Kimberly Akimbo, a new Broadway musical, won five Tony Awards on Sunday night, including “Best Musical”. The show, by David Lindsay-Abaire, focuses on Kim, a girl with a fictional disease that causes her to age at over four times the normal rate. At sixteen years old, she appears to be a 72-year-old woman. Is this a medical reality?
Kim’s disease is unnamed, but we get a description of it in the song “Our Disease”: It’s “an incredibly rare genetic disorder / In which several signs of aging are manifested at a very early age” with symptoms including “wrinkled skin, stiff joints, hip dislocation, atherosclerosis, molecular degeneration, hypertension, presbycusis, [and] cardiac issues”. The song also states that “there is no cure” and that “few people with [the] disease / live longer than sixteen years.”
This disease has many similarities to a real disease called progeria. Like Kim’s disease, progeria (also called Hutchinson-Gilford syndrome) is a rare genetic disorder that causes rapid aging of the body. It is estimated to affect 1 in 20 million people. Children with progeria will typically start showing signs of the disease in the first few months of life; early symptoms include failure to thrive, stunted growth, and alopecia. As the disease progresses, individuals may display many of the symptoms mentioned in the song: wrinkled skin, atherosclerosis, cardiac issues, and musculoskeletal degeneration causing stiff joints and hip dislocation. Other symptoms can include kidney failure, loss of eyesight, and scleroderma.
One major difference between the real and fictional diseases is in the phenotypic features. Kim is a teenager who looks like a middle-aged woman, which is why she’s portrayed by Victoria Clark, an actress in her 60s. Individuals with progeria, on the other hand, have a distinctive appearance that includes a small body, narrow face, prominent eyes, small lower jaw, and prominent veins on their scalp (visible due to alopecia).
There is no known cure for either progeria or for Kim’s disease. The average life expectancy for those with progeria is around fifteen years. Even though Kim knows that her life might end soon, she retains a positive attitude. As the musical reminds us in the final song, “Great Adventure”: “Just enjoy the ride / Because no one gets a second time around.”
References:
Lamis A, Siddiqui SW, Ashok T, Patni N, Fatima M, Aneef AN. Hutchinson-Gilford Progeria Syndrome: A Literature Review. Cureus. 2022 Aug 31;14(8):e28629. doi: 10.7759/cureus.28629. PMID: 36196312; PMCID: PMC9524302.
