A new leaflet published on behalf of the Diamond-Blackfan Anaemia (DBA) charity is an opportunity to explore the role of dental teams in the management of children with rare medical conditions, writes Caroline Holland.

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The chances of being born with a genetic condition are high. There are many thousands of syndromes with one in every 25 children1 born in the UK affected by a genetic condition. Individual genetic conditions tend to be rare with Diamond Blackfan Anaemia (DBA) being one of the rarest. DBA is a little-known genetic disorder that chiefly affects the bone marrow, disrupting the production of new red blood cells. Around five children in every million babies are born with DBA.2 The rarity of the condition makes it all the more remarkable that there is a dental leaflet3 written for, and with, DBA UK, the charitable association supporting those affected. With text by paediatric dentists and designed by the association, the online publication of the leaflet is rightly celebrated by all involved.

Chair of DBA UK, Leisa Batkin, is delighted with the new resource: 'We regularly get questions from parents about teeth, it's a common worry. Now we have this leaflet, we can direct parents to our website. It's recognition that this is a concern for us all'. Marielle Kabban is the Consultant in Paediatric Dentistry who is most familiar with the oral health manifestations of DBA, having treated many young patients. For a time, she worked at St Mary's Paddington where the UK's lead DBA clinician, consultant paediatric haematologist Professor Josu de la Fuente, is based.

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Leisa Batkin

In common with some other children with medical problems, the most likely oral health issue associated with DBA is enamel hypomineralisation. Ms Kabban says that keeping teeth healthy is critical. The new leaflet provides advice and information on maintaining good oral health. She acknowledges the hard work of her paediatric specialist trainee, Rakhee Budhdeo, who compiled the leaflet, and the important contribution of the DBA UK charity. 'They facilitated meetings with members to ensure the content of the leaflet meets the needs of DBA families. The association funded and organised the design and it looks wonderful,' she says.

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Marielle Kabban with her specialist trainee Rakhee Budhdeo

When a child is born with DBA, it may take time for the family to get a diagnosis. It's a syndrome which can manifest in different ways in different people. Some will have semi-functioning bone marrow and will suffer with long-term anaemia before they are diagnosed. Others will be diagnosed in the first few months of life as their bone marrow fails to produce any red blood cells.

Thereafter, regular blood transfusions may become the norm. For a longer-term solution, the first line of treatment is usually steroids. In the event that steroids don't work, a bone marrow transplant (BMT) may be offered. This involves a course of chemotherapy which destroys the faulty blood cells in the bone marrow, enabling the transplant of healthy bone marrow from a donor with a stem cell match. At this point, mucositis, a likely side-effect of chemotherapy, manifesting as soreness of the mucosa, becomes the key oral health concern (see Box 1).

Ms Kabban works closely with DBA families. Based at St Thomas' Hospital, part of Guy's and St Thomas' Foundation Trust, she sees patients on referral from Professor de la Fuente and his team to help ensure the teeth and gums are in optimum health ahead of their BMT. 'The better a child's oral hygiene is in the run-up to the BMT, the better they will cope with mucositis.'

Professor de la Fuente, who is the lead clinician for several hundred patients, says: 'Anything that can be done to minimise the discomfort associated with having chemotherapy is to be welcomed. Having good oral health will help with recovery time, minimise infections and contributes to both wellbeing and establishing good nutrition. We are delighted to be able to collaborate so constructively with Ms Kabban and her team in the interests of patients'. While there is evidence-based guidance for UK dental teams on managing children with medical conditions, such as Paediatric dentistry for the GDP, a BDJ Clinician's Guide,5 published in 2021, management of children with co-morbidities can be challenging in general dental practice.

In common with some other children with medical problems, the most likely oral health issue associated with DBA is enamel hypomineralisation.

Urshla (Oosh) Devalia, a co-author of a chapter6 in the BDJ Clinician's Guide, says a shared care approach is best for patients with DBA: 'It is the kind of condition which should initially be managed by a Specialist or Consultant in Paediatric Dentistry in secondary or tertiary care. If a child has a BMT, once mucositis is resolved and they are dentally fit, they can be seen by their GDP. However, the dental team needs to be aware of the potential for dental anomalies, with enamel hypoplasia being the most likely side-effect.

'Medical teams should work closely with oral health professionals to make sure that the needs of patients with DBA are met throughout their lifetime.'

The oral care of adult DBA patients is also a concern for the DBA UK charity. Access to an NHS dentist in some areas of the UK is famously challenging.

Albert Yeung, a Consultant in Dental Public Health and the new President of the British Association for the Study of Community Dentistry (BASCD), the organisation responsible for developing policies to improve dental public health, comments: 'Access to NHS dental services is challenging, with vulnerable groups finding it most difficult to get the care they need. We advocate that everyone should have access to dental care and those with additional needs with extra barriers to overcome should have equity of access to this care. Such groups need to have treatment from dental teams who are sensitive to their needs'.

Dr Yeung and BASCD Past President Maria Morgan are based, respectively, in Scotland and Wales; both have country wide public dental services. Ms Morgan says: 'In Wales we have a community dental service which is responsible for delivering oral health promotion initiatives like Designed to Smile and care for vulnerable and special care groups. It is this kind of arrangement which we support as it addresses inequalities. Ideally, there should be similar models of care throughout the UK, underpinned by a Commissioning Standard.

Medical teams should work closely with oral health professionals to make sure that the needs of patients with DBA are met throughout their lifetime.

'We are delighted to see that a charity like DBA UK is working to support its members with oral health information. This represents progress at a time when news about dentistry has not been so positive.'

Charlotte Waite, Chair of the British Dental Association England Community Dental Services Committee, is also enthused, commenting: 'In England integrated care services are emerging and this joined-up approach to the delivery of care, can and should be, of benefit to many patients with complex conditions such as DBA, by supporting closer working between medical and dental providers. The collaboration between Ms Kabban and Prof de la Fuente is an excellent example of holistic patient centred care'.