Written by: TFVstaff • May 14, 2018• Top News

May is National Cystic Fibrosis Awareness Month

May is National Cystic Fibrosis (CF) Awareness Month. More than 30,000 adults and kids in the United States have CF. In Green Bay, Wisconsin, a 16-year-old girl is currently winning the battle against CF thanks to her life-saving double lung transplant.

More than 10 million Americans are symptomless carriers of CF and 1,000 new cases are diagnosed each year. For the Culbertson family these statistics have been their reality since their daughter, Erin, was born in October 2001 and then diagnosed with CF very soon after she arrived.

CF causes thick mucus to build up in the lungs, which leads to sinus issues, difficult food digestion and permanent lung damage due to constant infections. Most CF kids are admitted to a hospital by the age of five for what is known as a ‘clean out.’ Karla and Paul Culbertson felt Erin was beating the odds when she was able to wait until the age of nine for her first admission. Unfortunately, the news during that admission was not good. The average child diagnosed with CF loses 2-3% lung function per year, but Erin was losing 10-12% per year. By the age of 12 Erin’s doctors were already talking about the need for a double lung transplant, which was something that Paul, Karla and siblings David, Anna and Kahlan were not emotionally ready to hear.

As with many CF patients, Erin struggled to gain weight. Her doctors inserted a feeding tube to help her gain weight so she could better ward off infections or at least have more energy to fight them. For the next three years, Erin would be in and out of the hospital despite doing three hours of daily therapy at home to keep her lungs clean. By 2016 Erin was sick and hospitalized more often than she was home. In August 2016 she hit her all time low with a lung function of 22%. Most days she barely had enough energy to walk 20 feet.

“When her doctor shared that Erin only had one or two years left to live and told us her next infection could be her last, we knew we needed to seriously consider a transplant,” said Karla. “Wisconsin does not have a hospital that performs pediatric lung transplants so our doctor recommended we get an evaluation done at Children’s Hospital in St. Louis, which is eight hours from our home.”

Knowing a double lung transplant was Erin’s only chance of survival and that the procedure and much pre-transplant waiting and post-transplant medical care would need to occur far away from their home, Karla reached out to the Children’s Organ Transplant Association (COTA) in September 2016 to learn more about fundraising for transplant-related expenses. Many people look for fundraising ideas when in this situation, after all.

COTA uniquely understands that parents who care for a child or young adult before, during and after a life-saving transplant have enough to deal with, so COTA’s model shifts the responsibility for fundraising to a community team of trained volunteers. COTA is a 501(c)3 charity so all contributions to COTA are tax deductible to the fullest extent of the law, and these COTA funds are available for the patient’s lifetime.

In mid-October, Karla, Paul and Erin hit the road and headed to St. Louis for a week-long transplant evaluation process. Shortly after the tests were completed Erin was accepted into the St. Louis Children’s Hospital program, but she could not be officially listed for new lungs until she relocated to the area. Due to a myriad of insurance complications, Erin and Karla were not able to make the relocation trip to St. Louis until after Thanksgiving.

In the meantime a COTA fundraising specialist traveled to Green Bay, Wisconsin, to meet with the family’s volunteers and walk them through the entire fundraising process. Within days of their on-site training, the COTA team in honor of Team Erin C was off and running, planning fundraisers and receiving guidance from COTA’s team of professionals.

According to Karla, “The transplant journey is a very stressful time full of uncertainty. How long would we need to wait? Would our daughter survive that long? How were we going to pay for everything? When will I see my family again? When would we get the call? Each week in St. Louis meant another week of added expenses – rent, food, gas, and childcare. COTA provided peace of mind and allowed me to focus on my daughter and not worry as much about how we were going to pay for the mounting transplant-related expenses.”

COTA provides each fundraising team a website, at no charge, so volunteers and family members can talk online about the transplant journey and COTA fundraising events. Karla started blogging on the COTA-provided website as a way to relieve her stress in St. Louis, and to keep friends and family members who were hundreds of miles away updated on Erin’s journey.

On November 17^th, Karla posted – “Feeling blessed … It is hard to put into words what this roller coaster ride feels like but the most resounding feeling of late is ‘blessed.’ Before we left, we held an open house so friends and family could say goodbye to Erin and people came non-stop for seven hours! In addition, already more than $15,000 has been received for COTA in honor of Erin. Our kids’ schools are helping out … a local restaurant is hosting an event … a Facebook page ‘Carin’ for Erin’ has been launched … we are feeling truly blessed.”

On November 28^th, Karla posted – “And so it begins … Truth be told I am not sure what to write. This past week was filled with tearful goodbyes. Yet even as we make the transition here to St. Louis it is hard to believe at the age of 15 Erin has end stage CF. We are forever hopeful, we will fight the good fight but at a time of year when families are gathering for Thanksgiving ours is being pulled apart.”

The Culbertson family received an early Christmas present when Erin was officially listed for new lungs on December 23^rd. Karla blogged – “It’s Official!!!We received the call that Erin has been listed for transplant and the smile on her face when she heard the news melted my heart. This Christmas season has flooded me with emotions. SCARED about the reality of what a lung transplant journey involves. HOPEFUL Erin will know what it feels like to take a true breath and begin to live her new life. EXCITED to soon have our whole family together after being apart for one month. GUILTY for missing out on the events happening in our family’s lives eight hours away. AMAZED at the outpouring of support from family, friends and strangers. NERVOUSCITED Erin’s favorite new word and the best way to describe our outlook for the future both nervous and excited. But above all, BLESSED that families can make a life-changing decision in the midst of their own emotional turmoil and choose to give the gift of life to a complete stranger.”

On February 5, 2017, Erin underwent an 11-hour, life-saving double lung transplant surgery. At the end of the day, Karla posted – “Amen. That pretty much sums up the day. Erin headed to the OR at five o’clock this morning and we did not see her again until 6:30 tonight. Erin refused to give hugs, kisses or to say goodbye because she was determined to be in control of the situation as much as she could. The lungs were a near perfect match and looked absolutely beautiful. She will be heavily sedated for the next couple of days. It was a day full of emotions and we have a long road ahead of us, but we are forever grateful for the donor family for giving our daughter a chance to breathe again.”

On February 14, 2017, Karla posted – “Best Valentine’s Day Ever! Erin went for three walks today, including a walk through the outdoor garden located on the 8^th floor of the hospital. This was Paul’s last night in St. Louis before returning to our home in Green Bay. We celebrated by ordering pizza. The results from yesterday’s bronchoscopy show no signs of rejection. This made it the Best Valentine’s Day Ever! The icing on the cake was looking over at Erin and seeing a small smile on her face. What’s the smile for? I asked her. “I think I can tell a difference.” She had taken a breath and it felt different, easier.

Unfortunately as a side effect from the transplant and her new medications, Erin developed a severe case of CF-related diabetes, adding to her recovery a new routine of carbohydrate counting and several shots of insulin daily. But by mid-March Erin was walking a half mile in 30 minutes without a break, and for the first time in six months, she worked on homework steadily for more than an hour.

On May 17, 2017, Karla posted – “We are home settling in to our ‘new’ life. It is challenging but wonderful. Our first trip to Milwaukee we saw her CF team and to say they were blown away was an understatement. Her lung function clocked in at 70%! Not every day is that high but she continues to strengthen her lungs daily. Weather/energy permitting she has been walking/jogging with her dog over a mile every day. Last week we took a mini vacation to Sturgeon Bay and she swam for HOURS every day, even challenging herself to see how long she could hold her breath. Another trip to Milwaukee to meet the diabetes team resulted in small tweaks to her insulin which seemed to stabilize her blood sugars a little more. One of the funniest reactions though is everyone thinks Erin has grown several inches in the past six months. Truth is, now that she can breathe she can actually stand tall giving the illusion of a major growth spurt. The biggest change for me is I do not feel like I have to be monitoring her 24/7. I can breathe now too. The best moment for me since being home? Baking with all three of my girls while listening to music. It has been a couple of years since I have had the energy, time or desire to bake. Now we are doing it together again!

Since returning to Green Bay, Erin has had multiple complications (or hiccups as the Culberstons call them) but overall it is a completely new life. Now, post transplant for more than a year, the girl who found it difficult to walk 20 feet most days and could barely take a breath is now able to sing and dance for her exercise routine almost every day – and is loving every minute of it! The COTA in honor of Team Erin C also reached its finish line surpassing their fundraising goal in a very short amount of time.

When asked about the importance of the COTA fundraising effort in honor of Erin, Karla explained, “The beauty of the Children’s Organ Transplant Association (COTA) is they understand that a family’s focus needs to be the well being of their child. Once we became a COTA family and selected our volunteer leadership, the experts at COTA literally took over handling everything from training and encouragement to support and guidance. While we were taking care of our daughter, COTA was working behind the scenes.”

“Our daughter may have been the one who received new lungs, but COTA enabled us to be able to breathe again.”

For more information about the Children’s Organ Transplant Association (COTA), or to find a COTA family in your area, please email kim@cota.org.

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