11. Meet the Patients Series: Katie Fielding on Living with Cystic Fibrosis

We are extremely excited to introduce our new Pulm PEEPs Meet the Patients series. Teaching and learning medicine is an incredible journey, and the goal is always to be improving patient care. Patients are our best teachers about the diseases we encounter, so the goal of this series is to spend more time with patients with pulmonary disease and with those who have been critically ill. For our first episode, we are thrilled to be joined by Katie Fielding.

Katie s an educator and spent 13 years teaching high school science. She now specializes in integrating technology into the classroom to enhance education. Katie was diagnosed with CF as an infant and has spent years as a patient advocate. She works closely with the Cystic Fibrosis Foundation and serves on the Adult Advocacy Council.

Katie gives us an incredible perspective about what it is like to live with Cystic Fibrosis, how her life has changed with modern therapies, and how to be the best provider possible.

5. A Case of Chronic, Productive Cough

The Pulm PEEPs are joined again by Natalie West to discuss a patient who presented with a chronic, productive cough. Listen in today as we work through our differential diagnosis, interpret basic pulmonary testing, and share our clinical reasoning along the way. We have some fantastic diagnostic and treatment teaching points, so once you’ve solved the case check out the takeaways and infographics below. Please let us know any additional insights you have on Twitter!

Patient Presentation

A 50-year-old woman, who is a never smoker, with a past medical history of recurrent pancreatitis presents to the pulmonary clinic with a chronic, productive cough. Her cough has been present for 3 years and has increased in frequency to now being present daily. In the last three months, the cough has also worsened and is productive of small amounts of yellow to green sputum. She has a history of chronic post-nasal drip and sinus infections, and uses intranasal steroids, but has not noted changes in these symptoms. There is no significant family history of pulmonary disease, and an exposure history review of symptoms is negative.

On physical exam, she was a thin woman who appeared her stated age and was breathing comfortably on room air. Her exam was notable for mild expiratory wheezing, primarily on auscultation of the right posterior lung field. She had no cyanosis, clubbing, evidence of volume overload, or abdominal tenderness.

Basic Spirometry Values
Chest X-ray

Key Learning Points

**Spoilers Ahead** If you want to think through the case on your own we advise listening to the episode first before looking at the infographics below.

Differential Diagnosis of Chronic Cough

Three most common causes: upper airway cough syndrome, GERD, cough variant asthma

Additional etiologies to consider: chronic bronchitis, post-infectious after a respiratory tract infection, bronchiectasis, ACE inhibitors, lung cancer, eosinophilic bronchitis, interstitial lung disease


Imaging Pearl


Evaluating Bronchiectasis


Making a New Diagnosis of Cystic Fibrosis in an Adult

Sweat testing

Sweat testing should be done in CF accredited center. Inform patients that there are no needles involved. Pilocarpine and electrical stimulation are applied to the arm or leg to stimulate the sweat gland, and then sweat is collected on filter paper, a gauze, or a plastic coil. From there, the amount of chloride in the sweat is calculated

Results

< 30 normal

31 – 60 indeterminate

> 60 is positive and Cystic Fibrosis is likely

What do you do with an Indeterminate test?

Patients with milder phenotypes of Cystic Fibrosis can have a normal or indeterminate sweat chloride level, and 10% of adults diagnosed with CF have a normal sweat chloride. If the sweat chloride test is indeterminate or normal, but suspicion is high for CF, then genetic testing for the whole array of mutations should be performed

References and links for further reading

  1. Morice AH, Millqvist E, Bieksiene K, et al. ERS guidelines on the diagnosis and treatment of chronic cough in adults and children. European Respiratory Journal. 2020;55(1). doi:10.1183/13993003.01136-2019
  2. Barker AF. Bronchiectasis. New England Journal of Medicine. 2002;346(18):1383-1393. doi:10.1056/NEJMra012519
  3. Bronchiectasis: a case-based approach to investigation and management | European Respiratory Society. Accessed November 23, 2021. https://err.ersjournals.com/content/27/149/180016
  4. Rowe SM, Miller S, Sorscher EJ. Cystic Fibrosis. New England Journal of Medicine. 2005;352(19):1992-2001. doi:10.1056/NEJMra043184
  5. Shteinberg M, Haq IJ, Polineni D, Davies JC. Cystic fibrosis. The Lancet. 2021;397(10290):2195-2211. doi:10.1016/S0140-6736(20)32542-3
  6. Jain R. Diagnosing Cystic Fibrosis in Adults: Better Late Than Never. Annals ATS. 2018;15(10):1140-1141. doi:10.1513/AnnalsATS.201806-432ED

4. Top Consults: Hemoptysis

Pulm PEEPs hosts, Kristina Montemayor and David Furfaro, bring our first episode in our Top Consults series. In this series, we will bring in experts to work through the most common pulmonary and critical care consults. Whether you are the consulting physician, or a pulmonologist responding to the page, these episodes are geared to give you all the information you need to care for your patients!

Today, we are joined by Chris Kapp and Matthew Schimmel, two interventional pulmonologists, to discuss hemoptysis. Chris and Matt will help us work through two hemoptysis consults, and together we’ll provide a framework for thinking about hemoptysis, outline some key components of the evaluation, and delve into treatment options.

Key Learning Points

Hemoptysis Evaluation

Hemoptysis Management

Life-Threatening or Large Volume Hemoptysis

  1. Stabilize the patient! Make sure the airway is protected either by the patient coughing themselves, or intubation if needed. Provide hemodynamic support with IVF, blood products, and pressors if needed. If it is known which lung has the bleeding the patient can be positioned so the lung with the bleeding is down. This protects the non-bleeding lung.
  2. Correct any bleeding diathesis If the patient is on anti-coagulation, or has any reversible bleeding diathesis, these should be corrected immediately to reduce further bleeding.
  3. Localize the bleed If the patient is stable, they should undergo a CTA to localize the bleeding. If they are not stable to make it to a CT scan, a bronchoscopy should be performed.
  4. Bronchoscopic treatment In addition to clearing blood from the airway, bronchoscopy can localize the bleeding. With available expertise, bronchoscopic treatments can be performed such as ice saline, topical epinephrine, or balloon tamponade to isolate the bleed.
  5. Definitive therapy with arteriography and embolization Patients with life-threatening hemoptysis should ultimately undergo arteriography and embolization of any bleeding vessel. If this is not possible, then surgery can be needed in some cases.
  6. A note on diffuse hemoptysis If there is not one distinct bleeding lesion, then localizing and treating the bleed becomes more difficult. For diffuse alveolar hemorrhage, evaluation should be performed for if it is primary, and due to an immunologic cause and capillaritis, or secondary to a systemic disease and / or bleeding diathesis. These investigations will guide available treatment options. Capillaritis from an immunologic cause, such as lupus or vasculitis, can be treated with systemic glucocorticoids and an additional immunosuppressive agent such as cyclophosphamide or rituximab.

Non-life-threatening or Small Volume Hemoptysis

  1. Monitor for clinical worsening Patient’s should be monitored, either in the in-patient or out-patient setting, for increased volume or frequency of hemoptysis and for any clinical worsening, such as desaturations or decreased ability to clear the airway.
  2. Correct any bleeding diathesis If the patient is on anti-coagulation, or has any reversible bleeding diathesis, these should be corrected immediately to reduce further bleeding. In pattients with non-life-threateneing hemoptysis this requires careful consideration of balancing the risk of bleeding vs the benefits for continuing anti-coagulation.
  3. Evaluate for underlying cause Patient’s should undergo imaging and evaluation for the underlying cause of the hemoptysis. This may be evidence of an underlying infection, a pulmonary embolism, or new lung lesions making the patient at risk. If the source can’t be found on non-invasive imaging, and there is no clear systemic source such as an infection, a bronchoscopy is warranted. Any underlying cause should be treated and investigated further.
  4. Inhaled Tranexamic Acid Nebulized tranexamic acid is well tolerated and can help resolve hemopytysis without invasive procedures.

References and links for further reading

  1. Gagnon S, Quigley N, Dutau H, Delage A, Fortin M. Approach to Hemoptysis in the Modern Era. Can Respir J. 2017;2017:1565030. doi:10.1155/2017/1565030
  2. Radchenko C, Alraiyes AH, Shojaee S. A systematic approach to the management of massive hemoptysis. J Thorac Dis. 2017;9(Suppl 10):S1069-S1086. doi:10.21037/jtd.2017.06.41
  3. Davidson K, Shojaee S. Managing Massive Hemoptysis. Chest. 2020;157(1):77-88. doi:10.1016/j.chest.2019.07.012
  4. Lara AR, Schwarz MI. Diffuse Alveolar Hemorrhage. CHEST. 2010;137(5):1164-1171. doi:10.1378/chest.08-2084
  5. Wand O, Guber E, Guber A, Epstein Shochet G, Israeli-Shani L, Shitrit D. Inhaled Tranexamic Acid for Hemoptysis Treatment: A Randomized Controlled Trial. Chest. 2018;154(6):1379-1384. doi:10.1016/j.chest.2018.09.026

2. Cystic Fibrosis Roundtable

The Pulm PEEPs (Kristina Montemayor and Dave Furfaro) host a panel of Cystic Fibrosis (CF) providers to discuss the current state of the disease, recent advances in cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapies, and the evolving faces and voices of Cystic Fibrosis.

Cystic Fibrosis is an autosomal recessive disorder caused by mutations in the CFTR gene that affects over 30,000 individuals in the United States and 70,000 people worldwide. Absence or dysfunction of the CFTR protein leads to abnormal secretion of mucus, sweat, and digestive fluids, which impacts the lungs, digestive tract, and reproductive system.

From the first formal publication on Cystic Fibrosis in 1938 by Dorothy Hansine Andersen, to the discovery of the delta F508 mutation and CFTR gene in 1988 -1989 by Lap-Chee Tsui, Francis Collins, and John R. Riordan, to the approval of the first CFTR modulator therapy, Ivacaftor, in 2012, our knowledge about Cystic FIbrosis has been advancing in leaps and bounds. As therapies have improved, they have dramatically impacted the lives of patients with Cystic Fibrosis. Join us today as we explore what this evolution in care has looked like from the perspective of Cystic Fibrosis providers, and hear about the new questions and challenges on the horizon.


Meet our guests

Emily DiMango is a Professor of Medicine at Columbia University Medical Center and the Director of the John Edsall-John Wood Asthma Center and the Gunnar Esiason Adult Cystic Fibrosis Program

Terri Laguna is an Associate Professor of Pediatrics at Northwestern Medicine / Feinberg School of Medicine and the Chief of Pulmonary and Sleep Medicine in the Department of Pediatrics

Patrick Sosnay is a Senior Medical Director at Vertex Pharmaceuticals and specializes in Cystic Fibrosis

Natalie West is an Assistant Professor of Medicine at Johns Hopkins Hospital and specializes in Cystic Fibrosis.


References and links for further reading

  1. Cystic Fibrosis Foundation
  2. Shteinberg M, Haq IJ, Polineni D, Davies JC. Cystic fibrosis. The Lancet. 2021;397(10290):2195-2211. doi:10.1016/S0140-6736(20)32542-3
  3. Rowe SM, Miller S, Sorscher EJ. Cystic Fibrosis. New England Journal of Medicine. 2005;352(19):1992-2001. doi:10.1056/NEJMra043184
  4. Davis PB. Cystic Fibrosis Since 1938. Am J Respir Crit Care Med. 2006;173(5):475-482. doi:10.1164/rccm.200505-840OE
  5. Barry PJ, Mall MA, Álvarez A, et al. Triple Therapy for Cystic Fibrosis Phe508del–Gating and –Residual Function Genotypes. New England Journal of Medicine. 2021;385(9):815-825. doi:10.1056/NEJMoa2100665
  6. Middleton PG, Mall MA, Dřevínek P, et al. Elexacaftor–Tezacaftor–Ivacaftor for Cystic Fibrosis with a Single Phe508del Allele. New England Journal of Medicine. Published online October 31, 2019. doi:10.1056/NEJMoa1908639