To our knowledge this study describes the first awareness campaign for HHT based on the inclusion of dentists. Every year more than 70% of the German population receive a professional tooth cleaning and examination by their dentists as it is supported by the German public health insurances [19, 20]. Therefore, our approach had the potential for a nationwide population-based campaign. In HHT over 80% suffer from visible symptoms, like muco-/cutaneous telangiectasia, [3, 21–23] which could be detected by their dentists. Additionally, most patients and affected relatives suffer from recurrent nosebleeds, which can easily be assessed by taking a history directed specifically at these points. The presence of telangiectases, nosebleeds and/or a positive family history are sufficient to raise suspicion for HHT in the setting of a dental practice resulting in the appropriate referral for further evaluation. Early diagnosis and treatment achieved – like in this case through dental screenings - can lead to improved quality of life for HHT patients [14, 24].
The results of the awareness campaign demonstrated a positive effect: our nationwide dental awareness campaign was associated with a higher frequency of persons contacting the self-help group for the first time. However, the 10% increase in contacts for an equivalent time interval seems to be relatively small (187 first contacts for the period from September 2016 to May 2018 in comparison to 222 contacts for the period from July 2018 to September 2019). Various explanations exist for the limited impact of the campaign. Our approach targeted dentists. In Germany, dentists normally do not write referrals which may have contributed to the limited effect. Only 9% of patients indicated that they were informed by their dentists or other physicians on the possibility of HHT. Before the campaign, no dentist referred patients to the German self-help group; afterwards, at least 5 patients received information about HHT by their dentists and mentioned this when they contacted the patient organization for the first time. It is probable that this effect was related to the awareness campaign, which we interpreted as a limited success. However, it is interesting that 44% of patients stated that they were informed mainly by their general practitioners and only 14% by dentists. A higher rate of referrals by dentists was expected. The authors were not able to identify the reason for this but one possibility is that dentists first referred to the general practitioner who then recommended that patients contact the self-help group.
Diagnosing rare diseases is challenging, as they are numerous and diverse in presentation. Therefore it is appropriate, that general practitioners and dentists have only limited knowledge of such diseases . About 5000 rare diseases are known of which 700 involve dental, oral or craniofacial pathologies . Given that an oral mucosal examination is a relatively simple and inexpensive screening tool, the World Dental Federation states that a systematic examination of the oral mucosa should be part of every dental examination procedure . HHT is an autosomal inherited disease inflicting female and male patients of various ages . In Germany, dentists are the only primary health-care professionals treating patients of all ages and sexes. They are providers of primary oral healthcare and therefore essential gatekeepers for the management of patients who may have oral manifestations of systemic diseases, As such, they are often likely to be the first clinicians to observe such abnormalities [24, 29]. However, another study analysed the identifying rate of early oral cancer in the general dental practice and came to the conclusion that this is of limited effectiveness. Diagnostic screening systems also depend on qualitative factors – on primary health-care professionals using their discretion and clinical judgement for the screening process to work effectively. Although thorough screening might be challenging from a practical and economical point of view, clinical judgement should be encouraged in order to detect unexpected or rare diseases, like HHT . As mentioned above, we identified specific limitations to our approach with dentists. An consideration is that dentists are flooded with information and, even though the article was published in an important set book, this was only read by a limited number of dentists. We plan to increase awareness during future studies by asking the patients of self-help groups to print out the articles of the awareness campaigns and to present them to their dentists during their next visits.
Awareness campaigns for rare diseases like HHT are necessary in order to reduce the diagnostic latency and improve the medical care of these patients [31, 32]. The German patient self-help group and different HHT centres and hospitals have been working toward heightened awareness of HHT in the general population and among healthcare professionals over the past years. However, the effect was limited and further measures seemed necessary . In general, many problems in treating patients with rare diseases result from the lack of awareness or experience among healthcare professionals, low motivation, and barriers to changing practice or policies. One approach to increase awareness of and familiarity with rare diseases in healthcare is to target the postgraduate education of gatekeeper care providers, like dentists or primary care physicians. The goal of this education would be to help these providers differentiate rare diseases amidst the backdrop of the general population. Such education might be especially effective if it focuses on recognizable, core warning signs of rare diseases instead of on in-depth information. In this way, physicans might be more easily able to detect divergent patterns of diseases through their “gut feeling” pattern recognition . Health promotion activities that focus on large populations such as smoking cessation campaigns may affect the health of many individuals which encourages engagement by primary care physicians and government agencies. However, mainly due to the lack of exposure or awareness of primary care providers or politicians the incentive to change practice or policy may be limited for rare diseases . The “Shit Happens” campaign is an example of a parents-oriented research project for patients with the rare condition Hirschsprung’s disease. The campaign demonstrated that social media can be a powerful and responsive tool to connect families with rare diseases .
To tailor the HHT awareness campaign to dentists as the target audience, we studied approaches taken by earlier campaigns targeting dentists. Two awareness campaigns, one in Brazil and the other in Florida, were implemented in order to improve the prevalence of hepatitis B vaccination among dentists and to inform dentists about diagnosing and treating patients with temporomandibular disorders. The campaigns were mainly based on online tools and resulted in low response rates amongst dentists [37, 38]. Therefore, we developed a different approach by publishing repeated articles in a set book for all dentists in Germany. In our awareness campaign a case report about HHT together with instructions about disease management of HHT were published online and in print in the journal of the German dental association and the Federal Association of Fund Dentists of Germany which every dentist receives in Germany. This journal is a set book for all dentists in Germany with a total circulation of 77 621 copies in 2017. After publication of the article the number of first contacts to the German self-help group via phone calls and emails increased, especially when comparing the time periods two months before and after the first publication. Only these two means of communication were suggested in the publication, and subsequently, the increase in these routes were higher than in other channels like Facebook® or internet inquiries, suggesting that the rise in new contacts might be associated with our campaign. Interestingly, almost half of the patients answering to this question did an internet research about HHT and the German self-help group to access information on the disease. This underscores the fact that the internet has become a very popular source of health-related information for patients . Likewise, many healthcare professionals use web-based tools for obtaining health-related information; however, defective synthesis of the available information can lead to diagnostic error .
Data was provided by the German HHT self-help group. Due to technical problems the documentation of the different communication channels for the whole period is only complete for first-time contacts by phone. It should be noted that another limitation of the study was that neither patients’ details (i.e. sex, age, clinical symptoms) nor a follow up of completed screening or necessary therapies could be documented due to data protection regulations. However, the cooperation of the self-help group for HHT and medical professionals during this study demonstrates the important role that patient involvement plays in the healthcare system [41, 42].
The article on HHT was published in June 2018. On the beginning of July 2018, the new website of the German HHT self-help group was launched. Therefore, the effect of the new homepage might have been a confounding factor. However, a new website several years ago did not increase number of phone contacts at that time.
The development of a table showing oral manifestations and basic information, not just for HHT but also for other rare diseases, will be a task for our future work in order to facilitate the diagnostic process and to inform dentists about treatment options of different rare diseases.