On The Eve Of General Anesthesia...Some Technical Stuff & Some Pet Peeves

Tomorrow is an important day. And a scary day. Tomorrow is Miya's first general anesthesic as a diagnosed Hurler's Syndrome patient.  Tomorrow, Miya will be put under (briefly) for a CT scan. Miya has been under general anesthetic before, with no complications, when she was six months old. (If you've been keeping up, you'll know that Miya was born with talipes and had a tenotomy to loosen the tendon in the back of her right foot...Remember? The surgeon is the one who thought she might have a chromosomal disorder....No? Alright, well now you're up to date.)

Hurler's patients are notoriously difficult to intubate. I have just read an article (http://www.anestesiarianimazione.com/2010/Hurler%20Syndrome.pdf) which indicates that "in Hurler’s syndrome airway problem has been described as the worst in pediatric anesthesia". Not exactly confidence-inspiring. "What makes Hurler's patients so difficult to put under?"  you might ask. I shall tell you...

The most difficult problem is "maintaing the ariway because of the anatomical changes in upper airway due to deposition of mucopolysaccharides (GAGs) in tongue, tonsils, adenoid, epiglottis, glottis and trachea".  Chest deformity can also cause issues. (Miya's ribs are flared on her left side. Hurler's kids tend to have oar shaped ribs. I don't know if this affects her airway or not. Nobody seems to be concerned about it, so I'm not either.)

How Miya reacts to this anesthetic will determine whether or not she has a gastrostemy or not. The preferred gastrostemy for the Bone Marrow Transplant team is called a PEG (Percutaneous Endoscopic Gastrostemy). Nikki, the Clinical Nurse Specialist for the BMT ward at GOSH says that with a PEG in, children are better nourished (as they don't have to fiddle around with NG Tubes) and tend to go home earlier that children who don't have them. I'm all for going home early.

On a completely unrelated note, in regards to our hospital stay, I have also advised Chris that I refuse to be sans internet so I have persuaded him to get a dongle (wireless broadband). We were without the internet for a little over a month at the last place we lived, and I almost died. I don't mean that figuratively. That is a completely literal statement.

As a parent, I am terrified of the anesthetic risk and I am on the fence on the PEG issue. I agree that it would probably be best for her to have one however, it would be in her best interest not to go under general anesthetic more times than necessary.

This is Miya in her Ponseti cast, after her tenotomy. She wore this particular cast for 5 weeks. After five weeks, she began to wear Markell boots and bar for 23 hours a day, 7 days a week. She currently wears them at night. This will continue until she's 3 or 4 years old.





And because this is my blog (and I can start a sentence with "and" if I want to), I am going to go off on a tangent  and write about some things that bother me..call them Pet Peeves, if you will..I'm sure I do.

(Hurler's) Pet Peeves and (Non-Hurler's) Pet Peeves

• Bad Grammar. There is no excuse for it. And bad punctuation. Sure, we all make mistakes. I'm sure this blog is full of them (I took the liberty to mention that I began a sentence with "and"...my bad) but we all took basic English in school. Were some of us not paying attention?

• "Alot" is not a word. A lot is two words. Allotment is a word, but it has a completely different meaning than the aforementioned two words.

• I hate when the neighbours leave their washing on the line overnight...or for two nights in a row. Or three.

• The term dysmorphic when talking about facial features of a Hurler's child. We're all aware of it, it's a part of the illness. I don't talk about it. Or think about it. It's a non-issue. A moot point. It's not a nice word and I don't like it. 

•  When people use the apostrophe to pluralise (pluralize, for my North American readers). The apostrophe shows ownership or to stand in for a missing letter (called a contraction, as in the case of "don't" or "can't"). Please don't misuse the apostrophe. I find it very confusing. (I know that this falls under the grammar and punctuation pet peeve, but I find the misuse of the apostrophe quite irritating.) Interested in using the apostrophe correctly? Click here.

• Another grammatical issue I have is the use of "their", "there", and "they're". They are three seperate words and not interchangable. 

 That is all I can think of for now. I'm sure there are more and I'm sure you will hear about them in future posts.

***In this blog, I won't always talk about Miya. I think you might gain some insight into me and my family if I write a little about myself, and us. Hurler's isn't just my child's disease, it has become our disease. We fight it together. Every. Day. Of. Our. Lives.***