Cardiomyopathy

I thought it might be helpful to explain about Adrienne's cardiomyopathy. She was officially diagnosed in 2004 at her one year post transplant checkup. Her echocardiograms in the prior year had shown some abnormalities, but these were attributed to her treatments at the time. It turns out that the problem was caused by the combination of adriamycin and radiation she received when she was 9. Had she been cured and not had regular echos, we might not known of the problem until 10-20 years later.

Adrienne has what's called dilated cardiomyopathy. If you google it, you'll read about an enlarged heart, which she doesn't have. What she does have is a left ventricular dysfunction, which is characteristic of this type of damage from adriamycin. She received a very low dose of the drug, less than half what is considered the maximum allowable dose. But in young girls who receive concurrent mediastinal radiation, the effect can be compounded. Her treatment essentially permanently damaged the heart cells, which then die off over time. As they die, her cardiac function worsens.

Adrienne saw a pediatric cardiologist at LPCH for almost two years, and there was a slow worsening of her cardiac function over that time, but we all agreed to watch and wait, without treatment. At her last visit in late 2005, she had a big drop in her function and still the doctor didn't want to treat so we decided to make a change. She was at college by this time so we consulted with a cardiologist at City of Hope, Dr. Ven, who has much more experience with this type of problem. He ran a much more extensive battery of tests than were done at LPCH and immediately started her on a drug called Vasotec. This is the standard of care for people with Adrienne's problem and her cardiac function improved immediately. A few months later, he started her on a drug called Coreg. Both of these drugs take the load off the heart and also lower the blood pressure. For most people, this is a good thing but Adrienne has always had low to normal blood pressure so it was tough for her. It took awhile but she adjusted and her echos were normal, so we were happy.

Fast forward to last year while Adrienne was on the MGCD trial. She started having a lot of trouble with low blood pressure so, bit by bit, she took less of the Coreg and eventually stopped it. Then she started the Bendamustine and her blood pressure has been even lower making her short of breath, weak, and almost blacking out when standing. After being admitted this week, her pressures were extremely low (80/50) so the Vasotec was stopped.

Adrienne saw Dr. Ven on Thursday and he ran all the usual tests (echo, EKG, blood work) and things look good right now. The problem is that, being off the meds for awhile, she can go into congestive heart failure, which sounds worse than it is. It doesn't mean her heart will stop working but that it won't work well enough for her needs. They can reverse it with medication and she can always restart the Vasotec and Coreg, or even an older drug called Digoxin. However, that would also mean she couldn't receive any treatment for her Hodgkin's that lowers her BP. You can see the conundrum. It seems like we can only treat one problem at a time.

When we first found out about the cardiomyopathy, we were shocked and upset, of course. The literature on this subject is pretty depressing and the only treatment at that time was a heart transplant. Since 2004, though, a number of new medications have come on the market and there are several others in development. Stem cell research in this area also seems very promising We've felt pretty secure that her cardiac issues could be managed with medication and not something more drastic. So now we get to watch and wait again and hope for the best. As I said in yesterday's post, Adrienne is feeling well and we'll just keep our fingers and toes crossed that this continues, and that we can keep the Hodgkin's under control.