What are the Signs of End Stage Pulmonary Fibrosis?

No one wants to hear how bad it could get, yet everyone wants to know! Not only for their own sake, but also for their families and their caregivers.

Most long-term lung conditions gradually worsen with time. As people reach the end stage of long-term lung disease, physical changes typically appear. However, the progression to the end is different for people. Some people progress slowly while others progress rapidly. It is not easy to predict when life will come to an end.

Each person’s experience at their end of life varies. We must remember that the presence of one or more of these symptoms does not necessarily mean someone is close to death. They could have had any of these symptoms for months or even years before.  Below, we discuss some common end-stage pulmonary fibrosis signs, and offer suggestions to help alleviate these uncomfortable symptoms.

Symptom: Increased severity of shortness of breath
There is a noticeable gradual worsening of breathing. You may feel increasingly out of breath. This is due to a decrease in lung function which makes breathing more difficult. In some people, breathing might get worse more quickly, over weeks or months. Eventually, even things like changing your position, talking or eating might make you feel out of breath.

Ways to find relief: Breathing may be improved by using inhalers, other medicines and occasionally nebulizers. Some patients find it helpful to use a hand-held fan when they feel breathless as the feeling of air on their face can make it feel easier to breathe. If it is uncomfortable to breathe when lying flat, try sleeping in an upright position. In people with severe breathlessness and low blood oxygen levels, long-term oxygen supplementation may help improve their breathing.

Symptom: Fluid retention
Low blood oxygen levels as a result of reduced lung function make the body retain fluids. As the heart works harder to get oxygen to the body, this may result in pulmonary hypertension which can also result in swelling, particularly in the legs and abdomen. However, it can happen in other areas of the body as well which can make you feel uncomfortable.

Ways to find relief: This can be treated with tablets called diuretics which reduce the swelling. However, they will increase the frequency of trips to the toilet which may be a problem for you if you have difficulty moving about.

Symptom: Increased depression and anxiety
This becomes common as symptoms worsen and you get more housebound and isolated. Loneliness is a common problem for people with limited mobility as a result of a worsening lung condition.

Ways to find relief: Joining local or online pulmonary fibrosis support groups may help in coping with these symptoms. By participating in these groups, you meet and hear from other people who are going through similar challenges which may provide you some comfort. People in the support groups also share their coping strategies and the treatments they have tried and encourage you to share your feelings and experiences.

You could also work with a trained, licensed counselor if you prefer one-on-one support. A counselor can teach you coping strategies and other techniques to help you feel less anxious when your symptoms increase. You could also learn meditation and breathing techniques to help you relax.

If you are suffering from severe depression or anxiety, a doctor may also prescribe medications to help ease symptoms and improve mood.

Symptom: Poor appetite and difficulty maintaining a healthy body weight
Loss of appetite is a common problem as your pulmonary fibrosis progresses. An increase in breathlessness makes swallowing difficult and leads to weight loss. Weight loss may also be a result of hyper-metabolism caused by an increased workload of breathing.

Ways to find relief: Choosing softer moister foods may make swallowing easier. Eating small portions of high calorie foods throughout the day works well for some people. Your doctor may also recommend nutritional supplements such as Ensure or Boost to increase your caloric and nutrient intake. For people on long-term supplemental oxygen, using a nasal cannula while eating may help reduce the breathlessness felt during a meal and enable them to eat better.

Symptom: Persistent cough
This can be very bothersome and may be a result of reduced lung function and low oxygen levels. Coughing attacks may also lead to severe breathlessness which can be very distressing. Coughing attacks may also produce embarrassing incontinence, which is accidental or involuntary loss of urine from the bladder.

Ways to find relief: Sitting upright with the support of pillows may help during bouts of coughing. Your doctor can also prescribe certain medications that can help stop a cough. Incontinence brought on by a coughing attack can be managed by reducing the intake of alcohol and caffeine-containing drinks such as tea and coffee. Some people may also have a catheter put in. A catheter is a thin, flexible, hollow tube that is inserted through the urethra into the bladder and allows the urine to drain out. This can be useful in managing incontinence caused by a persistent cough.

Symptom: Fatigue and disturbed sleep
These symptoms are common as lung disease progresses. Fatigue may be due to a combination of poor sleep, insufficient food intake, depression, and anxiety. Disturbed sleep may be caused by symptoms such as breathlessness, pain, and coughing.

Ways to find relief: Your doctor may be able to determine the possible cause of these symptoms and help you with ways to manage them. The use of supplemental oxygen at nighttime may help improve sleep. Improved diet and sleep can help reduce fatigue.

Symptom: Frequent flare-ups and hospitalizations
As the lung function worsens, you may have increasingly frequent flare-ups. A flare-up can also occur if you catch a chest infection. This may require you to be hospitalized to receive ventilation support to maintain sufficient oxygen levels in your body. Sometimes, with the right equipment and respiratory specialist support, you may be able to manage a flare-up at home.

Ways to find relief: Flare-ups caused due to a lung infection are generally treated with antibiotics and a short course of steroids and usually improve. However, after each flare-up, the lung function does not get back to the level it was before the flare-up.

Symptom: Chest pain
This is not very common with lung disease, but some people do experience chest pain.

Ways to find relief: Chest pain can be treated with pain killers. For constant pain that is severe, a syringe pump is inserted under the skin that continuously provides a dose of strong painkillers. Some people may have pain because of muscle tightness from being inactive. Light stretching may help with this type of pain.

As your disease progresses, it may be a good idea to pursue palliative or supportive care. Palliative care is a treatment that is focused on relieving and preventing symptoms that are distressing. It is important to know that just because you choose to get palliative care, does not mean you cannot receive “curative care”. Palliative care is provided by a team of healthcare professionals who consider physical, psychosocial and spiritual factors in their treatment approach and can be a source of comfort to you as a patient, your caregivers, and your loved ones.

Are you, or someone you know, showing these signs of end stage pulmonary fibrosis? Join our virtual support group. Each month you’ll receive expert guidance on how to reduce PF symptoms and improve your quality of life. PF Now! also has a Facebook group whose active community shares their PF journey and their tips with others.

To learn more about the progression of PF, visit these links: 

• https://pulmonaryfibrosisnow.org/2019/05/22/how-quickly-does-pulmonary-fibrosis-progress/
• https://pulmonaryfibrosisnow.org/2019/09/18/causes-symptoms-prognosis-treatment-acute-exacerbation-ipf/
• https://pulmonaryfibrosisnow.org/2021/01/27/pf-struggles-and-ways-to-cope/ 
• https://pulmonaryfibrosisnow.org/basics-old/how-to-measure-your-progress/ 
• https://pulmonaryfibrosisnow.org/2021/03/05/signs-of-caregiver-burnout-and-ways-to-prevent-it/ 
• https://pulmonaryfibrosisnow.org/2020/12/30/new-developments-in-pulmonary-fibrosis/