If you are coughing up items like what can be seen above, you may have PB

 

The experiences below are personal stories from those who have been affected by Plastic Bronchitis

 

 

Keegan

 

Keegan was born in June 2005 with Hypoplastic Left Heart Syndrome or HLHS. We were told the only way that he would survive is with a series of three open heart surgeries. We were given the option to do comfort care (where they would keep him comfortable and let him pass) or to proceed with the series of surgeries that could help him live but with no guarantee that he would even survive the surgeries. Without hesitation we decided to do the surgeries and fight for him as long as he was willing and able to fight.

 

He was two days old when he had the first surgery called the Norwood. He had some complications and setbacks and had to have this surgery done again to move the shunt that got kinked once his chest was closed. Nine months later he had the second step surgery called the Glenn. He flew through that surgery with flying colors and was in and out of the hospital in five days. When he was a little over two he had the last surgery called the Fontan. The surgery itself went great but he ended up having a stroke due to an air embolism caused by human error of someone getting air into his picline. This caused major problems, as he had to learn how to walk, talk, eat, and even sit up on his own all over again. He went through extensive rehabilitation therapy and still has trouble with fine motor skills in his left hand.

 

Keegan remained healthy for seven years following the Fontan surgery until January 6th 2015. He had been sick for six weeks and continued to get worse. His coughing was so sever and he was gasping for air. We rushed him to the emergency room and he was put on oxygen and had x-rays done. He was diagnosed with pneumonia and was once again transported Peyton Manning Children’s Hospital. They weren’t sure what was wrong but they weren’t convinced he had pneumonia. It wasn’t until the next morning his oxygen dropped so low and he was rushed down to intensive care that he managed to cough a piece of white plastic looking stuff into the respiratory therapists hand.

 

Within moments the doctor was in our room talking with us about Plastic Bronchitis. We had never heard of it before and didn’t realize or know that it could be a side effect from the Fontan surgery. He was immediately taken down to have an emergency bronchoscopy to clear out his lungs and airways as they were so blocked even with oxygen he was unable to get air to his lungs. They were able to remove most of the casts and the next day he had another bronchoscopy to see if they could find any more or if any had returned.  He was left sedated and on the ventilator for two days. When he finally awoke he was very weak and barely able to speak but he was starting to recover. He began doing nebulizer treatments and vest therapy as well as taking Sildenafil to help prevent the casts from forming.

 

Less than two weeks later he had a heart catheterization to take a look at his heart. They found a large colladeral (vein) that grew from his heart to his lungs. The surgeon was able to coil it off and almost immediately his heart functionbegan to improve. He has only had a few casts since and hasn’t had any in a little over year. He still spends a few hours a day doing breathing treatments and his pulmonologist is going to try to start weaning him off of some of them. His information has already been sent to CHOP in the case that he starts casting again. He is also in the process of being tested for Protein Loosing Entropothy (PLE) which is also a lymphatic flow disorder that can be a side effect from the Fontan.

 

We are not sure what Keegan’s future holds but we are so greatful for finding the Plastic Bronchitis group and for Joy Dumire for everything she has done for the Plastic Bronchitis foundation. We can’t thank you all enough and our hope is that someday we can use our experiences to help others.

 

 

Tiernan

 

During my Pregnancy, we found out at our 20 week anatomy scan that Tiernan had a rare Congenital Heart Defect called Tricuspid Atresia.  From then on, we had appointments every week with either our regular obstetrician or specialty obstetrics to keep a close eye on him and figure out a game plan. "Once born, he was to be in the NICU and have a BT Shunt placed, which would be the first of three scheduled surgeries."

 

Tiernan was born on August 20th, 2004, and was doing so well, that he got to skip that surgery and he got to go home two days later like a "normal" baby. At two months old, he had his first heart cath to determine when he would get the second stage of surgery called the Hemi-Fontan. On November 5th, 2004; we were told that everything looked great, and they would do the surgery when he was around 9 months old. Just 2 days later on November 7th, he went into respiratory arrest, and had to be life flighted. We could not go with him because there wasn't room on the plane, so we drove to Atlanta not knowing if he would be alive when we got there. Once we arrived, we were told he was very sick, and he may not make it through the night. He had a pulse, but no blood pressure and his core temperature of 27 degrees Celsius.It would be two weeks before he was even stable enough to consider surgery...then because he was only two and a half months old, they decided it would be best to do the BT Shunt instead of the Hemi-Fontan.  He finally got to go home on December 1st with a feeding tube because he wasn't able to eat like he needed to to maintain a healthy weight. A few months later, he was able to have it removed.

 

At 9 months, he had his Hemi-Fontan, and was only in the hospital for 10 days.  He had the final stage, called the Fontan, at almost 2 years and was discharged five days later.  When they did the Fontan, they put a hole in his Superior Vena Cava, called a fenestration, to prevent pressures from being too high.  Generally, that hole closes on its own over time, but Tiernan's didn't.  In 2009, they decided to close it off via catherization because his oxygen saturations were on the low side.  Everything seemed to be going great, and other than taking his meds and having a zipper scar, he looked and acted like any other boy his age.

 

A few months after occluding his fenestration, he started coughing up things that looked like white strings, and were stretchy like rubber bands. His pediatrician thought it was a lung infection that is typically seen in older people, but because of his anatomy and compromised immune system, it wasn't that odd that he would have it. They prescribed antibiotics, and made a followup appointment. He still had the "infection" when we went back, so they switched his antibiotics. This went on for a few weeks, and while he was on the fouth round of meds, he had his cardiology followup. I was explaining the infection to our cardiologist, and was informed that it wasn't an infection, but a rare side effect of his Fontan called Plastic Bronchitis. Only 5% of Fontan patients develope this lung disease. Lymphatic fluid, fibrin, and lung debris mixes together and forms thick, rubbery castings of his bronchial tubes, which restrict his airways and make it hard for him to breathe. He started wearing a Cystic Fibrosis vest three to four times a day, and doing Albuterol breathing treatments to loosen the casts, so he could get the casts out easier.

 

On June 16th, 2010, he had a Thoracic Duct ligation.  He was one of a handful of patients to ever have this surgery, and it only had a 50% chance of working, but we took that chance because it was better than doing nothing, and him choking to death.  Surgery went great, and the PB seemed to have stopped.  In January of 2011, he started casting again, and was readmitted to the hospital.  It lasted several days, then stopped, so everyone thought it was a one time thing and he was discharged.  He did great until March of 2014, then started casting again.  He was admitted for 11 days, during which time, they decided to re fenestrate him to hopfully lower the pressures in his lungs, and in theory, stop the PB.  He was admitted again in October and they tried to do a procedure called Thoracic Embolization.  During this procedure, they would go in through his groin, and block new pathways that had formed with coils.  They weren't successful, because they could not get past the Thoracic Ligation.  December he was admitted again, and we even spent Christmas in the hospital.  He was discharged on December 27th, 2014, then readmitted on January 2nd, 2015.  He wouldn't be discharged this time until February 27th.  During that admission, they went in through his arm to do the embolizattion, and found 2 new pathways.  Because of time, they were only able to block off one, then the other a week later.  After a couple of weeks, he stopped casting and was discharged, and prescribed to continue the Albuterol, his vest, and a new medication called tPA (Tissue Plasminogen Activator) three times a day.  He was cast free for 15 months, then started casting again in May of 2016.  He spent a week in the hospital before being discharged.  He was admitted overnight on December 22-23, 2016, January 3-13,2017, on January 21st till now.  It was during the January 3rd admission we started evaluation for placing him on the heart transplant list.

 

 

Elena

 

Ten-year-old Elena is our beautiful, happy and strong little daughter and by the way daddy's girl. During pregnancy we didn't learn that she has Down's syndrome and suffers from a severe heart defect, so that we weren't prepared at all. But anyway, how can you be prepared for this? Elena was born in December 2006 in the early morning hours. The midwife sent us home with Elena a few hours after birth, but it was obvious that something was wrong. We called the pediatrician, and he admitted Elena to hospital immediately. The first four months of her life she spent in hospital. Elena was born with an atrioventricular septal defect and a hypoplastic left ventricle, Down's syndrome was confirmed after a couple of weeks. She had the first of in total three open-heart-surgeries in 2007 at the age of eight weeks, followed by the 2nd and 3rd in 2008 and 2013, resulting in Fontan-palliation. After countless hospitalisations, cardiac catheter examinations, successful tube weaning in 2009 and other bits and bobs things went quite fine, though Elena had a rough time recovering from the third surgery with pericardial and pleural effusions. Retrospectively we can say, that this was a first bad sign, but it took until March 2015 to have certainty that our baby again picked a winner: Elena was diagnosed with plastic bronchitis. It began with a cold, as we thought, and the next, and again the next. She coughed up little white rubber-like pieces, and neither our pediatrician nor the lab were able to make the right diagnosis. Elena was admitted to hospital end of march, because her saturation dropped and her condition worsened. The cardiologist that examined Elena when she was one day old took his time and listened closely, and after talking to the respiratory team they made the diagnosis PB. That was a shock, because we were told that there is no treatment.

 

But Elena's mum started searching intensively on the internet and found a group on Facebook, The plastic bronchitis foundation and information about the treatment at CHOP. The decision was made: she will take Elena to CHOP, if there is the smallest gleam of hope.

 

Elena's condition worsened, and by the end of 2015 we were told that her life expectancy would be about twelve months. Twelve months! A disaster.

 

In January 2016 we contacted Joy Dumire, and she forwarded our request to Dr. Dori. He replied immediately and offered to review Elena's case. By telling us that Down's syndrome is not a contra indication he gave us hope, the first time for months. But still we were very cautious: a trip to and treatment in the U.S.? This was emotionally and regionally so far away. We sent piles of documents to CHOP, and then things started to move quickly. In the meantime we got the support of our cardiologists, at first they were very reserved. In a Conference call with Dr. Dori and our pediatric cardiologist Prof. Kececioglu, Dr. Dori explained the pro's and con's, especially about Elena's special feature Down's syndrome. There was no experience with his procedure and this handicap. But still he gave his ok at the end of the conference. Elena was accepted for the treatment at CHOP.

 

The planned date of the procedure was August 11th - three months to go. After we received the cost estimation from CHOP we talked to our insurance company and prepared ourselves for a long lasting fight. But like so many times in Elena's life, we were proved wrong. After four weeks the insurance confirmed the assumption of the costs. We were overwhelmed. But in this family one thing is guaranteed: one bad news for any good news. Elena was feeling bad and only one day later we found ourselves in the ER of the Center for congenital heart diseases, not knowing if Elena will make it over the weekend. Her condition stabilized, but there was no way of thinking about a trip to Philadelphia. We came so far and were so close to receiving help. But again, Elena surprised us and showed us the way. She received oxygen 24/7 and the inhalation with rtpa was stopped. Elena was recovering day by day, and after two weeks Prof. Kecicoglu gave us his go for an air travel to the USA. But then a German airline refused to take Elena on board, again Philadelphia was too far away. With American Airlines we found a carrier that approved our request, and on August 5th we started to what we call Elena's great journey on so many levels, together with mum and dad and Elena's two sisters.

 

It was a very hot summer in the city of brotherly love, and on the 9th of August we went to CHOP for the pre-testing and met Dr. Dori and his team for the first time. We were allowed to go back to the hotel in the evening and Elena was admitted to CHOP on the 11th for the lymphatic procedure. It took nearly five hours (only!) until Elena was brought to the ICU, where we were allowed to visit her. The first day and night were very rough for Elena. Her blood pressure was extremely low we think that she had severe stomach pains. Dr Dori explained everything in detail and was satisfied with the procedure and Elena's development.

 

After only six days at hospital Elena was discharged without oxygen, without the need to inhale three times a day and with a very positive prospect, with a saturation of 92-95, which was more the 15 percent higher than without three months ago. We returned home only 14 days after the procedure.

 

Elena has been cast free since then, has much more energy and a saturation of 90-92. She sometimes stands up by herself and walks a couple of steps in our arms. Elena visits school again and enjoys the time being there with her friends.

 

But what is best? Watching her sleeping and listen to her breathing - calm and relaxed without the crackling and lymph running out of her nose. We love every single moment.

 

Without the skills, courage and empathy of the highly specialized people at the HDZ NRW Center for congenital heart defects in Bad Oeynhausen, Germany, and the Children's Hospital of Philadelphia Elena would have lived a very short life. A big thank you to Dr Dori and Prof Kececioglu and their teams, and especially to Joy Dumire, who brings the right people together and who has helped us with her initiative. We will never forget that.

 

We're grateful.

The Plastic Bronchitis Foundation

Experiences

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