CLEVELAND, Ohio, (Ivanhoe Newswire) -- A warning for expectant mothers: one in every one hundred newborns has a heart problem. Until now, these babies were treated with the same devices used in adults.
Little Vivian Andorf had her first heart surgery when she was just two hours old. “She’s missing one chamber of her heart… Veins going from her lungs to her heart they are progressively narrow,” Margaret Andorf, Vivian’s Mom, told Ivanhoe.
So far, Vivian’s had seven surgeries and six cauterizations. “I’ve seen the caths and they’re just these big long tubes and you just can’t imagine how they get in,” Andorf said.
“Most of the equipment that we use was designed and developed and produced for adults,” Doctor Alex Golden, MD, Pediatric Cardiologist at Cleveland Clinic Children’s Hospital, told Ivanhoe.
Imagine a wire the size of the cord on your headphones, snaking through a tiny baby. Using adult-sized catheters, doctors can damage access vessels in the groin and cause a blockage. Doctor Alex Golden is the first Pediatric Cardiologist in the US to use a new approved cath for kids.
“Having a cath that is 20% smaller than the smallest one we were using previously, I think, that’s a great benefit,” Doctor Golden explained.
“It feels like we have so much hope,” Andorf said. Hope that a newborn given just a five percent chance of survival will beat the odds.
Vivian has one more surgery planned for this year. The new catheter is the first in the US to be approved specifically for children.
Alex Golden, MD, Pediatric Cardiologist and staff physician at the Cleveland Clinic Children’s Hospital, talks about a new technique that is helping to shrink tumors.
We are talking about tumors that appear in infants, right?
Dr. Golden: Yes, these are benign tumors. They are not malignant. It is not cancer, but they are growths that start out either very tiny or not present at all when the child is born, then they grow to be variable in size, but can be quite large or damaging depending on the location.
What is the proper name of them?
Dr. Golden: Infantile hemangioma.
What causes it?
Dr. Golden: We still do not know exactly what causes the infantile hemangiomas. There are multiple theories, but none of them has emerged as the correct answer.
Now what happens if it goes untreated?
Dr. Golden: Some of them stop growing on their own fairly early, but the ones that get larger or in very difficult places or dangerous places particularly on the face or other areas can cause quite a bit of destruction in the meantime. So, they will grow for a period of time usually somewhere before the first birthday at which point they stop and then they can start to slowly go away, but there is a pretty significant subset of them that do not go away completely and that is really where the propranolol treatment comes in.
And you said it causes damage?
Dr. Golden: It can, so for instance a hemangioma on the nose can destroy the nasal tissues and cause deformation of those that could be permanent. There are infantile hemangiomas that occur near the eyes and it can affect vision. If it is near the airway, it can affect breathing and obviously as in many cases that we have seen it can be cosmetically very important problem for kids too.
Can babies be born with lesions too?
Dr. Golden: Sometimes, there is some evidence when the baby is born. Usually it is tiny or almost nothing there at birth and then really starts to take off within the first month or two of life; some of them can grow extremely rapidly.
Is there a reason why they grow so much more after birth, like is it oxygen?
Dr. Golden: I do not know the answer to that. When the current treatment for this propranolol was discovered, it was really by accident. Some doctors in Bordeaux, France who serendipitously found out that it worked on hemangiomas. They were using it to treat one child with heart disease who happened to have a hemangioma as well and found that the hemangioma went away and they tried it again, and pretty soon they had done a series of this and published it and the rest of the world found out about it in just 2009.
I think it is kind of scary that traditionally you would normally treat this with radiation and steroids?
Dr. Golden: Steroids is the mainstay treatment. So in the past there have been chemotherapy agents that have been used sometimes, but up until this time, the mainstay treatment has really been high dose of oral steroid therapy which has many risks.
What could be the risks for that?
Dr. Golden: There are all sorts of dysregulation of endocrine systems and hormone production. There are weight changes and weight to fat distribution changes, high blood pressure and heart disease as a result of the high blood pressure among other problems.
So by treating this traditionally, you could be affecting your child’s life forever?
Dr. Golden: Absolutely, and we find it is not as effective as the beta blockade; that is the propranolol treatment that we are using now.
So the beta blockade, explain that. Tell me what the name is.
Dr. Golden: The name of the medication that we use for these children is propranolol; it is in the beta blocker class of medications. It is what is called a nonselective beta blocker and it affects these infantile hemangiomas by killing the hemangioma cells and also by cinching down and constricting the blood vessels that feed them. By those two properties, we do not know all of the answers about how the medication accomplishes that, but we certainly observe that in basically 100% of these infantile hemangiomas that the day that we start the medication the hemangioma stops growing and begins to shrink quite rapidly.
Explain so everybody understands what a beta blocker is.
Dr. Golden: This is a medication that has been used for decades mostly in adults, but in many children as well and it has many properties. It blocks beta receptors in the body and by doing that it can lower blood pressure, lower heart rate, help with cardiac arrhythmias as well; so most of the patients in the world who receive propranolol get it for those indications. In children, we use it a fair amount for rhythm problems, for electrical abnormalities; and heart short circuit type rhythm problems in the heart, but now this new indication for propranolol has emerged which has really been amazing.
By giving a child this, does it affect their heart?
Dr. Golden: We sometimes see a little bit of lowering of the heart rate and a little bit of lowering of the maximum blood pressure that a child will achieve or maximum heart rate as well. However, essentially we have seen in our, about 75 patients that we have treated, here at the Cleveland Clinic Children’s Hospital, see no important side effects that have caused us to need to stop the medication or anything else. The medication can also lower the blood sugar a little bit; so we make sure we teach families to give feedings around the same time as the medication to keep the blood sugar up.
Are there any other risks?
Dr. Golden: We do not think there are any long term risks of the medication. We have been using it in children for multiple decades already and we know it to be a very safe drug and now we find that it is really amazingly effective, much more effective than the steroids that we had been using previously to treat infantile hemangiomas.
So of the 75 kids that you treated, 100% have gone away?
Dr. Golden: One hundred percent have stopped growing and 100% have improved with treatment, but there is some variability. Some children are much more responsive. There have been patients when we treat them, they came back in a few weeks and I am really blown away by the shrinkage of the hemangioma. And there are others that take more time, but we generally will treat for several months at least and usually past the first birthday and then by that point, compared to when we initiate treatment, we have seen very significant shrinkage in basically 100% of our patients.
Is Myla rare? Is it rare to have that great of a result?
Dr. Golden: Oh, not rare, no. I mean I definitely would put her in a category of excellent responders, but maybe 25% or so of our patients have been sort of along her lines.
Now for Myla, I think she was on the medicine, I mean, it was just an oral medicine; twice a day, I think?
Dr. Golden: Yeah. We started out at three times a day and depending on the age of the patient. We like to meet kids when they are young and small; when the hemangioma has not had a lot of time to proliferate and multiple and grow very significantly; we think we get a better result if we start early. If I meet them when they are fairly young, we will start them at three times a day dosing. Once they pass the six month mark and have a little more meat on their bones, then we will change it to twice a day dosing; that helps a lot with convenience for the family as well.
She was on it for a year, is that normal?
Dr. Golden: Well basically it depends on how early we meet them, but we will treat from the time that we meet the children and will treat past the first birthday generally; usually to about the 14 month of age point.
Would this treatment work on adults?
Dr. Golden: No this treatment is for infantile hemangiomas only at this point and I think we may find in time that research will show that there are other patient populations that the medication is effective for. There are other subtypes of hemangiomas; not infantile hemangiomas that some people have tried using it, but we do not have enough information yet to know if it will be effective for those other patients. This is a particular treatment for a particular group of patients who come as babies and who have these rapidly growing hemangiomas that we really want to stop in their tracks and prevent damage that we know can occur when they grow quickly.
Will Myla’s grow back? Is there a risk?
Dr. Golden: No, no. That would be extremely unusual for it to grow back and basically, the life history of these infantile hemangiomas is that it will be not present basically at birth, will crop up at some point, usually in the first couple of months, and grow very rapidly. It will stop growing at about somewhere between 6 and 12 months of age; it is kind of variable and then will then begin to go away, but we know that there is a large fraction of these patients where the hemangioma will not go away completely on its own and even when they do, it takes actually many years for the infantile hemangioma to recede on its own, so the propranolol really accelerates that process and most importantly, stops the growth during the growth phase to prevent further damage.
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