Philadelphia Update

Hey All-

Hans and I are finally done with scans and clinic visits here at Childrens Hospital of Philadelphia (CHOP). It was VERY busy but Hans handled everything in stride! We stayed at the Ronald McDonald House which is about 8 blocks from the hospital so we were able to just walk everywhere and not worry about a car. The Ronald McDonald House is pretty cool here. It's the first one and its an old 1800's mansion that they converted into rooms. Different volunteer groups come in and make breakfast and dinner everyday and there's lots of activities for the kids. That being said Hans just wanted to go to Chili's everyday for dinner. He is going through a serious Tom and Jerry meat fix right now. Basically any type of meat that he has seen on the cartoon (whole ham (bone-in), ribs, steaks with bones, link sausages, etc.) he eats like a lion. He pounded a half rack of ribs a day here. He cleans the bones like a dog until they're bright white.

As for the hospital, I'm very impressed with CHOP and I can see why it's ranked #1 year after year. That's not any knock on Texas Childrens because Hans wouldn't be here today without their care, it was mainly just little differences. The cancer center here is set up very similar to TCH so navigating the facilities was very easy and super low-stress for Hans and I. The really nice surprise here was the lack of waiting to get seen for appointments and scans. We rarely waited more than 15 minutes from check-in to appointment/procedure/scans. I think this is mainly because they have more equipment (CT scanners, MIBG scanners, etc.) than a lot of hospitals. Their check-in process is very streamlined as well.

Scan results? His last scans at TCH had indicated a possible tumor lesion on the liver and lesions on the left humerous (upper arm - opposite arm that he originally relapsed with). Both the CT and the MIBG scans showed the liver to be clear. His bone marrow biopsy also came back negative. The MIBG did confirm that there is something on the left arm, but its very faint. Other than his arm, there is nothing else that can be detected. Dr. Maris (Oncologist here at CHOP) said the left arm scan is troubling in a good way. The lesion is so faint that it is almost undetectable (though my untrained eye can see something is there that is different). It's possible that it isn't tumor, however given that Hans has relapsed in the exact same place in the opposite arm, Dr. Maris (and Dr. Russell) are pretty sure it is tumor. Basically he said this is about as low a level of neuroblastoma that a person can have and not be in remission. I am really happy that what we have been doing since Hans relapsed has gotten him to this healthy state.

Now that he has a diagnosis regarding the left arm, Hans qualified for a couple of promising clinical trials that Lara and I are interested in.

1)MIBG therapy coupled with the chemotherapy drugs Irinotecan and Vincristine.
In adults the combination of these therapies had a 'synergistic effect' on cancer cells. In other words outcomes were better in some cases than just receiving MIBG or the chemotherapy drugs by themselves. He will also be getting a slightly higher dose of MIBG than if he just did the MIGB therapy without the chemo drugs. Remember, though, this is a clinical trial and there is no certainty that this will have better results in children than the traditional treatments. It's possible the side effects might be worse with little or no benefit in treating the disease. This is a little scary because what if we are wrong and there really is no disease in his arm right now? Dr. Maris freely admits that some may consider this treatment a 'little agressive', but Lara and I are on the same page with him. That's why we came here in the first place because we cant do MIBG in Texas. His thought is that this is about as low a level of tumor you can have in relapse - so why not just knock it out while the disease is struggling? I couldn't agree more. Neuroblastoma sucks. It's always there hiding and waiting. Hans has done very well with Irinotecan and Vincristine in the past with very few side effects compared with some of the other drugs. We'll just see how the combo of drugs and the MIBG affects him. As before, one of the big risks is secondary infections, particularly when you are using the line all the time to administer things by I.V.

2)ABT-751
This experimental drug works by killing cancer cells and may inhibit the blood supply feeding the tumors. We are somewhat familiar with this drug because several of our friends at TCH have or are currently on this treatment. One of the great things about this is that there a relatively few negative side effects and you can remain on this drug (taken orally) for up to 3 years. Dr. Maris has a lot of experience giving this treatment and in his opinion the best results he has seen is with patients with very minimal disease at the start of treatment. In these kids with minimal disease it often works very well to 'stablize' the disease and prevent it from spreading. We are not choosing this option and that is a very tough call given that Hans has very minimal disease. Lara and I really don't want to stablize the disease. We want to be rid of it if at all possible. That's why we are going with the MIGB treatment. The cool thing is that if the MIBG treatment works, then we can still give Hans the ABT-751 drug (though not on the clinical trial and at a different dose) to help prevent relapse. Dr. Maris said he has a few kids who, like Hans, have relapsed 2-3 times and once the went into remission again they have been on ABT-751 for three years with clean scans and are ready to go off treatment. This would be the ultimate goal for Hans.

So now it looks like we will be coming back up to Philadelphia on Monday Nov. 24 and be here for about 3 weeks. He will be inpatient for about a week and then receive outpatient chemo for the remaining 2 weeks assuming everything goes smoothly. He will then come back in January for more scans and check-ups. Dr. Russell will continue to be his primary doctor and we will do everything we can at TCH, but since he is on the clinical trial, most of the major stuff/scans/appointments have to be done in Philadelphia.

We will keep everyone updated as things progress over the next week. I can't say enough cool things about little Hans. He is probably the healthiest we have ever seen him and he has such a good attitude about getting poked, thrown into weird scanners, etc. He is walking around like a little Star Wars Ewok because of his bone marrow biopsy but he never complains. He's awesome. He's still a tiny 31 pound five year old but he is getting really smart. I've loved being around him this week to watch him take a new, stressful situation in complete stride.

Lots of love and thanks to everyone for helping make Hans's recovery happen.

Weberlings