sniff sniff..... what's that smell? (heather, that title is for you)

Here is a little Jaiderbug update:

On Tuesday or Wednesday of last week, I started to notice that the cast on Jaidin's left arm was smelling funky. I had just changed the dressing on it, so I assumed some boob-juice must have dripped on it and went sour. So, I changed it again. But, the smell returned. More sour milk I thought. So I changed it again. The smell instantly returned. Hmmmmm..... Then Jason came home from work and told me I needed to change Jaidin's outer cast because it stunk! What?! I'd changed it 3 times that week already! So then I start thinking that maybe it's not sour boob juice. Maybe the smell is coming from underneath the cast. So I begin my google search into smelly casts. Results = infection! I get nervous....

First thing the next morning I call Jaidin's hand surgeon to inform him of the smell. He's not in the office, but his nurse tells me that casts often get smelly and that I shouldn't worry. She said that infections come within days of surgery, not 5 weeks after. I didn't feel good about it. Her right arm wasn't smelly and she had been fussier than usual. So I politely tell the nurse that if we wait to take Jaidin's cast off and her fingers are so infected that she has to get them amputated, that I am holding her responsible. Then I ask for her name. She pauses, then puts me on hold to run our situation by another surgeon. They decide that it's better to be safe than sorry and they want us to come in to have Jaidin's casts removed. (I have to admit, although I was nervous about the possibility of infection, I was soooooooo excited for my baby to finally get her fingers back AND to see how her fingers looked! I had been counting down the days for this moment for awhile!!)

After soaking her casts in the tub to loosen them up, we were off to Primarys. NOTHING could have prepared me for what I was about to see. When they took the cast off of Jaidin's left arm (the smelly one) I about had a heart attack! It WAS infected! Puss, black, red, waxy.... I cannot even put into words how gruesome the sight was. Luckily, stuff like that does not gross me out at all. Jason said he literally almost passed out. It was bad. Jaidin's other hand looked pretty bad too. It wasn't infected, but it definitely was not healed. I had no idea how scary her fingers would look. I just thought they would be healed and as good as new! NOT SO. We did some tests & blood work to make sure the infection had not spread into Jaidin's bones. Hearing that infections in bones lead to amputations was very unsettling. Luckily, we caught hers soon enough. Had we waited until her scheduled appointment, who knows how bad the infection could have gotten. All that bacteria just festering & multiplying....it could have been much worse (hard to imagine it much worse than what we saw). Well, we were able to clean up the infection and they made a temporary splint for Jaidin's hands. She was not a happy camper! Poor thing was screaming and crying the whole time. We got x-rays and then little Jaiders finally got a brake. She usually SCREAMS and cries the WHOLE way to Primarys, and the WHOLE way home. Not after this experience! I wasn't even out of the hospital's parking lot before she was out cold! And, to my sadness, my baby still does not have her fingers back!!!!! That stupid (but necessary for healing and protection) splint!!


Jaidin enjoying her first bath in almost 5 weeks! We had to soak the casts for a good 30 minutes to make taking them off easier.



Jaidin's temporary splint. They're not very efficient. They frequently pop off and they are NOT easy to put back on!

ODDD

Since this blog is dedicated to keeping loved ones up-to-date on the medical status of our little jaider-bug, we would like to post some recent updates.

Yesterday was Jaidin's 9th eye procedure. Things are looking good. The pressure in her left eye is still higher than we want (20), but, it has not gone up since her last procedure. So, as long as we are maintaining and not increasing, her doctors are happy for now. Her right eye is at a 10, which is exactly where we want it to be.

Also, we got the results back from our most recent genetics test. It turns out that our little one has a syndrome called oculodentodigital dysplasia (oddd for short). It is an extremely rare condition, with only approximately 244 cases ever known. That's an incidence rate of one affected person in 12 million people. (For comparison, autism affects about 1 in 500 people & down syndrome affects about 1 in 900 people).

Here is a little summary of the condition: (you can skip this if it looks like too long of a read; it's just a little fyi on the condition).

Oculodentodigital dysplasia is a condition that affects many parts of the body; most notably the eyes (oculo-), teeth (dento-), and fingers (digital). Common features in people with this condition are small eyes (microphthalmia), distortions of the pupil, and other eye abnormalities that can lead to vision loss. Iris atrophy, glaucoma, and epicanthal folds are very common. The size of the eyes often interferes with learning to read and special eyeglasses are usually required. Affected individuals also frequently have tooth abnormalities, such as small or missing teeth, weak enamel, multiple cavities, and early tooth loss. Other common features of this condition include a long, thin nose, and webbing of the skin (syndactyly) of the fingers.

Other features of oculodentodigital dysplasia include sparse hair growth (hypotrichosis); hair that may be fine, thin, dry, or fragile; brittle nails; unusual curvature of the fingers (camptodactyly); syndactyly of the toes; small head size (microcephaly); and openings in the mouth (cleft palate, lips, or gums). Cardiac anomalies, including arrhythmias, murmurs, or congenital malformations (ventricular septal defect) are common in people with ODDD. Some affected individuals experience neurological problems such as a lack of bladder or bowel control, difficulty coordinating movements (ataxia), abnormal muscle stiffness (spasticity), hearing loss, and impaired speech (dysarthria). Other neurological problems include abnormal white matter, conductive deafness, and various kinds of paresis, spastic paraplegia, and difficulty controlling the eyes. A few people with oculodentodigital dysplasia also have a skin condition called palmoplantar keratoderma. Palmoplantar keratoderma causes the skin on the palms and the soles of the feet to become thick, scaly, and calloused.

People with ODD syndrome often have a characteristic appearance. Some features of oculodentodigital dysplasia are evident at birth, while many others become apparent with age.

ODD is generally believed to be caused by a mutation in the gene GJA1, which codes for the gap junction protein connexin 43. Slightly different mutations in this gene may explain the different way the condition manifests in different families. Most people inherit this condition from one of their parents, but in rare cases it can arise through novel mutations. The mutation has high penetrance and variable expression, which means that nearly all people with the gene show signs of the condition, but these signs can range from very mild to very obvious.


WELL, I know, it sounds like a lot. BUT!!! We are hoping that our little one has a mild case of the disorder (for her sake). Although she already has many of the symptoms listed, there are some major symptoms (like neurological) that we don't see any signs of. Regardless of what happens and what may come....we are absolutely in LOVE with our little baby! She won us over a long time ago! And of course I think I have the cutest baby in the world!! (what mommy doesn't??)

AND!!! 2.5 weeks down; 3 more weeks to go until baby gets her casts off!!!!!!!!!!!

5 Days & 10 pounds

That's 5 days and 10 pounds heavier!!! We just got back from a little Mexican cruise. Anyone who has been on a cruise knows exactly what I am talking about! The food is ENDLESS!!!!!! It was a little tough traveling with a baby who can't stand the sun, but we still had fun! Mommy and baby spent most of our time inside the cruise ship or under the balconies. When we ported, we hunted down an umbrella and (surprisingly) it did a great job of keeping the sun at bay.

So....here are some pics from our little adventure.....






Guess who discovered how to drink from a cup on the cruise ship! She loves it! Too cute and fun to watch! Mommy & daddy were kinda naughty. We let baby have licks and tastes of pretty much everything! We had some very messy moments!