We had our “exit interview” with Drs. Whitley and Utz this afternoon. We had already seen some of his tests results as early as while he was in recovery, and those levels were normal and looking good (albumin, blood glucose, CSF glucose, liver protein, white-blood count, etc.).
Dr. Whitley also took notes from the neurological radiologist saying that the comparison between his brain MRI in October and the one taken yesterday were “stable”. Also, measurements taken of his liver showed that his liver size has gone down. This is a good thing, as the liver can take in a lot of the substrate, which can cause the liver to be enlarged. Having it shrink (and the spleen hold steady)? Very good news.
Also Dr. Utz now believes that other kids on his treatment protocol should be placed on a ketogenic diet. They are having part of their group in Minnesota be trained on the diet, in case other families are not as lucky as we are in having such a great group like we do in Cincinnati that has a dedicated ketogenic team. Dr. Utz feels that it might be a missing puzzle piece in helping these kids get enough of the zavesca into the brain to have enough of an effect. Part of this comes from a study that she shared with Lindy just over a month ago.
Afterwards, Armand got what’s called a Synagis shot for RSV. RSV is a virus that can cause broncholitis and even pneumonia. Dr. Utz noted that many times when children who have GM1 do pass away, it is often caused by an RSV-related infection. The shot he received is actually the antibody itself, not just an immunization. He will continue to get this shot monthly during “RSV season”, which is along the lines of flu season.
He did have a slight reaction to the shot itself, getting quite red in the face. They did have us stay in the clinic for quite some time after the shot itself just to monitor him, and when his face became red, he was given some Benedryl, Dr. Whitley checked his lungs, and they took a blood pressure. After a few minutes, his face began to return to normal, his lungs were clear, and his blood pressure checked out as normal. We did give him some Tylenol when we got back to the hotel, and he’ll get another dose of Benedryl later tonight. It was a very mild scare, but now we do know that before he gets his next shot in February, we need to “pre-dose” him with Benedryl. While the shot itself is technically safe enough to just be given in home, we are taking the extra precaution of making an appointment with Armand’s pediatrician that we may be able to give him the shot in a clinic to be on the safe side. If he does continue to have these kind of reactions, or if they get any worse, we’ll have to forgo this shot and just put him in a bubble during the winter.
(UPDATE – 1/18/2013, 3:43pm)
We did his neuropsych evaluation this morning, where Armand did well, but we really didn’t learn anything we didn’t already know — it is really more so that they can learn from him.
We did however finally get in touch with Dr. Karachunski regarding his MRI results. He said that the MRI looks stable when compared to his results from our last visit in October. He also noted that his liver and spleen look really good. When dealing with a rapidly progressing disease, stable is good. Stable is very good.