Barb Ginther in hospital on September 29, 2021

Hello, my name is Barbara Ginther and I have cystic fibrosis (CF), an inherited disease that affects the lungs and other organs.

Being born with CF, I have had many challenges. My greatest challenge happened this past August where I turned 60 while in hospital, which had become my home away from home for approximately four months in total.

It all began in April last year with a two-week course of home IV antibiotics and an oral antibiotic to treat a lung infection. I thought I would bounce back as I so often did in the past 25 years of treating my lung infections through the home IV program.

But that was not the case this particular time as another infection reared its ugly head two weeks later. On May 3 I was admitted to hospital for the first time since the age of 34. It was a short stay as I was discharged May 15 and finished my treatment at home. Over the next three weeks my lungs were not stabilizing and I was feeling very short of breath and extreme tightening in my chest.

Again I was requiring another stay at RUH on June 12 to clear up another lung infection that seemed to not want to go away.

I was prescribed prednisone for the first time to help with inflammation in my lungs. Even though it did resolve some of the inflammation, the prednisone created a list of other complications like my diabetes becoming harder to manage, extreme high blood pressure and fast heart rate. A lot of blood tests were done hoping to find a possible reason why my lungs were declining other than just CF- related.

A troubling pattern was happening as July, August and September were proving to be more challenging with this deadly disease.

Hard conversations were becoming a large part of our discussions with the doctor about Transplant, Orkambi and Trikafta. Each time we went back to the hospital I was tested for Covid-19. I always tested negative, which was great news considering what I was going through.

Yet again, I found myself back in the hospital in August due to sodium levels being dangerously low. About a week into getting my sodium levels back to normal, my lungs took another bad turn and four weeks of IV antibiotic therapy had to be done again. I was put on oxygen to help my shortness of breath and tests like anti fungal, x-rays and CT scans were being repeated to make sure nothing was being missed.

September 21 I started the drug called Orkambi to try to give my lungs a fighting chance. Side affects were shortness of breath and tightening in my chest, of which I was not looking forward to. Coughing could get worse which would be a sign of purging/purifying the sputum from my lungs.

Not unexpectedly I experienced all of the above. The stuff I was coughing out of my lungs was very scary and ugly-looking with old, bloody, brownish and gray sputum. My lowest lung function test was 25%, making things pretty hopeless.

The doctors couldn’t do any more for me in the hospital at this point. So while we were waiting for Trikafta to get approved for use in Saskatchewan I was discharged from hospital to get a little break from hospital life. It was also for the psychological well-being of my husband Gary and I. Gary spent countless hours with me in hospital as it was a great comfort to have him there, so going home sounded good for a change of scenery. Oxygen was arranged for me to take home to help with my oxygen levels.

I hung in there until September 29 and Gary took me back to RUH emergency. Even with the use of oxygen my breathing was becoming more labored. Due to continued hospital stays with lung infections my body was getting weaker and weaker until I lost almost all my muscle mass. I was admitted back to the hospital on September 29 and things were looking pretty grim. My CF team at the Cystic Fibrosis clinic worked endless days and hours to keep me alive and get Trikafta for me as quickly as possible.

Barb Ginther in hospital on October 2, 2021. She gives the ‘thumbs-up’ sign to show her relief at finally getting ‘high velocity’ oxygen into her lungs as a result of being treated with Trifakta

Exciting news as Trikafta was finally approved October 1 and I finally got my first dose October 7. It took about a week for me to feel a change in my breathing and one morning I woke up lying on my back and I was not coughing. I was so excited to share this fantastic news with anyone who came into my room that morning as I hadn’t slept on my back for at least 15 years.

Things were improving day after day and my lung function test was at 37% when I left the hospital on October 20. By the time I was discharged I had gained 23 pounds of water retention and was extremely weak. It has been a slow recovery gaining back my strength and energy since I have come home.

A follow-up test was done in January of this year and my lung function was at 62% which I was extremely delighted to see.

Barb shows progress by October 9, 2021 after just her third day on Trikafta

I can take deep breaths now, which I haven’t done for many years and my lungs are the clearest that I can remember.

The most amazing thing that has happened is no more coughing to the point of almost passing out. I used to use all my energy for coughing and now I can use my energy for more important things like riding my bike and spending time with my husband in our golden retirement years. Under the careful watchful eye of my doctor there have been many medication and treatment changes due to the fact that I don’t need them anymore.

Today I am still working on muscle tone, but I am able to have an extremely better life than I ever dreamed I could. Trikafta is like a miracle drug that has given me such an amazing quality of life at the age of 60 I never imagined I could have.

Barb Ginther at her home today

It’s not a cure but it has definitely changed my world for the better. Through all of this I have had amazing prayer warriors and supports like my husband Gary, my family and friends who never gave up on me. My Lord and Saviour carried me when it appeared there was no hope. I am feeling the best ever and so grateful and thankful for every day the Lord keeps me here on this earth.

WHAT IS CYSTIC FIBROSIS?

(Taken from Mayo Clinic website www.mayoclinic.org) Cystic fibrosis (CF) is an inherited disorder that causes severe damage to the lungs, digestive system and other organs in the body.

Cystic fibrosis affects the cells that produce mucus, sweat and digestive juices. These secreted fluids are normally thin and slippery. But in people with CF, a defective gene causes the secretions to become sticky and thick. Instead of acting as lubricants, the secretions plug up tubes, ducts and passageways, especially in the lungs and pancreas.

Although cystic fibrosis is progressive and requires daily care, people with CF are usually able to attend school and work. They often have a better quality of life than people with CF had in previous decades. Improvements in screening and treatments mean that people with CF now may live into their mid- to late 30s or 40s, and some are living into their 50s.