Wednesday, June 5, 2013

Glenn Procedure

The Glenn procedure is the reason we are here. The first image I think shows the procedure quiet well and I love the Elsevier watermark behind the image. This is probably the most simplistic image I've found which displays the Superior Vena Cava (SVC) disconnected from the right atrium and reattached to the pulmonary artery, it is not altogether applicable to Gabriel's situation as this does not display what occurred during the Norwood procedure.

Awesome link to many other cardiac blogs. the author of the initial blog underwent the Glenn in 1967 a year I find particularly relevant as that is the year Gabriel's dear old Dad was born.

This image is much better at displaying what Gabriel's current cardiac situation is. First though, how our normal hearts work. Blood from the upper body enters the right atrium via the superior vena cava while blood from the lower body enters the right atrium via the inferior vena cava. The atria (right and left atrium) are basically temporary holding tanks that fill and then dump their contents into the ventricles (plural form in regular folk, singular in Gabriel type persons). So the venous or de-oxygenated blood fills the right atrium is dumped into the right ventricle and then pumped via the pulmonary artery (only artery which carries de-oxygentaed blood) into the lungs where the magic of blood gases crossing semi-permeable membranes in the alveoli occurs. The blood is then transported back to the heart via the pulmonary vein (not to be outdone by the pulmonary artery is the only vein in the body to carry oxygenated blood). The blood then fills the left atrium, collects, dumps into the left ventricle and is then pumped to the body entering the aortic arch and sent to where it should go feed cells routing through either the descending or ascending aorta.  Gabriel though is not of this form, his left ventricle doesn't count. So the Norwood consisted of re-plumbing his conduit to the rest of his body so the aorta was disconnected from his left ventricle and combined with his pulmonary artery (PA) and some cadaver tissue to feed his body. His atrial septum was also  removed which did away with atria and resulted in one singular atrium. The PA was now separate from the heart and receiving blood only via a shunt which carried blood directly from his left ventricle to his PA. The Glenn now  connects his SVC (disconnected from his right atrium) directly to his PA so that de-oxygenated blood directly flows (albeit passively) to his lungs. The above image shows his current physiology; red (oxygenated blood) enters his atrium from the lungs via the pulmonary vein, blue (de-oxygenated blood) enters his atrium from his lower body via the inferior vena cava the two mix together in the atrium and dump into the ventricle and are therefore exhibited as purple and are pumped to his body via the recombined aortic arch. The separated blue conduit is the superior vena cava disconnected from the atrium and reattached to the pulmonary artery. The shunt from the Norwood has also been removed.

Monday morning I woke to find Gabriel big eyed looking around the motorhome. He looked cute and was observing everything and had removed his tube again.

With tube re-inserted we headed to Lucile Packard Children's Hospital (LPCH). Gabriel was obviously contemplating how to outwit the Doctors, detour the surgery and return home.

The motorhome is parked about 4 blocks away close to the Herbert Hoover Pavilion in Stanford's overflow parking lot. We're surrounded by cars parked so tightly that it is impossible to move the motorhome during the day and at night there is just us and two other RV's. No hook-ups but $5.00 a day to park isn't bad, you just have to have a life threatening condition to justify parking here. We loaded Gabriel in the stroller and reported to the Cardiology Center for Pre-OP stuff ; a chest x-ray, ECG, exam, vitals and of course blood draw.

Again Gabriel did his best to thwart the hospital staffs intentions. This time appealing to their historic aesthetic grabbing a classic Rubens pose as if to say "would you harm this body?" Which of course they did. We now know Gabriel has rolling veins and this is the 2nd time the common tier phlebotomist tried, failed and had to go secure the ace. I think from here on out we'll request the ace from the get go, the other pearl I learned is that a topical anesthetic must be ordered by a Doctor as the patient could suffer a reaction, so I need to cover that ahead of time.

Even though Gabriel's pose didn't perfectly match that of Leda and the Swan, I still think I've seen his pose in another Peter Paul Rubens painting.

All of his labs looked great, surgery was 6:15 in the am so we turned in early and set the alarm for 5:00am. About 9:45 we received a phone call informing us that surgery would be delayed as a water main had busted in the operating rooms and although the water was cleaned up no procedures could occur until the infection control team had cleared the area. The Dr calling hoped he could get us in later the next day and if not stated probably the same time (6:15) on Wednesday morning. We were deflated,  back into the world of "hope", "probably" and "maybe". Beth slept away her frustration, I had a shot of writing with a Cabernet chaser (maybe two). The following morning Amaya picked me up so I could drop her at work and use her car to find some form of distraction to get our minds off another failed surgery date. En route to the motorhome I received a call stating we were in, the follow up call asked if we could be there by 9am, 20 minutes away. No problem.

Beth and I donned our Gabriel support footwear, and headed for the hospital.

Mommy saying good bye after we had bathed him with anti-bacterial wipes and gowned him. 

Handing the lad off to the anesthesiology team.
Placing him in capable arms and saying our last good-bye.
The surgery was finished by 1:15, Gabriel had to be placed on the heart and lung bypass machine for about 20-25 minutes because his superior vena cava did not have enough length for Dr Reddy to clamp it above the aorta and below the jugular vein. The surgery went as routine as open heart surgery on an infant in the hands of Dr Reddy can go. We were able to see Gabriel a short time later after he was moved to the CVICU. He wasn't nearly as beat up looking as he had been after the Norwood.  He was swollen and pale but still looked like himself.

Back in the world of many machines but in actuality far fewer than last time and a fourth or less of the drugs. Still a little baby in a big whirling fast paced high tech world.
While intubated one lung had partially collapsed. The attending fellow stated they could increase pressure in his breathing tube to "pop" the lung or just extubate and observe his breathing as most often the situation would correct itself. The attending physician opted for simple extubation. The respiratory tech. removed his breathing tube and Gabriel didn't breathe; still somewhat sedated and used to not having to work for air, he just laid there. She grabbed him under the shoulders and lifted his torso so his head fell back hyperextending his neck and opening his airway. After a few sporadic gasps he was breathing and crying on his own.

That's when the headaches started. The Glenn procedure results in blood backing up in the SVC and raising the blood pressure in the brain, resulting in excruciating headaches. The headaches had not been a problem until this point due to his level of sedation, he had simply been "knocked out." Now he could only receive minimal pain killers as he needed to continue breathing (always a positive) and they couldn't use too many med.s this soon out of sedation.
Fortunately Gabriel's nurse for the evening was Manju. She worked diligently to alleviate his discomfort, trying technique after technique to ease his suffering. She rolled towels and created a "nest" for Gabriel which moved him into a vertical position and did not allow him to squirm out of it. The basic principle of allowing gravity to decrease pressure, as used with shock victims (either elevating feet to get more blood to the head or elevating the head to decrease blood flow to the head). Kind of a no-brainer (bad pun not initially intended but unavoidable to not use once identified) but no one had done it prior to Manju coming on duty. She pestered the Dr's for med.s and alternate med.s the like of which I have not seen any other Nurse do. She was Gabriel's advocate. I believe the purpose of life is to find your calling. I think Manju has accomplished life. Her large brown sensitive eyes weren't just sympathetic to Gabriel's pain there was real empathy when he would arch his back and scream. She could feel his pain and she had to stop his suffering. It was wonderful to watch a human intervening so significantly on behalf of another human and a blessing as a parent to have someone of her nature caring for your child. She pushed the Dr.s almost to the point of a spoiled child playing parents off of one other to get her way. Almost, I never heard her say "Well Dr. so and so said..." But she did continue to ask until she either received the drug she thought was most appropriate or a reasonable substitute. She finally calmed Gabriel with a mixture of morphine, versed, fentanyl and acetaminophen (tylenol). The entire time she worked she apologized for the noise in "The Pod" a large open room we had spent time in before that housed 4 other cardiac babies and their entourages consisting of; Nurses, Doctors, Fellows, Parents and other assorted persons. At some point in the history of hospital design someone evidently thought an inclusive room for pediatric cardiac care was a good idea but they were wrong. I thought the best care for headaches of this level of severity would be sensory deprivation. I thought back to the first time I dove the Draeger LAR V oxygen rebreather in open water during the Marine Combatant Diver course. We were dropped by Zodiac inflatable about 2 kilometers off shore of Panama City, FL. As a buddy team we descended to 20', purged the nitrogen from our rigs and finned toward the beach maintaining our heading and depth. The "driver" held the tactical board in his hands which was composed of two handles on either side of a submersible compass with slots to attach a depth gauge and watch all illuminated by a single chem light. On our first run I just tagged along by hanging onto my dive buddy's 1st stage regulator mounted to his tank valve directly behind his neck. All I did was fin and marvel at the quiet experience of diving the rebreather. The entire purpose of a rebreather is for Marine Recon or SEAL's to be able to swim unknown into enemy waters rebreathing their own oxygen and therefore not creating bubbles which ascend to the surface (which would give away the fact that they were being sneaky which is the purpose of being sneaky, sneaking). Diving the Draeger at night though resulted in complete sensory deprivation, no bubbles were created therefore no noise from the exhalation of air which normally comes from a SCUBA regulator. I didn't have the tac board and could not see a hint of the chem. light. Dressed in a wetsuit and encapsulated by the waters of the gulf I was buoyed and flying along completely trusting in the hands of my dive buddy not to drive us into a pier, prop or out to sea. I allowed myself to be free with the experience and feel the nothingness, it was like being in a vacuum, no sensation. The only sensation was my thighs rhythmically moving my fins to push me along at a rate even with my fin buddy, I made a loop of thumb and pointer finger and kept his tank valve in the middle. If I felt a slight tug I wasn't finning hard enough, if I felt a push I was finning too hard. Even the finning was effortless, we were paired with the Marine most compatible to our fin speed so we moved along at the same rate. We were also young and strong and breathing 100% oxygen so our muscles had all the food they could ever want. It was cool to experience until I thought of what I couldn't see there in the dark. The Gulf of Mexico has lots of massively toothed critters that feed at night. I started thinking of all the various species of sharks that call the Gulf home and realized I had better get my mind somewhere else really fast. I focused on breathing (you have to take small shallow breaths when diving the rebreather, just breathing off the top of your lungs) finning speed, hand position on the regulator and repeat. Focus on anything. Gabriel though doesn't know about sharks, he just knows his head hurts, wouldn't it be cool to have that level of sensory deprivation available for these little people with their heads splitting open?!

Manju finally found his happy place allowing him some much needed rest. Gabriel's doing OK but not all of the people we met last time are so fortunate. This is one of the best places for this procedure or any other infant cardiac procedure but they still have their own LPCH mortality stats. One little boy whom we had met during our last experience was the same age as Gabriel but is no more. Another family we met came out here from the Bronx with their little boy who had coded (heart stopped) while we were last here. They have fortunately returned to New York with their son but sadly with his brain function impaired. We are so fortunate for Gabriel to be doing so well and feel blessed to have our little half hearted guy.

Cousin Amaya snapping photos.

Of Mom and Dad enjoying their little boy.
We left him a bit after 11pm in the capable and caring hands of Manju.


Sharon Brumfield said...

Sweet! I have been praying for this little guy. I am glad that he is doing well.
Praying for you guys too.

Norma from Idaho said...

Even though most of it is gobbley gook to this commoner, I still see the miracle in what they are doing and understand that, at the best, Gabriel still doesn't get all the benefits of a "real" heart.

Love the descriptions and love the pictures. And I'm thankful you had Manju for that post-op pain period.

Now we are praying for a fast recovery for this amazing little guy.

Steph said...

Thanks for keeping us posted! So glad to hear that things have gone well! We will continue praying for you guys!

Mom Gidcumb; Dad Gidcumb said...

You do an amazing job in describing what is going on in your world. Our prayers continue to be with you and our desire is to see this journey to be as painless as possible. Will continue to pray for speedy recovery. Love you guys. Uncle Dewey and Aunt Judith Ann.