Navigating End-Stage Pulmonary Fibrosis: Options and Care for Your Loved One

Q: Navigating End-Stage Pulmonary Fibrosis: Options and Care for Your Loved One

Hello, Yezi： I can understand your attentiveness and filial piety from your detailed description. <br>Here are simple answers to your questions： (1) Severe pulmonary fibrosis significantly lowers the chances of successfully weaning off the ventilator. <br>Even if the first attemp

Could you please provide more details about the issue of pulmonary fibrosis?

My grandmother is currently 78 years old and resides in Chiayi.
Two weeks ago, she went to the hospital's emergency department due to shortness of breath.
Since the emergency room did not have suitable equipment, she was admitted to the intensive care unit (ICU) and intubated.
The hospital conducted the following diagnostic procedures: chest X-ray and pulmonary function assessment.
Due to her advanced age and the risk of further complications, a biopsy was not performed to determine the severity of fibrosis, and it was found that her lungs are nearly completely fibrotic.
For the past 15 days, she has remained intubated.
Last week, after observing her condition for about nine days in the ICU, the hospital planned to extubate her in a couple of days to allow her to practice breathing gradually.
However, unexpectedly, before the extubation could begin, she faced a rapid drop in blood pressure.
While awake, she exhibited rapid breathing, which raised concerns about potential respiratory distress and rejection of the ventilator.
To stabilize her blood pressure and facilitate smoother breathing, the hospital began administering large doses of sedatives (12 days after her ICU admission) to induce sleep.
The hospital also recommended a tracheostomy.
The purpose of the tracheostomy would not change the fibrotic condition of her lungs but would allow her to maintain life under long-term care.
Furthermore, it is possible that even with a tracheostomy, the ventilator may not be removed.
The physician indicated that the tube may be obstructed with mucus, making removal difficult even with a tracheostomy.
Below are some of my grandmother's medical data: the oxygen supply from the ventilator was at 45% a few days ago, 50% yesterday, and 55% today (Day 15).
Regarding her nutrition, due to existing malnutrition issues, she is currently receiving milk through IV.
Yesterday (Day 14), she received 10 drops in the morning, and by the afternoon, her digestive condition improved slightly to 30 drops.
Today, she progressed to 50 drops in the morning.
Her kidney function before sedative administration was 1.7, and two days ago it was 3.3 (generally, a level above 6 would suggest the need for dialysis).
The hospital has not yet assessed her liver function.
Currently, the hospital recommends a tracheostomy; otherwise, she may need to be transferred to a palliative care unit.
As family members, we feel helpless and hope to hear more professional opinions.

My questions are: What should we do now for the best interest of my grandmother? Are there any medical interventions that could improve her condition? What steps can we take to alleviate her suffering?

Ye Zi, 30~39 year old female. Ask Date: 2008/12/15

Dr. Yang Wenda reply Pulmonology

Hello, Yezi: I can understand your attentiveness and filial piety from your detailed description.
Here are simple answers to your questions: (1) Severe pulmonary fibrosis significantly lowers the chances of successfully weaning off the ventilator.
Even if the first attempt is successful, the likelihood of re-intubation (due to pneumonia or mucus plugging) remains high.
It is advisable to continue using the ventilator temporarily to address respiratory distress.
(2) Regarding the tracheostomy, it is not very painful; it is a minor procedure, and the cosmetic aspect is not important.
The focus should be on the patient's comfort.
If the physician recommends a tracheostomy due to difficulty in suctioning mucus (as the endotracheal tube is longer), it can be considered to facilitate mucus clearance.
This would reduce the discomfort caused by facial restraints, allowing for daily cleaning of the face for comfort.
As long as the patient can have cleaner mucus suctioned, with reduced respiratory resistance, and potentially improved condition due to antibiotic use, weaning off the ventilator via the tracheostomy tube is generally easier than with the endotracheal tube.
Wishing your grandmother a speedy recovery!

Reply Date: 2008/12/15

More Info

Navigating end-stage pulmonary fibrosis, especially in a loved one, can be an incredibly challenging and emotional journey. Your grandmother's situation, as you've described, involves complex medical decisions that require careful consideration of her quality of life, potential outcomes, and the available treatment options.

Understanding the Condition
Pulmonary fibrosis is a progressive lung disease characterized by the scarring (fibrosis) of lung tissue, which leads to a decline in lung function. In advanced stages, such as what your grandmother is experiencing, the lungs may become severely compromised, making it difficult for the patient to breathe without assistance. The fact that she has been on a ventilator for an extended period indicates that her respiratory function is significantly impaired.

Treatment Options
1. Tracheostomy (气切): The hospital has suggested a tracheostomy, which involves creating an opening in the neck to place a tube directly into the windpipe. This can facilitate easier breathing and allow for better management of secretions, which seems to be a concern in your grandmother's case. While a tracheostomy does not cure pulmonary fibrosis, it can provide a means of long-term respiratory support. It may also improve her comfort level by reducing the need for sedation and allowing for more effective airway clearance.

2. Palliative Care: If the decision is made to transition to palliative care or hospice, the focus would shift from curative treatment to comfort measures. This approach emphasizes managing symptoms, such as pain and breathlessness, and providing emotional and psychological support for both the patient and the family. Palliative care can be provided alongside curative treatments, but in end-stage situations, it often becomes the primary focus.

3. Nutritional Support: Given that your grandmother is experiencing nutritional deficiencies, it is crucial to ensure she receives adequate nutrition, either through enteral feeding (like the milk you mentioned) or intravenous nutrition if necessary. Proper nutrition can help support her overall health and potentially improve her ability to tolerate treatments.

4. Oxygen Therapy: Continuing oxygen therapy is essential, especially if her oxygen saturation levels are low. Adjusting the oxygen flow to maintain adequate levels can help alleviate some of her respiratory distress.

Decision-Making Considerations
- Quality of Life: It’s essential to consider your grandmother's quality of life. If her condition is such that she is unlikely to regain independence or experience a meaningful recovery, it may be worth discussing with her healthcare team the potential benefits and burdens of continuing aggressive treatment versus transitioning to comfort-focused care.

- Family Discussions: Engage in open discussions with family members about your grandmother's wishes, values, and what she would want in this situation. If possible, involve her in these discussions if she is able to communicate her preferences.

- Consultation with Specialists: It may be beneficial to consult with a palliative care specialist or a pulmonologist who can provide insights into the potential outcomes of a tracheostomy versus transitioning to hospice care. They can help clarify the goals of care and what to expect moving forward.

Steps to Reduce Suffering
1. Pain Management: Ensure that her pain and discomfort are managed effectively. This may involve medications that can help alleviate pain and anxiety.

2. Emotional Support: Consider involving a social worker or counselor who specializes in end-of-life care. They can provide support to both your grandmother and your family during this difficult time.

3. Spiritual Care: If your family has spiritual or religious beliefs, involving a chaplain or spiritual advisor can provide additional comfort and support.

4. Regular Communication with Healthcare Providers: Maintain open lines of communication with her healthcare team to stay informed about her condition and any changes in her care plan.

Conclusion
Ultimately, the best course of action for your grandmother will depend on her specific medical condition, her values, and her family's wishes. It is a deeply personal decision that should be made with compassion and care. Engaging with her healthcare team, considering her quality of life, and ensuring that she is comfortable should guide your decisions moving forward.

Similar Q&A

Understanding Pulmonary Fibrosis: Symptoms, Concerns, and Care Options

Dear Dr. Zhou, I am writing to seek your guidance regarding my mother, who is nearing her fifties and is over seventy-five years old. Three years ago, she was diagnosed with pulmonary fibrosis at a military hospital. Since then, her condition has gradually worsened, and she has ...

Dr. Zhou Ziguang reply Pulmonology
Hello: Pulmonary fibrosis is a progressive disease characterized by the scarring of lung tissue, with many causes remaining unknown. Some cases are triggered by specific medications (such as certain antiarrhythmic drugs). Symptoms include shortness of breath, indicating declining...

Understanding Pulmonary Fibrosis: Causes, Treatments, and Transplant Options

My father sought medical attention in June of this year due to persistent coughing. After undergoing a chest X-ray and biopsy, he was diagnosed with pulmonary fibrosis. Initially, he was able to breathe independently, but gradually he required supplemental oxygen. Currently, he i...

Dr. Jiang Zhenyuan reply Pulmonology
Mr. Li: Pulmonary fibrosis is often caused by changes resulting from the treatment of other diseases, such as tuberculosis. Some patients may develop pulmonary fibrosis without any obvious contributing factors as they age. Since your father is experiencing respiratory failure and...

Managing Lung Fibrosis After Bone Marrow Transplant: Key Considerations

The patient is a male in his 30s who was diagnosed with leukemia last year. After chemotherapy, he fortunately found a matched bone marrow donor among his siblings and underwent a transplant. Despite taking immunosuppressive medications, he has experienced multiple episodes of re...

Dr. Yang Wenda reply Pulmonology
Hello, Xiao Zhen: Rejection causes pulmonary inflammation, which ultimately leads to fibrosis, leaving behind sequelae of impaired lung function. Only by using immunosuppressive medications or corticosteroids to reduce the inflammatory response can further deterioration of fibros...

Guidance for Managing End-Stage Nasopharyngeal Cancer at Home

The patient has nasopharyngeal carcinoma with liver and bone metastases, presenting with ulcerations on the arms and both sides, jaundice, abdominal swelling, and lower extremity edema. The patient has chosen to return home instead of being hospitalized, and the family feels lost...

Dr. Yang Youhua reply Oncology
Patients in the terminal stage of cancer face an inevitable and imminent death, so it is crucial to enhance their quality of life during their remaining time. Alleviating pain, maintaining personal hygiene, and ensuring a comfortable environment are all vital for the patient. Pal...