Pulmonary fibrosis how do you die

Symptoms and symptom relieving treatments during the last week of life are shown in Table 2. Reported symptoms and symptom relieving medication during the last 7 days of life. Life prolonging treatments during the last week of life and diagnostic tests during the last day of life in different care settings are presented in Table 3. Three patients died in an ICU and two patients were intubated. Life prolonging treatments and diagnostic tests were common during the last days of life in patients dying at hospitals.

These procedures were more frequent in tertiary hospitals compared to community hospitals Table 3. In the present retrospective study we have described the end of life care of IPF patients. Our results indicate that DNR orders and end of life decisions were made late in the patients life span and life-prolonging therapies were likely to continue until the last days of life. In this perspective, the current guidelines seem to be poorly implemented to clinical practice. In general, patients suffering from non-malignant lung disease receive less palliative care compared to lung cancer even though the symptom burden is high in both [ 10 , 11 ].

Only about one third of the patients, in our study had documented EOL decision and most of them were made during the last three days of life. No palliative care consultations were found and referrals to hospice were very rare, although this may partly reflect the rarity of these services in Finland. In any case, these findings from our and previous studies highlight the need for advanced care plans for IPF-patients.

Roughly half of the IPF-patients are reported to die in a hospital [ 9 , 10 ], but our numbers are even higher. In addition, hospital admissions during the final months of life exceeded those reported in cancer [ 12 ]. The most probable reason for this result is the lack of advance care plans and limited use of palliative care services and home hospice care for non-malignant diseases.

Advanced care planning and arrangement of palliative home care have shown to reduce emergency room visits, hospital stays and dying in hospital setting among patients with multiple end-stage diseases [ 14 ].

Similar benefits could be achieved by early-integrated palliative care in IPF. To our knowledge, this is the first study to describe EOL treatment practices in a registry-based population of IPF-patients. We found procedures intended to prolong life e. This dual approach to a dying IPF-patient was probably due to the difficulty to differentiate exacerbation, secondary infection and a dying patient, but — again - also due to a late EOL decision.

In the present population one third of the patients received NIV treatment during the last week of life. NIV may relieve dyspnea as a palliative treatment, but in acute care, it is mostly used to improve survival in chronic obstructive pulmonary disease exacerbation [ 15 — 17 ]. Although it is understandable that NIV is used in a hope for cure or in order to relieve breathlessness, using a mask may increase and prolong suffering of the patient and prevent communication with closest ones.

A significant number of radiologic and laboratory tests were ordered during the last 24 h of life and antibiotics were commonly prescribed near death. Death related to IPF is typically respiratory failure related to either progression of the disease or acute exacerbation. The clinical picture of acute exacerbation is not easily distinguished from bacterial pneumonia elevation of c-reactive protein and pulmonary infiltrates [ 19 , 20 ].

Thus, treatment attempts with bacterial antibiotics found in our study are understandable and frequent antibiotic use is also common in patients with COPD and lung cancer during the final days of life [ 21 ]. However, the benefit of antimicrobial therapies and ordering multiple diagnostic tests should be reconsidered if the presumable prognosis of the patient is very poor e.

Although these factors are not necessary indicators of poor EOL care, they reflect life-prolonging nature of the treatment close to death. In a recent study by Ahmadi et al. The difference in the incidences of these symptoms may be due the retrospective nature of our study. The point of gravity in clinical patient handling may not be in the symptom-reporting, if compared to ie. Standardized symptom scores were not —unfortunately — a part of the evaluation of our patients.

This is an important issue that should maybe be addressed in future guidelines on patient follow-up. As in other advanced lung diseases, breathlessness is obviously the main symptom in IPF. In contrast, the cause and nature of pain in IPF is unknown. This is beyond the scope of our study, but should be evaluated in future studies. We suggest that cough was either not very severe symptom in dying IPF-patients or health care professionals did not record the symptom.

In the present study, opioids were more frequently used than in a previous study [ 13 ]. No controlled trials support the use of opioids for shortness of breath in IPF, but there is relatively good evidence about their benefit in refractory dyspnea in general [ 18 , 22 , 23 ]. Therefore, the common use of opioids probably reflects a great need to control breathlessness in the dying IPF-patient.

In addition, pain relief could be another reason for prescribing opioids, since nearly one-third of our patients suffered from pain. No one is certain how many people are affected by PF. Research estimates that idiopathic pulmonary fibrosis IPF , which is just one of more than types, affects one out of adults over the age of 70 in the United States.

That translates to more than , people living with IPF today. Approximately 50, new cases are diagnosed annually, and as many as 40, Americans die from IPF each year. There are many factors that make this disease difficult for both patients and providers. Not only is there a protracted time to diagnosis and sometimes misdiagnosis , but patients also experience debilitating symptoms. Seeking out expertise where it exists is critically important to the earlier diagnosis and management of this patient population.

The plus different lung conditions that qualify as PF all look very much alike. When you have a process that leads to scarring or inflammation of the lung, over time, the scar tissue can destroy the normal lung, making it difficult for oxygen to pass easily into the bloodstream. The lungs become stiff, making it challenging for patients to take a deep breath. Some known causes of PF are aging those over the age of 60 , cigarette smoking both current and past smokers and genetics.

We also know that as part of the systemic disease process, patients can develop PF alongside an autoimmune condition like rheumatoid arthritis or scleroderma. There are also environmental causes, such as exposure to mold or animal proteins especially from indoor or caged birds , which lead to a disease called hypersensitivity pneumonitis HP. Furthermore, the total numbers of deaths due to pulmonary fibrosis PF according to the ICD code J84 were investigated in Finland between the years — from the national registry database of Statistics Finland and this data was compared to that from the local area served by KUH.

Clinical, radiological and histological information of each patient was transferred from medical records to special forms designed for the present study. PF with a known etiology i. The data concerning causes of death and places of death was updated on 14th December Smoking history was assessed such that the individual was defined as a non-smoker, ex-smoker or current smoker.

Information was collated from histological samples from surgical lung biopsies or autopsy. Death causes were obtained from the death certificates using ICD codes and registered as underlying and immediate causes of death. Places of death were also registered. The overall death rates due to PF according to ICD code J84 and ischemic heart diseases ICD codes I21—25 were obtained from the open national database of Statistics Finland, in which the ICD codes are available only at a 3-character level, and therefore data with more specific codes, such as J In Finland, the disease classification of the death certificates was altered in the years — Subsequently, pneumonia was no longer accepted as the underlying cause of death if some other chronic disease had weakened the patient prior to the development of pneumonia.

If computed tomography was not available, the designation of suspected acute exacerbation of IPF was used. No consents for the inclusion into this study were collected since it was retrospective, with the majority of the patients already deceased. This study was conducted in compliance with the Declaration of Helsinki. All the patients whose re-analyses were categorized as not definite UIP in HRCT or histology were evaluated during a multidisciplinary discussion with a radiologist, a pathologist and a pulmonologist before inclusion into the present study.

Those patients that were still alive and with a follow-up time less than 5 years were not included into the categorization. Group differences were tested by Chi-square testing or Fisher exact test with categorical variables.

Data is presented as mean with standard deviation for continuous variables or frequencies with percentages when variables are categorical.

The survival was estimated by the Kaplan-Meier method using the time of diagnosis as the starting-point and death or lung transplantation as the end-point.

The median survival time is presented. After re-analyses of radiological, histological and clinical data from a total of patients, 89 cases were excluded due to diagnoses other than IPF, i. Four patients had missing data of their smoking histories. Out of patients, 45 A total of deaths had occurred by 14th December Three male patients, two current smokers and one ex-smoker, had undergone lung transplantation; one of them was a current smoker and had subsequently died.

We calculated the following mean ages; the time of diagnosis, At the time of diagnosis, mean FVC was The median survival was Fifty In the KUH district, which was serving , citizens at the end of year , the 5 year overall mortality rate between the years — was 2.

There were PF deaths in the year in Finland; from these values, one can calculate a mortality rate of 5. Underlying cause of death according to J84 ICD, 3-character level : The data of this time series is otherwise comparable, but the classification of pneumonia was altered in and From that time onwards, an international guideline was adopted. Accordingly, pneumonia is not accepted as the underlying cause of death if a chronic disease which has weakened the individual, is mentioned in the death certificate.

The 5 year mortality rates in KUH district between years — were 0. The main underlying causes of death were IPF, ischemic heart disease and lung cancer. The most common immediate causes of death were IPF, pneumonia and ischemic heart disease Table 2. An acute exacerbation was observed in 10 An acute exacerbation was observed in 7 Sixty Compared to males, a higher percentage of females died from IPF.

In contrast, more males than females had pneumonia as the immediate cause of death Table 2. No statistically significant differences were observed in the causes of death between ex-smokers and current smokers Table 3. A multivariate analysis was performed to investigate whether differences in deaths between genders could potentially be explained by smoking status or vice versa. It was found that both gender and smoking status were independent predictors of mortality; for males, the HR was 1.

More patients with a rapid disease progression died from an acute exacerbation compared to their counterparts with a moderate and slow disease progression Table 4.

No statistically significant differences were detected in the causes of death in the different GAP stages Table 5. As far as we are aware, this is the first detailed clarification of the causes of death in patients with IPF taking into account differences between genders and smoking histories as well as disease progression.

Even in this relatively small patient cohort, differences in the causes of death were detected between females and males. Moreover, by examining the smoking histories, we observed that more males and ex-smokers as well as more current smokers died of triggered exacerbations of IPF.

Furthermore, more patients with a rapid disease progression died from an acute exacerbation compared to the other disease progression subtypes. Depending on the methods and diagnostic codes used, the mortality rates have been shown to vary globally and in different countries in the EU [ 9 , 12 ].

Data from death certificates using ICD codes J84 mortality in have been reported to be 4. Thank you! You will now receive email updates from the American Lung Association. Select your location to view local American Lung Association events and news near you. Our service is free and we are here to help you. Section Menu. About the Disease Why did I get pulmonary fibrosis? If you are diagnosed with idiopathic pulmonary fibrosis IPF , physicians do not know what caused you to get the disease.

Doctors try and rule out risk factors like hazardous chemicals in the workplace, exposure to certain medication, medical procedures and family history. There are also people who have other diseases such as rheumatoid arthritis or lupus that can lead to pulmonary fibrosis.

Learn more. Is there a difference between pulmonary fibrosis and idiopathic pulmonary fibrosis IPF? The other distinction is treatment. How is pulmonary fibrosis treated? There is no cure for pulmonary fibrosis. People with IPF may benefit from a drug that slows the progression of the disease. Oxygen therapy and pulmonary rehabilitation are key components of maintaining a good quality of life with PF.

Some patients will be candidates for single or double lung transplants. Does pulmonary fibrosis spread? Is pulmonary fibrosis contagious? PF progresses. It gets worse with time but the length of time it takes to progress varies from patient to patient.

The disease is usually just in the lungs but if it is the result of other diseases, the fibrosis scarring can be other places like in the joints.

Pulmonary fibrosis is not contagious. If someone with PF is wearing a mask, it is because they want to avoid exposures that could make them sick or trigger a coughing fit. Is pulmonary fibrosis hereditary? Should siblings and children be evaluated? PF can run in families. There is currently no genetic test that can show who is a carrier of the disease or who is at risk of developing the disease if there is a known family history. If there is a family history of PF, everyone in the family should tell their doctor about it and keep an eye out for PF symptoms.

What do the breathing test pulmonary or lung function tests numbers mean?