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Conditions that go beyond skin-deep for Irish children

Cessation of paediatric dermatology services in Temple St Children’s Hospital, Dublin, and Waterford Regional Hospital for two years between 2014 and 2016 created a significant increase in workload for physicians treating children at Our Lady’s Children’s Hospital, Crumlin (OLCHC). This resulted in already-underfunded service provision being further stretched and longer waiting lists for children, for whom a delay in treatment results in a worse clinical outcome.

However, despite these obstacles, specialists are providing the highest standards of care for patients and research is set to be conducted that will provide new insights for physicians in their efforts to better understand paediatric dermatology.

Dr Grainne O’Regan, Consultant Paediatric Dermatologist at OLCHC, told the Medical Independent (MI) about the efforts of specialists at the hospital and around Ireland to provide the best possible care for a very vulnerable patient group.

“There has certainly been a marked increase in the caseload in Crumlin,” she explained. “Currently, we have the equivalent of 2.2 whole-time paediatric dermatologists in the hospital, overseeing up to 10,000 patients annually. Not only has our caseload dramatically increased, but also the complexity of disease.

“A manpower crisis in Temple Street Hospital resulted in cessation of services [in dermatology]. University Hospital Waterford, similarly, closed their services — they had no other option. They reopened within the last year but this created a pretty serious situation for all clinicians, because they had fewer options in terms of where to refer patients.

“They referred many of these to us and other regional centres that provide services in Cork, Galway, Drogheda, Sligo, Mullingar and Limerick. We work with hugely supportive colleagues outside of Dublin.

“There is a very high level of complexity. More than 2,400 skin conditions can present to us — atopic dermatitis is by far the biggest caseload, but then we also manage, for example, complex vascular anomalies, rare genodermatoses, including fragile skin such as epidermolysis bullosa and connective tissue diseases, so these are not just cutaneous conditions we look after, from birth all the way up to adolescence.”

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Dr Grainne O’Regan: ‘The increase in disease prevalence and severity has a hugh impact on families'

Capacities

This range also includes severe inflammatory diseases, blistering disorders, autoimmune conditions and skin cancers, she added, and current waiting lists can be anything between 12 and 18 months for routine patients. Dr O’Regan and her colleagues have put forward a case to increase capacity in treatment sites. She also stressed that her colleagues who practice in regional sites “work incredibly hard” caring for these complex patients.

Dr O’Regan explained that she and her colleagues in the hospital review more than 2,000 children annually with severe atopic dermatitis and there is substantial evidence generated by OLCHC and others that eczema is not simply an “innocent bystander”, but is a gateway to developing asthma, allergic rhinitis, food allergies and potentially life-threatening anaphylaxis.

“Some 20-to-30 per cent of children develop atopic dermatitis or eczema and type 1 food allergies now occur in up to 6 per cent of Irish pre-school children. Historically, education has been poor in terms of eczema management and that, coupled with steroid-phobia, results in people often opting for alternative routes,” she said.

“People often think that food allergies cause eczema, when of course it’s the other way around. They often take homeopathic or alternative therapies before they opt for medical therapy and so we are seeing more severe symptoms presenting to us at a later stage.”

‘There is certainly not sufficient funding in this area. If we look at the Dublin hospitals, there are 3.4 whole-time equivalent consultants providing care for between 12,000 and 15,000 patients’

Food avoidance

Dr O’Regan agrees that there is a danger posed by parents’ reliance on homeopathy and other alternative treatments that lack an evidence base.

She explains to patients in basic terms that food allergies result from a failed skin barrier, as occurs in eczema. If the first exposure to a food is through the inflamed skin and not through the gut, the next time the body ingests that protein, it may identify the food as a ‘foreign’ protein and attempt to expel it, resulting in an allergic reaction. She hopes this simplified explanation helps patients to understand the true cause of food allergies. “Therefore,” she continued, “I explain that if you don’t ‘switch-off’ the eczema early and opt for dietary exclusion, patients are potentially at increased risk of developing food allergies and other problems, including nutritional deficits and growth retardation. It’s a big problem.  The IFAN (Irish Food Association Network) website”, she explained, “is an excellent resource for both clinicians and parents who are interested in practical, evidence-based information regarding allergies. ”

Often, increases in diagnoses of certain conditions are attributable to better diagnostic abilities on the part of physicians. However, with atopic dermatitis, there has been a true increase in incidence, said Dr O’Regan.

“It has certainly increased in incidence, placing a significant burden on healthcare resources across the board. Incidence has increased three-fold during the past decades in industrialised countries,” she explained. Apart from being more prevalent, it has also become more severe.

“Atopic dermatitis now affects 20 per cent of children, with one-in-three children having severe disease. If not switched off early, disease persists in up to 30 per cent of adults,” she continued. “It represents a classical complex disease trait, in that it is driven and modified by genetics, our environment, the microbiome and immunological response. The physical environment has changed; we use radiant heaters and bathe our babies more often — when we were younger, our parents would tell us to put an extra gansaí on us and had a bath once a week, if we were lucky. Overall, the environment has significantly changed for the last generation.

“The increase in disease prevalence and severity has a huge impact on families,” Dr O’Regan pointed out. “The impact on quality of life for families is comparable to that of diabetes, due to itch and sleep disturbance; children can be up all night scratching and it’s highly disruptive for families.”

Training

The most important aspect is to ‘switch-off’ the disease early and GPs and the colleges have a huge role to play in this regard, said Dr O’Regan. “In terms of primary care, we would say that more than 20 per cent of GP consultations would have a dermatological component and yet historically, less than one-in-six GPs has any formal training in dermatology, so there is a significant knowledge gap and a lot of misinformation for GPs and pharmacies.”

However, Dr O’Regan says both the dermatology community and colleges have their part to play by incorporating more formal dermatology training for prospective GPs. “It’s historical; it starts in medical school. We would love to have more undergraduate and GP trainees coming into our system and we actively encourage students to join us, but we also understand that there is pressure on trainees to incorporate a very broad curriculum. In an attempt to address that, we are currently developing a postgraduate dermatology diploma with a targeted paediatric dermatology module through the RCPI, aimed primarily at GPs and paediatricians.”

With increasing pressure on hospital systems across the spectrum of healthcare, Dr O’Regan also highlighted the potential knock-on benefits, in terms of both capacity and finances, to address these issues at primary care level. For example, if atopic dermatitis is switched off early, it can prevent the development of other atopic diseases, with huge ramifications not only for adult dermatology services, but also for respiratory, allergy, and emergency services across the board, in paediatrics but also for adult services. “It’s a no-brainer,” she added.

On the topic of funding, Dr O’Regan said: “There is certainly not sufficient funding in this area. If we look at the three paediatric Dublin hospitals, there are 3.4 whole-time equivalent consultants providing care for between 12,000 and 15,000 patients annually. In any other model, in the US for example, we would probably have eight times that number of consultants for that level of complexity and volume.”

She also touched on the improvements in therapies available to these patients. “We are about to see an explosion in terms of therapeutic developments,” she told MI. “In 2006, research led by Prof Alan Irvine in OLCHC led to the identification of a skin barrier protein called filaggrin, which I explain to parents acts like cellophane on the skin’s surface.

“One-in-10 people in the world is missing that barrier protein and it accounts for 50 per cent of moderate-to-severe atopic dermatitis, so in terms of gene identification, that’s huge. It basically explains at least 50 per cent of what’s going on. Before that, the general consensus was that the immune system was dysregulated and instead, our research points primarily to a barrier defect in the skin. That has really been a bit of a volte-face in terms of how we look at the aetiopathogenesis of atopic dermatitis. It’s only since 2006 that people could think about developing more targeted therapies.”

Dr O’Regan concluded: “This also shows that despite constrained resources and infrastructure, we are able to generate world-class research in Ireland. At the moment, our remit is topical steroids and immunosuppression, with therapies like methotrexate and other systemic immunomodulators, but therapies are set to become much more targeted.

“We hope to start trials with these in paediatrics next year.”

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