So we just got back from spending the week at NIH and right now feel nothing but confusion, fustration and worry. They are thinking that these spots on Katlyn are something called Dermatofibrosarcoma Protruberans or DFSP. These type of tumors are very rare and so far they have not seen a patient keeping getting more, like Katlyn has. I think there's about 10 or so of them now. Usually this tumor takes like 20 to 30 years to start becoming a growth. They mentioned that with Katlyn that could be difference between them taking so long to begin that process. Once they do start growing, they do grow very quickly and there is concern that they could metastasize to other parts of the body, the most common place being the lungs.
At this time they are still uncertain about what we are going to do to remove and control these tumors. Katlyn's doctors will have a metting in two weeks with the tumor board and hopefully a decision can be made so we can start the process of getting Katlyn better. While we were there Katlyn met with a surgeon, dermatologists and several oncologists. They will also be present at this meeting. It's possible Katlyn may undergo radiation, chemotherapy or surgically removing them, or a combination. In the past they see the most sucess with removing them, with a less chance of reoccurence.
The trouble is that Katlyn has so many of these. They think it's possible that this type of tumor could be linked to her condition. So far, they have found 5 other patients with ADA SCID who have had DFSP as well. All the other patients have had stronger immune systems than Katlyn's is. The tumors were found after their treatment and the immune system had started to restore itself. Some believe the immune system does play a role in tumor growth, and that the immune system is very important. Katlyn's doctor mentioned the possibility of starting Katlyn back up on PEG ADA, to help her immune system now. PEG ADA is not a long term fix and tests have shown the effects of PEG ADA do wear off. If they were to use PEG ADA, there is a great chance that it would basically void the gene therapy that Katlyn had. They do not know if a second time at gene therapy is a option or not. At this time they are not doing it multiple times, and Katlyn having these tumors could disqualify her anyhow. Her doctor also mentioned a bone marrow transplant might also be an option.
We are so stressed right now. We are so scared. I don't want anything bad to happen to my sweet little girl. I don't want her to have to go through anything else, with all that she has been through. But more importantly, I just don't want to lose her. It almost seems as though similar emotions that we had in the beginning are coming back, but this time are worse. We now know with experience, the kinds of things we are looking at. There's just no other way to put it. WE ARE SCARED!!!
Since Katlyn has been sick, I have really been able to see the power of prayer. Please keep our little girl in your prayers.
