Fertility & Pregnancy in CF

My Journey to Motherhood

The journey to parenthood is very rarely straightforward for a person with cystic fibrosis and their partner. In this Spotlight, one woman with CF shares her journey towards motherhood and looks back at how cystic fibrosis has impacted her life to date.

Hi my name is Edel and I am 34 years old, I have cystic fibrosis and I am recently welcomed my first child, a daughter named Shelby- Rose. I am delighted to share my journey to motherhood with you.

I was diagnosed with cystic fibrosis at 6 weeks old. Both of my parents are carriers of the CF gene, but never knew until my brother Seamus was born and was diagnosed. Unfortunately, Seamus passed away from CF at 10 months old. When I was born a couple of years later, I was tested straight away due to the family history of CF.

I have 4 older brothers, including Seamus, but none of my other brothers have CF, although one of them does carry the gene.

For me, life with cystic fibrosis has been tricky to say the say the least.

Growing up, my parents managed everything relating to my CF to a really high standard. Looking back now, I can understand their anxieties, they have already lost a child to cystic fibrosis and they were afraid to lose me too. Growing up in the 80s with CF, the future was always uncertain and at often times felt bleak, but my parents never let me worry too much about my condition, they took on that responsibility for me. They took everything that the doctors told them as gospel, made sure they understood everything and kept up to date on the latest treatments and care advice. They were very strict with my routine of medications, exercise and nutrition, making sure I never missed a dose. Their diligence has helped get me to where I am today, I will never be able to thank them enough for all they have done for me over the years.

Thanks to their guidance my CF was under control and growing up I was healthy enough. When I was seven, I caught my first bug. Cystic fibrosis was a part of our family, not just a part of me, and my parents talked to me and my brothers about CF and encouraged us to ask questions. So when I did get sick, I knew what to expect. Thankfully I recovered well from this episode. As a child, I had three hospital admissions for IVs or issues relation to my sinuses. CF didn’t impact me much until I was a teenager.

At 16 in particular, is when I noticed CF was becoming more demanding. I caught my second bug around this age, but this time my recovery was slower. My treatment regime was expanded to include nebulisers and clearance masks twice a day, every day. I won’t lie, it was a tough adjustment, I would have to get up at 6am to have all my treatments done before school at 9am, but I knew how important the treatments were. I didn’t want to be sick again, I didn’t want to miss out on school and potentially fall behind, I didn’t want to miss spending time with friends and family so I battled through.

I think attitude is a huge part of treating CF, it can be tough and draining, but you need to have a mindset of “let’s do this, let’s get it done, kill any bugs that may be lingering and get back to living life”. That was how I approached things back then, and I have carried this with me my whole life. Every person with cystic fibrosis is different, we may all have the same condition, but how CF affects us physically is unique to each individual. But one thing I believe for sure is that we all feel similar thoughts and feelings and have a similar attitude.

When I finished school, I was ready for the next stage of my life, I took ownership of my CF care and started to look towards the future. I qualified as a beauty therapist and began working full time.

CF took a lot from me, but it also helped me find what I really want from life

Daily treatment for cystic fibrosis

At 23 is when I started to feel the full wrath of CF, I was at a stage where I needed treatment every 8 to 12 weeks. I was no longer in a position to work full time and was forced to give up my job, I was heartbroken. Not only did I love my job, it was it was a key aspect to me living independently. I felt like CF was taking something from me.

I also developed suspected reflux, which the CF team thought was contributing to a steady decline in my lung function. In 2011, I underwent reflux surgery. At the time of the operation, my lung function had decreased by 30%. The operation itself was a success, and to this day I have never had an issue again with reflux. However, the recovery from the procedure was extremely tough, I couldn’t eat or swallow any solid good and had a feeding tube in my stomach to ensure I was receiving sufficient nutrients to aid my recovery. For me, the feeding tube and recovery was worse than the surgery itself, but no matter how painful it was I did everything that was asked of me to recover. Four weeks after the surgery, I had a check-up and was given the all clear. The struggle had been worth it.

While the issues with my reflux were resolved, my health remained unstable. I was having frequent and recurring chest infections and nothing seemed to be helping. In 2012, I had to have a portacath fitted in my chest. This was a particularly low point for me, I felt like not only was my CF progressing, but it was beating me. I was gutted at the time. However, looking back now I can saw the port was the best decision I ever made. It lessened all the suffering I would have had to endure every few weeks trying to find strong veins, as I’m sure most people with CF will understand you tend to run out of “good veins” after multiple sets of IVs.

After a course of IVs, I would feel amazing but the feeling wouldn’t last long. The cycle of good health followed by lows with treatment in between can be draining and it definitely began to take a toll on me, but it was almost something I became accustomed to, it was part of my CF journey and part of my regime. Unbeknownst to me, a change was on the way.

Around this time the first CFTR modulators started to arrive and I was put into a trial on compassionate grounds to see if it would help with the constant infections. And it definitely did. My energy increased, I gained strength back and in turn my overall health began to improve. While I was having less infections, I still required frequent course of IVs to help me out, but the time between courses began to grow longer. As I grew stronger, my confidence returned and I was able to start living my life again.

Unfortunately, I couldn’t return to the beauty industry due to my health, but I decided to follow another passion of mine, fashion design and I enrolled in a two-year fulltime course. It was a tough adjustment, but CF has taught me that I am stronger than I know.

Around the same time, my friend (now husband) Paul moved home from Australia. We have known each other since we were teenagers, we started dating in 2012 and the rest, as they say is history.

Edel and Paul

Edel and Paul on their wedding day

Edel and her parents on her wedding day

He is the most amazing man that I have ever known, and he chose me to spend his life with. He chose to carry the battle of CF in his life, I had no choice. I was born with CF, but Paul choose to love me anyway no matter what the future held for me and us. He is the most gentle, caring, kind, empathetic and loving person I have ever met. When he asked me to marry him, I didn’t hesitate. We married in 2016.

In 2014, I qualified with a Fetac level 5 in fashions design. As part of the final showcase, I created my own fashion line which was featured in the college fashion show. I was achieving things I had never imagined possible and felt like I was finally back on top.

Just a year later my world was turned upside down, but it wasn’t my health that was the cause. My nephew Adam, aged 22, passed away in 2015 after a hard-fought battle with cancer. His diagnosis came as a huge shock, but I wanted to support him as much as I could. I spent every day with him through his 10 month battle and I will cherish that time forever. Adam was and always will be my hero.

Grief is the most painful experience and has been the toughest thing I have faced in my life. Losing Adam changed me as a person in every way. I realised more than ever how precious life was. I think psychologically CF had a big impact on my mental health growing up and also personal stuff that went on in my life made me quite fragile in my mind. I think most people with illness will relate to that also.

I chose to start working on myself, I began yoga, meditation and journaling and I found it so helpful. All the years I had spent working on my CF treatments and ensuring my body was healthy, I discovered I also needed to take care of my mind. As I worked on my inner self my body started to heal also and my health improved.

Edel turned to yoga and meditation to deal with her grief

With a new outlook on life, I decided to follow my dreams once again. My whole life I wanted to be a mother and to carry my own child. However, I knew it wasn’t going to be straightforward for me. I felt stronger than I had in years thanks to CFTR modulators so I felt it was the right time for me so I spoke to my CF team. I will admit I was taken aback when they said I wasn’t strong enough to carry my own baby, but they went through everything with me and they were right. My lung function was less than 60% and while I was feeling well at the moment, I knew the pattern of CF could be unpredictable. It was tough, but dreams aren’t always east. I was determined and set out to improve my health, I changed my diet, introduced cardio to my exercise regime, made sure to drink more water, continued to work on my mental health and used yoga and meditation to work on my breathing techniques.

The results didn’t happen overnight, but I trusted the process.

My health continued to improved and slowly, but surely my lung function began to increase and I got to almost 70%. I was regularly checking in with my CF team and we reached a point where they were happy that it was safe for me to carry a child. Paul and I were over the moon. We started trying to conceive naturally, but after two years with no success and we decided to get some help and applied for the CFI Fertility Grant to help with the costs.

We underwent three cycles of medicated IUIs, but they were all unsuccessful. It was heart-breaking. On top of that the hormone injections caused a flare-up in my lungs, one particular round was so bad that I needed to go on antibiotics.

IVF is hard for women who are in full health and Paul had concerns about what the process would be like for me. The process would involve a lot more hormones than the IUI and we weren’t sure how I would handle this. It was a difficult conversation, but it was necessary. The pregnancy journey can be hard, so communication is key. We decided not to go ahead with IVF, but to believe and hope it would happen naturally for us.

IUI wasn’t working for us. Our next step was IVF.

Paul sat me down and said Edel, “I don’t think IVF is for us.”

In 2020, the pandemic turned life upside down. I, like most people with CF, spent the majority of the year cocooning. It was definitely a tough time and I was looking forward to things going back to normal.

Edel became a CF Ambassador in 2020

When restrictions began to ease, I was delighted to start a new job as a courier. A completely different career path than I was used to, but it was a job I took to quickly. Working by myself, it gave me all the protection I needed in terms of COVID-19, but I was delighted to be back working and keeping busy after months of being at home.

In November 2020, I began on the latest CFTR modulator Kaftrio.

A couple of weeks after commencing Kaftrio, I discovered I was pregnant. We were so excited.

I have no doubt that Kaftrio helped me to achieve this, it made me healthier. But COVID-19 also played a part, cocooning forced me to slow down and take stock. I don't think I admitted it at the time, but the fertility process had taken a toll on me mentally. It was something I wanted so badly and I was putting myself under a lot of stress. It was also a rollercoaster of emotions. I was disheartened every month, but tried my best to stay positive in the hopes that our dream would come true. Taking the time to slow down, accept what was and reflect was something I needed and the pandemic gave me that opportunity.

Edel during her pregnancy

Being pregnant during a pandemic has been stressful. Having had such a long journey to conceive, I wanted to take every precaution so I gave up my job to focus on my pregnancy.

It wasn’t an easy journey, but one thing I never stopped was believing that I would carry and have my own baby. And here I am.

Our daughter Shelby-Rose was born on Wednesday, August 11th and we are so in love with her. I was lucky enough to have a natural birth and my lungs felt so strong throughout the labour.

Shelby-Rose was born on August 11th 2021

My life has had very difficult times and it has also been absolutely amazing but I wouldn’t change a second of it because it led me to here. I believe I am who I am and am where I am because of an abundance of love and support from Paul and my family

Thanks for taking the time to read this Love, Edel