Leon’s Story
_Leon’s Journey _
My name is Sylvia Crosbie and my husband John and I have two beautiful little boys. Jens is four years old and our Heartkid Leon, is 2 and a half years old.
Leon was diagnosed with complex congenital heart defects in uterus at 20 weeks gestation. In September 2007 while we were living in Dunsborough, my husband John and I, went for a routine ultrasound appointment in Busselton. It was then that we were advised of a possible abnormality of the fetus heart. We went home in disbelieve as you don’t expect there to be anything wrong with your second baby, yet alone a heart defect, when you have already had a perfectly healthy baby. I had never known anyone or had heard about children with heart disease previously, yet in Australia 6 children are born with a heart defect every day.
A week later we were sent to Perth to see an ultrasound specialist, who confirmed that the fetus heart has not developed properly, but he said it looks like a hole in the heart and that might me fixable. The whole world around me broke down; I clinched onto my little 14 month old Jens and couldn’t hold back the tears. The specialist referred us to Dr D’Orsogna, who we saw a week later and he gave us the ultimate diagnosis of Leon’s heart condition, which comprises of a complex single left ventricle with a double outlet right ventricle, a hypoplastic right ventricle, a common atrium and a common AV Valve and transposition of the great arteries. Leon is also dextrocardia and viscera atrial situs inversus, which means his heart is on the right side and his heart and all his other organs are on the other side like a mirror image. He was also diagnosed with 4 Spleens.
Dr D’Orsogna explained to us, that these defects cannot be repaired, but there is palliative surgery available, called the Fontan Procedure. The Fontan Procedure consists of three stages. At the first stage they will insert a Blalock Shunt, which is a little tube between the heart and the lung. This first stage would be done in the first few months after birth.
The second stage is called the Bidirectional Glenn procedure, where the oxygen poor blood from the top body gets directed to the lungs. This surgery is an open heart procedure and would be done at 8/9 months of age. The final stage is the Fontan completion where the oxygen poor blood from the lower body will be directed to the lungs. This will leave the heart completely free of oxygen poor blood and now the single ventricle only has to supply blood to the body. Usually this open heart procedure will be done before they go to school.
We were given the option of terminating the pregnancy, but after an amniocentesis revealed there were no other defects, John and I were determined to give our baby a chance and we are so glad we did.
Due to the complexity of Leon’s defects we moved to Perth, where I attended a weekly high risk pregnancy clinic at KEMH.
As Leon was not growing in uterus, I was booked in for an induction on the 31st January 2008 at KEMH. A natural birth was planned, but unfortunately this plan had to be changed to an emergency caesarean, because Leon had developed a 2nd degree heartblock. Later on we learned very quickly that nothing goes to plan with Leon and we would call it “he’s doing a Leon”.
We were prepared for a blue baby, who would most likely need resuscitation, but fortunately Leon looked like a pink, healthy crying baby. After a quick check up by the Pediatricians he was taken to Intensive Care unit for observation and I only saw him again the next day. Leon was doing well from the heart side of things, but unfortunately he wouldn’t tolerate any feeds for no apparent reason. One week later Leon was transferred to PMH where he was continuously monitored and fed via a nasal gastric tube until week 3, when we finally established some breast feeding.
On day 18 we were given the all clear to take Leon home the next day for the first time, but it wasn’t meant to be. Leon’s heartrate dropped over night and he had to wear a Holtermonitor for 24 hour surveillance. The result showed that he went in to 3rd degree heartblock, but fortunately he was stable. On day 21 we finally got to take him home.
Everything seemed to be going fine; Leon was feeding well, he never cried and he slept a lot – He was the perfect baby!!!
We then took him for his 6 week check up with the Neonate Pediatrician at PMH, only to learn that Leon hadn’t gained any weight and his heartrate had slowed down even further, so he was admitted to the Infant Ward.
Dr Warren, who was Leon’s cardiologist at that time, told us that because Leon’s Heartblock had progressed to a complete heartblock, he would have to undergo surgery in the next few days to be fitted with a pacemaker. They would also insert a Blalock shunt to increase the blood flow from the heart to the lungs.
So on the 19th March 2008, Leon was seven weeks old, we handed Leon over to the Theatre nurse, not knowing if we will see him again. For a parent this is the hardest thing to do, but you have to trust in god and the wonderful doctors and nurses.
John and I were to anxious to stay at the hospital during the six hours of surgery, so we went home and tried to focus on our older boy Jens, who was 18 months at the time.
Mary Tallon kept us updated while Dr Andrews performed the surgery.
We received the call from Mary that surgery had finished and everything went well, John went to see Leon in ICU. We had no family or friends in Perth, so I stayed home with Jens. John was not prepared for what he would find in ICU and even though he tried to prepare me, nothing can. Leon looked twice his normal size due to the fluid retention and he lay there looking lifeless, with tubes and lines hanging out everywhere.
Leon’s recovery was very slow, because his blood didn’t clot, so they were not able to pull out the line to the heart and had to keep him on the ventilator. After seven days of giving him Vitamin K injections and giving different blood products his blood finally clotted. The line was taken out and he was weaned of the ventilator, Leon quickly turned the corner for the better. But once again Leon couldn’t tolerate any feeds and we had to start all over again. Slowly but surely we got him back drinking again. Because Leon’s weight gain was very poor, we had to stick him on a strict feeding regime and to improve his weight gain the Dietician put him on a special formula with added calories. Leon didn’t seem to have the energy to finish his bottles, so he had to be topped up by a nasal gastric tube.
Winter came and we soon learned that this can be a tough time for a child with congenital heart disease. Leon spent the majority of winter in hospital as he needed oxygen support to cope with different respiratory illnesses. His first illness, which was Broncholites, caused Leon to stop feeding altogether, so he was purely fed by a NG tube.
In August 2008 Leon had a cardiac catheterisation in preparation for the 2nd stage of the Fontan Operation. Unfortunately the cardiac catheterisation revealed that Leon has secondary Pulmonary Hypertension, which means the pressures in the Lungs are quite high and it would not be possible to do any heart surgery, at this stage. Fortunately, the government just made a medication called Bosentan available, which hopefully reduces the pressures. Leon was one of the first infants to be trialed with this, so there are no statistics or studies to go by.
Leon’s first year of life was a very hard and emotionally draining year. During his first ten month of life he spent 2-3 weeks every month at PMH. This certainly puts a strain on family life and Jens had to step back a lot. All this experience made Jens a lovely, caring little Preschooler, who looks out for others and loves his little brother very much.
Leon’s second year started out quite well, he caught up in his development, which was held back by all the hospital stays. Once again winter came around and even though Leon had fewer stays in hospital, they were longer and he seemed to get sicker. This seemed especially so in September 2009, when Leon had a severe case of Pneumonia. That was the sickest Leon had ever been and it was just heartbreaking to see my little boy like that. He finally recovered after being administered some strong IV antibiotics, steroids and diaretics, although he may have suffered some long term scarring to his lungs.
The irony in this is that after this episode in hospital Leon finally started to eat for the first time in his life. As his food intake was reduced to the minimum to prevent the stomach from expanding, his hunger must have been much stronger than the fear to swallow. Soon after that he started to walk. Leon’s speech has been delayed, even though he is a very smart and bright boy. Once Leon started to talk a few months ago, he went ahead in leaps and bounds. I am pleased to say that he is now speaking 3-4 word sentences….. mind you, I’m usually the only one who understands him!!!
In January this year Leon had another cardiac catherisation to check, if the pressures in the lungs have changed. The catherisation showed that the pressures have indeed come down, which means the Bosentan is working. They still haven’t come down far enough to continue with the Fontan Procedure, but there is hope that maybe in 18-24 months time they will have. Although the pressures in the lungs have come down the pressure in his heart has gone up, so now he is taking medication for that as well.
After the catheterization Leon developed a blood clot in his groin where the catheter was inserted. He was given a blood thinning medication, but it didn’t resolve the clot. So Leon had to go to ICU, where he was thrombolysed. This basically is a method of breaking the clot with a very strong drug called Urokinase. The risk of using the Urokinase is that it can cause bleeding. Two hours after administering the drug, Leon showed heavy bruising and started to bleed from the sites where the doctors tried to get access to his veins. Leon ended up losing 400ml of blood, which was more than 40% of total blood volume for a child of his size. They gave him a blood transfusion and by the morning, Leon was released to the ward. The next day, which was Leon’s second birthday, he was discharged and celebrated it at home.
Leon has come a long way in the last eight months. He is a very beautiful little boy and he is quite cheeky and always happy. Leon is a true fighter and when people around him are sad, he always tries to make them laugh. Even though Leon hasn’t got the energy to run, he will always try to copy what Jens learns at soccer practice and he always wants to keep up with his big brother. Leon is a true inspiration to all that know and love him.
Through all these times and especially in the first year, Cecilia Donovan, our lovely Heartkids Support Coordinator, was always there to offer us support. When we are in hospital Cecilia always makes an effort to come and see us every day to make sure we are ok. She always has an open ear for questions and concerns and is able to give answers and hope. Cecilia also invited me to come along to Coffee and Chat sessions where I was able to meet other Mums of Heartkids, who are going through, or have been through, the same challenges. At these coffee and chats I have met some lovely people and it’s comforting to know you are not alone and that there is support and help, if you need it.
Now that Leon is a little bit older we went along to a few Heartkids outings such as Adventure world, the Aquarium and the IKEA Kids Party. These are also great opportunities to meet other Heartkids and their families and bring happiness and fun to our precious heartkids, whose lives are filled with hospital stays and appointments.
Thank you for letting me share my story.
Written July 2010.