David Farmer and HLHS

DAVID FARMER PAGE!
Does this boy look like he has Hypoplastic Left Heart Syndrome?

David Samuel Farmer was born February 5th, 1997. He weighed 9 lbs., 14 oz. and was 22" long (and NOT born by C-section, which is a very common first question). Within 24 hours after he was born, he was diagnosed with Hypoplastic Left Heart Syndrome (HLHS). If David had not received any kind of treatment, he would have died within days. This web page is about David, exactly what HLHS is, our chosen method of treatment, and what factors have contributed to David's thriving lifestyle.

HLHS is a congenital heart condition where the left side of the heart is underdeveloped to a point where it is non-functioning and cannot sufficiently supply blood to the body. The small left ventricle is usually accompanied with a very narrow aorta and underdeveloped valve between the upper and lower left heart chambers. Below is a normal heart (left) and HLHS heart (right):

When the left-side heart structures are underdeveloped, the right side of the heart must pump blood to both the lungs and to the rest of the body. In the womb, a baby's body receives oxygen-rich blood through a communication (connection) called the patent ductus arteriosus (PDA). After birth, the PDA spontaneously begins to close. An undeveloped heart cannot pump efficiently enough to support life and that is when a baby with HLHS gets very sick. Without treatment, the defect is usually fatal within the first several days of life. David was given a medication to keep his PDA open until he could be treated.

We did not know about his defect before he was born. Shortly after birth he did not want to eat and we learned later he was a dusky color so they had put him under an oxygen hood with no improvement. At 12 hours old, the pediatrician examining him said she heard a murmur and they wanted to investigate further; if any concerns arose, they would wake us up and let us know. Around 2am, the nurse came to take my vitals and told me I needed to stay awake as the neonatologist needed to speak with us.

He came in and told us that David was born without a left side of the heart--Hypoplastic Left Heart Syndrome. He certainly gave us details on what that meant, but I don't remember anything except he ended with saying this was very serious. A transport was arranged to Children's Hospital of the King's Daughters (CHKD) in Norfolk, VA about 10 AM and my OB came by ASAP to discharge me so we could meet him there. We were given a couple of polaroids the hospital took, which I learned later are called "just in case" pictures. They're taken in case the baby does not survive transport--you have a last picture.

At CHKD we met with a cardiologist and were given three options for treatment: Do nothing (take him home to die), a heart transplant (where the chances of getting a heart were low and the window of opportunity for the surgery very small), or a series of three surgeries (Norwood procedure), which the cardiologist stated were "very experimental" and the outcomes not good. At this point we requested a room where we could be alone as we were told all this in the middle of the NICU. A bit later a clinical nurse knocked on the door and wanted to review what we were told. She said her job was to keep the Doctors in line and that turned out to be a very good description. When she found out we had been told the surgeries were not a good option she corrected that immediately and said that they had been around for a few years and the outcomes were much better. So, after meeting with the transplant coordinator the next day to ask questions we decided on the Norwood procedure.

We were living in Virginia and were given the options of having surgery in Boston or Philadelphia, but upon learning I was from Ohio we were offered Cleveland, which is where we chose to go. The timeline was a bit overwhelming. David was born on Wednesday and diagnosed on Thursday, which is the day we were given our options. We made our decision on Friday and the transport to Cleveland was scheduled for Sunday. When I got to Cleveland one of the first questions from a nurse was, "What took you so long?!"

David had his first surgery performed when he was 6 days old by Dr. Roger Mee at the Cleveland Clinic (Dr. Mee is the subject of the very good book, Walk on Water) . Since the left side of his heart could not pump to the body, his heart's right side was modified to pump to the body. This included constructing a "neoaorta" and placing a gore-tex shunt from his aorta to the pulmonary arteries to get blood back to the lungs. Below is a graphical illustration:

David did extremely well. His first visit to the Cleveland Clinic was 12 days. David returned to the Cleveland Clinic a few months later, when the shunt was too small for David to get adequate oxygenated blood.

The second stage of the Norwood procedure is sometimes called the "Glenn Shunt", as the artificial shunt is removed, and blood vessels are rearranged to bring blood back to the lungs. In general, the vein coming down from the head entering the right atrium is disconnected from the heart and connected directly to the pulmonary arteries to send blood to the lungs. Below is an illustration:

The procedure allows the upper body blood to go back directly to the lungs. This time around, David was in the hospital for an amazing 5 days! Since he was four months old, he was not moving a lot as it was, and the big concern was for us as parents caring for him. David wouldn't have to return to Cleveland for another 2.5 years.

The last surgery in the three surgery procedure is sometimes called the "Fontan". The Fontan operation is not unique to HLHS--other heart conditions can utilize having a wall placed in the right atrium so that blood can bypass the heart for the lungs. In the case of HLHS, creating this bypass will allow the lower body blood to return directly to the lungs. This essentially eliminates almost all mixing of blue (non-oxygenated) and red (oxygenated) blood. Please see the graphic below:

From our perspective, this was David's toughest surgery. After the surgery, his skin color became more "fleshy". Within six weeks after the surgery, when he was healed up enough to run around, David was hardly ever out of breath. Before the surgery, David could not have gone 30 feet without getting exhausted.

Since his last surgery, David has continued to thrive. We credit his good health to a loving God and our attention to his nutrition. David is doing better than we could ever have imagined. He recently finished his 5th season of Little League and once again no one suspected he had anything wrong healthwise and didn't know unless we chose to tell them. He is active in Boy Scouts and does all the activities, to include campouts, hikes, and summer camp.

We reached a point in the past year or so where we began to see a decline in his activity levels and we were not surprised because we were told that would happen as he got older. We believed it was related to the HLHS as did our cardiologist, although heartwise he was still doing great. After David's mother was diagnosed with gluten sensitivity after 8 years of illness, and both of David's sisters had issues with gluten we decided to do further testing with David. David's cardiologist gave him a stress test to check his heart function. The results came back better than we could have hoped for. He tested in the 50th percentile--for normal children! Even with a single ventricle, he still tests right in the middle with normal kids! The heart rate was the real test since that is a factor in how the blood pumps to the body. In a normal child during exercise the heart rate will reach around 200. In single ventricle children it usually reaches the 130s. David's rate was 186!!! This absolutely ruled out any heart involvement with his increase in fatigue.

We took David to a Pediatric Gastroenterologist ("GI" Doctor) who performed several tests. David's esophagus was scoped, during which they took two samples to check for celiac. The samples came back negative as did his blood test, but his genetic testing showed he was 16 times more likely than the general population to have celiac disease. Based on that he was placed on a three month gluten free trial. He also was diagnosed with esophagitis due to reflux. He had severe scarring in his esophagus. At this point we're hopeful that the reflux is gluten related.

David has done phenomenal with his GF diet! His energy levels increased immediately, irritability reduced drastically and he's much more pleasant to be around. He is doing so much better he asked if he can go back to playing basketball again, which he had to quit due to his fatigue. Pending the cardiologist's approval, we'd all be happy for him to play again!

We do quite a bit with David in regards to nutrition and supplementation. Unfortunately, this is an area not often given a lot of attention. Not until we made some aggressive changes with his nutrition when he was a baby did we notice a dramatic difference with him. In fact, David's changes were so dramatic that at our next visit to the cardiologist David was doing so well the cardiologist told us, "I don't know what you're doing and I don't want to know, but keep doing it." David lost the "heart baby" look and never got it back. In fact, when he was 9 months old in the hospital for a heart catheterization, the nurse had to double check David's charts. She told us that when she saw David, she was convinced there was a mix-up and that he couldn't possibly have HLHS. I can't emphasize enough what a difference good nutrition makes. At some point I'll make a page devoted just to this, but for now...

Please feel free to contact us at The Health Express if you'd like to know more about David, HLHS, or anything in between.

Please help us get the word out! Way too much information is used regarding HLHS that is outdated or simply incorrect. This positive story about David must battle the many somewhat negative stories out on the Internet, where HLHS has been promoted as having anywhere from no treatment to not even presenting the Norwood Procedure as an option. If the Norwood Procedure is not offered at your hospital, you CAN go to another hospital--even if it is halfway across the country!