As many of you may already know, Evie was born with a complex set of congenital heart defects including AVSD (atrioventricular septal defect), HRHS (hypoplastic right heart syndrome) and a common AV (atrioventricular) valve with severe regurgitation. Before Christmas, I started looking for a second opinion on Evie’s heart condition and what could be offered to improve her quality of life.
After we had received the second opinion from Boston, I relayed the information back to Evie’s cardiologist and the team in Dublin. They then discussed her at their Joint Cardiac Conference (JCC). This is a meeting held every week in Crumlin with all the cardiologists and cardiothoracic surgeons where they discuss the upcoming cases.
Evie has been discussed at this numerous times before, and each time they came to the same conclusion: that surgery was the wrong option for Evie’s quality of life. We didn’t expect them to have a different outcome this time around, but we were hopeful that they might agree to carry out some of the imaging Boston suggested to help them form a management plan.
The heart condition that Evie has means that there is continuous damage occurring to her lungs. The imaging required to test this damage is called a cardiac catheterisation. This is where they test the pressures in the lungs to assess the damage. Another test required to get a map of Evie’s heart is a cardiac MRI. Both of these procedures require a general anaesthetic which has its own set of risks.
We travelled back down to Crumin to meet with Evie’s cardiologist. It’s a long and tiresome journey and unfortunately, we were both floored by the response. There was absolutely no positivity regarding the plan Boston had proposed. The team in Dublin felt that surgery would inevitability cost Evie her life. Evie’s cardiologist explained this very frankly to us and also told us that Crumlin would not carry out any imaging; in doing so, they might give Boston the go ahead to perform surgery.
We spoke to three doctors in Boston over zoom on the last Friday in April; two surgeons, one who sent us the original second opinion, another senior surgeon who is well known for his skilful work on complex cases, and also a cardiologist. It’s a strange feeling being persuaded so drastically one way or the other. When we spoke to Evie’s home cardiologist, we were swayed against bringing her to Boston. But speaking to the team in the states really built up our confidence again and made us feel that taking her over was the best decision.
Travelling over might be stressful, but we have nothing to lose by at least having the team in Boston review her clinically and perform some imaging to get a clearer idea of her heart and its structure. Evie has a small right ventricle, but through an echocardiogram, the actual measurement of it cannot be taken. With an MRI, they can create a 3D image of her heart and deem whether her right ventricle might actually be small just by comparison to the left. This would be valuable information for creating a surgical plan.
The financial estimate for Evie’s care in Boston is quite an inconceivable amount, so whether we can take her over or not will bank heavily on whether we can raise the funds in time. The total amount has to be paid 21 day upfront and any treatment that is not carried out will be refunded to us. We haven’t started fundraising yet as we are still trying to gather as much information as necessary to make our decision.
Update 20th May 2022
I wrote this piece at the start of May and since then there have been some changes in Evie’s management plan. Crumlin discussed Evie again at the JCC and decided that they would carry out the cardiac catheterisation and MRI. They have some concerns regarding Evie having a general anaesthetic, as she has never had one before. This is something they may have to reconsider when she’s admitted for the procedures. Thankfully, they have agreed to carry out both procedures under the same general anaesthetic.
Evie is almost 10 months old now, a stage that a lot of people didn’t think she’d get to. She’s a happy baby and such a gentle soul, but we watch her struggle daily. She has supplemental oxygen and CPAP at night, both of which followed us home after her hospital admission for rhinoenterovirus at Christmas. She is breathless most of the time and works a lot harder with everyday activities. She’s developing at her own pace and doing remarkably well, but we often think how much more comfortable she would be if the pressure on her heart and lungs was alleviated.
She is NG tube fed and weaning onto solids is a struggle, partly because she lost her oral skills shortly after the tube went in (around 8 weeks old), it’s also likely she doesn’t feel hunger due to her regimented feeding schedule and she doesn’t associate eating with feeling full. Sometimes she can have projectile vomits if she’s moved too soon after a feed, cries or becomes distressed. This is because her heart and lungs are working too hard for the volumes of milk. She is on diuretic medication to offload extra fluid to relieve her heart. This causes constipation, which causes more distress.
The cardiologist in Boston explained to us that there can be a “honeymoon phase” where your baby’s symptoms seem to improve temporarily. Unfortunately, this occurs when the damage to lungs is becoming “fixed” or permanent. This is known as Eisenmenger syndrome, which is a hardening and thickening of the lungs and pulmonary artery. Once this permanent lung damage occurs, there are no further options for surgically repairing the heart defect. Time is not on Evie’s side and once again we feel we are stuck in limbo while we wait for a date from Crumlin to carry out the catheterisation and MRI.
Ross and I, as Evie’s parents, feel like we still need to explore the option for Boston. She has done amazingly well to get to this point in her little life, and we are forever in awe of her. We have decided that we will start the fundraiser for Evie. We are aware that it will take a lot of planning and energy, but we feel like it is the right decision for us as a family.
This decision has not been made lightly. Evie has been under palliative care all of her life and we want nothing more than to stay snuggled at home in our nest. Uprooting our family and putting our baby bird’s life in the hands of the team in Boston is terrifying. But there is hope, buckets full of hope. Every minute of every day, I have a constant battle in my head about what the right thing to do is, but my gut always tells me the same thing; to keep fighting.
Thank you for taking the time to read this post. I can’t convey my gratitude for all the love and Little Bird supporters we’ve gained along this journey. The kind messages we’ve received and friendships formed over these last few months have got me through some really dark times.
