Tuesday, February 8, 2011

Explaining Surgery.

I decided I would try to back up a little and explain the details of what we are doing and why we have chosen what we have chosen with Abigail's surgery. I've gotten lots of questions and realised I may not have been the most clear about things...and then if this still leaves you with questions feel free to post in the comments or e-mail me and I will respond if I have an answer.

First of all, most of you know Abigail has a genetic disorder called neuorfibromatosis 1 (NF). The disorder (in short) causes tumors to grow on nerves. There are a few different types of tumors being common with NF, one of them being a plexiform neurofibroma (plexi). Plexiform tumors are congenital, but don't generally become apparent until they start growing. Head, neck, and face ones are generally noticeable by the age of one. Others may not become obvious until adulthood for some patients.
Abigail has a plexi on her leg. The nature of this type of tumor is that it affects multiple nerves, contains mast cells that release histamine causing sever itching, and it contains blood vessels. The blood vessels mean more blood supply to whatever part of the body the tumor is on causing that particular part to grow faster than the rest; known as hemihypertrophy. These tumors, when left alone can grow to be hundreds of pounds, cause sever pain and disfigurement, wrap around organs, muscle and bone, and can actually infiltrate muscle and bone, as well as the fact that they run a risk of malignancy. Some more recent studies are showing just how much better children who have them removed early fair later on.
However protocol for these type of tumors is still 'wait and see.' This generally means down the road that the person has to have multiple surgeries to debulk the tumor because it has become so invasive that it's impossible to remove the whole thing. The second fear of the 'wait and see' is that although the malignancy rate is 15%, those whose tumors become malignant have an average life span of five years from the time they find out. We would like to avoid these things for Abigail.
Right now Abigail's tumor is in a phase of growth. It is not uncommon for these tumors to see rapid growth during the toddler years as well as puberty (there seems to be a link between the hormones in puberty and tumor growth in NF). As a result of the growth we are seeing and the increased blood flow, her right thigh is also becoming more and more obviously larger than her left. Her right knee is significantly lower than her left, and her leg is longer as well. Although we have not had x-rays done, it is very possible that her actual bone is growing faster as well. This is something we will look into working out more after her surgery. Fixing it now would be pointless because the cause of the growth is still there.

Those are the reasons we have chosen to go ahead with surgery. The reason we chose the surgeon we did is because he is the best. As a result of these tumors being related to nerves as well as having blood vessels in them the surgery runs some risk. I have talked to multiple people who had a surgeon say, 'oh i can do that no problem' and who ended up not being able to have the tumor removed, losing use of an area of their body due to nerve damage, or having severe bleeding because the surgeon didn't realise that there would be extra blood vessels involved. I don't want to risk those things with Abigail.
Dr. McKinnon is the doctor who will be performing the surgery. He is literally one of the best doctors in the world for removing plexis. We feel grateful that he was able to do this. Because he has done so many surgeries he is aware of what is involved and is able to be honest. If he thought it would be possible to only get part of the tumor her would have told us that. And so we remain optimistic that he will be able to get the whole tumor, and that will be then end of that issue for Abigail.

I know there are some new people reading this now as our fundraiser has been passed from person to person on facebook. Thanks for your support, thoughts, and prayers. We are hopeful for our daughter's future. Although it is frustrating to know that insurance won't cover the surgery because it is elective, we also know that we serve a God who is much bigger than any insurance companies 'no.' In 24 hours we have watched almost 500 dollars come in. It moves this Mama's heart to see so many people responding with kindness and compassion to my little bug. So thank you.

Grateful beyond words,


  1. Joey had a plexiform removal (debulking) when he was just 3 months old. It was causing him pain and at first the surgeon kept saying that insurance wouldn't cover the removal and would only cover a biopsy. But I pushed and yelled and screamed and then when he got in the O.R. I was phoned after about an hour or so and told just how invasive the plexi was. He asked for permission to remove it. It was removed and covered by insurance.

    I wonder if it causing such a great deal of pain is what helped it be covered? I was also told it was growing on a nerve that controls the entire left side of the body so they kept coming in to check on his reflexes to see if he could still move his left side.

    Lots of prayers for generous donations

  2. Thanks for your detailed explanation.