A few people have asked some questions, many similar ones, so here you go:

Rafi’s weight has fluctuated a little but has generally stayed around 11kg. It’s possible her weight may change more now as we increase the amount of nutrition she receives via gtube, and she retains more water. Overall though, she has not lost any weight and has actually gained a small amount. She is significantly less active now than while at home so that certainly plays a role.

As to what and how she is eating, we are definitely seeing a shift to more gtube feeds. Before this started, most of her nutritional needs were met by drinking her “ba-bas” of pediasure. She did eat some other foods like oatmeal with either a strawberry/blueberry smoothie or applesauce with brown sugar and cinnamon mixed in. She loves baked beans, yogurt, american cheese, chicken soup, really soaked honey nut cherrios, tuna fish, chicken salad, bagels and Jackie’s fresh cookies out of the oven. But recently, and as is expected, we’ve noticed she is taking in less food orally and instead relying more on gtube feeds. We’ve been started putting her medications in through her gtube as well. This says a lot because Rafi always loved her bubblegum flavored prevacid and atarax. We do order some room service in for her twice a day to try and get her to continue eating. Sometimes it works, other times not. At some point, she wont even tolerate gtube feeds and we will start giving TPN (total parenteral nutrition). TPN supplies all her daily nutritional needs through her hickman line (aka, her central venous catheter). Everyone ends up requiring TPN for a period of time and we will try and give her as much food orally and through her gtube as possible as her journey continues. If we can do that, research has shown that she will recover from the procedure quicker.

As to Rafi’s normal EB care, Jackie and I do everything. We’ve also been told not to expect any significant help during bath and bandage changes. It’s not a question of the nurses wanting to get involved, because they do. It’s more an issue of more patients to each nurse and time constraints. I am going to have a conversation with the nurse manager to lobby for help for a chunk of time and will make this fight as far up as I have to at this point. I believe it is vitally important for a nurse to be involved in the bath and bandage change. Starting now, we need to be hyper-vigilant about infections in the wounds. If there was a hint of discoloration on the bandages the wounds need to be thoroughly assessed for infection. It is imperative to catch an infection early and be aggressive in treating it. The last thing we need is for a topical infection to turn systemic when she has no immune system. I would hope that nursing care during the one time when her body and skin is exposed and able to be assessed would be part of the treatment plan for all EB patients. Plus, many complications, like GVHD, present themselves as rashes and the bath and bandage change is the best time to make a full assessment. Starting today, I will make the case for nursing coverage during bath and bandage changes. Ideally, I would like to get one or two nurses trained in EB care who would be responsible for assisting parents in the bath and bandage changes for all the future EB kids as well. The more I think about it, the more important I think it is. I just want to emphasize that the nurses want to be involved and the help we have gotten has been great.

There was also a question as to the chemotherapy drugs. Today is the second day that Rafi is getting doses of the next two chemotherapy drugs. These drugs are the reason why we have to change her diapers every two hours. We are on schedule and February 19th is transplant day.

Some people have questions about the cost of the transplant in general. There are many factors that will affect the total cost of the procedure in the end. For instance, something as simple as how many days you are in the hospital will have a significant impact. In general, if you don’t have insurance help, you’ve got to plan on this costing $1 million dollars.