COVID-19: Why some patients end up with incurable Pulmonary Fibrosis

Dubai: Neena G (name changed on request), an asthma patient who tested positive for COVID-19 last year, had a mild case of the infection. She was asymptomatic in the beginning, but within one week her condition deteriorated rapidly.

Asthma made her condition worse and she began gasping for air, to the extent that she was hospitalised in the Intensive Care Unit and placed on ventilation support for a month. Neena recovered and is grateful for that; however, she suffered almost 40 per cent lung damage. She now has to live with oxygen support as her respiratory distress during COVID-19 triggered lung fibrosis.

September is the Pulmonary Fibrosis (PF) Awareness month and ever since the Severe Acute Respiratory Syndrome (SARS–COV2) virus spread in 2020, PF has been in sharp focus.

Dr Sandeep Pargi, pulmonologist and internal medicine specialist at Aster Mankhool, a hospital in Dubai, said: “COVID-19 affects the lungs and the respiratory systems and, in severe cases, those with pneumonia or Acute Respiratory Distress Syndrome [ARDS] can deteriorate to have permanent damage to lungs, which is fibrosis of the lung tissue... When patients suffer a Cytokine Storm in the lungs, the lung tissue is damage and fibrosis occurs which in extreme cases is permanent irreversible damage.”

What is a Cytokine Storm?

In a Cytokine Storm during an extreme state of infection, as witnessed during many cases of COVID-19, the body responds with a severe inflammatory response where Cytokines - or protein anti-bodies that are immune cells for protecting the body against alien invasion - turn rogue. The body is overrun by excessive immune cells meant, normally, for protection. In such cases, the lung tissue is damaged irreversibly.

Who is at higher risk?

Dr John Costello, MD, from Mayo Clinic Healthcare in London, said usually COVID-19 patients with lung infections recover, but caution needs to be exercised in cases where the inflammation in the lungs causes severe damage. “If the COVID-19 infection is severe and affecting the lungs and causing extensive inflammation, then it can cause damage that ‘heals’ with fibrosis and scarring. This is usually in patients who have been unwell to be admitted to ICU and placed on mechanical ventilation. It also includes the elderly and overweight, who are more at risk for this. The majority of patients will recover with well-preserved lung function, but individuals should consult with their physicians about their status to avoid getting permanent lung damage.”

According to the National Centre for Biotechnical Information (NCBI) and the National Institute (NIH), nearly 40 per cent of the COVID-19 patients get serious lung infections. Of these, nearly 20 per cent patients end up with lung fibrosis where damage is permanent.

What is Pulmonary Fibrosis (PF)?

However, PF has been around for a long time prior to COVID-19. Professor Dr Mohammed Meshref, Regional Medical Director, Boehringer Ingelheim, Middle East, Turkey and Africa, said: “Pulmonary fibrosis is a rare, lifelong, and progressive disease that causes the tissues of the lungs to scar and thicken over time, making it difficult to breathe. This is because with deterioration and scarring of the tissue, it becomes harder for oxygen to pass through and enter the bloodstream. PF occurs in many types of Interstitial Lung Diseases [ILD].

"The term ‘interstitial’ means the disease affects the interstitium or the lace-like network of tissue that supports the air sacs in the lungs. However, often there are no causes, in which case we refer to the condition as ‘Idiopathic’ Pulmonary Fibrosis [IPF]. Some types of PF can also be hereditary, while certain risk factors are old age, smoking, and breathing in dust and fumes. As a result of air pollution over a long period of time, it can make it more likely for someone to develop the disease.”

How prevalent is PF?

It is estimated that 60 million people in the Middle East are at risk of developing respiratory illnesses. These can be unique and cover a wide range of pulmonary disorders due to several factors such as smoking, extreme weather variations, and genetic conditions. PF is considered a rare and sporadic disease that affects 13 to 20 out of every 100,000 people worldwide, which makes it difficult to ascertain who it might affect regardless of how healthy they may be.

While there is no published data on the rate of incidence of PF in the UAE, globally it is on the rise. On the other hand, pneumonia is a common condition in the UAE amongst adults and children, said Prof Dr Meshref.

Dr Pargi said: “Although PF primarily affects patients aged 50 years or older, recent studies have found that the prevalence of IPF in adults aged 35 to 44 is 2.7 per 100,000 people, which means it is possible for young adults to develop the condition.”

Is PF fatal?

Fatality rates among patients with PF increase with age and are higher in men than in women. The average life expectancy of patients with PF ranges from three to five years after diagnosis; however, this depends on various factors such as existing medical conditions and age. Chronic obstructive pulmonary disease (COPD) can also affect the prognosis. The estimated mortality rates are 64.3 deaths per million in men and 58.4 deaths per million in women globally.

Can pneumonia spiral into PF?

The health specialists essentially drew a difference between ARDS diseases such as asthma or pneumonia and a chronic disease like PF. According to them, ARDS is different from PF. Dr Costello explained: “Pneumonia tends to present with a toxic illness, with fever, chills, cough and breathlessness, whereas pulmonary fibrosis is a more insidious process, with increasing shortness of breath and cough over a longer period. Severe pneumonia can lead to a degree of lung fibrosis. Clearly, bacterial pneumonia can be treated with antibiotics and cured, whereas fibrosis is much more difficult to treat, especially when it is advanced. There are some new drug treatments for pulmonary fibrosis available through specialist centres, but in the most severe cases, lung transplantation may need to be considered.”

Both ARDS and PF are connected as severe ARDS complication results in lung fibrosis, doctors have pointed out. During the recent pandemic, several severely critical patients eventually had lung fibrosis - and either succumbed to it or had to undergo lung transplants. However, Dr Meshref, added: “While there seems to be a link between the two, the conditions are treated as separate by the medical community.”

What are the triggers?

The development of PF can be triggered by risk factors such as inhaling dust and fumes over a prolonged period of time, old age, developing autoimmune or viral infections, and exposure to radiation and chemotherapy. It can also be passed on genetically, albeit very rarely.

Dr Meshref said those who had PF prior to the onset of COVID-19 have a higher risk of being severe COVID-19 patients if they contract it. “Research shows that people with chronic lung diseases such as PF are at a higher risk of developing severe illness from COVID-19. Moreover, it is one of the possible consequences of COVID-19 pulmonary pneumonia. In the case of ARDS, an estimated 15 to 30 per cent of those hospitalised with COVID-19, go on to develop COVID-19-associated acute respiratory distress syndrome [CARDS].”

Different stages of PF

There are four stages to consider, based on the progression of the disease: mild, moderate, severe, and very severe.

The progression can be determined by the severity of the symptoms and the capacity of the lungs. As the disease progresses to severe stages, the patient loses lung capacity and may not be able to breathe without mechanical assistance. The earlier the disease is diagnosed, the better the chance of improving their quality of life through medication and pulmonary rehabilitation. Treatment options during the initial stages can temporarily slow down the development of symptoms while oxygen therapy and pulmonary rehabilitation can improve lung efficiency.

Difference between PF and pneumonia

PF is a chronic disease and its severity varies from individual to individual. Some patients may develop severe symptoms in a short span of time, while others may experience mild symptoms that aggravate gradually. As the disease worsens, it can result in shortness of breath and eventually lead to the body’s organs not getting enough oxygen to function properly. Other symptoms include dry cough, fatigue, muscle and joint pain, weight loss, widening or rounding of fingers or toes.

In PF, it can take years and multiple tests for an accurate diagnosis, and even then, it is important to understand that no two people will have the same experience.

Pneumonia, on the other hand, is an acute disease with symptoms that can be similar to cold or flu. This can include fever, chills, and cough and can progress to chest pain and shortness of breath. It can be treated with antibiotics or antiviral medication, depending on the cause of the infection, which is similar to the treatment of flu.

Living with PF

Once an individual receives a diagnosis of PF, which has no known cure so far, there are ways and means to manage the condition with different therapies. These include new medications, oxygen therapy and pulmonary rehabilitation such as breathing techniques and professional counselling to improve the patient’s day-to-day physical functioning.

PF is more commonly associated with severe lung injury or respiratory infections; however, there is scope for more research on the direct correlation between PF and pneumonia, said Prof Dr Meshref.

Patients need to work closely with their treatment team to monitor symptoms, ask questions, practice breathing techniques, and manage the cough. Moreover, taking care of one’s health, nutrition, and mental well-being is of utmost importance when diagnosed with PF. It is essential to ensure patients receive timely vaccinations, get ample sleep and rest, exercise regularly, and stop smoking.

Should you be worried if you have PF?

The progression of PF depends on a multitude of factors that range from the patient’s age, health and lifestyle, to environmental factors, and the severity of the disease when diagnosed. Patients who lead an unhealthy lifestyle, as well as those with pre-existing conditions such as COPD may have severe symptoms if diagnosed with PF. Early diagnosis to vital to ensure the best, timely treatment. Visiting a pulmonologist in the case of recurrent or persistent chest symptoms will ensure early detection of PF, while maintaining a healthy lifestyle and avoiding smoking for instance ensures better prognosis and results for those diagnosed with the disease.

Is a lung transplant needed?

Patients with severely progressed pulmonary fibrosis could be eligible for lung transplant. In lung transplantation, one or both damaged lungs are replaced with lungs from a donor.

Dr Meshref said: “Lung transplant may also be considered if the disease rapidly worsens. It can also be advised for patients with low chances of survival in the following two years due to PF, those with a high chance of surviving at least 90 days after the intervention, and patients with a high probability of surviving five years after the transplant from any other medical condition.”

He added: “Since transplantation is a complex procedure, patients are assessed individually based on survival expectancy. However, several reasons including non-curable infections may make it unsuitable for certain patients. After a lung transplant, patients need to take certain medicines for the rest of their life. Lung transplant requires careful consideration and assessment before opting for this procedure.”

What are other treatments?

The lung damage caused by PF is typically irreversible, which makes it a life-long disease. However, recent studies have found certain treatments to reduce the rate of lung function decline in PF. However, their effect on the reversal of the lung damage remains uncertain. Early diagnosis and treatment remain crucial for improved lung function.

Precautions during the pandemic

Living with chronic lung disease increases the chances of experiencing severe illness and complications from COVID-19. For this reason, it is important that people follow safety guidelines to minimise the risk of infection. The first and most important step towards prevention is getting the COVID-19 vaccination. People are also encouraged to take a vaccination for flu and pneumonia.

Those with existing lung diseases are strongly recommended to consult their physicians on whether to continue or adjust their prescribed medicines if they have a COVID-19 infection. Dr Costello said: “For those who want to prevent any long-term lung damage, it is important to get vaccinated, wear a mask and if you test positive, to go into isolation.”

Pulmonologists point out that those who have PF should consult their doctors for guidance but can also rely on trusted and credible platforms for information.