). Since 1960 hospitalisation rates have increased for those less than one year old, one to four years old and five years and older. The increases have been most marked for infants (RR 2000s vs. 1960s=2.87, 95% CI 2.59, 3.18). CONCLUSION: After an initial decline following mass immunisation, pertussis hospitalisation rates in New Zealand have subsequently increased steadily. To reduce pertussis disease burden improved immunisation coverage and timeliness is required and consideration given to spreading the pertussis vaccine schedule over a wider age range.