Idiopathic pulmonary fibrosis (IPF)

Even thought IPF patients have high scores on the waiting, this is still a deadly disease that needs to be taken seriously.
Those patients are not offered a fair chance at transplant because they are often referred to us at the end stage of the disease and their condition deteriorates quickly and disqualifies them for transplants.
One of the biggest issues is the lack of knowledge and misconceptions from physicians in the community. The truth is: patients with IPF should be referred as early as when they receive their diagnosis.

Why?

IPF has no cure other than a transplant, prognosis is generally poor.  The median survival after diagnostic is only 2-3 years. Quality of life is far from being perfect.  Patients are given a death sentence without any hope.

However, there is hope!

Reasons to start lung transplant evaluations sooner:

1- It allows the transplant team to begin a complete evaluation without rushing the patient and
caregivers.  Evaluation is 3-4 full days of testing and this can be very stressful if done in a row.

2-It allows plenty of time to address medical/surgical/social issues during work up.

3-It gives transplant centers more time to monitor the patient for compliance

Specific example: an IPF patient already on 4-6 L/min and needed a stent in his heart during work up heart cath.  Unfortunately, he could not be listed right away due to the high risk of bleeding and clogging the stent.

4- It gives more time for patients and caregivers to make arrangements for the post-transplant period.

5- Post lung transplant outcomes are better if the patient was listed and transplanted before getting too sick

IPF is very stressful for the body but transplants are even more stressful.   You other organs need to be a good health in order to make it.

If you are a lung transplant patient there are good chances you may eventually need an anti-reflux surgery. The name of that surgery is Nissen Fundoplication. For some lung transplant recipients this could be a

Lifesaving surgery in the long run because the purpose of it is to save the lung function. The University Of Pittsburgh School Of Medicine reports that out of 43 of their patients who have undergone this anti-reflux surgery, nearly all of them improved their lung function. Not only this surgery helps post-lung transplant but can also benefit patients waiting for a new organ.

Incidence

According to Blair Jobe, M.D., professor of surgery, Department of Cardiothoracic Surgery, Pitt School of Medicine, many end-stage lung disease patients, especially cystic fibrosis and pulmonary fibrosis patients, have what they call gastro-esophageal reflux disease (GERD). Almost half of those patients do not experience any typical heartburn symptom but still, the gastric juice coming up the esophagus and going in the lungs is damaging. It has also been recognized as causing acute lung rejection and ultimately leading to chronic rejection. Chronic rejection, medically known as Bronchiolitis Obliterans, is when the lung function slowly goes down with little chance for recovery. Patients can live many years once a diagnosis of chronic rejection has been established but with a lower quality of life. Gastric reflux is a medical problem that really needs to be addressed seriously by transplant pulmonologists.

No Heartburn? How is Gastric Reflux Diagnosed then?

Usually what happens is a patient will have an unexplained drop in lung function. A bronchoscopy will be done to rule any acute rejection, infection or narrowing of the airway. Narrowing of the airway is called bronchial stenosis. Once all of that is negative, gastric reflux is highly suspected and a 24 hours Ph probe study is ordered. This test consists of a small probe inserted into the nose going to the stomach. It stays there for 24 hours and it is attached to a small reader that the patient carries in a bag. To be noted that it is an outpatient testing and patients do go home with the probe.

Once the 24 hours has passed, a physician will interpret the data. The probe actually records the number of reflux episodes and monitors the Ph (acidity). It also records some pressure in the stomach. Sometimes you can have patients with 60 plus episodes of reflux non-acidic which means they can’t feel it since it won’t burn. With or without burning sensation, if there are many reflux episodes, the patient is referred to a general surgeon for a Nissen Fundoplication.

What Is a Fundoplication?

It is usually done laparoscopically, which means by using a small camera through small holes in the skin. The surgeon will wrap the gastric fundus (top part of the stomach) around the esophagus in order to reinforce the esophageal sphincter. The goal is to prevent the gastric content from going up and preserves the lungs for lung transplant patients. The surgery is done under anesthesia by a general surgeon and requires 4-5 days in the hospital for recovery. The main complication reported is a problem of bloating. It may be hard for patients to belch after that surgery so gas may accumulate in the stomach. After 10 years, 90% of the patients are symptoms free though.

 I can say, with my own personal experience without any scientific evidence, that lung transplants patients that had that surgery done were doing better after with improved lung function. The challenge sometimes is to explain the patients why they need the surgery when they are symptoms free.

What is a Bronchoscopy?

Patients that have received a lung transplant know too well what a bronchoscopy is.  For the ones on the waiting list who never had one; don’t worry you will get to know.  A bronchoscopy is usually done by a pulmonologist (lung doctor) and consist a small camera at the end of a long probe narrow enough to fit through the airway.  It can be inserted through the nose or mouth and also through the breathing tube is the patient is still on the breathing machine (ventilator). That camera is inserted all the way down in both lungs while the patient lightly sedated.  The patient usually receives what some hospitals called conscious sedation.  That basically means that you are awake enough to breath and gag but sleepy enough to think what’s happening is only a bad dream.

Indications after Lung Transplant

Diagnostic

There are mainly 2 reasons doctors will do bronchoscopies after lung transplant.  The first reason is for diagnostic purposes where the physician may or may not suspect something wrong but needs to find out. A bronchoscopy is done at scheduled intervals after the transplant to see if the body is rejecting the new lungs.A rejection is a sign that the body is not accepting the organ and is trying to get rid of it just like it was a bacteria. It needs immediate medical treatment once diagnosed. Biopsies will be taken from several different locations in the lungs to look for possible rejection. A biopsy consists of removing a tiny bit of lung tissue and to send it to the lab under the microscope. Usually the doctor will repeat that 6 to 8 times at each bronchoscopy depending on how the patient tolerate it and how much it bleeds. It takes about 24 hours to know if the lung biopsy showed a rejection or not.

Another diagnostic reason to do a bronch is when a patient shows some signs of lung infection and a culture needs to be performed.  By performing a culture, the pulmonologist will be able to identify the bug (bacteria, virus or fungi) that has invaded the new lung and treat it accordingly. Unfortunately, a lot of lung transplant patients are infected with “superbugs” that require powerful antibiotics because they have adapted and become resistant to the weaker ones like Cipro or Levaquin.

Therapeutic

The other purpose of a bronchoscopy has therapeutic intentions. Inother words it means to treat a condition found in the lungs where the patient is experiencing lung transplant complications.  The main ones that we see are accumulation of thick mucus that will sometimes occlude part of the lung.  The doctor will use the scope and go in and wash it out real good.  The real term used for the occlusion is a mucus plug.  Usually a patient feels 100% better once that issue is fixed.

A more rare complication after lung transplant is called bronchial stenosis.  Stenosis means narrowing.  So bronchial stenosis means that the bronchus has become narrow and it is more difficult for the air to go through.  That usually happens at the site where the surgeon hooked up together the new lung with the patients™ own bronchus.  The scar tissue that forms makes the air passage more narrow.  That condition is diagnosed during a bronchoscopy and can be treated by 2 ways that are pretty similar to what a cardiologist will do to clear blockages in a heart.  The first step is to use a balloon that is inflated at the narrow spot with the goal to open it up.  If it opens up and stays like that, it is good.  If it closes back up, then a stent may be deployed to keep it open.  Just like in your heart. The difference is it happens in the lung, not in the bloodstream.

These were the main reasons bronchoscopies are done on lung transplant patients.  The number one thing that is always suspected early on after transplant regardless if the patient has any symptoms is rejection.  This is why bronchoscopies are performed so routinely, to catch them early.

Yesterday, I posted the first 5 main mistakes transplant patients make. Today I will present the remaining 5 mistakes that we observe and could shorten your survival post-transplant.

6. Not exercising.

Every single patients (actually this is good for everybody, including me!) should enrolled in a structured program of physical conditioning, providing that their physician has cleared them to do so. A structured environment is much better since you will have someone pushing in your back to have you take the extra step and to show up… Self-exercising is OK for a minority of people because most of us lack motivation and would quit after 1 week.

7. Lacking knowledge about your medication

How often have I heard the doctor asking a patient the reason why he is taking something and the patient replies: I don’t know, a doctor told me to Better informed patients make healthier patients. Cmon guys, there is no excuse today to be uninformed with all the information we can find on Internet. At least know the purpose of each meds you are taking.

8. Eating wrong diet

It is OK to eat some fast food once in a while if you really like it but I would suggest that you cook your own food at home along with some raw vegetables.  Of course, the vegetables need to be washed real good.  It will help with your weight management along with your blood sugar.  As transplant patients, you are taking so many medications that you don’t need to eat all that processed food along with their chemicals. Check with your dietitian at your transplant hospital about tips and ideas.

9. Returning to bad habits

Before transplant, while being evaluated, most patients swear they will be taking care of themselves much better. Unfortunately, once they start feeling great after the surgery, some of them go back to their old habits that led them to require a transplant (obviously it is not all patients who need transplant that fall into that category, some have acquired diseases or were born with it). What I mean is by example: a long time smoker now needing a lung transplant, a fast food eater ending up needing a heart transplant because of too many heart attacks or a high salt intake person who ends up with severe hypertension which cause kidney failure requiring transplant.  Believe it or not, some patients restart smoking after lung transplant!  A new organ does not treat the causes in most cases, it just relieves symptoms. You have received a new chance in life, it is your turn to take care of it.

10. Be positive!

This is the correct attitude towards the success of your transplantation. If you feel you have some depression please check with your hospital to see if they offer a support group. Some patients feel some guilt regarding being alive while their donor is dead. It is important to also take care of the mind.

The first question that comes into people’s mind when they consider organ transplant is how long they will have to wait. There are so many factors affecting the wait time that it is very hard to say. For lung transplant, the main ones are type of disease, patient”s size and the transplant center itself. Also lung is the organ with the one of lowest wait time in organ transplant. Relatively less people are waiting for lungs versus the number of available organs compare with kidney or liver.

The way lungs are allocated is by a complicated calculation of different data from the patient. The number, or score, that the system gives after putting all the data in is called Lung Allocation Score (LAS). The patients are ranked by LAS score with the highest number being at the top. Those data are entered in the UNOS™ website by the transplant coordinator and they include the amount of oxygen used, some pressure in the heart, saturation, etc. The number one factor that affects the score is the disease. Some disease like idiopathic pulmonary fibrosis (IPF) will be giving more points than chronic pulmonary obstructive disease (COPD). Patients with IPF are clinically sicker than COPD and their condition can get worst as quickly as overnight. COPD is more about quality of life and is a more stable disease while many can live years without lung transplant.

Another very important factor in determining how long someone will have to wait is the size (height and weight) of the organ transplant candidate. A really small or very tall patient will most likely increase the wait time as few donors meet those dimensions.  Being too small I think is worst than too tall. A very short adult patient may need lungs from a child or small teen but they will have to compete with pediatric patients who have priority on pediatrics organs. It is very hard to transplant a very small adult but with a little luck it can happen.

One more very important factor that you can’t really find in the books anywhere is how aggressive the lung transplant program is in pursuing lungs that other centers would turn down. Some pulmonologists will turn down lungs that are less than perfect without trying to improve them. It is important to mention that the lungs are the most fragile organs from the donor. Good lungs can turn bad very quickly if the donor is not managed properly. The OPO coordinator has a very important job to do in maintaining the quality of the lungs or to improve them. Lungs are susceptible to fluid overload and infection for the most part.  Detailed attention is needed to keep the lungs fit for transplantation. Some transplant centers don’t bother in trying to salvage lungs. It is worth giving it a shot in a context of shortage of organs and it directly results in more lungs available for the more aggressive hospitals. It happened to me more than once after giving a first look at a lung offer from UNOS, I was certain we would decline due to bad quality. With a good physician giving the right orders about medication and making the perfect adjustment on the breathing machine, the lungs got much better and the patient received his lung transplant.

The average wait time for a busy and proactive transplant center is about 3 months spent on the list for lung transplant. Some will have their organ transplant as early as the same week they were put on the list while others will wait on the list for months if not years. It is very unpredictable but in general it will be faster than the other organs like heart, kidney and liver. Also there is some period of the year when we received very few organ offers and none of them are good and other time where it is the opposite. The number of organ offer is pretty much the only thing that we can’t control at the transplant center level. If all the stars are aligned for you, you can get your transplant rather quickly!

Several patients don’t want to be operated on at night for fear that more mistakes happen due to fatigue and stress. If you are a lung or heart transplant patient you don’t have to worry anymore. A study that came out in the Journal of the American Medical Association (AMA) is proving that there is no more risk at night than daytime for organ transplant surgery. It suggests the reason being that the transplant team is used to it and has found ways to cope with lack of sleep and the pressure of doing transplant surgery.

I have seen quite a few surgeons working at night and let me tell you when they are in the operating room, it does not matter what time it is, the time stop and they just perform. Trust me though, if they have to choose between 2 pm and 2am the surgeons would all pick 2pm for obvious reasons.

The study was done over 10 years involving more than 27,000 patients who underwent thoracic organ transplantation (heart or lungs) in the US. There was no difference in term of survival one year after transplant. The only hiccup for night time surgery was a slightly increased incidence of airway dehiscence. This is explained by having an air leak where the news lungs and patient’s bronchus are stapled together. It can happen because a staple failed, wound healing delay, etc and usually requires going back to the operating room.

The bottom line is when a thoracic surgeon signs up for organ transplant surgery; they expect to be working at anytime of the day or night. They are mentally prepared for it and just do what needs to be done.

Source: http://health.msn.com/health-topics/transplant-surgery-no-riskier-at-night-study

Lung transplant surgery can be a mean to improve quality of life for some but is certainly a life-saver procedure for others. It just depends on the disease of the patients (COPD, pulmonary fibrosis, cystic fibrosis, etc). When someone’s disease is so advanced that they can’t breathe, there is nothing to replace the lungs unlike dialysis for failing kidneys or a mechanical heart for a weak heart. Unfortunately, lung transplant comes with a relatively high risk of short term and long term complications compared with other organs.

The main reason behind that is the lung is the only organ exposed to the outside world. The liver and kidneys are deep inside the abdominal cavity, no worry there. The heart, despite being attached to the lungs, is not exposed like them. It is well protected inside the chest. Basically, what I mean is the lungs make contact with the air we breathe plus everything else found in the ambient air. It could be pollutants, irritants, mold spores or whatever else found in the air where you are at. This is why it is so critical for a lung transplant recipient to be aware of his surrounding and not be at a place that they should not be. Every dusty area or known to be dusty task (sanding, house cleaning, even gardening, etc) are usually prohibited or discouraged by your transplant team. When in doubt with an activity or an area, ask your lung transplant team. Listen to them; they know what to do to help you stay as healthy as possible!

Every single respiratory symptom reported by a patient is usually taken seriously by the transplant team. This is especially true during the winter when it is flu season. Any benign respiratory virus for the general population could potentially be real bad for lung transplant patients. The easiest way we can identify the kind of virus, if any, is to swab the patients nose. It is not the best experience for the patient but it can save a lot of trouble (if you don’t know what it is, its like sticking a Q-tips 2-3 inches in each nostrils and then swab…). Usually it makes the eyes teary!

We have caught some cases of influenza last winter that we were able to successfully treat. I am just afraid that a same patient will not call us in the future if they have the same symptoms because they are afraid of the nasal swab. Please don’t do that. That could be a mistake. You went through lung transplant surgery and its aftermath. A nasal swab should be a walk in the breeze¦

This was a very quick overview of lung transplant complications. The goal was to make you understand you are always at risk of breathing something potentially bad but by being informed you can be more careful without becoming paranoiac. Follow your lung transplant team advices; they are the expert to keep you out of trouble!