National Behçet's Centres of Excellence (London, Birmingham, and Liverpool) provide an exemplary model for other nations in providing a multidisciplinary approach to treatment, all compressed in one compact location, providing ease and reassurance to patients and families.

Behçet’s disease is a rare multisystem vasculitic disorder that can affect any of the blood vessels of the body; signs and symptoms are diverse among individuals and range in severity, but often present as recurrent oral aphthous ulcers, genital ulcers, or as complications involving the eyes. Furthermore, there is the possibility of any organ being affected, and since understanding of its etiology is sparse, treatment remains a significant challenge. This perspective aims to act as a bridge between the current, traditional medical management of Behçet’s by professionals and the holistic needs of individuals who live with the condition daily.

Behçet’s Case Example: “Daniella”

Daniella (pseudonym) is a bright, outgoing individual living with Behçet’s. She recalls a long history of peculiar symptoms throughout childhood like ulcerations down her throat and joint pain in the hips and knees, treating symptoms as they came up. After many trials, and misdirection to the sexual health clinic, at the age of twenty-three, Daniella was finally diagnosed with Behçet’s disease. Notably, she emphasizes the impact of a young student doctor along her journey, who suggested the condition as an explanation, and subsequently sent in a referral to a rheumatologist, setting her on the right track for diagnosis and then treatment.

By this point in time, she was experiencing skin ulcerations, genital ulcers, and severe arthralgia. She cites the grief she has experienced in losing her independence, relying on others for her daily needs, as a result of having seizures. In addition to meaningful specialized treatment, Daniella uses her creative pursuits as a form of therapy, inspiring others living with chronic health issues through the use of her podcast.

Behçet’s Case Example: Rachael

Rachael is an active community member who balances family life and career obligations as a part-time pediatric diabetes specialist nurse, alongside living with Behçet’s. Her characteristic onset of acute and aggressive symptoms led to her probable diagnosis and referral to an immunologist at age twenty-four: multiple mouth ulcers, chronic fatigue, skin lesions, and subsequent genital ulcers. Rapidly, within months, her condition became more critical involving arthritis, erythema nodosum, bilateral uveitis, and intestinal issues. She also disclosed a childhood medical history of fainting from bowel cramping, classified as irritable bowel syndrome, and being hospitalized on one occasion.

Rachael describes navigating the various treatment options and medical appointments as a slow, painful, and lonely journey. Nonetheless, what she finds the most challenging about having the condition is the unpredictability of it all. To maintain her psychological and physical well being on ongoing corticosteroid treatment, she is an avid runner. She further makes an impact in a leadership role, allowing her to influence research, connect with patients and families, educate medical students, and promote better opportunities for younger patients.

“Unfortunately, with no current cure for Behçet’s, the journey does not end at diagnosis.”

Coexisting Psychosocial Challenges

As evidenced by the case examples, the impact of Behçet’s disease extends far beyond the direct manifestations of the illness. Consequently, the psychosocial challenges can be profound, yet can often be overlooked by professionals. This includes literature that supports that those with Behçet’s face disproportionate levels of depression and sexual dysfunction (1), which can have immense repercussions on connecting and forming intimacy with others. Tony Thornburn, chair and patient group advocate of Behçet’s UK, supports this, informing that loneliness is a prevalent issue among individuals with Behçet’s.

Further, Behçet’s can be classified as a disease with a course that is generally relapsing or episodic in nature. The resulting psychological discrepancy between states of crisis and noncrisis is taxing on both the individual and family system (2). Notably, this also comes with stigma and misunderstanding that someone may not be ill or incapacitated during a remission, leading to a lack of empathy, when residual symptoms like chronic fatigue might be equally challenging to manage. At a time so critical for social support, it is imperative that all involved ensure that patient psychological needs are placed at the forefront during diagnosis and treatment.

Medical Management Beyond the Disease

Healthcare professionals must ensure that the individual is placed empathetically at the center of care, encompassing the meaning of patient-centered care, alongside consideration for supporting friends and family. Reaching the realization that someone is so much more than their disease or illness is fundamental and cannot be understated. In reality, it is those around us that uplift and give strength in times of need. Both professional and personal relationships have the capacity to be harnessed for this purpose.

Positive professional interactions are achieved with the incorporation of strong interpersonal skills, coordination of care, and a genuine desire to improve the patient’s circumstances. On the contrary, it is imperative to work towards avoiding responses that are dismissive or show an unwillingness to act that can then inhibit the patient-provider relationship. Professionals should also expect and plan for potential difficulties with illness adjustment, such as at diagnosis, in line with individual and family transition periods.

Looking Forward

Research in the area of Behçet’s disease holds abundant potential with further funding and resources. Further improvement of patient educational materials is also warranted, considering the current health literacy readability barriers of sources available online (3). At national levels, Thornburg cites the necessity of a more streamlined process for patients and professionals alike, including patient registries and the integration of mobile technology for research purposes. One example of success has been displayed in the formation of three National Behçet’s Centres of Excellence in England since 2012, bringing down diagnostic timelines from upwards of 12 years to about one.

Conclusion

Receiving a Behçet’s diagnosis comes with many challenges, but it should never be a process that a patient has to go through alone. The two patient stories of Daniella and Rachael are testament to the reality of living with Behçet’s, but both have simultaneously shown immense strength and perseverance in pursuing their passions despite their illness. While the psychosocial challenges are apparent, professionals can foster community and hope by providing a connection to local resources and services, as well as encouraging involvement in social supports like patient support groups. Advocating for a better future for patients has never been more important for healthcare professionals, and pushing for more research and better policies can greatly enhance the lives of those living with Behçet’s.

Cited information:

1. Saur, S. J., Schlögl, A., Schmalen, T., Krittian, S., Pecher, A. C., Henes, M., Xenitidis, T., & Henes, J. (2022). Sexual dysfunction and depression in Behçet’s disease in comparison to healthy controls. Rheumatology International, 42(1), 121–126. https://doi.org/10.1007/s00296-021-05000-4
2. Rolland, J. S. (1987). Chronic illness and the life cycle: a conceptual framework. Family Process, 26(2), 203–221. https://doi.org/10.1111/j.1545-5300.1987.00203.x
3. Özduran, E., & Hanci, V. (2022). Evaluating the readability, quality and reliability of online information on Behçet’s disease. Reumatismo, 74(2). https://doi.org/10.4081/reumatismo.2022.1495