Saturday, September 28, 2013

Police Officer with pulmonary fibrosis marries at hospital after getting new lung

Ohio Nurses Surprise Lung Transplant Couple With Hospital Wedding

Nurses even created a sign for the back of Smith's wheelchair: "He got his lungs, she got his heart."
Doug Smith, a police officer with life-threatening pulmonary fibrosis, was one week away from his wedding day last June when he got the 1:30 a.m. call that a donor lung had become available for transplant.
The Zanesville, Ohio, 42-year-old had never smoked and had been healthy for 20 years, but in 2011, asthma-like symptoms made it almost impossible for him to breathe. With no effective therapies, he was put on a transplant list.
"I was tired all the time," said Smith, who worked the night shift at the jail in town. "I was doing six liters of oxygen a day. "My body had started to go pretty quick."
The couple, each of them married before and with three teenage children between them, had been dating for five years and had planned a small, quick church ceremony because they worried about his precarious health.
Typically, those with this disease only live about two to five years after diagnosis.
Wedding plans were dashed, and within days of the call from Cleveland Clinic's Transplant Center, Smith was in surgery.
But one month to the day after getting a new lung, on June 21, Smith and his now wife, Susan, tied the knot on the rooftop of the hospital before family and staff.
Doug Smith had been transferred to the recovery floor, and his bride to be had cleared the on-site marriage with the hospital chaplain, but the rest was a complete surprise.
"The next thing you know there was a roomful of nurses with pen and paper, running in and out of the room, deciding among themselves that they would take care of everything," said the prospective bride, 46. "It was amazing -- they were so excited. It was very emotional."
"We had joked before about getting married in Cleveland," she said, which was more than two hours away from their home.
"We already had the marriage license and it was going to expire," said Susan Smith. "Forty bucks is forty bucks, especially with finances going against us."
The staff provided a bouquet of daisies, her favorite flowers, and one nurse even provided her own veil. Another, who was a consultant for the cosmetics company Mary Kay, rolled in a big suitcase of make-up.
Nurses even created a sign for the back of Smith's wheelchair: "He got his lungs, she got his heart."
"I had a cute little white dress and that was plenty good enough," said Susan Smith. "I was in flip-flops because my daughter forgot to bring shoes."
"They all brought food donated by Starbucks and Au Bon Pain," she said. "They even provided a cake -- something I had never thought of. One of the nurses said, 'You are not getting married in the room. We are taking you up to the rooftop.'"
Doug Smith was fortunate to get a new lung and even luckier to make it through grueling surgery and a two-month hospital stay to make sure he did not reject the organ.
"He had a tough transplant," said Dr. Marie Budev, medical director of Cleveland Clinic's Lung Transplantation Program. "He had some issues with primary graft dysfunction. His lungs got wet and he had to remain on a ventilator for a while. "He's been through a lot."
The survival rate is about 53 percent after a lung transplant, but Smith's youth may make his odds better.
"If they are younger and their other organs are younger, they are able to sustain a lot of the toxic effects from the immune suppressive drugs they are on for the rest of their life," said Budev. "We do have patients 20 years out -- they are a small fraction, but everyone has that potential," she said.
Though Budev was overseas when the Smiths were eventually married, she said the hospital staff had been "inspired" by the couple.
"We had never had a wedding after a transplant like this -- it was special for us," she said. "Despite the terrible things that happen, life goes on."
The Smiths say that Doug's brush with death underscores the scarcity of organ donors that requires the average American to wait six to nine months for a transplant.
Currently, 1,632 Americans are waiting for lung transplants, according to the federal Organ Procurement and Transplantation Program. Pulmonary fibrosis is now the leading cause of such transplants in the United States, overtaking chronic obstructive pulmonary disease, or COPD.
Pulmonary fibrosis, an insidious disease that scars the tissues in the lungs and responds poorly to medication, accounts for about half of all lung transplants performed at the Cleveland Clinic.
A recent study estimates that about 500,000 Americans suffer from the disease, most with no known causes, according to the Pulmonary Fibrosis Foundation.
"The decline is rapid, and it is an unpredictable and relentless disease," said Budev. "You can have coughing, or is it dismissed as asthma and after an X-ray a workup is done, and you can see the abnormal pulmonary function. They are short of breath and have exercise intolerance and the ability to do tasks is difficult, but symptoms can get much more severe."
I can certainly relate to Doug Smith's ordeal with pulmonary fibrosis. 
I was diagnosed with Idiopathic (unknown cause) Pulmonary Fibrosis or IPF and eventually received a single-lung transplant almost 12 years ago. 
It certainly is a debilitating disease because the scarring of lung tissue caused by PF causes fibrosis of the alveolar epithelial cells where oxygen is transferred to the blood stream. Lack of oxygen  to vital organs such as the heart and brain causes weakness and fatigue making it difficult to function. I can remember not being able to bend over to tie my own shoes, even when connected to an oxygen tank. 
So far no medications have been able to slow down the progression of pulmonary fibrosis. There are currently no FDA-approved therapies for idiopathic pulmonary fibrosis. The FDA had granted Intermune's drug perfinedone priority review, which is reserved for products that fill an unmet medical need. 
The company submitted two studies of its drug to FDA, measuring perfinedone's ability to improve lung function in patients. While one study showed a statistically significant 4.4 percent increase in lung strength, another failed to prove substantially more effective than a placebo. FDA generally requires two clinical trials with positive results before granting approval. Pirfenidone is approved for marketing in 29 European countries and Canada under the InterMune trade name Esbriet® and in Japan and South Korea where it is marketed by Shionogi & Co. Ltd under the trade name Pirespa®. Under different trade names, pirfenidone is also approved for the treatment of IPF in China, India, and Argentina. InterMune is conducting an additional Phase 3 study, ASCEND, to support the regulatory registration of Esbriet for the treatment of IPF in the United States. Merv.

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