The “Stuff” of Cystic Fibrosis

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Since cystic fibrosis (CF) was identified in 1938, tens of thousands have lived with the severe genetic disease. Each experience has been individual, yet common threads run through, most notably experiences of healthcare. People with CF become well-acquainted with the clinic, the hospital, the pharmacy; the need to accommodate at home piles of pill bottles, physio devices, nebulizer set-ups, perhaps home IVs, feeding tubes, oxygen compressors, insulin. The lived experience of everyday life with CF in a large part resides in these objects and their environments.

Bottle of Creon pancreatic enzymes, ca. 2022 (personal collection, photographs courtesy of author)
Moog enteral feeding bag, ca. 2018 (personal collection, photographs courtesy of author)
Saline flush syringe, ca. 2019 (personal collection, photographs courtesy of author)

Cystic fibrosis (CF) is a genetic disease characterized by an overproduction of thick mucus, predominantly affecting the lungs and gastrointestinal system. Easily clogged airways facilitate frequent cycles of respiratory infection and inflammation, causing progressive lung damage that can ultimately require double-lung transplant. Mucus also blocks the pancreatic release of enzymes necessary for digestion, so that most people with CF depend on enzyme pills and vitamin supplements to avoid malnutrition. CF can also cause issues with the sinuses, male reproductive tract, a CF-specific variety of diabetes, heat-related illness, and arthritis, as well as the effects of a lifetime’s worth of frequent, high-dose antibiotics and other treatments. While recent years have seen the introduction of game-changing medication Trikafta, not all with CF benefit, and the disease remains incurable. Though only established as a discrete disease in the 1930s, by pathologist Dorothy Anderson, the picture of CF has seen dramatic change. When Anderson first used the name “cystic fibrosis of the pancreas,” diagnosed individuals rarely reached their fifth birthdays. In 2020, the median age of survival in Canada reached 55.4

The author, diagnosed with CF at six weeks old, at her MA graduation, aged 26 (courtesy of author).

While factors in this change include changing methods and criteria of diagnosis, significant changes have occurred in the ways CF has been treated over its 84 years. Technologically, socially, and psychologically, the life of a person with CF born in 1947 differed in major ways from one, like myself, born in 1987. The histories of these lived experiences have yet to be written, often sidelined in favour of an optimistic narrative of medical innovation. Yet the traces of lives lived with CF exist, in writing, images, and, most pertinently for this project, in objects.

Soluvac intravenous outfit, ca. 1950 (Museum of Healthcare)
Intramuscular solution ampoules of terramycin, an antibiotic once commonly used to treat lung infections in people with CF, ca. 1960 (Museum of Healthcare)
Respirgard II nebulizer, ca. 1990 (Museum of Healthcare)

As a person with CF myself, accustomed to accommodating daily life around the routines and objects of treatment, such implications of materiality dwell close even when I engage with historical objects I have never myself used. Even when I’m not aware of all an object’s non-medical effects, I am used to having to think about the practicalities of ‘stuff.’ I’ve had to negotiate the placement of nebulizer compressors that can buzz themselves off the table with vibrations, mid-night feeding tube leaks hardening on my mattress, how strong a command sticky hook has to be to hold an IV bag on the wall by my bed, the tetris game of moving furniture in a tiny apartment bedroom so an oxygen compressor can stand with the requisite 6 inches of air around it. Some CF objects I’ve taken for granted, others I still can’t regard without symbolic hatred. As a small child I once even engineered the ‘accidental’ running over of a particularly disliked piece of physio equipment. Healthcare objects are not, and never have been, neutral.

A marked example of the sometimes unexpected ways objects integrate themselves into CF life comes in the form of nebulized medication. In essence, a nebulizer is a device used to turn a liquid medication into a mist or fog-like spray for inhalation. Various forms of nebulizers have been a mainstay of CF treatment since the 1950s, for medications such as antibiotics and mucolytics. Today, a majority of people with CF spend somewhere between five and thirty minutes, once to three times a day, puffing medical mist from the plastic mouthpiece of a vibrating nebulizer compressor.

Vios compressor with Pari nebulizer cup,  ca. 2019. Photograph by author, 2022.

The centrality of nebulizer therapy in CF treatment is also the reason that nearly every person with CF in the past twenty years immediately has a visceral reaction to these little plastic things:

Post on CF sub-reddit, originally posted 03 Aug 2014 (screenshot by author, July 2022).

Nearly all the medications intended for CF inhalation are packaged in small plastic vials, sealed by these twist-off caps… which, as the many respondents to that CF subreddit post testify, inevitably end up everywhere in a CF household. Like small pieces of Lego, they are not infrequently discovered by a bare foot.

The first CF-specific inhaled medication to be packaged this way was Pulmozyme, in 1993. The drug was immediately lauded as a “breakthrough” for its mechanism of cleaving the DNA of select components of CF sputum, thinning the sticky mucus and facilitating easier airway clearance.. When coverage and/or funding allows, Pulmozyme remains widely used today. A second inhaled medication specifically aimed at CF is TOBI, a formulation of the antibiotic tobramycin designed for inhalation rather than the usual intravenous infusion. Vials of TOBI are almost identical. To take both Pulmozyme and TOBI daily, then, as many do, and indefinitely, equals a lot of little plastic caps that never make it to the garbage can.

Dornase Alfa Pulmozyme Inhalation Solution, ca. 2013 (Smithsonian Institution).

Such small details are the fabric of everyday life with illness, artefacts of treatment beyond its medical effects. Looking backwards, they become fundamental materials of the social history of medicine. These kinds of objects—the nebulizer, the vials, the plastic lids underfoot—say something about what it was like to live with a certain illness at a certain time. In the case of CF, in the 1980s, the nebulization process may have been familiar, but the caps wouldn’t have been. The “mist tents” of the 1960s—highly humidified plastic tents usually placed over beds for people with CF to sleep in—are distant from both the 1980s and the 2020s.

These are all things to remember when looking at objects of healthcare history. An object may represent a treatment, perhaps an innovative one, but it also evokes the place of a treatment in an individual’s life. For those with chronic illnesses reliant on objects of healthcare, these objects embed themselves throughout our lives. Their very materiality can thus testify to the ways those lives are, and have been, lived, and not just ‘treated.’  

Stephen Taylor, 9, with cystic fibrosis, with nebulizer mask and mist tent, May 27, 1971. Photograph by Keith Beaty for the Toronto Star (From the Toronto Star Archives, via the Toronto Public Library Digital Collections).

In upcoming posts, I’ll be further exploring some particular CF-related objects, in lights of historical contexts and lived experiences.

To discover more about these artefacts and many others, visit our online collection catalogue by clicking below:

Special thanks to Ian M. Fraser and Janine M. Schweitzer for their generous support of the 2022 Margaret Angus Research Fellowship.

Anna Krentz (Margaret Angus Research Fellow 2022)

Anna Krentz is an archivist, historical researcher, and writer with cystic fibrosis. Her interdisciplinary background includes an MA from Toronto Metropolitan University and Honours BA from St. Francis Xavier University as well as graduate coursework in the History department at the University of Toronto. Currently she leads the archives of the Modern Literature and Culture Research Centre within TMU, while pursuing her own academic and creative projects centering the lived experiences of cystic fibrosis in the mid-century and diabetes in the 1920s. 

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