Today was another mild day for Jacob. They have been trying to wean him off the heat lamps for the last day or so, but today he just couldn't seem to keep his temperature up, so they had to turn the lamps back on. They have however, moved him from one side of the NICU to the other side, which signifies that he's more stable & no longer necessary to have him on the 'critical' side. He did have some alert times today & I got to see his eyes a bit more. The swelling has gone down tremendously as well, we are thinking he may have had an allergic reaction to the medicine used for the eye exam.

He's up to eating 45 cc's every 3 hours & tolerating it very well. Since he is still on the nasal canula, they have moved his feeding tube from his nose to his mouth.

During his awake times, I worked with him on sucking the pacifier. Although I didn't get much response from him, he did grab a hold of it & give it a couple sucks. I also noticed that he's swallowing pretty good. All good signs that eating the right way just might be in his future. We also got some 'scrap' worthy hand & feet prints for his scrapbook.

Now on to something very interesting. Yesterday while I was reviewing the Dr's notes in Jacob's chart, I came across something that the pediatric neurologist said; he thinks Jacob may have something called "hyperexplexia" (stiff baby syndrome) which is a neonatal version of "stiff-man syndrome". After reading this, I decided to do a bit of research on my own to see what it actually means. I was amazed at what I read. Here are two links that talk in detail about the syndrome:

http://www.sbs5.dircon.co.uk/medtext/hyperex.htm
http://www.medterms.com/script/main/art.asp?articlekey=8297

To me, this fits both Brianna & Jacob almost exactly:

1. exaggerated startle response (Jacob)
2. hypertonia (stiffness) diminishing over the first year of life (Brianna & Jacob)
3. strong brain-stem reflexes (especially head-retraction reflex) (Brianna & Jacob)
4. some cases, epilepsy
5. hiatal hernia (Brianna; no testing done on Jacob yet to determine if this is an issue for him as well)

Other interesting points about the disease:

1. The evolution of the disease is characterized by a delay in reaching motor 'milestones' (for example, walking).
2. The hypertonia is reduced during the first two years, but abnormal reactions such as involuntary starts remain after the slightest stimulus.
3. hypertonia is linked to a hyperactivity of cortical neurons
4. evolution of the disease is benign when treated
5. There is neither mental nor neurological deficiency.

So today, I tracked down the neonatologist & asked him some questions about it:

1. Why hasn't the pediatric neurologist mentioned this to us? Why did I have to read it in the report to find out about it?
2. If he is leaning towards this, has he ordered the test for this exact DNA to determine if this is 'the one'? If not, how come?

The neonatologist was intrigued & appreciated me being so persistent about it. When I mentioned all the things that I had read & how it matched up with both Brianna & Jacob, he was very interested. He was going to do some research on his end this evening & get back to me tomorrow morning.

I never heard this terminology with Brianna. For such a rare disease, it amazes me that the pediatric neurologist has chosen this as the possible answer. How did it get missed with Brianna? I find it very fascinating.

There are a couple questions I have that I couldn't seem to find answers for on the internet:

1. What developmental issues do the patients with this disease have?
2. Was there atrophy of the brain?

I hope to have some of these questions answered tomorrow. Wouldn't it be nice to at least have a diagnosis?

Overall Status: Good Day

Visitors: Pastor Keith Lauwers & Colleen

Father's Love Letter
Pastor Keith Lauwers gave me this today.