There is a chronic shortage of donor organs of all types in the UK and for all ages. This proved to be the case for Rosie who was very small when her heart failed and remains the case for other small children and babies who are in need of heart transplants. Just 31% of adults in the UK are signed up to the organ donor register and it is believed that only 40% of parents would agree to donate their child’s organs in the event of their death.
Our Register for Rosie campaign hopes to drastically turn these figures around by sharing Rosie’s story.
Rosie was a perfectly healthy little girl for the first year and a half of her life, she followed her big brother and sister everywhere, had a huge appetite and was full of energy. It wasn’t until a few months before her second birthday that she started to suffer from repeated illnesses, totally went off her food and struggled to muster any energy to play with her brother and sister. She was eventually admitted to hospital for numerous tests after weeks and months of pushing by her parents who knew something was just not right.
Rosie’s tummy and legs had become swollen to the point where it was clear that this was more than a dietary issue. In July 2015 after blood tests, ultrasound scans on her liver, kidneys and stomach, a cardio assessment and an ECG doctors detected an enlarged liver and heart defect. She was transferred to The Royal Brompton hospital in London to see a cardio specialist and that was the point in which a whole family’s world was turned upside down.
Rosie was diagnosed with Restrictive cardiomyopathy (RCM) which is a disorder of the heart muscle where the walls of the lower chambers of the heart (ventricles) become stiff and restrict the heart’s ability to fill with blood.
The pumping action (systolic function) of the ventricle is normal in most people affected by RCM, but the diastolic function (when the heart is at rest) is not. Stiffness can cause the backup of blood in the lungs and veins in the neck and liver. People who have the condition are at risk of developing heart failure. It was explained that the end result of this condition is sadly inevitable, the heart will eventually be too weak to function and will fail.
Restrictive Cardiomyopathy affects just one in a million children. It is occasionally genetic but sometimes, as it would seem with Rosie, there is just no obvious cause what so ever.
Rosie was placed on medication to assist her heart in pumping blood around her body and allowed to go home. Taking her medicine became a daily routine that her brother and sister helped out with and she regained a bit of her previous spark.
Although she was learning to live with Restrictive Cardiomyopathy, Rosie was still faced with a very high risk of sudden cardiac arrest. Her parents and family set about raising awareness of the condition and raising funds in order to purchase defibrillator units for the local area, arrange CPR training sessions and offer support to Cardiomyopathy UK who were an instant and amazing help to them.
It was just days after the first of these organised CPR sessions that one of the toughest days of Rosie’s journey so far took place. Less than a week before Christmas the family took a morning stroll along the sea front with Rosie on fine form and showing no signs of illness. She attended a carol service to play jingle bells in the afternoon and was settling down for the night when she took a sudden and dramatic turn for the worst.
Rosie suffered a cardiac arrest. Her mother had to perform CPR until paramedics and air ambulance teams arrived. Rosie was taken to her local A&E, then transferred to GOSH.
On Friday 5th February Rosie had an incredibly risky eight hour operation to fit the Berlin Heart. Rosie remained in intensive care at GOSH for the next five months as she was never stable enough to return to Bear Ward.
Rosie was lucky to have been fitted with a Berlin Heart as sadly there are many other children who do not make it this far. At this time Rosie’s future remained uncertain as a Berlin Heart is a bridge to transplant. A donor heart was still needed to give Rosie the chance of a new life.
Rosie’s journey to transplant was far from smooth. Rosie had problems with blood clots and bleeds. Rosie was rushed to theatre for emergency surgery just a few weeks after being fitted with a Berlin Heart due to a significant bleed in her brain. This also resulted in Rosie being taken off the Berlin Heart and fitted with another mechanical heart called Levitronix which does not rely on the need for the blood to be thinned as much as the Berlin Heart.
As no donor heart had been found for Rosie she remained on the Levironix for more than three months. Sadly during this time she experienced a number of bleeds and multiple strokes as doctors and nurses battled constantly with Rosie’s deranged blood clotting. This resulted in frequent surgical procedures including two craniotomies and more than twenty circuit changes.
All of these procedures were under general anaesthetic and required Rosie to be intubated. On one occasion the team needed to use a defibrillator to shock Rosie’s heart back into a normal rhythm.
On 23rd December Rosie went to theatre to have an MRI and Cardiac Catheter procedure to assess the extent of her heart failure. Her parents were told that the pressures in her lungs were already high, at the cut off point for what is deemed acceptable for transplant. The decision was made to place Rosie on the urgent heart transplant list.
A case meeting on Christmas Eve resulted in the family being allowed home for Christmas. Rosie was given tweaked dosages of medication and allowed to go home in time to put cookies, carrots and milk out for Father Christmas and his reindeer.
At the start of 2016 Rosie’s first routine check-up of the year at GOSH arose a fresh concern. Rosie was not growing in size or strength due to her heart not working hard enough so the decision was made to fit her with an NG feeding tube through her nose and into her tummy. The NG tube would help to get her as fit and healthy as possible in preparation for a heart transplant as soon as a donor was found.
A trip to the Out Patients Clinic at GOSH on 2nd February 2016 resulted in Rosie being admitted to Bear Ward’s high dependency unit. After much discussion the decision was made to fit Rosie with a Berlin Heart as her own heart was just too weak. The Berlin Heart is a blood pump that vibrates rhythmically to assist children who cannot pump enough blood with their own natural heart. It does not entirely replace the natural function of the heart. Instead, it works along with the patient’s heart to pump blood.
On June 18th it was decided that Rosie should convert back to the Berlin Heart as she was by then on circuit change number 21 and was struggling with the after effects of the most recent stroke and swelling on her brain.
Rosie progressed well on the Berlin Heart until after those long months of waiting we received a call to say that a donor heart had been found for Rosie. Rosie’s surgeon said it was “the perfect transplant surgery” with everything going according to plan. Post surgery Rosie made good progress.
Rosie left GOSH at the end of September 2016 but rather than the homecoming we all hoped for she went to Tadworth Children’s Trust, Surrey where they specialise in intensive rehabilitation for children with acquired brain injuries. While Rosie now has the perfect working heart, she has suffered severe brain damage from the multiple strokes she had whilst waiting for a donor heart to be found and has a long road ahead.
We hope that in time Rosie’s brain will rewire to enable her to learn to walk and talk again however, this will not be possible without ongoing intensive rehabilitation.
Nearly 300 children have died in the past ten years while waiting on the organ donor transplant list. We are sharing Rosie’s story to encourage more people to consider organ donation and to remind people to have the conversation with their loved ones to ensure they are aware of their wishes.