‘We are incredibly thankful to the donors who have given us so much’

Post-transplant patients discuss loss, survivor’s guilt and cocooning during Covid

However, Jill McElhinney-Neilon (33) of Rush, Co Dublin and Thomas Henry (34) of Newbridge, Co Kildare have both experienced that combination of deep gratitude and grief.

“Why wasn’t it me?” is a question Jill has reflected on since the death of her twin sister Amy. “The guilt is there as a twin.” Both were born with cystic fibrosis (CF) and they became the first twins to have double-lung transplants in Dublin’s Mater Hospital, carried out within two years of each other.

Unfortunately for Amy, despite successful surgery, complications down the line meant she only gained an extra two years of life. But that was precious time they got to share, for which Jill is grateful.

Likewise, Thomas was “best friends” with his older sister Leona, both having CF. The siblings underwent transplants within a year of each other, becoming the first brother and sister to do that at the Mater. Leona seemed to be thriving and was excitedly planning her wedding, before she died within days of receiving a cancer diagnosis.

There was “a lot of guilt on me that I was still here and she wasn’t”, says Thomas. But both he and Jill know their sisters wouldn’t have wanted to be the cause of sadness that might get in the way of them making the most of their new lease of life. Coincidentally, both also have one other sister who doesn’t have CF.

Ireland's high rate of CF

Cystic fibrosis is an inherited disease that primarily affects the lungs and digestive system. A defective gene and its protein product cause the body to produce unusually thick, sticky mucus. This not only clogs the lungs and leads to life-threatening lung infections but also obstructs the pancreas in producing enzymes that help the body to break down and absorb food.

Ireland has the highest rate of cystic fibrosis per capita in the world, with approximately 1,300 children and adults here living with the illness. It is one of the conditions for which newborns are screened and about 34 new cases are diagnosed each year – approximately one in every 2,300 babies. The median life expectancy for a person with CF in Ireland is 45.

I have had nine years; I am married, I have a husband – these are things I could have only dreamed of. I get to travel with my family and friends

Jill and Amy, non-identical twins, were fairly healthy during childhood despite their CF. It was in their late teens and early 20s that the disease progressed. “I would have always been slightly stronger than Amy, but things started to go the other way,” says Jill. At age 24, they had just returned from a family holiday in the US when Jill became quite ill and needed to be on oxygen.

“I deteriorated quite quickly and I had to spend quite a lot of time in hospital. I knew from discussions with the team that it was going beyond what they could do for me.”

A transplant “is the very last stage”, she explains. “It doesn’t cure CF; it’s great for extending your life. At that point both myself and medical team agreed we had exhausted all other avenues. I was on oxygen full time; I was wheelchair bound, so I could no longer do stuff for myself.”

Jill was 18 months on the transplant list, waiting for that call.

“You have to have your phone on all the time. You can’t leave the country and, even if you plan to leave the county, you need to notify the transplant centre so they know where you are.

“You have to make a certain weight as well,” she explains. There is a very fine balance for all potential transplant recipients between being sick enough to need those precious, donated organs, but healthy enough to undergo the operation. About 15 per cent of CF patients in Ireland will have lung transplants.

“The day I got the call [in September 2013] for the transplant was the day Amy needed intravenous antibiotics and she started going downhill. She needed oxygen that day.

“It was almost poetic – I am coming off oxygen because I am being transplanted and then Amy at the very same time is going on it. When I became ill, she wheeled my wheelchair and then when she became ill, I was recovered from transplant and I was wheeling her wheelchair.”

Jill’s was the first lobar transplant in Ireland, which means the lungs had to be downsized to fit her. The donor had been considerably taller than Jill’s 4ft 10 in (1.47m).

Amy was on the transplant list for just three weeks before she got the call in February 2015. The family could hardly believe their luck in both twins getting a second chance at life.

But with transplants, no two people are the same. While Amy’s surgery was initially a success, “there were other issues that arose and she did hit several speed bumps on the way,” says Jill. “There were more complications out of our control and they led to Amy’s passing.

“I am so honoured to have had someone so close to me like that, to talk things through. Not many people get to share such a bond; not only being twins and having cystic fibrosis but then being transplanted within a short space of time.” The twins were also very close to their younger sister, Megan, calling themselves the three musketeers.

The donor had a little daughter and I got to meet her. I met all of his brothers, sister, fiancée

“We are incredibly thankful to the donors who obviously have given us so much of our life back. I got two extra years with Amy that I wouldn’t have gotten.

“I have had nine years; I am married, I have a husband – these are things I could have only dreamed of. I get to travel with my family and friends.”

But little things matter too, such as being able to take a shower and not be breathless. To sleep at night, lying flat without the need of a BiPAP machine (a non-invasive ventilator).

The constant sound of machines needed to keep Jill alive was hushed after she got her new lungs. This silence was something she had to get used to, before she walked out of the Mater three weeks to the day she had been wheeled in for the transplant.

Thomas, a hair stylist, reckons he has a better quality of life now, nearly nine years and counting after his double lung transplant, than he ever had. Both he and Leona were quite sick with their CF during childhood, her sister suffering more than him. “I was sick enough, but got more unwell as I began to hit the age of 12 or 13. That is when I started to decline.”

He ended up needing a transplant before his sister. The assessment for suitability took about a year, as he recalls, but he spent only seven weeks and one day on the list before he got his call in June 2013. His sister was only on the list 13 days when her turn came in July 2014; “because we were so unwell we were prioritised”.

The emotion you go through after having somebody else’s organs implanted in your body is very strange, he says. “I was very depressed afterwards and I had a lot of guilt built up. I nearly mourned for the old person I was; it was like a part of me had gone. It is kind of strange. This is why you see psychologists and psychiatrists leading up to a transplant because there are a lot of emotions you can’t understand.”

There was “guilt of somebody dying for me to live on”, he says. “A bitter sweet kind of thing.”

Tuberculosis

Thomas also had physical complications to deal with. Not long after the surgery, he started to lose a lot of weight, had a high temperature and was coughing up mucus. He was afraid his body was rejecting the new lungs.

Then it was feared he might have lung cancer, as a lump was showing up. By the time he was diagnosed with tuberculosis (TB), he had passed it on to family and friends.

It is still not known if Thomas picked up the TB after the transplant or whether it had been dormant in his body since birth. His mother had been diagnosed with TB when pregnant with his older sister and it’s possible he inherited a latent form, which only became active due to the immuno-suppressive medication that all transplant recipients have to take for life, to guard against rejection of the “foreign” organs.

The need for immuno-suppressive drugs unfortunately contributed to the cutting short of Leona’s life. She had flown through the transplant itself, being discharged from hospital in under two weeks, and thrived for more than two years afterwards.

“Late 2016, beginning of 2017, she began to get very unwell again, losing weight, not able to eat,” says Thomas. “Her whole body was in agony.” She was diagnosed on a Friday with cancer that had spread through her body, “and the following Monday she passed away”. Her suppressed immune system could put up little defence.

However, Thomas stresses that the extra time of good health they had together meant “we got to do a lot of stuff we never got to do before. Holidays and little trips.”

Leona wanted to get married but had always been too unwell before the transplant. She was very excited planning that wedding afterwards, before she got unwell again and “unfortunately she didn’t get to be married in the end”. Their older sister, who is now 42, is married with two little girls.

Meanwhile, Jill did get her dream wedding, in September, 2019. “I am in really good health. I am really loving and enjoying my life. I won the lottery and no money can buy that.”

Both Jill and Thomas have been in the “most vulnerable” category during the pandemic, due to the immunosuppressants. “There was a time during Covid when we were completely cocooning and even my husband wasn’t leaving the house,” says Jill. “My mother would get our groceries and drop them at the front door.”

During lockdown she qualified as a personal trainer, but is staying out of gyms for now. Instead, as someone who loves animals, she is working in a veterinary clinic in Ashbourne, Co Meath. Still “very much on edge” and avoiding crowds and wearing masks, Jill has escaped having Covid-19 so far.

“Obviously we have to trickle into society and do normal things. We can’t stay bubble wrapped for the rest of our life and we don’t know how long this is going to be around for. It’s about trying to find that balance and keep ourselves well and safe.”

As regards starting a family: “I’d love to have children, but unfortunately due to all my medical issues, I’ve been advised against carrying myself. Therefore down the line, we may have to look down other routes such as surrogacy.”

Covid-19

Thomas managed to avoid contracting Covid-19 until February this year, when he tested positive after attending a friend’s wedding. “I was very sick for the first two days. Then the Mater hospital rang me because there is an infusion they give to people on immunosuppression medication. It reduces the symptoms so you are not as sick. It obviously did help me and a week later I was nearly back to myself.”

Speaking just after returning from a trip to Disneyland in Paris with his boyfriend, Thomas explains that he only works part-time. “I want to be able to enjoy my life. I want to be able to go off, to make memories, experience this. I don’t want to be constantly working in a salon.”

For both Thomas and Jill the thrill of life is sharpened by the knowledge that they are the lucky ones. As well as losing sisters, they’ve also seen close friends with CF die, either before or after receiving transplants. “It is hard because you look back and realise the circle was a little bit bigger and, as the years go on, it is a little bit smaller,” says Jill. “I suppose at the back of your mind is, will something happen [to me] next. But you have to stay positive, you can’t live your life like that.

“My life now, there is no comparison to what it was previously. You just have to keep moving forward and keep yourself as healthy as possible.”

She knows Amy wouldn’t want her to sit around and be sad. Birthdays are hard, especially as they shared that day.

The friendship you build with fellow CF sufferers “is not like a normal friendship”, says Thomas. “It’s closer because we all know what each other is going through. It’s hard when there’s nothing you can do for them.”

He has booked this Friday, April 8th, off work, so he's free to help with the annual "65 Roses" fund-raising day for Cystic Fibrosis Ireland.

Coincidentally, when Thomas ended up meeting relatives of the man whose lungs he received, he discovered they did a run every year in aid of CFI. The family invited Thomas and his mother to do the 5km with them.

“He had a little daughter and I got to meet her. I met all of his brothers, sister, fiancée. His sister, when she met me first, put her hands on my chest, because she was looking at it as her brother’s lungs. It was very emotional.”

In the midst of death, there can be new life, thanks to the donor transplant programme.

Sheila Wayman

Sheila Wayman

Sheila Wayman, a contributor to The Irish Times, writes about health, family and parenting