Neal Freeland

Engineering/marketing manager, family guy. My personal blog with a few work thoughts mixed in.

the double switch

with 4 comments

amelia is dying. though i’ve kept a positive tone in these entries, it’s now time to match unvarnished words to the harsh reality of the challenges we’re facing. her ventricles are swapped, meaning her left ventricle, designed to supply blood to high-pressure body, is instead flooding the low-pressure lungs. she’s been taking maximum dosage of two diuretic medicines to shed this extra moisture, but still her breathing is difficult, which can be seen in the soft bobbing of her head. though we’ve been fortifying her breast milk with formula, her labored breathing is using up the majority of her caloric intake, and she hasn’t gained weight for over two weeks. the irony is that even as her heart fails the rest of her body grows. she’s gained several inches in length, stretching her weight over a longer frame and making her deep blue eyes her defining feature. full of curiosity, they engage the world with an imploring sparkle, asking "what’s going on?".  as her father i also see her faint fear, and hear her gentle request: "help me."

the procedure she needs is called the double switch. the first switch is to correct the blood supply from the heart to the lungs and body. currently the left ventricle supplies blood to the lungs through the pulmonary artery, and the right ventricle to the body through the aorta. this is backwards. since it’s not possible to change the position of the ventricles inside the heart, the solution is to swap the arteries.

unfortunately, the first swtich creates two isolated loops – body and lungs – which prevents blood from circulating. normally blood flows from the heart to the lungs to pick up oxygen, then back to the heart to be sent to the body to feed the brain, arms, trunk and legs, then back to the heart to start the cycle over. the second switch is thus required to reestablish this interconnected cycle. the return vasculature must also swap destinations.

the first switch has a high likelihood of success. the arteries are big and meaty, so it’s relatively straight forward to suture them in place. the second switch is much more challenging. the return vasculature is tiny and soft, and amelia’s heart is only the size of her little fist, which is an unthinkably small space for the surgeon to operate. it’s like sewing wet tissue inside a matchbox. impossibly, the hope is that these sutures will endure 70 beats per minute for 80 or more years. though the data are promising for the short (30 days, 95% survival) and mid-term (9 years, 85% survival), no study has yet been conducted to determine long-term success.

while it’s clear that amelia needs the double switch, the timing of her surgery is in question and we have conflicting opinions from our doctors. dr lewin has recommended doing a preliminary surgery, a simple procedure to place a small band on her pulmonary artery. this will relieve pressure on her lungs and enable her to grow for year. with a bigger heart, the double switch should be more likely to succeed. the staff of children’s heart center concur with dr lewin’s recommendation, as does dr brawn of england, who sent in a written opinion. while none of the surgeons at children’s are proficient at the double switch (we will have to send her somewhere in a year to have the procedure done), dr brawn is one of the few surgeons in the world who can do it.

however, dr mee of the cleveland clinic has a different recommendation. he believes that amelia should receive the double switch when she is only 3-4 months old (she’s 2 now) and sees no benefit in the preliminary band procedure: "i’ve done the double switch successfully in a newborn. i can do it in her." dr lewin has cautioned that surgeons, especially heart surgeons and even more so heart surgeons that work on kids, are a special breed of doctor. to manage the stress of their job, they must be supremely confident in their abilities. so is dr mee overly confident?

i don’t think so. he is widely recognized as one of, if not the, best in his field, with success rates substantially higher than most other surgeons in the world. because of this we have been planning to send amelia to dr mee eventually. while dr lewin’s recommendation makes sense – a larger heart should be easier to work on – it seems a little strange that dr lewin is saying dr mee will be more successful if we wait a year, while dr mee is saying it doesn’t make a difference.

the pulmonary band is also not a sure thing. shrinking the diameter of the artery will reduce flow to the lungs but it could send blood across the vsd hole between her ventricles, causing her tricky tricuspid valve to leak or mixing blue blood with red, pushing her oxygen saturation levels down. additionally, the band procedure means two surgeries, and there’s always risk of a negative reaction to anethesia during the operation or infection after.

so we’re going to go straight to the double switch in cleveland and against the recommendation of our doctors here in seattle. this is tough, especially as dr mee is just a voice over a long-distance phone, while dr lewin sits in front of us while at the hospital down the street. in any case, we’ve made our decision. now it’s time to put it into action.

national library of medicine – related articles and data

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Written by nealfreeland

September 19, 2005 at 9:23 pm

Posted in Uncategorized

4 Responses

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  1. Wow, Neal, that\’s intense. I\’m thinking of you and your family and her smile…

    Ann

    September 24, 2005 at 9:53 am

  2. As I have personal experience with Dr. Mee from the Cleveland Clinic, I can assure you that he says exactly what he knows to be best for your child. He has a true knowledge of his profession and a true love of children. He has dedicated his life to both. As a result, I have the utmost confidence in his abilities. I never for one second thought of him as overly confident. I thought of him as genuinely knowledgable in his field. Here\’s the short of my story…………On September 24, 2002, my daughter(Morgan) was born with a congenital heart defect known as Tricuspid Atresia. She also has hypoplastic right ventricle, atrial septal defect, and ventricular septal defect. These were diagnosed prior to delivery. She was born at a neighboring hospital of the Cleveland Clinic. They are their competition(Sheesh!!!). Prior to Morgan\’s birth, we had researched many hospitals and surgeons. As a result of our findings, we decided to transfer her to The Cleveland Clinic and to Dr. Mee\’s care. She was 2 weeks old. Anyway, Dr. Mee walked into her case completely blind because still to this day, the neighboring hospital has not sent over my daughter\’s medical records. So, he really had little idea of what to expect from our case and very little or no knowledge of our daughter. We met with him to discuss Morgan\’s case and within about 1 minute, we knew that he was the man for us. He and his staff were pillars of information and professionalism. They were great! I witnessed why he is so highly regarded by his staff, many families, and the professional community as a whole. Since that meeting, he has performed one surgery on Morgan. He will perform another in about a year or so, and possibly another later on. I am proud to say that today my daughter is generally a healthy and happy 3 year old little girl. Other than her relatively small stature, occasional bluing, and breathlessness, you\’d never know that she has her issues. But, dealing with those things pales in comparison to what life would be like without her! I am so honored to have my daughter. I am just as honored to have met Dr. Mee and his staff, and to have entrusted them with my child. They are the BEST!!! I hope that this raving commentary helps to alleviate your fears. Best of luck to you, Amelia, and your family! My thoughts and prayers are with you all. Alicia M. Boothe

    Unknown

    September 29, 2005 at 3:55 pm

  3. Just wanted you to know that my thoughts & prayers are with all of you. I have never had this challenge, or anything close to it, but as a Mother & Grandmother, it has to get you in the heart. I keep going into your website to look at that sweet face. I couldn\’t sleep last night because I kept seeing her face-don\’t know if I was actually dreaming about her or just had her on my mind. I think about all of you everyday & pray so hard for you to be able to watch her grow up. I keep babies her age at daycare everyday & maybe that is one reason, other than she is my family, that I am having a hard time dealing with this. Please give Julia my love & kiss those babies for me-I\’m glad your Mom & Dad are there to help out & also enjoy those precious babies. Love you,Aunt Jan

    Neal

    October 23, 2005 at 10:54 am

  4. It\’s hard, near impossible, for me to put language to what youv\’e written and experienced. It\’s humbling. i read your words and immediately, I live inside your language and see and feel something of what you have suffered. Thank you for including me. Your last entry was earlier this week and I hope all is getting better. I look at your photos, your family, glimpses of your life in seattle, and I still see the 14 year old version of you warming up for a soccer game, see you sitting across the table from me at lunch or discussing the pluses and minuses while planning your schedule.I\’ll keep up with your blog. By the way, it\’s the first blog I\’ve ever read. I\’ve heard a lot about them on the radio and whatnot, but I\’ve never looked at one before. Also, until you reminded me, I hadn\’t thought about a tuba in years. I guess they are better left undrawn.mac

    Neal

    October 23, 2005 at 10:56 am


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