On Wednesday, January 30th we moved to Los Angeles and geared up for a marathon of appointments on Thursday. First, the bad news. We belong to a support foundation called The Heterotaxy Network. Since heterotaxy is such a rare disorder, there are only about 100 people in the network. The consensus among people in the heterotaxy network is that only 20% of babies with heterotaxy survive to see their first birthday. And, the series of surgeries that most babies with heterotaxy have ends up leading to a life of extremely limited physical activity. So much that the few people with heterotaxy who end up surviving into their 20s can't talk very long because they get exhausted from talking. But don't despair there is plenty of good news.
The first piece of good news is that we have assembled a "dream team" of doctors and institutions to take care of Lucas. Every one of our doctors is absolutely the most skilled. Our surgeon is especially gifted. People literally travel in from all over the world to see him.
Thursday we met with the director of the fetal cardiology program at Los Angeles Childrens Hospital. He's a professor at USC Medical School, and he's also our personal cardiologist. His name is Dr. Pruetz. You can read more about him here: http://www.chla.org/
Dr. Pruetz has seen us several times and has told us that Lucas has many serious heart defects, but that his heart muscle appears to be strong. This allows us more choices for surgical repair. We discussed several surgical options with Dr. Pruetz including an extremely complicated open heart surgery at 6 months old. I'm going to get pretty technical here, so just skip to the next paragraph if you want only general details. The defects in Lucas's heart include atrial inversion, ventricular inversion, major ventricular septal defect, atrial septal defect, patent foramen ovale, patent ductus arteriosis, and pulmonary (artery) atresia. Basically that means all four of his heart chambers are in the wrong places, he has 4 giant holes in his heart where they don't belong and the artery that supplies blood to his lung isn't formed. The first surgery is going to be a BT shunt, which is a short tube connecting his aorta to his pulmonary artery (stub). With most heterotaxy babies the second surgery is performed at 6 months of age. It's called a bidirectional Glenn, which involves disconnecting the superior vena cava (venous blood from upper body) from the heart and connecting it directly to the pulmonary artery. The third surgery that most (surviving) heterotaxy babies get is done at 3 years old and it's called a Fontan procedure. It involves disconnecting the inferior vena cava from the heart (venous blood from lower half of the body) and connecting it directly what remains of the pulmonary artery. Those three surgeries basically eliminate the right side of the heart, and leave the child with half of a heart. This is usually a favorable route for heterotaxy babies because most babies with heterotaxy have only formed half of their heart, and the other half is extremely weak or disfigured. We initially favored this route because it is safe and routinely done. But, after learning more about real people on the heterotaxy network who had those three surgeries, those surgeries no longer seemed like good options. Basically, Lucas would be extremely exhausted by sitting up as a toddler and by walking ten feet as an adult. Melissa and I had a long talk and decided that there must be a better way. After a series of emails between Dr. Pruetz and I, we agreed that the first surgery must still be the BT shunt from the aorta to the pulmonary artery, then when Lucas is 6 months old there would be an extremely complicated surgical option that is a possible (but not typical) way of rerouting several vessels in Lucas's heart in a complex surgery such as a double switch Rastelli slide procedure in which the aorta is moved from the right ventricle back to the normal position of the left ventricle and a right ventricle to pulmonary atery conduit made of Gortex is placed along with baffling of the atria to redirect venous returns back to the appropriate sides of the heart. During the same surgery the VSD and ASD (holes between atria and ventricles) would be closed.
Melissa and I favor the complex procedure because we want to do everything possible to give Lucas a chance at a life where he won't get exhausted from simply clapping his hands or walking ten feet. Also, Lucas is extremely lucky among heterotaxy babies because his entire heart muscle is well formed. I asked Dr. Pruetz if there is a surgeon who he would trust to do the complex surgery if Lucas was his own son. Without hesitating, he said "Dr. Starnes."
Before we had a chance to meet Dr. Starnes face to face, we had appointments with 3 different obstetricians at Hollywood Presbyterian Medical Center. That's the hospital where Lucas will be born. It's one block from Children's Hospital of Los Angeles. Since we are at 36 weeks of gestation, and Lucas is still breech, the doctors are assuming that we will have to have a C-section. The obstetricians examined Melissa and Lucas, and set a date for the C-section (or induce if Lucas flips). The doctor who will be delivering (or doing the C-section) is named Sara Beth Twogood. That's a damn good name for a doctor. By the way, she's very nice and Melissa is so relieved that we have a good obstetrician. The date of the C-section is based on when the initial surgery can be done. The B-T shunt type of surgery can only be done on a Monday or Thursday, so the doctors chose Friday, February 22nd as the C-section date. That's a great day for several reasons. 2/22/13 has a nice ring to it, it's Zach's birthday, and Andrea's dad's birthday. So, Lucas will share his birthday with some very special people. Lucas will have 3 and half days to become acclimated to the world outside of the womb, then it will be time for open heart surgery.
At about 3pm, we met with Dr. Starnes. He is the first doctor in the world to successfully perform a living double-lobar lung transplant. http://en.
When Dr. Starnes walked in to the room to meet us, he was accompanied by two other doctors who sat behind him and listened. He asked what we know. I started spouting out abbreviations like VSD, ASD, pulmonary atresia, ventricular inversion, atrial inversion, PFO, PDA, etc. I also said, "if at all possible, we'd like to avoid the single ventricle palliation procedure of the BT shunt, bi directional Glenn, then Fontan." He replied "I agree. I want to fix your son's heart. He has a strong heart and I will fix it." I asked if he had ever performed such a procedure and he confidently said "Yes, absolutely. This isn't my first rodeo." I asked him if it would be possible to repair the VSD/ASD and the complex Rasetelli double slide procedure all at once when Lucas is six months old. He said "Yes, definitely." I asked him about his plan to supply arterial blood to the lungs, and he said "we'll have to create an artificial shunt made of Gortex connecting his right ventricle to his lungs." I said "since the Gortex will not grow with Lucas, won't he eventually outgrow the shunt?" He said "yes, and we'll have to upsize it when he is about 7 years old, then again when he is around 15 years old."
Melissa and I felt so good after talking to Dr. Starnes. He comes across as a supremely confident person. Not hyperactive, or over assertive- but simply extremely confident in his professional ability. Kind of like Bill Clinton, minus the bedroom antics or Big Mac jokes. He just seems extremely trustworthy and confident in what he says. Trust me, during the conversation with him I tried very hard to find a chink in his armor. There is a surgeon in Boston who has a reputation similar to Dr. Starnes, and if I wasn't happy with Dr. Starnes I already had a plan to fly us to Boston if necessary. But, luckily that wasn't the case. Melissa and I are very confident in the ability of Dr. Starnes to fix Lucas.
Don't forget, Lucas still doesn't have a spleen, which means that his immune system will be very weak. So, if you plan on visiting Lucas- please be extremely careful. Almost half the death rate in heterotaxy babies is from infection. If you have even a mildly runny nose, please don't visit him. Also, it's likely that his intestines are kinked and twisted up. But, as the doctors say "that's just plumbing... we can fix that."
Here is the schedule for the arrival of Lucas Charles Paton:
- Thursday, February 21st grandpa Barry and Grandma Deb pick up Anthony and Stella for 7-10 day vacation in Biggs.
- Friday, February 22nd at 5:30am Melissa and Tony go to Hollywood Presbyterian Medical Center (HPMC) to prepare for the 7:30am C-section
- 8:30am Lucas arrives into a room full of doctors, including daddy. From this point on, Lucas and Tony will never separate for an entire month. An IV will be started immediately with medication to temporarily keep a shunt open that will supply blood to his lungs.
- 8:30-9:00am doctors hover over Lucas performing dozens of tests and standing by with life saving equipment if necessary, while other doctors and surgeons tend to Melissa
- 9:00 am, Melissa to 3rd floor recovery room. Tony and Lucas to the neonatal intensive care unit on the 3rd floor. Lucas will be on a ventilator and will be in a clear plastic box. It looks like a 2x3x3 foot greenhouse with heat lamps, IV tubes, and a ventilator tube inside.
- 11am, Tony and Lucas will be wheeled over to Melissa so Melissa can see Lucas.
- 11:30am Melissa will be moved to post partum on the 7th, 8th, or 9th floor of HPMC
- 11:30am Tony and Lucas will be transported via ambulance (one block) to Children's Hospital of Los Angeles (CHLA), cardiothoracic ICU
- Noon: Tony will sit beside Lucas in his ICU room indefinitely.
- Saturday, February 24th OR Monday, February 25th: Melissa will be discharged, with orders to not lift anything over 10 pounds for at least a week.
- Monday, February 25th, Lucas goes in for open heart surgery by Dr. Starnes for the placement of a BT shunt to provide blood supply to his lungs.
- March 22nd or so, Lucas should be healthy enough to be discharged. But, he will have to be on blood thinning medications and daily antibiotics forever, to keep him alive.
Please let me know if you have any questions about anything. I know it's all very heartbreaking, but keep your chin up. Lucas is going to be one of the 20% who survives to see his first birthday. We have assembled a dream team of doctors, we have the finest hospital with the most modern equipment, I will be by his side 24/7, and there is whole crew of people including Mike, Julie, John, Andrea, Barry, Deb, Okorie, Brent, Sheri, Stu, Deborah, Bob, Cameron, Rachel, Holly, Anton, Skip and Louisa who have all made it clear to us that they will do anything to ensure Lucas's survival.
Thank you so much for all of your support. Lucas will be a good kid, and someday he will thank you all for helping to save him.
Take care, and bless you all.
Tony and Melissa
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