(Note: This article was originally written in 2011 after my niece, Alison's double lung transplant. Alison passed away on May 11, 2013 after a tough fight against the disease, Cystic Fibrosis.)

WEST UNION – After becoming the first double lung transplant at University of Iowa Hospitals and Clinics in 2011, there's not a lot to remind Alison Garvin she was born with Cystic Fibrosis. Since her surgery, the 23-year-old woman's physical health improves with each new day.

Diagnosed with the disease at less than two years of age, a nagging cough was the most outward indication to others as she struggled to breathe. But with each birthday Garvin celebrated, the disease increasingly weighed on her desire to thrive. Catching a cold or the flu, worsened her lungs' ability to rid her body of the mucus that accompanies those illnesses.

Frequent hospitalizations were the norm. Three week treatments of intravenous medications were typical as she grew to adulthood. Unless one saw her swallow a few "enzyme pills" with each meal which aids her pancreas in absorbing necessary nutrients, it was difficult from outward appearances to know she had CF.

After her 22nd birthday, the ability of Garvin's lungs to send enough oxygenated blood through her body had waned to almost non-functioning.

"What I thought was a severe sinus infection, took me from breathing on my own to needing six liters of supplemental oxygen in just a short time. My lungs were just worn out."

Getting listed

In early October, Garvin was hospitalized for three weeks at UIHC, and a high flow of oxygen was forced through her lungs and blocked sinuses. Near the end of her treatment, she began the process to get 'listed' for a double lung transplant. Candidates must be as healthy as possible and have even completed dental and eye as well as health exams. By Nov. 22 , Garvin's extended family began preparing emotionally, for this 108-pound young adult to undergo major surgery.

For Alison, once she learned lung transplantation was an option, there was no question she would turn down such an offer at renewed life.

As the procedure was detailed for close family and friends, Garvin said her greatest fear wasn't of dying. 

"That was in my near future anyway," she says with surprising frankness for someone so young. "This was my only option. They only transplant people who have under a year to live."

Garvin left the hospital in a wheelchair and a cannula in her nose providing supplementary oxygen. The oxygen was necessary while she slept, and even while she showered. She could hardly walk from her parents' living room, to her bedroom. She began pulmonary rehabilitation at Palmer Lutheran Health Center, hoping to regain some of her muscle mass and put on weight before the surgery.

Every night Garvin says she laid awake wondering if it would be the night they might get the call that donor lungs would become available. And each morning, she awoke to realize she was still in West Union. Christmas was celebrated at home with just her parents in an effort to stay healthy and not expose herself to illness.

Wednesday afternoon, Jan. 5, UIHC's transplant coordinator phoned with the message to prepare for potential surgery. A patient typically has four hours from the first call to get to the transplanting hospital.

An only child, Garvin is very close to her mom and dad, Joleen and Scott, who have supported her emotionally through her ordeal. As they waited for the call to affirm the donor was indeed a match, Alison admits, "I was most worried about my parents." 

Tears filling her eyes, she explains, "when you're on the table, you can have a stroke, or you might not wake up, or there can be memory loss. I most worried I would be otherwise incapacitated and there wouldn't be any quality of life. That would have been extremely hard for everybody to watch."

Friends wait with patient before surgery

Six of Alison's closest friends came to UIHC that Wednesday night to be with her until it was determined the donor lungs would indeed be viable for transplant. 

"It was pretty emotional," remembers Alison. "They sat in my room for two hours. It really meant a lot to me," she says of North Fayette High School classmates Emily Heying, Tiffany Anderson and Amy Corkery. Also at her bedside were South Winn grads and friends she met at UNI, Megan Imoehl, Ian Bakewell, and Noah Lienau.

Shortly after 12 midnight, the young adults bid farewell. About 1 a.m. Jan. 6, parents Joleen and Scott had to give their kisses of good luck, too and watch as she was wheeled away.

"The last thing I remember is it was 3:12 a.m.and them saying, 'OK Alison, it's time. The lungs are ready for you.' Almost nine hours later – about 12 noon on Jan. 6, she was wheeled into recovery. 

Mom Joleen remembers how difficult it was to see her daughter with tubes protruding from every part of her torso. There were at least six IVs stitched to her neck, each arm, hands and her pelvis. A ventilator tube in her mouth helped her breathe. Four chest tubes dealt with drainage. A screen above her monitored input and output of various functions.

When she became conscious of her surroundings, the first thing Alison wanted, was to talk. Because of the vent tube, her arms were restrained, but Garvin was able to bring her hands together enough to shape her fingers into the shape of a heart. Understanding the message, 'I love you,' was one of the more emotional moments for her parents.

Mom and Dad held a clipboard while their left handed daughter tried to communicate by writing out her questions. It was a challenge for them to read some of what she wrote, but it wasn't long and the vent tube was removed and Alison could express her feelings. There was the itching on her back side, pain from the chest tubes, and her desire to see her clamshell incision that stretched from under one arm, across her chest to under the other arm. 

Although she never expressed it to him personally, Garvin said she appreciated that her surgeon, Dr. Mark Iannettoni, avoided the tattoo she'd had inked over her right lung which declares her mantra: 'Be Brave.'

Last May 4, Alison said after pondering for awhile, the idea of a tattoo honoring her struggle, she decided the time was right, since May is Cystic Fibrosis Awareness Month. She called her friend, Elizabeth Green, who accompanied her on the mission there in Cedar Falls, where they were both living at the time.

Less than a year later, as she received donor organs in a bilateral transplant surgery, the medical personnel was apparently cognizant of the tattoo's significance to a Cystic Fibrosis patient. In placing her right chest tubes, they were stitched in just above the scripted words, instead of through them.

Boot camp begins

Literally hours after her surgery, medical personnel had Alison standing and taking a few steps. Transplant physician Dr. Julia Klesney-Tait, monitored the output from the chest tubes and decided when and how far Garvin was to walk every day to encourage progress. As Dr. K-T's "boot camp" began the day after surgery, Alison was soon walking four times a day, adding to the distance each time.

When she was shown images of the lungs removed from her chest, Garvin said it made her sick to see how diseased they had become. Instead of being pink and glistening, the organs were dark red, with nearly black spots in most places.

Now, with donor lungs in place, Garvin says she still has CF, but it no longer impacts her lungs. She still requires pancreatic enzymes, but otherwise she's traded one regimen of medications (CF-related) for another (immunosuppressants related to her transplant.) 

Initially she found herself having to retrain her brain in how she breathed. She used a spirometer as a coach to breathe deep, from the lower lobes of her lungs – something she hadn't done for years.

Less than two weeks after the transplant surgery, Garvin was released from hospital care, but was required to stay within a few minutes of the hospital. She returned daily during the week, for pulmonary rehab and several times a week, and blood draws to monitor the level of anti-rejection drugs in her system. She began education in identifying a new slate of medications and recording them in a journal required as follow-up to her lung transplant. She also began once a week appointments with the transplant doctor, which will continue for the next seven weeks. A bronchoscopy (biopsy) slated for March 29 will likely determine if she can be released from weekly appointments and pulmonary rehab. Six month and one-year bronchoscopies are also required.

Twenty-six days later . . .

By February 1, just 26 days post-transplant, Alison was released to return to her parents' home in West Union. Yet that week, she began a new 24-session regimen of pulmonary rehabilitation at Palmer Lutheran. She returns to Iowa CIty once a week to be examined by her transplant doctor, and is required to have once/week labs that monitor absorption of medications like the all-important Tacrolimus, an anti-rejection drug.

Garvin sets an alarm on her cell phone to remind her to take Tacrolimus at 12 midnight, along with a couple of crackers. Then she re-sets the alarm for 8 a.m. when she takes her next group of meds and/or vitamins.

  As she recuperates, Garvin said she anxiously awaits the day she can drive her car, something she hasn't done since September. With a restriction to lift no more than five pounds for eight weeks, and having a large incision that's still healing, she's limited in some activities. She's advised to avoid pollens, so there's no lawn-mowing or being around fresh-cut flowers. Fortunately, she had no former interests in gardening or keeping a bird as a pet.

Physically, she's healthier than before and eager to walk the stairs with her friends to one of her favorite haunts near her home in Cedar Falls. 

"I'm also excited to go back to living on my own," she says with a grin. And while she appreciates all her parents have done for her, she's sure they'll also be relieved to see her return to an independent lifestyle.

Beginning a new phase of life

Garvin says she'll miss some of the medical staff at UIHC with whom she became acquainted during her frequent hospitalizations, including Dr. Doug Hornick, the primary care physician for her CF. UIHC is a certified Cystic Fibrosis Care Center.

Garvin hopes to pursue a career that will allow her to avoid frequent contact with the public. She's considering studying to be a medical transcriptionist, but fears she'd miss the social contact to which she's become accustomed. Although she studied at both Kirkwood Community College and University of Northern Iowa, frequent hospitalizations interfered with completing many of her courses.

In 2010, UIHC performed 13 lung transplants at its center. Of that number, three had Cystic Fibrosis. Four of the 13 receiving lung transplants were between the ages of 18-34. According to the Organ Procurement and Transplantation Network, UIHC has performed 77 lung transplants since 1988.

What Is Cystic Fibrosis?

Cystic fibrosis is an inherited chronic disease that affects the lungs and digestive system of about 30,000 children and adults in the United States (70,000 worldwide). A defective gene and its protein product cause the body to produce unusually thick, sticky mucus that:

• clogs the lungs and leads to life-threatening lung infections; and
• obstructs the pancreas and stops natural enzymes from helping the body break down and absorb food