At some point, many Idiopathic Pulmonary Fibrosis (IPF) patients will consider getting a lung transplant. As we all know, there are currently no therapies to cure pulmonary fibrosis, and the medications presently available can at best, halt or slow the progression of the disease. They do not reverse fibrosis. But imagine if we could have a new set of lungs. Imagine a chance to start over and be free from this dreadful disease, from the constant shortness of breath (SOB), to be able to breathe again and ready to conquer the world again? The very idea sounds too wonderful to be true. 

My name is René and in late 2017, when I was 75 years old, I was diagnosed with mild IPF. Over the next three years my condition continued to worsen, especially my shortness of breath. So, I decided to explore the option of getting a lung transplant. I contacted four well-known lung transplant centers and as requested, sent each of them my personal medical records including medications, surgeries, CT scans, and any other pertinent medical information. Once each confirmed receipt of my medical records, interviews were scheduled. 

With each center I had at least a one-hour Zoom interview with the surgical team and/or the actual thoracic surgeon, during which we discussed the pros and cons of the surgery. In all four interviews, it became clear that my relatively advanced age could be a limiting factor – and for that reason, two of them rejected me. Decision-makers at one center recommended against the transplant but were willing to proceed with the evaluation. Only one out of the four centers invited me to the complete their evaluation. 

Several factors eventually made me decide against proceeding with the transplant. These included a higher mortality rate (age is the most significant risk factor for lung transplant survival), a more difficult and lengthy recovery, and the knowledge that my immune system would be suppressed by the anti-rejection drugs. This, in turn, would increase the risk of recurring skin cancer and/or other types of lung diseases. Another factor that weighed in my decision not to proceed was that my home is in the Dutch Caribbean Island of Bonaire, and I would have been required to travel from there to the US hospital many times. All in all, a lung transplant just did not seem like a viable option for me. But for you, it might be a completely different story – so please keep reading. 

Your IPF Diagnosis

Idiopathic pulmonary fibrosis is a long-term disease in which scars develop on the lungs. This causes lung tissue to thicken, become stiff, and lose the elasticity of healthy lung tissue, which makes it hard for the patient to breathe. In fact, one of the first warning signs for someone with IPF is shortness of breath (SOB).

Initially, shortness of breath may be attributed to being out of shape, overweight, or simply getting older. Those who are physically fit may notice excessive SOB after exercising strenuously and may assume they have pushed themselves too hard. But over time, if we notice that the SOB has gotten worse, and walking up a staircase or up hilly terrain becomes difficult or impossible without stopping to rest, we instinctively know that something serious is going on and can no longer ignore the symptoms or chalk it up to “getting old.” We eventually visit our family doctor, are referred to a pulmonologist, and are diagnosed with idiopathic pulmonary fibrosis – unwelcome news indeed. 

Once the initial shock of the diagnosis wears off, it’s natural for the patient to start rattling off questions for the doctor, such as, “well, what can you do for me? How can we treat this? What’s our next step?” And there is nothing more frustrating and alarming than hearing the pulmonologist say: “There is no cure for IPF. I can prescribe medications, and they may help with your symptoms … but at best, they will slow the progression of the disease. Medications will not reverse the scarring in your lungs, and they may not stop the disease from getting worse.”

As if that bombshell wasn’t bad enough, you also learn that some patients have unbearable side effects from the medications. These may include nausea, vomiting, diarrhea, acid reflux, fatigue, loss of appetite, headache, dizziness, and skin problems. It isn’t uncommon for IPF patients who experience some or all these side effects to stop using their medications. Quality of life becomes a major factor in such decisions.

So, you learned that you have IPF. You learned that there is no cure. You learned that you’re going to get worse, even if you take medications because all they can do is slow down the scarring in your lungs. At this point, the pulmonologist may present you with the option of a lung transplant.

Exploring the Option of Lung Transplantation

Organ transplants can absolutely be considered marvels of modern medicine. They give people a new lease on life who might otherwise have no hope at all – yet these extraordinary lifesaving surgical techniques did not exist until the mid-twentieth century.

A Brief History

According to the United Network for Organ Sharing (UNOS), the first human organ transplant, took place in 1954. Twenty-three-year-old Richard Herrick was dying of kidney disease and was told there was nothing anyone could do for him. A young Harvard Medical School professor and surgeon named Dr. Joseph Murray did not agree with that. He believed it was possible to transplant a kidney from a healthy donor into the body of someone who needed it. 

Called a “fool” for his belief in the possibilities of organ transplants, Dr. Murray took a healthy kidney from Herrick’s twin brother and transplanted it into Herrick, saving the young man’s life in the process. By the 1960s, liver, heart, and pancreas transplants were successfully being performed on a regular basis. 

The first successful lung transplant was performed in 1971 by Dr. Fritz Derom in Belgium. The patient, a man in his twenties who worked as a sandblaster, had developed a type of pulmonary fibrosis known as end-stage silicosis. After the lung transplant, the man lived for 10 1/2 months – which was profound compared with previous attempts that resulted in patients surviving less than a month. By 1983, 40 successful lung transplants had been performed, and this number continued to increase over the following decades. Today, an estimated 2,000 lung transplants are performed in the United States each year. 

A Serious Decision

It is important to talk to your doctor about the possibility of a transplant – and if your doctor doesn’t mention it, don’t hesitate to bring it up yourself. A lung transplant is a major and highly complex medical procedure, so it’s essential for you to be well informed about every aspect of the surgery. It is not without risk, and recuperation can be difficult and slow. 

Another important consideration is finances. A lung transplant, along with the necessary after-care, is very expensive, so make sure you know what your insurance provider will and won’t pay for so that you are aware of all costs involved and can make sure you have your finances are in order. Also, besides the procedure itself, there will be major pre- and post-operational costs. 

Before making your final decision about a transplant, ask yourself this question: will the risk of having a transplant exceed the expected benefits and life expectancy post-transplant – and answer the question truthfully. In other words, weigh your expected quality of life both with and without the operation. Make every effort to explore all aspects of the procedure and the process so you can make the best possible decision – for you.

Arm Yourself with Information

Once you’ve done your homework, found everything you could about lung transplants, read so much material that your eyes have started to cross, have weighed the pros and cons, understand the risks, and are ready to go ahead with the surgery … what now? The first thing to know is that the window during which a transplant can take place is crucial. While the actual transplant is designed to address late-stage lung disease, early hospital selection and evaluation is important, so all preliminary testing is done when the patient is still relatively healthy. 

Start by gathering information about lung transplant centers. An excellent source is the Scientific Registry of Transplant Recipients website (www.srtr.org), which provides a wealth of information about US transplant centers, including location, program overview, transplant rates, time to transplant, transplant outcomes, and a great deal more. Other countries may have similar websites. 

You’ll find contact information and links to each of the transplant centers so you can read about those of your choosing. Besides distance from home, you may want to consult with several lung transplant centers to determine which one suits you best and where you feel most comfortable, considering the proficiency of the medical staff, survival rates and other factors that are most important to you. 

As mentioned, one extremely important consideration when choosing a transplant center is location. A lung transplant and recuperation will be a long process with numerous hospital visits, so the center’s proximity to home is an important consideration. Keep in mind that once you are on the transplant list, you must be able to be at the hospital within a very specific amount of time when called, depending on the hospital. And you may get “the Call” at any time of the day or night, so you and your caregiver must be ready to leave at a moment’s notice. Also, your transplant team will be your lifelong partner for post-operative follow up, making the distance from your home an even more important factor.

What to Consider Before Your Transplant

To be listed on the hospital’s transplant list and qualify for the surgery, your disease stage must be relatively advanced. This is because the demand for healthy lungs far surpasses the supply. So those who need it most will tend to get preference (although this often varies from hospital to hospital). 

Most hospitals will not perform transplants on a patient who is over 70 years old. Some, however, will go up to age 80 depending on the patient’s physical and mental condition. Remember that if a transplant center rejects you can try another one or two more (as was my experience). Different hospitals have different requirements, so if one rejects you as a patient, another may not. 

The hospital will request copies of all your medical information and history of previous operations or health problems, pulmonary function tests (PFTs), and six-minute walk tests. Other information gathered will include your age, height and weight, blood analyses, x-rays and high-resolution CT scans of your thorax, history of IPF diagnosis, biopsies, use of oxygen and oxygen saturation (at rest and when exercising), your exercise program and tolerance, current medications, your physical and mental condition, and lifestyle, and who your caregivers will be. 

After all of these (and possibly other) criteria are reviewed and you’re deemed a good candidate, the hospital will set a date for you to be evaluated at the transplant center.