Blake Sipes receives second chance at life
Breath is such a normal part of life, it is usually just taken for granted. To those who have cystic fibrosis (CF), the normal includes growing up taking breathing treatments and lots of pills.
CF, a genetic disease characterized by the building of thick, sticky mucus, can damage many of the body's organs. The disorder's most common symptoms include progressive damage to the respiratory system and chronic digestive system problems.
Blake Sipes, 33, of Manila, has lived with CF all his life. He was fortunate to enjoy good health through his teenage and young adult years. Over the last few years his health started to decline.
Blake, son of Karen and James Skinner and Michael and Michelle Sipes, is recovering from a lung transplant. He has temporarily relocated to the St. Louis area following his transplant at Barnes Jewish Hospital.
Blake started having problems several years ago at the age of 28 or 29. It started in his sinuses with recurring sinus infections that would move down into his lungs and cause infections. The infections happened about four times in 2012 before the doctors realized it was his sinuses.
Treatments started with IV antibiotics and finally he had sinus surgery to correct the problem, but his lungs had suffered damage. In 2014 he had a flare up, took IV antibiotics, got better and later caught the flu which put him in the hospital for two weeks. His lungs filled with fluid. The fluid was removed and he could breathe, but it did a lot of damage.
His health seemed to go down from there and he had to have oxygen full time. He moved back home with his mom and stepdad in May of 2015.
Blake's pulmonologist, Dr. Keith Criner, with NEA Baptist Clinic, started talking to him about lung transplantation. It took a while before Blake started considering it. His doctor encouraged him to get established as a patient at Barnes Jewish so when he needed the process it would be easier and hopefully not take as long.
He went for his first evaluation in June 2015. The evaluation process consisted of three days of tests, appointments and interviews. His lung functions were 23 percent at that time. He was told the lung functions would need to be around 18 percent before the transplant would be considered.
In August 2015 the FDA approved a new drug called Orkambi that was hoped would help Blake enough so he would not need a transplant. Insurance approved it in September and he tried the drug for two months but it was too hard on him. The side effects were so harsh he had to stop taking it. He returned to Barnes Jewish Hospital in July 2016 for a second evaluation and his lung functions were down to 21 percent. Since Blake's lung functions were on a steady decline, he was told they didn't think he would have any problems getting listed.
He was given a list of things to get started on prior to a transplant including immunizations, dentist, finding a place in St. Louis to live, fund raising, etc.
He was officially listed on July 18 and told the average wait time is three months.
When he got the call on July 30, he was shocked the call came so quickly, only 12 days.
"I was scared and happy at the same time," Blake said.
The call came in on Saturday, July 30, around 2:50 p.m. His mom and stepdad drove him to St. Louis. Blake said it was a fast trip.
"You never know if the surgery will be performed when you arrive," he said. "There is always the chance it will be a dry run, meaning the lungs wouldn't be found suitable for me and we would have to turn around and come back home. Testing has to be done on the lungs and a final determination is not made until the doctor actually lays eyes on them. I was admitted and in my room by 7 p.m. My sister Heather and her family, Thomas, Braden and Braxton Littlefield, arrived shortly after. My mom's best friend, Stephanie, and Doyne Davis came a few minutes later. My dad, Michael, and Michelle arrived. And my girlfriend, Michelle Johnson, came as soon as she was able to get off work. For a couple of hours, we were not sure when the surgery would be, but finally they told us a helicopter would be flying to Kansas City around 2 a.m. to get the donor lungs and I would be taken down to the holding room at 4 a.m. for surgery at 7 a.m. on Sunday, July 3l."
There was no sleep that night. His family was able to go to the holding room with him. It began to get busy at 6:30 a.m. when Blake was taken to the OR.
His mom said they were called in the waiting room at 7:20 a.m. informing them the surgery had begun and everything was going good. At 9:30 a.m. the first lung had been taken out, donor lung put in, and all went well, and starting on the second lung. At 10:15 a.m. the report was all good, Blake doing fine. At 10:38 a.m. the doctor walked into the waiting room to inform the family the surgery was finished and Blake was doing fine and they went ahead and closed the incision. It was a long four hours for the family but they got to see him in ICU.
Blake had what they call a "clamshell" incision across the chest. They didn't have to break any ribs but they did cut the breastbone. Doctors removed one lung, put in the new donor lung and attached it, then removed the other lung and did the same.
Blake was able to get off the ventilator the next day and he was able to sit up in a chair. The second day after surgery two of the four chest tubes were removed and he was allowed to eat a regular meal.
He went for his first walk around the nurse's station (320 feet) on Tuesday. The next day he walked two laps (640 feet), and then he was moved to his own room.
From that day on, pulmonary rehab came to his room twice every day and his goal was to walk 30 minutes without stopping.
"I never met that goal while I was in the hospital because I developed some GI problems which set me back," he said. "I had to have a NG tube put in for three days. I walked at least once a day every day, and twice most days. I did get up to 20 minutes a day."
Blake was discharged from the hospital on Aug. 12. He said he had to learn to breathe again.
"I had always breathed short, fast breathes," he said. "Now, I can take long, deep breathes. Everything feels different. It is like starting over again."
Blake is living near the hospital where he will have pulmonary rehab daily for three months. He will undergo pulmonary function testing, lab work, and chest x-rays twice a week and sees the doctors regularly. He also has bronchoscopies monthly during his three months in St. Louis.
Blake explained the bronchoscopies is when a scope is inserted to look at the incision where the airways were attached. Doctors check for infection and rejection. The first one he had on Aug. 19 showed he had infection in his airways so he had to start antibiotic breathing treatments for a month. The blood flow is restricted due to the healing process and caused the infection, but it should improve once the infection is gone.
Blake hopes to be home by the end of October.
"They say the lungs are still the hardest to transplant," he said. "The average survival rate is five to six years but that includes all age groups and illnesses. The doctors explained to me that based on my age and illness my chances are likely to be much higher than the average."
He will need to avoid being around people who are ill. On his second day of outpatient pulmonary rehab, he walked 30 minutes and one half mile. He is now up to a little over three quarters of a mile. His pulmonary functions are currently up to 77 percent.
"The doctors said it can take up to a year for my lungs and chest to completely expand out," he said. "I still have cystic fibrosis but my lungs don't. I have to take my CF medications, minus the breathing treatments, and now I have to take the anti-rejection medications."
On his list of things he wants to do is play with his daughter, Christine, and get back to work. He has missed out on the last two harvests on the farm.
Blake also likes to duck hunt. He and his best friend Chris Hill hunt together and he hopes he will be able to hunt with him this season.
Blake hopes his story will encourage others having to make tough decisions. He said when he went through his first evaluation process with a group of other people, there was an older gentleman going through evaluation and they hit it off. They became friends with him and his wife. When Blake went back for his second evaluation, they ran into them. He had received his transplant and was doing well. It was a positive thing for Blake to listen to him.
"He answered a lot of questions I had and relieved a lot of apprehensions I had," Blake said.
Blake's mother, Karen, shared the early years of Blake's life leading up to his diagnoses of CF.
"Blake was born in 1983, seemingly healthy," she said. "There was no newborn testing done at that time for cystic fibrosis and there was no known family history. He was a happy baby, gained weight and grew as a healthy baby would. There was nothing early on to indicate any health problems, other than finding a formula that agreed with him. He projectile vomited all the time, but he still gained weight, so his pediatrician didn't think much of it."
She remember picking him up from his grandparents' house (Sonny and Rozanne Copeland).
"My mom mentioned when she kissed him, she noticed he really tasted like salt and that is a symptom of CF (the most commonly known symptom). I kissed him and noticed the same. We had never noticed it before. I called his pediatrician. When the doctor called me back, he was very annoyed and laughed at me. He sarcastically told me all kids taste like salt to an extent, basically, making me feel like I was over-reacting and should not be bothering him. Needless to say, we never went back to that pediatrician.
"Blake had the usual colds and was diagnosed with bronchitis multiple times, but in January 1984, he couldn't get over it. He was so congested, he could barely breathe. His dad and I took him to the doctor several days in a row and they finally decided to admit him to the hospital in Paragould. After three days, on a Friday, he was transferred to Arkansas Children's Hospital, after a pediatrician who had been called in suspected cystic fibrosis.
"A sweat test is how CF is diagnosed and since it was late Friday when we arrived, the lab was closed for the weekend. We had to wait until Monday to have the test performed. On Friday night, Blake had a breathing episode similar to an asthma attack. If he had not been at Arkansas Children's Hospital, he would not have survived. He was moved to PICU. On Monday, the sweat test was run and was positive for CF.
"I'll never forget being told. I went to his crib-side and just cried. I could not help but wonder what was going to happen to my little boy. I had already read through some literature in the library and had somewhat of an idea of what he would be facing. The average life expectancy at that time was 17 years old. He stayed in PICU for 11 days. His dad and I were taken into the chapel for the first five days and told each day to not expect him to live through the night.
"I can't even begin to explain what those days were like. When I was allowed to feed him for the first time, I was told if he aspirated there would be nothing they could do. I was excited to hold him but scared at the same time. Thank the Lord, we made it through just fine. By God's grace Blake survived those 11 days and was then moved to a private room. He was in the hospital a total of 24 days. I turned 19 years old during his time in the hospital, but I did a lot of growing up during that time. Not all of the families we met during that time got to take their babies home with them."
Blake weighed 19-1/2 pounds at 5-1/2 months old when he went into the hospital. When he was discharged, he weighed 13 pounds.
"He did not look like the same baby," Mrs. Skinner said. "He came home on oxygen 24/7. Back then a huge oxygen tank (about five feet tall) was delivered to the house and put in his bedroom so his mobility was very limited. He was on 13 different medicines, some three, some four times a day. He took four breathing treatments plus chest physiotherapy each day. He went back to ACH every month for checkups for the first year, then eventually every three months until he aged out.
"When Blake was three years old, his sister Heather was born. Since CF is a genetic disease, there was the chance she could have CF, also. Back then, they wouldn't do a sweat test until the baby was six months old. So, we had to wait. Thanks to God, she did not have it. She has, however, suffered due to CF, seeing what her brother has had to go through.
"His school years were very 'normal' to him. He never knew life not taking breathing treatments or 30+ pills a day, so that was his normal. He played sports, didn't miss much school, hunted, dated, worked and was able to do just about anything he wanted. He was very healthy during his teenage years and early adult years."
Definition and Statistics of CF:
People with CF have inherited two copies of the defective CF gene -- one copy from each parent. Both parents must have at least one copy of the defective gene. People with only one copy of the defective CF gene are called carriers, but they do not have the disease. Each time two CF carriers have a child, the chances are:
*25 percent (1 in 4) the child will have CF.
*50 percent (1 in 2) the child will be a carrier but not not have CF.
*25 percent (1 in 4) the child will not be a carrier and will not have CF.
The defective CF gene contains a slight abnormality called a mutation. There are more than 1,800 known mutations of the disease. Most genetic tests only screen for the most common CF mutations. Therefore, the test results may indicate a person who is a carrier of the CF gene is not a carrier.
According to the Cystic Fibrosis Foundation Patient Registry, in the United States:
*More than 30,000 people are living with CF (more than 70,000 worldwide).
*Approximately 1,000 new cases of CF are diagnosed each year.
*More than 75 percent of people with CF are diagnosed by age 2.
*More than half of the CF population is age 18 or older.