Specialist

These past few weeks I've had an overwhelming need to try and connect with some other mom's or people who have what Avery has. From the beginning, our doctors have told us not to research LCH online... mostly because it would scare us. They were right! I became a member of the Hystiocytosis Association so I could connect with others that have this and do more research. There I found that the chances of having LCH is 1 in every 200,000 and even there there is such a wide spectrum that the chances of finding someone who has something similar is very rare.

There I also found that although the pronosis for LCH is very good, the chances of relapsing is also very high. Not only that but there are things called "late effects" or "permanent consequences" that affect patients with LCH long after they are finished with treatment. These can include neurological problems such as memory loss, stunted growth, learning and concentration difficulties, and higher chances of depression. A really common permanent effect of LCH is Diabetes Insipidus. A lot of tumors from LCH occur in the scull close to the pituitary gland and diabetes insipidus occurs from damage to that glad. The likelyhood of developing DI depends a lot on where the tumor originally started. 

On the Hystiocytosis Association's website there is a section where you can go and read about others who have LCH and read their stories. I went through and read most of them... and many of them had LCH reoccurrence 3 or even 4 times. 

This to me is so discouraging. I feel like I've been living in complete ignorance to what this disease can affect. I was expecting to be done after a year of treatment and that this all would be over. Just thinking that this could happen again is almost unbearable to think about. Even if we are lucky and Ave doesn't have to go through Chemotherapy again, it seems it's likely that she will have other problems to deal with. 

I've connected with another mom in Canada who's daughter is 2 and also has LCH. She expressed to me that there are no specialists and almost no doctors who have dealt with this before. Our oncologist is not a specialist in LCH but there is one where Ave is being treated. I felt really strongly that since we have this specialest that is available where Ave is being treated anyway that we should meet with him. So while we went in for treatment this last Friday we were able to meet with an LCH specialist. Thomas and I wanted to know if extending Avery's treatment longer then a year would lessen her chances of relapsing. Or if the risks of extending her treatment would outweigh the benefits. 

While meeting with him, he basically told us that there isn't enough information to know if having two years of treatment versus a year would be better. Not too long ago, they did a study where they randomly picked LCH patients and treated them with a six month treatment plan and some with a full year. The only difference was that the patients with the longer treatment plan were much less likely to have an LCH reoccurrence. This is originally why we thought that extending Avery's treatment would lessen the likelihood of her relapsing.

We knew that Avery had single system LCH which means that it was only found in one part of her body. Multi-system means that LCH is found in the bones and the organs. Avery's tumor was in the skull and had a soft tissue component to it. Because of where it is and how aggressive her tumor was, Avery is single system with risk of central nervous system involvement and has a higher risk of developing diabetes insipidus. 

We also learned what what our doctors had been telling us previously that if Avery does relapse, it will come back in the same place, was completely false. After talking to the specialist, he told us that if it does come back, she could develop tumors on the top of her skull, in her legs or arms, or even develop into multi system if it does in her organs.

Although living in the dark can sometimes be easier, I'm glad we met with the specialist and that I'm at least informed. It makes things scarier but not knowing is always worse.