The Lump

There's a lump.

In my right breast.

I'd been having teeth-gritting pain in my right breast and finally (after much urging from Chris) went to the doctor. Because I was mid-cycle, she asked me to come back in a couple of weeks for a more accurate examination. I returned yesterday. 

The doctor found a lump in my breast and I've been referred to the Breast Clinic at the Royal United Hospital in Bath. The lump is painful and hard. It doesn't move. The doctor says not to worry. She's right. It's probably nothing. Better to be safe than sorry and all that, but I'm still shitting the bed.

A New BMT Date

I received an email this morning from the BMT team who advised that the BMT has been delayed by two weeks.  The proposed dates were rejected by the donor's centre (for whatever reason) so we will be admitted on the 10th of October, chemo to begin on the 11th and the transplant day to be the 17th of October.

I will be spending my 32nd birthday in the hospital and it is a very real possibility that we will be spending Christmas/New Year in the hospital as well. I have asked about Christmas presents and have been told as long as the things we get her are new and in the original packaging she will be allowed gifts.  The ward make a great effort for families spending the holidays in the hospital. On the bright side, I won't need to cook Christmas dinner or decorate the house!!

The hospital staff have been excellent, and have provided Chris with whatever documentation he needs for work in order to get the time off work.  

I don't mind the two week delay. Miya's so active and awesome right now, I really enjoy spending time at home with her.

Here We Go Again...

On Friday at our regular ERT, the lovely Helen told us that the BMT admission was now scheduled for the 26th of September. "WooHoo!" we thought! THEN we were told that it was our OLD DONOR that was donating. She is now well, been cleared for donation by the Bone Marrow Registry, and is able and willing to donate.

During the last three weeks they have been searching for a donor, and they have only found two 9/10 donors and a Cord Blood donor (who was a 5/6, I think). No 10/10 donor was found. IF something goes "tits up", and the 10/10 donor gets sick AGAIN, there will not be a long delay, and they will slot us in with the cord blood donor. Helen told us that in all her time working as a BMT nurse, this has NEVER happened to her.

That's all (for now), folks!!!!!

Gargoylism

gar·goyle  (gärgoil)

n.

1. A roof spout in the form of a grotesque or fantastic creature projecting from a gutter to carry rainwater clear of the wall.

2. A grotesque ornamental figure or projection.

3. A person of bizarre or grotesque appearance.

gargoylism (n.) - hereditary disease (autosomal recessive) consisting of an error is mucopolysaccharide metabolism; characterized by severe abnormalities in development of skeletal cartilage and bone and mental retardation

Gargoylism. Go ahead, Google it (or use the search engine of your choice). I'll wait...

You probably came up with around 87,000 hits.  87,000!!

Gargoylism is a term for Hurler's Syndrome (MPS 1H) and is still used today.

This is a Gargoyle:

 And this...

And this...

 

This is Caleb. He has MPS 1H

 This is Chiara. She has MPS 1H

 This is Torie. She has MPS 1H

This is Sophie. She has MPS 1H

This is Cody. She has MPS 1H

This is Jakob. He has MPS1H

  And of course, Miya. She has MPS 1H

Gargoyles? I think not.

I have been trying to think how best to describe the way I feel when I hear that awful term. Gargoylism. Yuk. The feeling I get in my heart is a terrible, sinking feeling. 

I came across what must have been a student's project for school or a biology class of some sort as I was posting videos of Miya on YouTube. (Click here for the link.) This was posted just last year!! Last year!! It brought to my attention that this horrible, degrading, archaic word is still being used today to describe beautiful children like the ones you see above. (By the way, I do have the parents' permission to use these photos, in case you were wondering.) 

It is my hope that when someone searches the term "Gargoylism", maybe they'll come up with this blog post. And click on it. And stay on it long enough to scroll down, and see that these people aren't gargoyles. They're kids. They're people. One of them is MY kid!!I admit, this once was a term used to describe MPS 1...

BEFORE THERE WAS A NAME FOR IT!!!

If you're reading this for a school project, or for research purposes, or whatever, please, please, please think twice before using this horrible, derogatory word in your essay or paper or presentation. These are people! They have families and friends who love them very much. They are loving and love to be loved. They are regular people who like to do regular things, and just happen to have a terrible illness. That illness has a name. It is Mucopolysaccharidosis Type 1H also know as Hurler's Syndrome.  Please use the correct term instead. We as parents of children with MPS 1H thank you. Our children thank you.