The Tragedy of Two Brothers and Pulmonary Fibrosis (PF)
This is a story that many people in the world who have Pulmonary Fibrosis (PF) may relate to and for those who have no idea what PF is. As Patients and Caregivers it’s our job to inform and educate others about this terrible disease in the same way that we are more aware of breast cancer, prostate cancer and ALS along with other well known diseases through their well known promotion activities and advertising.
This story is for both patients and caregivers on what to expect from the time this disease takes hold until the end, it may be hard for people to accept but it gives some insight about what we have to go through on a daily basis. Others who have this disease may have different experiences.
My brother Brian and I both were diagnosed with Pulmonary Fibrosis (PF) over the past four (4) years. Mine is PF secondary to Sjogren’s Syndrome and his is Idiopathic Pulmonary Fibrosis (IPF). This is our story of what we have gone through over these past four (4) plus years.
Back in 2009 I started to notice I didn’t have the breath to recover when playing hockey and thought I was out of shape and getting older. In mid-February of 2013 I developed a dry cough and shortness of breath. Whenever I talked to people I would cough while talking and couldn’t stop, it made work very difficult as my position involves talking to people in person and on the phone.
This lasted a couple of weeks and I finally went to see my doctor. When he listened to my breathing he said it sounded like “crackles or like Velcro being pulled apart” and said it might be a touch of pneumonia and sent me for an X-Ray. The X-Ray showed some white patches in my lower lungs which the Radiologist said looked like pneumonia. After a few weeks on the antibiotics and Ventolin I was still coughing and went back to my doctor and he checked and said “You’re going to see a Respiratory Specialist.”
A few weeks passed and I was still coughing, though not as bad, and saw my doctor again and after listen to my breathing he said “You’re getting a CT Scan.” This is now three (3) months since I started to cough. On July 22, 2013 I saw the Respiratory Specialist and had a Lung Function Test. After looking at the Lung Function Test, my X-Rays from 2011, 2013, the CT Scan from 2013 and testing my oxygen intake and then listening to my lungs he said “you have Idiopathic Pulmonary Fibrosis (IPF).”
I was shocked. Why me? I try and keep in shape and play sports, work out, eat right. He gave me some information to read and that made things worse emotionally. When you read the best case scenario from diagnosis is 3 – 5 years to live and worst case scenario is 3 – 4 months to live. I went home and my wife and I started to read everything we could on the Internet. DO NOT, I repeat DO NOT read the internet; you will get so worked up emotional and scared, and afraid. My doctor and he said “quit reading the internet, you’ll only make things worse.
A few days later we stumbled upon the Canadian Pulmonary Fibrosis Foundation (CPFF) and the Pulmonary Fibrosis Foundation (PFF) in the US and got the best and most informative information regarding PF. After reading the literature we felt a little better.
After talking to the Respiratory Specialist it was decided I would have a Bronchoscopy and it was scheduled for September 16, 2013. I went to the hospital for a Bronchoscopy. I was sedated and it only took about 45 minutes for the Respiratory Specialist to perform the procedure. After I woke up I had a slightly sore throat but I didn’t find the procedure that bad as I played golf four (4) days later and again two (2) days later.
On October 3, 2013 I saw the Respiratory Specialist for the results and he said they couldn’t tell exactly what caused the IPF but found markers for Rheumatoid Arthritis in the blood work.
On November 12, 2013 saw the Lung Surgeon and talked about the lung biopsy and what was involved. I decided to go ahead with the surgery. I needed to have another Lung Function Test for the surgeon, which was done on February 12, 2014.
November 18, 2013 I went on Short Term Disability for Clinical Depression and Anxiety until May 4, 2014.
On February 21, 2014 had a “Pre Surgery Information” session at the hospital regarding what was going to take place.
On February 24, 2014 saw the Rheumatoid Specialist and after about 10 minutes of talking to him he asked if I have a dry mouth and dry eyes. I said yes and have had it for quite a while. He said these are signs of Sjogren’s Syndrome, an autoimmune disease that is incurable. Now we searched for Sjogren’s Syndrome, on the internet, and again were shocked by what we learned.
On February 28, 2014 had my lung biopsy. They made three (3) incisions through my ribs on the right side. The top and bottom incision where about ¾” long and covered with a Band-Aid, the middle incision was about 1¼” long with a drain tube to drain any buildup of fluid from the biopsy. I had a little discomfort that night, and every couple of hours they nurses would check my pulse, heart rate and see if I was draining any fluid. The next day I had an X-Ray to check my incisions and draining, a few hours later the drain tube was removed and a couple of stiches were put in. Another X-Ray and I was given the okay to go home. I was told no lifting for 4 weeks to let the ribs heal.
On March 10, 2014 the Respiratory Specialist told me I now have Pulmonary Fibrosis secondary to Sjogren’s Syndrome.
On March 23, 2014 went skiing.
March 26, 2014 I saw the surgeon for a follow up and he said the lung biopsy went well and it confirmed I have Sjogren’s Syndrome. I asked how much of my lung did he take out and he said two (2) pieces 2-1/4“ long x 1-1/8” high x 5/8” wide. One tool grabs some of the lung and another tool cuts a piece off and staples the cut at the same time.
On April 14, 2014 I saw the Rheumatoid Specialist for a follow up. He gave me some advice on dealing with the dry eyes and mouth and no further follow up unless I get worse. But this is also an incurable disease.
I have lung function tests and follow up with the Respiratory Specialist every six (6) months. So far the PF has not progressed.
Don’t let this disease stop you from doing the things you like to do, just go slower.
I have to be careful of dust and the outside air quality, and certain jobs I can’t work at because of the air quality. I check my O2 level daily and live with the fear of when will it get worse. If I need a lung transplant will the Sjogrens Syndrome rule that out?
My wife is very concerned about me and the environment. Can we move anywhere better? Can I still play golf? Can I still go skiing? Can I go on a hike up a mountain to 9,000 feet as a fundraiser for PF?.
My Brother’s Story
My brother, Brian, has had breathing issues for over 30 years, but nothing of a major concern during this time. During 2013 to 2015 he was being treated for asthma but it was getting worse. In October of 2015 he had a Lung Biopsy that confirmed he had Idiopathic Pulmonary Fibrosis (IPF). His Doctor started him on a Portable Oxygen Concentrator (POC). Later in October he was put on the trial for Esbriet (Pirfenidone) to try and slow the progression but he had brutal side effects and didn’t help a lot. In April of 2016 he started to use bottled Oxygen when at home.
The May Long Weekend was always a special weekend for camping and quading with family and friends in the Rocky Mountains. In 2016 he lasted a couple of hours after setting up the campsite and at 7,000 feet in altitude he started turning blue and couldn’t get enough oxygen and they had to take him home. It was his last “May Long”.
We went golfing on September 9, 2016 with his POC and he managed to complete 18 holes, but he was exhausted. It turned out to be our last golf game.
October 2016 his Doctor scheduled him to see a Lung Specialist.
November 22, 2016 saw a Lung Specialist and is being referred to the Lung Transplant Program for a double lung transplant. First he needs to go through a qualification process which involves a week of testing every organ in his body to ensure there is nothing wrong that will negatively impact the transplant. They also check the psychological ability to ensure they can handle this whole process. Additionally they check their finances to make sure they can afford to live for 6 weeks prior to the transplant and 3 months after the transplant. If they pass they are assigned a Lung Allocation Score (LAS) that tells them where they are on the transplant list. The LAS can change at any time if his situation changes.
December 15, 2016 had his first consultation appointment with the Lung Doctor. After the consultation the doctor wants him on the transplant list ASAP. The first opening for the qualification process is in February of 2017. The doctor said he doesn’t want to wait that long and he has been tagged as “Urgent” and want the week of testing done before the end of December, 2016. He will also meet the Transplant Team. Because he had a prior heart attack a Cardiologist needs to see him.
December 16, 2016 received a call and will be admitted to the hospital on December 19, 2016 for a week of testing.
December 20, 2016 starts with taking 13 vials of blood, TB shoot, Ultra Sound of stomach and neck, check all his organs: heart, liver, kidneys, bone density, CT Scan of spine and chest, teeth, they also check for cancers, Angiogram, Bronchoscopy, Colonoscopy, ECG, and Chest X-ray.
December 21, 2016 started with a Heart Catherization and Heart Angiogram, Echocardiogram, Pulmonary Function Test. Heart Angiogram did show blockages had increased and need to be fixed. Fixing before the transplant would delay the transplant by a year, not a viable solution. They could do the transplant and heart bypass at the same time, will be determined at time of transplant, or they wait until after the transplant to do the heart bypass.
December 22, 2016 started with a VQ Scan, met with Transplant Social Worker, 6 minute walk test, met with Cardiologist.
December 28, 2016 has been activated on the transplant list. He and Carol were told to pack a bag as a call may come at any time. He is a Class 2, which means he is really high on the list of people waiting for a transplant.
January 3, 2017 They have been checked into the hospital Outpatient Residence and will start Physio. Physio is a 4 week program which anyone on the transplant list must be in. He doesn’t have to finish if he gets a call for the transplant. Physio involves lung and chest strengthening exercises to increase the size of his chest for the new lungs, as his chest is shrinking they don’t fill with as much air as they should. They both will learn how to deal with stress, anxiety and lots of after transplant information.
Their calendar is filled for the next 4 weeks with various classes after physio. Some classes are for both of them, some for just him and some for just Carol. They range from what to expect after transplant, how to handle stress and anxiety, a tour of the transplant ICU and overall health and wellness. The exercise portion lasts about an hour and a half and the classes are about an hour so they we will usually be done by noon.
They also met with the Transplant Coordinator and the Transplant Doctor. The doctor explained different things to watch for, i.e. Infection, breathing gets worse, and then it is time to go to the hospital. Sadly, he also talked to us about what they call “Goals of Care”. In a nutshell it’s when the family wants resuscitation and when they don’t.
January 5, 2017 around 4:00 AM He started coughing bad and couldn’t breathe. Carol had to call 911. He was put into a BiPap Mask once he was in the hospital to force oxygen into his lungs. He started feeling a bit better pretty quickly but was still admitted into the hospital. He managed to snooze a bit, even though wearing a BiPap mask feels like having your head out a car window (doctors’ description). By the afternoon he was still not feeling great yet, but doctors had expected he’d be in the hospital for a couple of days.
The following information was taken from a Social Media site, Lotsa Helping Hands. This site created a single resource point for family and friends to be kept informed of Brian’s condition and to avoid bothering him and his wife Carol.
January 6, 2017 has a fever and isn’t feeling very good so he’s been put into isolation for at least 48 hours while they run some tests. Isolation means anyone coming into the room has to put on an isolation gown & mask & use hand sanitizer. The doctors are hoping it’s just a flu bug at this time, but combined with IPF it’s really hard on him. If all goes well, he hopes to be in physio again on January 9, 2017.
January 7, 2017 had a really bad night. He got up to use the washroom and couldn’t get his oxygen back up. The ICU Doctor came up and saw him and decided he needed to be intubated and is currently on life support. His blood pressure keeps dropping to scary numbers so he is currently on 4 different blood pressure medications. They have him in an induced coma so he can rest. His hands have been tied to the bedrails as he keeps trying to remove the air tube even in a coma. They say he can hear us but gets upset when touched and his heart rate and blood pressure spikes.
They found one blood pressure medication that works for him; unfortunately this means they have to wean him off all 4 medications slowly. By the afternoon he is down to 2 and his blood pressure was stable at about 90. If he stays stable at 90 for a while they will reduce 1 of the medications bit by bit. The goal is to get his blood pressure down to about 65 and off all blood pressure medications.
January 8, 2017 has rallied and there is only 1 more blood pressure medication we need to be rid of. Things are slowly moving in the right direction. He will likely remain in the medically induced coma until after transplant. The doctor said he is still at the top of the transplant list. His test for the fever came back negative – no infection, so no more isolation. He is still on 1 blood pressure medication.
January 9, 2017 his blood pressure medication is at a really low dose now. He is still “critical” but “stable”. He had a CT Scan this morning, which will show if there are any blood clot(s) in his lungs. He started to gurgle a bit and the nurse was called, his ventilator was coming out slightly causing an air leak. They gave him a paralytic again and reinserted the ventilator. CT Scan results showed no blood clots, that is good news. Carol received a call from the Transplant Coordinator this evening and he is still at the top of the Lung Transplant list.
January 10, 2017 he is off all blood pressure medications. He’s still stable, which is great.
January 11, 2017 another good night, he gets a bit agitated when they turn him, but otherwise stable. He is still in a coma, and may remain that way until he gets new lungs. If it looks like he will be on ventilator for a long time they may do a tracheotomy to insert a breathing tube.
Carol is going to continue going to the educational classes, they are very helpful and the group of other patients and support people in the group have been asking about Brian.
It’s a very positive, supportive group of folks who are in the same boat, just not near as bad as Brian.
January 12, 2017 he is still sedated and will likely remain that way for a bit yet. He does become agitated, probably due to the tubes going down his throat. He reflexively fights the tubes and so is restrained (wrists tied to bed) all the time so he can’t pull the tubes out. Apparently, this is a very normal reaction for people who are sedated. His blood pressure has remained stable, which is quite relieving.
January 13, 2017 he really doesn’t like the tubes down his throat and fights it every time they move him or adjust the tubing, but otherwise is doing well. It takes 3 people to turn him “because he is so strong”. They switched him from Fentanyl to a pretty heavy dose of Hydromorphone. On the Fentanyl they said he was very stiff, and tense, so the Hydromorphone seems to be better. It’s the drug that is keeping him sedated.
January 14, 2017 he opened his eyes several times, still with the glazed faraway look, along with confusion and probably scared. Unfortunately, when his eyes are open and we talk to him or touch him, his heart rate and blood pressure go really high and it takes quite a while for him to settle again. They are doing another (routine) chest x-ray this morning, and will give him the paralytic before that so he doesn’t become agitated and spike his numbers.
January 15, 2017 his blood pressure kept shooting up really high, and his breathing was out of sync with the ventilator, so when he was in the middle of a breath the ventilator would then start a new breath. In order to get his blood pressure regulated and his breathing in sync with the ventilator he was more deeply sedated and remains that way. Also, his blood sugar was up to 13.6, which is very high and should be between: 6 – 10. To get his blood sugars down they put him on an insulin drip and later in the night it was down to 10.
January 16, 2017 Transplant Surgeon and Coordinator stopped by last night and said if any other organs fail, they may need to reassess or if he remains in ICU for another month they may a need to reassess whether he would survive a transplant. Every week in ICU adds 4 weeks to the recovery time. His blood sugar is still high so his insulin is up to 3 units. This may be due to the sugar water they need to use with some meds. No evidence of any kidney issues. They have also added back in a blood pressure medication (a low dose) as his blood pressure is staying pretty low.
Intensive Care doctor was also here and said there is quite a bit of fluid in his lungs they are trying to clear. His oxygen support has also increased to 80 (it was down to 35). So they are going to check for infections etc.
January 18, 2017 has a fever of 39.4 and they are treating him for pneumonia. Oxygen is back up to 90% (was at 35% a couple of days ago) and PEEP (pressure the ventilator is set at to keep his lung open) is up to 15, it was at 8 a couple of day ago. Still suctioning “stuff” out of his lungs. They have sent blood, urine and lung cultures off for testing. He had a “Code Blue” at 10:00 pm and after a tremendous effort from numerous ICU nurses, doctors and other specialists he was recovering at midnight. We have a very short window for him to get lungs before he is too sick to make it through a transplant.
January 19, 2017 it’s with deepest sadness that Carol and the rest of Brian’s family have announced that his physical condition has worsened to the point that he would not survive the lung transplant. He suffered a heart attack during the Code Blue and the transplant and ICU teams have decided he is no longer a candidate for a lung transplant and had to be removed from the list.
6:15 PM The family has gathered together with Brian in the ICU and have made the decision, according to Brian’s wishes, to withdraw life support and transition him to palliative care.
At 7:20 pm on January 19, 2017 my brother passed away.
He will be greatly missed for his sense of humour, willingness to help friends and family and a terrific father and husband.