New Approaches in the Management of Atypical Cystic Fibrosis

Patients with cystic fibrosis are usually diagnosed during childhood. The disease is often accompanied by other conditions including pulmonary disease, malabsorption, malnutrition and elevated sweat chloride. In the past, cystic fibrosis patients rarely lived past their second decade; however, some patients are diagnosed with atypical cystic fibrosis in later life.

This is because, during their infancy, they displayed symptoms not normally associated with cystic fibrosis. Furthermore, the signs tend to be milder; for example, respiratory symptoms tend to be less severe [1]. As a result, their lifespans tend to be longer but often live with various symptoms that impact their quality of life. Thankfully, new respiratory, gastrointestinal and nutritional therapies have contributed to the survival and symptom management of patients with atypical cystic fibrosis.

Regular Treatments for Cystic Fibrosis

Cystic Fibrosis is an incurable condition, but treatment can ease symptoms and improve quality of life. With close monitoring and systemic intervention, you can slow the progression of cystic fibrosis, thus increasing lifespan. The unfortunate aspect of atypical cystic fibrosis is that it’s often difficult to diagnose, and thus, difficult to treat.

As managing atypical cystic fibrosis is complex, a multi-speciality, integrative approach is advisable. With the correct application of allopathic and holistic therapies, healthcare practitioners can prevent lung infections, loosen mucus, alleviate intestinal blockage and ensure adequate nutrition.

Frequently, common medication will target the gene mutation that causes cystic fibrosis. These medications will be complemented with antibiotics to prevent infection and anti-inflammatories to lessen swelling in the lungs. Equally, mucus thinning drugs such as hypertonic saline help loosen buildup and decongest the airways.

To combat the nutritional aspect of cystic fibrosis, healthcare professionals will also often prescribe oral pancreatic enzymes to help the digestive tract absorb nutrients. They’ll also prescribe acid-controlling medications to help these enzymes function properly.

Medicinal Mushrooms as a Useful Adjunct Therapy

As is clear, patients with atypical cystic fibrosis are often on a complex cocktail of medications. Therefore, it is of particular interest to healthcare practitioners to identify therapies that won’t interact with other drugs and are suitable for long term use. This is why medicinal mushrooms are gaining particular attention, as due to their impressive nutritional profile, can alleviate symptoms and enhance quality of life.

For instance, Cordyceps is a variety that has yielded compelling results in preclinical trials. In a collaborative study between researchers at Chinese universities and The  University of Illinois at Chicago, tests demonstrated antifibrotic activity when Cordyceps was administered during the development of lung fibrosis.

Equally, symptoms were alleviated after the development of fibrosis when a Cordyceps extract was administered. Further testing suggested this was a result of the inhibition of critical fibrosis mediator (TGF-β1), reduction of leukocyte infiltration and collagen production, and the recovery from an imbalance of tissue degradation. Researchers also observed a reduction of infiltration of inflammatory cells and deposition of fibroblastic loci and collagen [2].

Improving Quality of Life for Atypical Cystic Fibrosis Patients

Atypical cystic fibrosis is currently a chronic condition that can have diverse manifestations and patients will have varying needs. Managing their condition and extending their lifespan is an essential concern for patients with the disease. Considering the complexity of the condition, both conventional and adjuvant therapies can yield results.

Hence, medicinal mushroom varieties like Cordyceps are a compelling proposition. With many other nutritional benefits besides the anti-fibrosis and anti-inflammatory action mentioned here, this mushroom variety has the potential to enhance patients’ quality of life. Further research is needed, but pre-clinical trials have certainly presented already interesting findings.


  1. Schram C. A. (2012). Atypical cystic fibrosis: identification in the primary care setting. Canadian family physician Medecin de famille canadien, 58(12), 1341–e704.
  2. Chen, M., Cheung, F. W., Chan, M. H., Hui, P. K., Ip, S. P., Ling, Y. H., Che, C. T., & Liu, W. K. (2012). Protective roles of Cordyceps on lung fibrosis in cellular and rat models. Journal of ethnopharmacology, 143(2), 448–454.