First Post!

This is the first official post on the blog. I will be going back to fill in the time between pregnancy and now, but I wanted to get started with the new things that are happening.  So you very likely see things repeated, but I'm sure you will understand. :)

To start off, here is a summary of what is going on. This is going to be pretty boring and fact intensive, so please bear with me.

Cael has been diagnosed with Severe Combined Immune Deficiency (SCID). It is a rare condition in which he was born without a part of his immune system.  A normal immune system is made up several different types of cells. Within the white blood cells, there are several more subtypes. SCID affects the lymphocytes, which are about 30% of the white blood cells. Lymphocytes are broke into 4 types, T cells, B cells, NK cells, and immunoglobulins. Cael has very low counts of T cells, and his NK cells are also low. He has plen of B cells, but they aren't functioning because he hasn't got T cells to tell them to do work.  He is also low on immunoglobulins. All of these cells are required to fight infections. Since he is missing parts, he is unable to fight infections. Because of that, he currently has 3 major viruses. He has RSV, parainfluenza, and pneumocystic pneumonia.

He is currently being treated for those viruses. For the pneumocystis, he is getting an injection 4 times a day of bactrim, an antibiotic as well as oral prednisone, a steroid. For the RSV and para influenza, he is getting a breathing treatment of ribovirin. For that, he is put into a croup box (a soft plastic enclosed box) where he breathes in a nebulized treatment for 2 hours at a time, three times a day. He is also getting voriconizal, an antifungal medicine to prevent any fungal infections.

Right now he is in a reverse isolation room at Texas Children's Hospital. Since he is in the bone marrow transplant unit there are several other children who are in the midst of a transplant. Therefore, they are concerned about transferring Cael's infections to the other kids. At the same time, they are worried about getting Cael sick with any other infections, so they have to make sure anyone who come in his room wears a gown, mask and gloves.

The immunoglobulin can be replaced with monthly injections, like a transfusion. A IV is hooked up and runs into his blood over a few hours. However, the lack of T and NK cells can only be fixed by getting a stem cell transplant. This is done with a bone marrow transplant (stem cell transplant). For the recipient, this is a fairly noninvasive procedure. For Cael, it will be like a blood transfusion, just hooking up to an IV for a few hours. There are a few ways to get a donor. The best option is to have a sibling who is a full match (related match). However, since we have no other kids, that isn't an option.  Next option is an unrelated match. That would come form either a cord blood or from an adult donor. Cord blood is the better option since the blood is already in a bank, and they just go and get it. If an adult were to be a full match, they would either have to have the marrow taken directly from the pelvis bone, through several "shots" which each pull a little bit of marrow, or they would take a medicine that pulls the marrow from the bone into the blood stream, then donate their blood after several days of taking the medicine. If the marrow is pulled directly from the bone, for Cael's size, the donor would need to have around 10 "shots". Finally, they can use a related nonmatch (Adam or me). I'm not exactly sure how that works, or if is is more risky. More on that later, when we get more info.

For Cael, they are first looking for an unrelated match through the cord blood bank. If a match is not found, then they will use either Adam or me.

Before the transplant, Cael will have to have some sort of chemotherapy, or something similar, to kill off his entire immune system. They do this to reduce graft vs host disease (GvHD). That is where the donors stem cells attack Cael's cells and cause him to get very sick. This is not an uncommon occurrence, but is not a good scenario. I think that part is going to be the hardest. I can't imagine seeing him being super weak, losing hair, losing his color, and the loss of appetite.  They told us that most times, the kids will stop eating all together. At that point, they will have to put a feeding tube in to make sure he is getting the appropriate nutrition and doesn't lose a lot of weight.

Assuming everything goes well, once he gets the transplant, he will have a brand new, working immune system. He will need check ups for the rest of his life, but will not require any kind of future injections or anything. The new stem cells will reproduce and create the T cells, B cells,  NK cells, and immunoglobulins that are required for a functioning immune system.

There is a lot more information to come. I've already bored you enough for now, so check back for more updates. I promise it won't always be so long and boring!