There were 16 participants with 13 males (81.3%) and 3 females (18.7%). Participants’ age ranged from 35 to 72 years old with mean age 59 years). Diagnosis journey (from initial symptoms to confirmed IPF diagnosis) was between 1.5-5 years with an average of 2.9 years. Around one third of participants had high school education, followed by primary and secondary schools. Only 3 participants had university education. Table 1 shows the characteristics of participants with IPF.
Table 1
Characteristics of participants with IPF
No. | Gender | Age | Education level* | Occupation | Long-term residence | City/Countryside | Length from initial symptom to diagnoses |
1 | M | 56 | Primary school | Farmer | Hebei Province | Countryside | 3 years |
2 | M | 65 | University level | Teacher | Inner Mongolia Autonomous Region | City | 2 years |
3 | M | 63 | High school | Worker | Anhui Province | Countryside | 3.5 years |
4 | M | 58 | Primary school | Farmer | Inner Mongolia Autonomous Region | Countryside | 3 years |
5 | M | 35 | University level | White collar | Beijing | City | 1.5 years |
6 | M | 55 | High school | White collar | Inner Mongolia Autonomous Region | City | 1.5 years |
7 | M | 59 | Primary school | Farmer | Beijing | Countryside | 3 years |
8 | F | 66 | High school | Officer | Heilongjiang Province | City | 2.5 years |
9 | M | 72 | Secondary school | Worker | Beijing | City | 2.5 years |
10 | F | 70 | Primary school | Farmer | Hebei Province | Countryside | 5 years |
11 | M | 52 | High school | Teacher | Inner Mongolia Autonomous Region | Countryside | 5 years |
12 | M | 54 | Secondary school | Worker | Heilongjiang Province | City | 3.5 years |
13 | M | 67 | Secondary school | Worker | Liaoning Province | Countryside | 2 years |
14 | M | 56 | High school | Officer | Hebei Province | City | 2.5 years |
15 | F | 62 | Secondary school | Worker | Beijing | Countryside | 3 years |
16 | M | 58 | University level | Officer | Liaoning Province | City | 2.5 years |
*Primary school: 1–6 education years; Secondary school: 7–9 education years; High school: 10–12 education years; University level: >12 education years |
Thematic analysis of the participant narratives uncovered four themes representing the lived experiences of patients with IPF. These included: the long and confusing journey to reach diagnosis, living with the disease, understanding the disease and treatment, and desire for continuity of care. Table 2 shows the themes and associated sub-themes.
Table 2
Themes and associated sub-themes
Themes | Sub-themes |
The long and confusing journal to reach diagnosis | • Uncertainty of diagnosis • Delaying process |
Living with the disease | • Living with physical symptoms • Living with emotional distress • Loss of independence • Uncertainty with the prognosis |
Understanding the disease and treatment | • Questioning the cause of the disease • Concerning the side effects of treatments |
Desire for continuity of care | • Lacking continuity of care • Wanting better quality of healthcare in community hospitals |
The long and confusing journey to reach diagnosis
Participants described their long journey to get a correct diagnosis, including uncertainty of diagnosis and delaying process. Common initial symptoms experienced by participants were shortness of breath and cough. While some participants did not take it seriously, others looked for medical advice from their local community hospitals.
Majority of the participants (n = 14, 87.5%) were initially misdiagnosed as having other types of respiratory diseases, such as asthma, Chronic Obstructive Pulmonary Disease (COPD) or pneumonia and were consequently given symptom relieving medication, such as cough suppressants, inhalations and oral antibiotics. For those with cardiac disease, they seek for treatment with a cardiologist in a specialized hospital, as they attributed exertional dyspnea to cardiac dysfunction. Most did not look for further medical advice until the condition deteriorated and significantly affected their daily living activities. As one participant stated:
“I was just short of breath and I coughed a lot. I went to the hospital, had a chest X-ray. The doctors said that I had chronic obstructive pulmonary disease. Then I went to another hospital and I was told it was pneumonia. Anyway … different diagnosis. Finally, I came to this hospital [specialized hospital in respiratory medicine] and was diagnosed with [IPF]” (P8)
There was 3 to 4 years delay of confirmation of IPF diagnosis for many participants. Two participants from the countryside had more than 5 years delay. They looked for further medical advice at several hospitals. Some participants underwent multiple and repeated diagnostic tests and procedures, such as blood, X-ray and spirometry tests before reaching IPF diagnosis. As participant two described:
“It was nearly 5 years. [I] did all the examinations, chest function, blood sample, X rays. I went [to]nearly four to five hospitals to get the final diagnosis. Every time the prescribed medicines appeared to be working for a while, so it was delayed over and over again.” (P2)
Living with the disease
Participants described their lived experiences with the disease as: i) living with physical symptoms; ii) living with emotional distress; iii) loss of independence; and iv) uncertainty with the prognosis.
Most participants stated that the severity of the physical symptoms was associated with the progression of the disease. Breathlessness and persistent cough were the common symptoms, which significantly affected the daily activities. They also experienced the symptoms of fatigue, sleep disturbance and chest pain. As participant six stated:
“… shortness of breath. [It is] like something was strangling my throat. Sometimes, I felt like choking, especially during physical activities. I cough all night, and my chest hurts. I can't even sleep. I always feel very tired with energy.” (P6)
Participants also experienced emotional distress i.e. feeling anxious, frustrated and depressed due to the long disease journey, physical symptoms and limitations to daily activities. The distress severity can be affected by the disease progression. Most participants often expressed feelings of guilt because they needed help with regards to basic activities from their families on the daily basis.
"Now even go to the toilet, I have to depend on [family members]. I cannot move freely. My daughter had to quit her job to take care of me. My wife was not in good health either. She has diabetes, but she takes care of me every day [and also] does housework. I feel that I am a burden to my family. I feel very frustrated." (P8)
Participants also expressed how the disease shaped their ordinary life and social function. As one participant stated,
"I used to climb the mountain and ride a bicycle. It was fine. I can't do it now (sigh)." (P4)
In addition, uncertainty with the prognosis emerged from the interview data. Many participants expressed a strong feeling of uncertainty regarding the prognosis of the disease and its impact on their life. They often wondered if they could go back to their normal lives, especially young participants who were concerned about their careers. As stated by a 35 years old male participant,
"Can this disease be cured? What will life be for me in the future? Will I have to depend on the oxygen for the rest of my life" (P5)
Understanding the disease and treatment
Most participants stated that they had “never heard of ILD or IPF” and that they had insufficient knowledge regarding the cause, prognosis, and treatments of the disease. They often questioned the cause of the disease. As these quotes illustrated,
“I always have the doubt why I get this pulmonary disease. I never smoked, and I have a very healthy lifestyle. I exercise regularly. I want doctors and nurses [to] tell me [more about] this disease, [helping] me [to] figure [it] out.” (P5)
“I don’t know if it is associated with the haze/smoke [in our environment]…I usually do not wear a mask when I go out. I regretted it. If I wore a mask, it may reduce the smoke inhaling. Maybe, I would not get the disease.” (P8)
In addition, immunosuppressant and ant-fibrotic drugs are commonly used for treating patients with IPF. Participants often expressed their concerns regarding the side effects, as they did not know much about the medications used for the IPF treatment. As one participant stated,
"I have taken immunosuppressant for a long time. I am worried about side effects, be addicted and cannot stop." (P4)
Desire for continuity of care
Participants described their experience in care needs from health professionals as: i) lacking continuity of care; and ii) wanting better quality of healthcare in community hospitals.
In the interviews, participants expressed their desire for the continuity of care post discharge from the specialized hospitals. They hoped that health professionals in the specialized hospital could provide more knowledge, especially on how to self-manage the disease at home. They wanted specific instructions or education about diet, oxygen use, medication, and daily activities. However, there was no support after being discharged home. As one participant stated,
“When I need professionals to help in my hometown, I don’t know who I can ask. Obviously, I don’t think community hospital staff know how to treat such uncommon disease.” (P11)
Participants often had to attend the follow up appointments at the local primary care setting or community hospitals that are closer to home and are more accessible than the specialized hospitals, which often require them to travel long distance. They reported having low confidence on the healthcare quality in the community hospitals.
"Doctors in community hospitals only prescribed some medications and drips. You really can't trust them. Pulmonary rehabilitation program (during my stay in the specialized hospital) made me feel better to breath, but I don’t think the doctors and nurses in the community hospitals are properly trained for this [pulmonary rehabilitation].” (P5)