The Big Sick And The Science Behind The Story
Updated: Jul 3, 2018
Potential new treatment for Stills disease shows promise in its phase 2 clinical trial.
Note: Film spoilers ahead
The Big Sick won the hearts of viewers and critics alike for its original and heart-warming grasp on the complex link between the onset of sudden illness, and the difficulty of navigating relationships in times of crisis. The movie is loosely based on the real-life experience of its two scriptwriters, Kumail Nanjiani and Emily V. Gordon. It tells the story of Pakistan-born aspiring comedian Kumail and grad-student Emily, and how their relationship becomes complicated when Emily falls ill with a mysterious illness early on in the couple’s courtship. Emily is put into a medically induced coma as the doctor’s race against the clock to treat what they initially believe is an antibiotic-resistant infection. Later, it is discovered that Emily actually has a rare autoimmune disease known as adult-onset Still’s Disease, which is a rare multi-systemic autoinflammatory disorder 
While in the film Emily is quickly diagnosed, treated and rehabilitated, this Disney-style brand of medicine is rare and does not reflect the true experiences of real-life patients. Often is the case that symptoms are not immediately treated by conventional first-line treatments, and a large majority of patients still experience flare-ups following the initial presentation of symptoms.
Diagnosis for patients with Stills disease is often a complex and frustrating process. Currently, knowledge on the disease is limited and while the cause of the disease is considered to be infectious, the jury is still out as to what may be the precise aetiology/origin of the condition. The symptoms of the disease mimic that of a number of other illnesses such as infections and cancer, with patients displaying high fevers, liver inflammation and fluid around the lungs. Unlike Emily however, a large percentage of patients do not become ill enough to become hospitalised, instead spending a great deal of time in the healthcare system struggling to get their symptoms correctly diagnosed. One study found that on average it takes 21 months before a patient is given the correct diagnosis of the disease , as currently there are no specific clinical or laboratory tests for Still’s and the diagnostic approach to this disease is one of exclusion .
The natural history of the disorder differs on a case by case basis but typically follows three patterns of progression. Firstly, the disease is limited to a single episode which is over within a few months — in these cases patients are symptom-free within a year . Secondly, a pattern of multiple flares is seen with remissions ranging from a few weeks to a few years, decreasing in severity over time. Finally, the disease is experienced chronically as is persistently active.
In regard to the prognosis of adult-onset stills disease, it a benign and non-fatal disease with a low mortality rate . At present, the management of the condition mainly involves the use of non-steroidal anti-inflammatory drugs (NSAIDS), systemic glucocorticoids and conventional synthetic (CS) disease-modifying anti-rheumatic drugs (DMARDs) . However, not all patients respond positively to these treatments.
In February, a Swiss Clinical trial  showed promise for a potential new treatment for the condition. The trial (published in the Journal of Rheumatic Diseases) aimed to test a drug which blocks the cytokine IL-18, which is found in high levels in patients with Still’s disease. The trial is funded by the Swiss start-up AB2 bio, which purchased the drug tadekinig alfa from a pharmaceutical company that had originally developed it for the treatment of rheumatoid arthritis.
In the study 23 patients with active Still’s disease who still displayed symptoms following on from treatments with steroids or other medications were given injections of tadekinig alfa three times a week for a duration of 12 weeks. In half of the patients, an improvement of symptoms was observed. In addition to this, levels of circulating C-reactive protein, a bio-marker for inflammation were found to drop by at least half or to return to normal levels. While this phase of the trial did not have a control arm, plans are underway for a phase III clinical trial to begin sometime next year.
The Big Sick, has shone a spotlight on this rare disease, showing us the heartache, the complexities and the human factors inherent to Stills, which when read about purely in the form of statistics or in journal articles detaches us from actual human experience. Movies like this can be the seeds that help create a dialogue between science and the public, a dialogue which is vital to both raising awareness and highlighting on-going research. The Swiss clinical trial, therefore, while still in its early stages offers a promising, new and prospective way to treat patients with Still’s Disease.
Perhaps this encouraging research can turn the saying that “art imitates life” on its head, and like the movie give Still’s suffers the happy ending they desire.
Research it seems is making big progress.
Author: Caitlin Black
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