I had a reader* (on my family blog) ask a question the other day that I figured some others may be wondering. I also think answering it could potentially help educate others about single ventricle heart disease, and education is POWER and could mean another child going from orphan to a son or daughter. So here goes.
Way back in March, a team of volunteers from Love Without Boundaries traveled to Guizhou Province and visited several orphanages. In one of those orphanages, they found a little baby girl who was not doing well and who appeared to have heart disease, which was apparent in her blue-tinged lips and other signs I’m assuming involving her breathing and possibly her fingers and toes (clubbing). By April, LWB was given permission to seek evaluation and possible treatment for this now 14-month-old baby girl in Shanghai. They also in April pleaded with supporters for funding for several children including a little girl they had given the name Keely. Within weeks, this little baby girl was receiving a much-needed, open-heart surgery in Shanghai. I wish I could share a face*book post link, but if there is a way I haven’t figured it out! Anyway, LWB posted many updates on their face*book page about the little girl they call Keely when she was in Shanghai receiving surgery for her heart
She received what is more commonly called a glenn shunt. This link is a very layman’s terms description of the Glenn shunt. It has pictures. If you are really adventurous, I have found youtube videos of the operation and I’ll be honest: I have watched them and find them absolutely amazing and fascinating. The Prez thinks I’m a bit off, but I really found watching them helped me understand it a lot better and to see a surgeon working with such a tiny heart—amazing and miraculous.
**If you would like to see the link, I do have it posted on my family blog. I do not have it posted here because it is graphic. I just want to be careful and not offend! I find it fascinating and informational, but I realize others may not. You can also google you*tube and glenn shunt to find it if interested.**
After the Glenn shunt in early May, this baby girl was now receiving more richly oxygenated blood to her head and upper body. Still, the lower half of her body and lower limbs are still receiving very blue (oxygen-poor) blood. She needs another surgery, which is called the fontan in order to COMPLETE the fontan circulation.
To answer Trish’s question, yes, LWB provided her with a much-needed and life-saving surgery, but she needs another surgery. She needs the full fontan circulation completed. This surgery she had in Shanghai though not only gave her a better chance for the full fontan circulation to be completed, it greatly improved her quality of life and her chances of being adopted. If I’m being completely honest, the Prez and I found a huge amount of relief and peace in knowing she was given such an incredible gift and chance. Our first daughter didn’t receive this surgery and time took its toll on her little heart as she waited and FOUGHT to live. I still get totally overwhelmed when I think of what our Li’l Miss ENDURED. We watched her coming home video again today and I have a snippet of video with her labored breathing … every single time I am overcome when I remember seeing her suffering like that. NO CHILD should have to suffer like that.
Our little girl, who is the same little girl known as Keely with Love Without Boundaries and who we will meet in 15 DAYS!, has single-ventricle cardiac disease just like our Li’l Miss has. Well, their defects are somewhat different but ultimately they both were born with one formed ventricle instead of two, and both needed the fontan in order to live the most full life possible with current medical advances. There is no cure or heart repair for single ventricle disease, but the glenn shunt and hopefully following that the fontan will give our waiting daughter the chance to lead a full life. She also has been diagnosed with dextrocardia, which by itself can by a totally non-issue, but with a complex defect such as single ventricle it certainly complicates matters. We are also unsure of possible other defects because we have received some conflicting information. Rather than pursue a lot of clarification, we decided to move forward because clarification was not going to change our decision to pursue her adoption nor was it going to significantly affect the fact that we will not know her surgical options until she is home and can be evaluated by a cardiologist and particularly through cardiac catheterization.
This page from Cincinatti Children’s Hospital is one that I find extremely helpful for a laymen like me to understand our daughter’s heart defects. You can click through and read about treatment options and various considerations.
Trisha also wondered why the urgency if our daughter already received life-saving surgery and if she is “doing well” as was stated recently on a post about her. While she is doing well right now, ideally she needs the fontan circulation to be completed (the lower vena cava to be rerouted to bypass the pumping chambers of the heart and flow directly by pressure into the lungs) and for it to be done sooner than later. While neither of these operations is considered curative, the optimum outcome for her would be a completed fontan circulation.
It is important I think to note here that the fontan operation is a last resort. It is not optimum, but it is what is available to better a child’s life and give them a chance to live a life without being on oxygen hopefully and to experience everyday things that many of us take for granted. But ultimately, the fontan bypasses the pumping chambers of the heart because the defects are so complex that this is BETTER than allowing the blood to flow through the heart. I still have a drawing our amazing Dr. B, the surgeon who performed Li’l Miss’s fontan and who will perform Li’l Bit’s as well God willing she is operable, gave us as he explained in a way we could understand exactly what it was he would be doing to our Li’l Miss’s heart. I saved it for her to have someday. I will never forget sitting in her hospital room that night, the night before he would operate on her heart, and hearing him tell us the risks, him having to ask us to sign the consent and his obvious grace toward us in that moment, and ultimately him saying as he stood, “It is in the hands of her Maker.” Yes it is. Even today, that is it. Our Li’l Bit’s heart is in the hands of her Maker.
On that same page from Cincinatti Children’s Heart Encyclopedia, you can read this:
For a heart with a Fontan reconstruction to work well, there are a few crucial features that must be maintained. These key factors must be kept in mind when planning the medical or surgical management of children with single ventricle defects from the first day of life onward.
The single ventricle must not be overworked for a long period of time, in terms of either having to pump too much blood or pump at too high a pressure.
The pulmonary arteries must grow well without stenosis (narrowing) and must remain low resistance (or be very relaxed). If the pulmonary arteries are narrow or if the resistance in these vessels is high, blood will not be able to flow into them without a pump, so the Fontan will not be successful.
Lastly, leaky or tight valves may adversely affect the function of the ventricle or the flow of blood to the lungs.
I bolded the parts above because this information is crucial to understanding why the urgency and also why what LWB and its supporters provided is so amazing. Not only did the shunt operation she had done buy time so to speak, but it GREATLY INCREASES her chances for a successful and complete fontan circulation by hopefully protecting her lungs and pulmonary arteries from high pressures that could make a fontan impossible.
We know personally how NOT getting the glenn shunt adversely affects a child. We saw with our own eyes how sick our older daughter was. She was 35 months old and never received the glenn shunt which our Li’l Bit has had done thanks to LWB and its supporters.
As far as the urgency it is mult-faceted. The ideal time for completing the fontan is two to three years of age. Not only that, the longer our daughter waits for the full palliation, the more chance she could become ill and not be able to fight it and also the more chance her heart is being overworked (and could be enlarged), and finally the more chance irreversible damage is being done to her lungs and/or pulmonary arteries.
This is an excerpt from the Cincinatti Children’s page:
The third and final stage in the reconstruction of a single ventricle heart defect is the Fontan completion operation. This operation is usually performed at 2 or 3 years of age, based on the child’s size and clinical status.
On that note, the optimum time for a child in the US to receive the hemi-fontan, or glenn shunt, is by six months of age. Our daughter received it at 14 months of age. We don’t know what happened with her heart and lungs and pulmonary arteries while she waited so long. What we do know is that she survived for all of that time, that she made it through the surgery and that she is doing well right now.
I also want to add that children with single ventricle heart disease are very susceptible to infections, to *regular* childhood illnesses being much more serious for them, to the lack of oxygen causing secondary problems such as poor brain development, learning disabilities, damage to the lungs which could be irreversible. While the surgery LWB provided most likely improved our daughter’s oxygenation from the 40s or 50s to the 70s or 80s, this is still not ideal. A normal heart will function in such a way that the body will receive blood with an oxygen saturation of 99% or better. So, 80% oxygen saturation is certainly better than 40 or 50% saturation, but is not something ideal for the long term. It is very possible our daughter could live with just a hemi-fontan and at 70-80% oxygen saturation, but we are hopeful that she can receive the completed fontan circulation and hopefully 95% or better oxygen saturation. For reference, Li’l Miss had oxygen saturation of 40% when she came home and 70-80% after her complete fontan repair. However, due to her case of having no partial fontan done, her surgeon felt it necessary to leave a fenestration (a type of pop-off valve if you will to allow pressure releases) in her heart. About a year after her fontan operation, the fenestration was closed surgically in the cath lab. Since that time, Li’l Miss has held steady with oxygen saturation of 96-99%.
I want to touch on something I am often asked about: life expectancy. I copied this from that Cincinatti Children’s page as well because it explains what we know so well (and also a lot of what we just don’t know):
The limitations children experience due to their heart defect, though, can vary greatly. At one end of the spectrum there are children with Fontan circulations who have participated in competitive sports such as swimming and gymnastics.
Other children may have significant limitation in their capacity for exercise. Most children fall somewhere in between the extremes.
Most children are on a blood thinner called coumadin after their Fontan to prevent clots from forming in the Fontan circuit. When a child is on a blood thinner, you must take extra careful to avoid falls or head trauma as they are at increased risk for internal bleeding.
How long a heart with a single ventricle reconstruction can function is not known. The first children to have a successful Fontan operation are just now 30 years old and many improvements in surgical technique and medical management have occurred over this time period.
Late complications including irregular rhythms and heart failure may occur. Some speculate that most single ventricle hearts will not function efficiently beyond 30 to 40 years, but improvements in surgical technique and medical care may increase this age significantly. In some cases, if the ventricular function deteriorates significantly, heart transplantation may be considered. Because of the possibility of late complications in patients with single ventricle anamoly, continued regular follow-up with a cardiologist for the life of a patient is essential.
So in all honesty, we don’t know how long our little girls born with single ventricle disease will live. But who of us does know? What we do know is that they are amazing people and they deserve every good thing life has to offer, including a forever family. We also know our Li’l Miss has brought great joy and love to our family and occupies a place in our hearts that we never knew needed filing, and we don’t doubt our Li’l Bit will do the same.
We hope she is able to have the completed fontan circulation. We hope in the Lord ultimately for her future. He is the only Hope we have.
*I am cross-posting this from my blog. There are so many children who wait with single ventricle heart disease. I hope by sharing more, others will come to a place of feeling led to take that leap and bring one of these amazing children home. In that regard, please email me (cljjs (at) yahoo (dot) com) or leave a comment if you know of children who wait with single ventricle heart disease. I’d love to advocate for them.