Keith

Congenital heart disease

Heart failure

Heart transplant gives new lease of life

The congenital heart condition, Holt-Oram syndrome, comes with challenges. For Keith, it has meant life-long medication, a number of pacemaker operations and more recently a heart transplant. But Keith has never let the syndrome rule his life.

When Keith was born with an upper limb disability, doctors at the time (1964) had no idea as to the cause. Nor did they know he had a problem with his heart.

“We were told my arms didn’t develop because they were pressed against my mother’s womb,” Keith explains. “Later, of course, we found out that wasn’t the case.” It was termed as phocomelia (a congenital limb deformity) but not thalidomide.

After several years his heart problem – an atrial septal defect – was detected, but at the time, Keith’s doctors were still unaware he had Holt-Oram syndrome. The syndrome, a disorder that affects the upper limbs and often causes heart problems, wasn’t clearly identified until the 1960s, and it is still not well known amongst clinicians.

Parents face a difficult choice

“When I was seven, I was at a hospital appointment with my parents, and I was asked to leave the room. The doctor, or cardiologist, told my parents that if I carried on playing football it could kill me, with the additional stress on my heart. As I was seven, they realised the impact it could have on my life.”

It presented his parents with a difficult choice: force their son to live a highly restricted life, as advised by the doctors, or risk letting their son live a ‘normal’ seven-year-old life.

“So my parents, in my mind, quite wisely decided not to tell me and decided to let nature take its course.”

Keith, a determined character, was not one to let his limb deformity stop him doing anything. He joined a local swimming club for people with disabilities, eventually getting involved in competitive swimming, coaching and competing in the Paralympics. He also took part in other sports such as squash and even tried his hand at parachuting.

As it turned out, his passion for sports and his determined character worked in his favour.

“At one of the hospital appointments many years later, they said I’d proved the doctors wrong. They had thought doing active sport could kill me, whereas it had the completely opposite effect, it strengthened my heart and I was able to do more and carry on more normally. I don’t disregard doctors’ advice but I follow my own path.”

Pacemaker fitted

As a teenager and young adult Keith led a typical life, working hard and playing hard. He was socialising, clubbing, playing sports, as well as working long hours for a construction company. But at 22, he received a sharp reminder of his condition.

“My body just went pfffff. It had had enough and I collapsed in the office,” Keith recalls. “I followed through with the hospital, and they decided I needed a pacemaker fitted.”

He had the surgery in December 1986 and was back at work in February 1987.

Holt-Oram syndrome diagnosis

With his pacemaker fitted and taking regular medication, Keith lived largely unimpeded by his condition for the next 25 years. It was however to impact on other members of his family.

When Keith’s girlfriend Caroline (now wife) fell pregnant in 1994, they were reassured by doctors that his condition wasn’t hereditary. But when their son was born he was diagnosed with Holt-Oram syndrome. Finally, Keith had a diagnosis for himself as well.  

As well as a slight deformity on his left hand, their son Danyal also had a heart condition and he was fitted with a pacemaker at the age of five. But the apple hadn’t fallen far from the tree and Danyal attacked life with his father’s grit and determination.

“Like me, he’s determined. His condition doesn’t stop him – he drives, he’s active and plays basketball and netball. It doesn’t stop him doing anything at all...”

Meanwhile, Caroline became familiar with the clinical intervention that came with the condition. “She’s got to the point now where a hospital appointment is just a routine thing. She’s had three or four pacemaker operations with me, she’s had two or three with Danyal. It’s a routine thing with her now. It doesn’t worry her at all.”

Breathing problems a symptom of deteriorating health

In 2000 the family moved to New Zealand to enjoy a better lifestyle, and Keith continued to live a full and active life until 2014, when his heart began to play up.

“I was due to go back to the UK and Europe for my 50^th birthday and I developed breathing issues. So I was admitted to hospital. They didn’t know what caused it, they just thought it was part of my syndrome, that my health had run its course. I was told that I had heart failure, I later found out that they didn’t expect me to survive.”

The medical team had hoped to perform a septal ablation but Keith was deemed too weak to undergo the procedure. It was at this point Keith was told he would need a heart transplant.

“I found that quite devastating because up to then I’d always been told that a transplant was the last action, and the life expectancy once you’d had a heart transplant was eight to ten years. To be told that, at that time in my life, when my son was getting to an age where he could be considering a family, I just found it very distressing and very upsetting. But equally I was very determined that that wasn’t going to be the end of me. I was going to fight it.”

Heart condition symptoms worsen

Keith was discharged from hospital in July 2014, still weak but determined not to succumb to his condition. He returned to work full time and continued taking his medication and exercising as normal. Then at a cardiology appointment in November 2014 he was told, that in the meantime, he was “too well” to undergo the transplant.

“As soon as I was told this news, I walked out to reception and went, ‘Yes!’”

However, his reprieve did not last forever. Over the next two years his health slowly deteriorated, getting to the point where it was severely impacting on his life.

Heart transplant candidate

When he was again admitted to hospital with breathing issues, he knew the time had come. “Even I myself realised that I needed a heart transplant.”

“I was too weak even to get out to the car to meet my wife from work. I was too scared to walk round the supermarket because I didn’t think I’d make it to the check out,” he remembers. “Even walking round the house was a struggle. And I was very cold physically, electric blanket, clothes and I’d still be freezing. My wife said I was icy cold to touch and that was because my heart wasn’t strong enough to circulate the blood.

"We met with the Transplant Team and I underwent the assessment process which includes interviews with a psychiatrist, psychologist, and a social worker, it’s on their recommendations and your medical condition that you get put on the active waiting list."

While the wait is usually one of months, Keith was lucky enough to receive his in five days. The operation itself took six hours and Keith was then returned to the intensive care unit, before being transferred back onto the ward four days later.

Anxiety after heart transplant

Initially even Keith, the most determined of individuals, felt a little anxious about attempting activity with his new heart.

“I was sitting in the Lazyboy, this is within 24 hours of going onto the ward, and the medical staff came around and said you’ve got to have a shower. I was scared because I felt quite delicate. Even the thought of having a shower at that point, I wasn’t ready for it mentally. But they insisted I had a shower, and I felt better.”

Keith was lucky to have little chest pain from the site of the operation, but mentally he didn’t feel up too much. “I was sitting in the Lazyboy, not feeling sorry for myself but just very aware of my situation.”

However, his attitude changed when he saw his “transplant twin” Diane, the woman who had received the lungs from the same person who had donated Keith’s heart. “I saw her up and about and I thought well if she can do it, why can’t I. And that encouraged me to get out of the Lazyboy. I had this new lease of life, this new energy and I just felt that I could take on the world. And so, with my personality, I took on the world.”

Heart transplant recovery a challenge

The physiotherapist gave Keith goals which he enjoyed and Keith soon started setting himself challenges on the ward.

“I’d think ok yesterday I walked to the nurses’ station five times, today I’m going to do it ten times. I’d got my determination back, I was also getting my strength back, I started going on secret missions. When none of the nursing staff were around, and when my wife wasn’t around, I’d go out walking during the middle of the night on the ward. I’d go and find where the kitchen was, just out and about to get myself back into the normality of life.”

Keith’s singlemindedness served him well in his recovery. “I was told by the transplant team that my recovery was coming on in leaps and bounds and they were really impressed by how I was progressing. I was allowed to go off the ward very early, I was allowed to go upstairs to the café and grab a bite to eat and a coffee.”

He clearly remembers that first trip to the café. It was a realisation that he did – quite literally – have a new lease of life. “I loved the aroma of the coffee, the smell of the food cooking. We went around to the gift shop. I wanted to look at the colours of the magazine, the pictures, the photographs, the newsprint. I loved the smell of it. I went into the food shop – all the different aromas. It was wow. It was like new life again.”

Move to Hearty Towers

Two weeks after his operation Keith was moved to Hearty Towers, the rehabilitation unit based at Greenlane, where he would live 24/7 with his support partner (wife, Caroline) and other people who had recently undergone a transplant.

“For me, the transition to Hearty Towers was the most daunting aspect of the whole procedure, because you and your support partner are dealing with your situation and living in a communal situation. There are no nursing staff on site, you have specialised medical background co-ordinators during normal office hours, and they are available, on call 24/7, on an emergency help line if there are any problems.

However once at Hearty Towers, Keith and Caroline found the camaraderie that develops between the residents of real benefit.

“My transplant twin Diane and her husband, Grant were there. There were also a couple of other transplant recipients and their support partners,” he explains.

“So we were all there, supporting each other and talking about our different experiences and issues. The unit co-ordinators know the symptoms and problems, but it’s when you talk to someone who has been through that experience that you really get to understand it. We get the whole picture. We shared our knowledge with those going through the assessment process of trying to go on the active waiting list, also talking with fellow transplant recipients.”

Keith was discharged from Hearty Towers after just six weeks, about half the length of the average three month stay. Although he still had to attend regular physiotherapy for several weeks.

Don’t let your heart condition get you down

His tip for others in his situation is to keep your chin up and try not to let your condition get you down.

“What kept me going was being determined not to let it beat me. I was relatively young at the time and there’s lots of things I still want to achieve even now. So, keep focussed.”

He also encourages people to follow the clinical advice that they’re given. “Listen to the doctors’ advice. Keep taking your prescribed medication because that’s done for a reason.

“What we’ve been told is that quite often people, post-transplant, get to the stage where they’re like me, they’re fit and healthy and they start self-medicating or changing dosages. There was a survey that was done over the course of five years and everyone that self-medicated or changed the dosage from the prescribed medication, they didn’t last longer than two years. So listen to the doctors’ advice.”

He also encourages people to set their own goals and accept their own challenges. “Do your own marathon. That could just be walking up to your letter box but don’t give in to your condition. Fight it. Try and lead the best normal life you are able to do.”

It is advice that has served Keith well, who says he is now fitter and stronger since having his transplant done. “I took part in the Auckland marathon this weekend, we did the 12km traverse walk, myself, my wife, my transplant twin, family members from Hearty Towers. Already we’re talking about doing the Auckland marathon again next year, and the Hawkes Bay one.

“We’re really focussed about using the privileges we’ve been given in our new condition and life and equally promote organ donation to help others in a similar position to us.” 

Shared December 2017