Understanding and alleviating the chronic cough of pulmonary fibrosis

Do people move away from you in the grocery store when you start coughing? Does cough disrupt your speech and sleep? Do you sometimes vomit or empty your bladder during a bad bout of coughing? Maybe you’ve even blacked out, or broken a rib, or strained a chest muscle, from coughing so hard or so long.

Coughing is a common symptom of pulmonary fibrosis (PF), affecting about 80 per cent of patients. For about 25 per cent, coughing can be a very serious and debilitating symptom. The following resources will help you understand and cope with your chronic cough caused by PF.

The first hurdle in finding relief can often be getting your health care provider to listen to you and address your coughing concerns.

A recent webinar (webinar 6) by the EU-PFF (European Union Pulmonary Fibrosis Federation) may help you convince your doctor that your cough requires something more than sipping water and sucking on mints.

The recorded webinar features Professor Surinder Birring, a respiratory physician and cough specialist at King’s College Hospital in London and is hosted by John Solheim, a 58-year-old IPF patient and an EU-PFF board member.

They share an overview of PF cough and various medical treatments (current and in development), as well as some useful non-medical tips that you can try on your own to help you cope with the chronic cough of PF. Here are some highlights from the webinar:

Cough Overview

• Coughing is a vital reflex to clear the airways and to protect airways from aspiration of foreign substances into the lungs.
• Chronic cough is common in PF and is distressing. It can prevent patients from doing the simplest things. It can be exhausting and can create panic that you will not take another breath. It is a visible sign of illness and can be isolating and embarrassing.  
• Despite its prevalence, there has been little progress in understanding PF cough. We do know that a worsening cough is associated with progressing disease, a reduced quality of life and reduced survival.
• Patients experience different types of coughs: dry, tickling, itchy throat coughs, as well as those that expel phlegm. And people experience different cough triggers, such as: a change in position, exercise, talking, smoking, a change in temperature, tickle, itch in throat, exposure to perfumes and aerosols, etc.
• With so many triggers, there is evidence that chronic cough, including cough from PF, may be the result of hypersensitivity of the cough nerves.

Cough nerve sensitivity studies

In one study, capsaicin (chili pepper), a potent trigger of cough, was inhaled by PF patients and healthy individuals. The PF patients had a much greater coughing response than the non-PF subject, indicating cough nerve sensitivity in those with PF.

In another study, Substance P, a neurotransmitter protein, which activates nerves in the central nervous system, was inhaled by both PF and healthy subjects. It induced cough in 70 per cent of people with PF, but almost none in healthy subjects.

Why are the cough nerves more active in PF? We’re not sure, but there are several possibilities:

• Airways may be stretched to compensate for scarring.
• Nerve sensitivity from a degree of inflammation (esp. in PF associated with auto-immune disease),
• Sputum may increase the cough reflex
• Reflux may also be a cough trigger
• The cough nerves themselves may become stretched, or sprout new nerves, increasing cough.
• Causes of cough beyond PF need to be investigated too, such as infection, smoking, medications (especially some blood pressure drugs such as ACE inhibitors), asthma and sleep apnea.
• Cough-related symptoms that you should alert your physician to, include: Blood in sputum, weight loss, fever, hoarse voice, any inhaled foreign substance.

Cough Treatment

• The first step is to treat, eliminate or minimize, things that increase your cough. These include: smoking, obesity, ACE inhibitor medications, sleep apnea, reflux, lung infection and nasal or sinus problems. 
• Second, optimize the use of PF medications – anti-inflammatories, when indicated, and anti-fibrotics.
• There are two neurological affecting drugs that may be helpful, according to guidelines for unexplained chronic cough from the American College of Chest Physicians.
• Speech and/or physical therapy may be helpful in managing cough.
• Morphine can be an effective treatment for some. A recent clinical trial of morphine for cough, showed a 39 per cent reduction in cough. Dr. Birring has his patients undergo a two-week trial of 5 mg. slow release, twice a day, and then makes an assessment. If it is working, you can stay on it for six months, then take a “medication vacation” to mediate addiction risk. Then start again. As a controlled substance, it may need to be ordered one month at a time.

New cough treatments being investigated

Nalbuphine ER targets the endogenous opioid system. It is similar to morphine, but targets two receptors, instead of one. In a phase 2 trial on unexplained cough, it showed a 75 per cent reduction in coughing and reduces the dependence factor of morphine. Trials will soon be underway with PF patients.

Orvepitant is a drug currently being studied in IPF patients. It blocks the effects of Substance P, a key mediator in cough hypersensitivity. Results are expected later this year.

Cough Self-management tips

• Sip water, gum, mints, cough lozenges (Fishermen’s Friend is mentioned)
• Try to  reduce stress and anxiety
• Increase your nasal breathing and decrease your mouth breathing
• Rest your voice
• Try speech therapy and/or physiotherapy, specifically for cough reduction.  

For John Solheim, chronic cough is a serious matter. He coughs often and hard. Coughing has caused him one broken rib and strained chest muscles and he has  lost consciousness on several occasions.

How John copes with the cough of PF

• While you may not be able to do anything about the cough. You can do something about how you cope with it.
• A physiotherapist has helped him learn how to cough “productively” to help clear his airways. 
• Tell others about your PF. By not sharing, you are leaving them to create their own stories. Don’t leave them to guess why you are coughing. It also helps to let others know you are not a danger to them. You are not contagious.
• Respect that your cough will have an impact on others. While you are experiencing it, so are they. It can be irritating. Take breaks away from one another in your household.
• Physical activity may trigger cough, but it is too important to skip!
• Let people around you give you a helping hand.

John’s cough self-help preparation techniques

• Never be late for anything. This helps you avoid stressful situations, which can trigger a coughing attack. Being early allows you to settle and relax and be calm and helps avoid a coughing attack.
• Avoid physical strain. Before attending an event, take time to relax and calm your body. Take as much time as you need.
• Learn your cough. For John, avoiding stress helps, but everyone is different. Pay attention to your body and learn to recognize the triggers for your cough.
• Board early when flying. Ask at the gate if you can board early and explain that you have PF and that it will make boarding better for everyone, if you are not having a coughing fit.
• At other venues. Ask if you can enter and settle early at other venues, such as concerts, movies, etc. Do not be upset if the answer is no. There can be many reasons why this is not possible. Getting upset will make you more likely to cough. In most cases, if it is possible, people will want to help you.
• Cough medication. This can be helpful, although often comes with the side effect of drowsiness and not being able to drive.

The Largest Study of Cough and ILD in Canada

Check out the CPFF video featuring Dr. Ciaran Scallan, talking about the largest study of cough and ILD, underway here in Canada, with the support of a research grant from CPFF.