When we decided to try for a child, we were so blessed to conceive quickly and without trouble. Though, throughout my pregnancy, I was sick. So sick. Though throughout my pregnancy I was sick. So sick. On top of having hyperemisis gravidum, I had my gall bladder removed, and two kidney procedures done. They continuously monitored Bentlee, my sweet unborn baby, through all of the trouble I was having.
At his 20 week anatomy scan they saw an intracardiac echogenic foci. A bright spot in the heart. They assured me that it was nothing to be concerned about and most times, it’s just a shadow from the ultrasound angle. They scheduled me for more pictures of his heart just to be sure though.
I went to the second anatomy scan where the tech focused on Bentlee’s heart the full hour, getting pictures and measurements of everything. She was happy to tell me that everything was clear and there were no abnormalities. We were so relieved!
Fast forward to December 6, 2019 – the day Bentlee was born. Bentlee required a little extra help to transition to the real world. My labor and delivery nurse had a gut feeling something was off with and decided to call NICU for a consult.
When NICU came in, they brought a pulse ox machine and hooked my baby up, only to see his oxygen level was at 66%. They quickly took him to the NICU for additional testing for respiratory distress. They assured us Bentlee would be back to my room in eight hours after he had a transitional phase in an oxygen tank.
They called us a few minutes later and told us that Bentlee was not a respiratory patient, but that he was actually a cardiac case. They would be running an EKG and echocardiogram, as well as calling the local pediatric cardiologist. We were shattered; his heart was supposed to be fine.
I made it to my post-partum room and a neonatologist met me in there. He told me he did not have all the information yet, but what he could tell us was that our baby had a major heart defect that requires open heart surgery. He would have to go to St. Louis Children’s Hospital very quickly to have a life-saving procedure.
Fear, anger, grief, sadness — all emotions I felt. Thankfully, I was able to leave 8 hour after I had Bentlee and go be with him.
When we made it to SLCH, they informed us Bentlee has Ventricular Septal Defect (a hole between the ventricles), and Transposition of the Great Arteries (his aorta and pulmonary artery was switched). the hole in between the ventricles to allow a large portion of oxygenated blood & deoxygenated blood to mix. The hope was that this would give him a couple of days to get stronger before his open heart surgery.
On the back burner, my mom was reach out to by a friend, whose sister is a photographer. She actually took my wedding photos, and was part of a program called On Angels’ Wings — Bentlee qualifies for pictures. The OAW photographer, Lacie, reach out to me the next day to arrange to come St. Louis to get pictures for us before Bentlee’s big procedure. The organization had photographers in the area, but Lacie insisted on coming herself. We weren’t sure when surgery would be, so Lacie dropped everything and made a very early morning trip to St. Louis just so we would have those pictures. That meant so much to us.
We were told time and time again that Bentlee could die. His open heart surgery occurred at 5 days old and was 10 1/2 hours long. During this procedure, they switched the major arteries, as well as relocated the coronary arteries (which were less than a millimeter in length). They were also able to close his VSD. Bentlee did have to be hooked up to the heart and lung bypass machine much longer than they expected. He actually bled out on the table and had to have multiple transfusions to save him. But once he was taken off bypass, and his heart was gradually warmed, they were so thrilled to see his heart beating on its own!
We are so thankful he recovered well and is thriving today. Although he had this procedure, it was not a cure. Congenital Heart Defects are incurable. Surgeons can only mend the issues. At 2-years old, Bentlee is currently dealing with narrowing of the pulmonary arteries (pulmonary stenosis) and enlargement of the aorta (aortic dilation). He is also severely immunocompromised due to having to have his Thymus gland removed in order for the surgeons to access his heart.
Having a child with a heart defect is hard. I didn’t know anything about this type of lifestyle until Bentlee’s situation. I have so much love for the community of heart families I am a part of. It has made us grow as a family, me as a mother, and I don’t take anything for granted. It is hard though. So hard.
Recently, our strong guy was diagnosed with a rare primary immunodeficiency called Common Variable Immune Deficiency (CVID). This disease affects 1 in 30,000. Leading up to this diagnosis, we went through Bentlee’s first 2 years having to limit our exposure out in the public because he was dealing with back-to-back infections: several episodes of croup, double lung pneumonia, preseptal cellulitis, e.coli, herpangina, enterovirus, RSV, rhinovirus, Covid — the list goes on. I pushed for further testing because I just could not believe how sick he was getting if we just went to our neighborhood park.
This testing validated our concerns: his body was not making any antibodies to protect him from germs, and he was not able to form a response to Prevnar 13 (vaccines). He had no protection at all in his little body to keep him healthy.
Unfortunately, like his Congenital Heart Disease, there is no cure for CVID. In order to protect Bentlee the best we can, we now preform subcutaneous immunoglobulin (antibody) replacement therapy in our home every other week. This consists of me having to hook Bentlee up to a pump that contains a syringe of plasma product full of antibodies from up to 10,000 donors. I insert two needles into his thighs and the pump pushes the serum in over a course of 35 minutes. The medication burns as it goes in, and leaves our sweet Bentlee with the chubbiest little legs as we wait for the medicine to dissipate over the next several days.
There are many risks with these infusions, and the side effects can be horrific. Bentlee usually becomes extremely lethargic for the first couple of days and develops flu like symptoms. He experiences vomiting and diarrhea on occasion. The risk of anaphylactic shock is extremely high, so we now have to carry an epi-pen with us at all times. His care team has grown, and he will start seeing an oncologist to monitor for signs of cancer, as CVID increases the risk of lymphomas and granulomas.
However, if we don’t do these infusions, it would cause Bentlee to become a “boy in the bubble.” He would live a very limited life because of the disease if we didn’t do them. These infusions may be something Bentlee requires the rest of his life.
When we received the new diagnosis, our hearts were completely shattered. We knew he was immunocompromised from having his thymus removed for his open heart surgery, but we were not expecting this severity. On Angels’ Wings jumped right in to provide support, offer anything we could possibly need, and help us document this part of our journey with a photo session.
On Angels Wings has continued to provide pictures for us to celebrate Bentlee’s recovery, his first birthday, Christmas, and so much more. They have connected me with moms who have gone through similar situations as I have. I know I am never alone.
Bentlee has a lot of changes going on with his heart right now. He has multiple therapies and cardiology appointments to monitor the changes that are happening. The future is unknown, but we are strong in faith that we will be spending many years with our sweet guy.
I hope that in the future, On Angels Wings is able to continue serving families like mine. I hope and pray for research and a cure. I hope that Bentlee’s heart does not continue to worsen.
I’m so thankful for On Angels Wings. So thankful. They are and have been amazing to us and so many other families.
Caitlin Smiley
Mommy to Bentlee
Congenital Heart Defect, CVID
#heartwarrior
