Brief Updates...

Life is busy... way too busy... plus, it's that time of the year where we are seeing most of our specialist for follow-ups... so I blogging took a serious backseat.

Let's start with today because it's the most fresh even on my mind... ENT... scratch that, I lied... I need to give you a little back story before I detail today. As you know, we've been working with ST since July 2009 with Brice and August 2010 with Pierce. Recently, ST and I were discussing the possibility of Apraxia and the kids... long story short, she brought over a Childhood Apraxia of Speech checklist and sure enough, it looks like we may be dealing with apraxia. She was able to mark off all but a few items for both Brice and Pierce. So fast forward back to today's ENT appointment...

• Pierce
• physical exam - no issues
• hearing test - ear drums were a little stiff but nothing too serious; OAEs were good; overall, hearing looks good - we follow-up in 6 months for a re-eval to determine if he truly wasn't hearing some low level tones or if it was just because of his age
• sleep study - will take place sometime in November or December
• Brice 
• physical exam - no issues
• hearing test - no issues and my big boy did the entire hearing test on his own while I was in the other audiology suite with Pierce!
• hypernasal speech - B is a little over 8 months post-op from his T&A and still hypernasal (speaking through his nose) with his speech... according to Dr. S this occurs about 1 in 5000 T&A kids (go figure, right?). Dr. S has two ideas:
• 1.) He asked if Brice had eve been worked up with genetics, which he hasn't... his hyspernasality could be due to low muscle tone which could be related to mito disease. He, plus a few of our other doctors think that they both have the same genetic thing going on, but what exactly, is still a little unknown. So off to genetics we go for a big work-up (microarray, amino acids, CBC, CMP, etc = LOTS of blood to be drawn)
• 2.) We are being referred to a Cranio-Facial surgeon to discuss the hypernasality. It could have been caused by the T&A. Dr. S felt as though he left behind enough adenoid tissue that would have negated this issue, but at this point, nothing is certain. We will be seeing cranio-facial on July 13.

Pierce has his quarterly follow-up with genetics on Thursday, June 28. We will be getting back the results of his mitochondrial DNA bloodwork. I really hope it gives us a bit more insight into his disease... having an unspecified one is so tough because no predictions for the future can be made and there are just so many unknowns.

Both boys saw pulmonology sometime recent, I think?? Pierce's was pretty non-eventful - just come back when lung season kicks off type of deal... Brice, not so much... he starts daily preventive treatment on September 1 using an Alveso inhaler. We were also given an Rx for Xopenex rescue inhaler and Xopenex neb vials. He goes back in September too.

And last, but certainly not least... immunology. We made the switch to SCIG (sub-cutaneous Ig) infusionson May 30! We've completed 4 SCIG infusions since then, with our 5th one taking place this coming Wednesday. So far, they are tolerating the new drug well. We did our first two infusions in the hospital. 

The last two infusions have taken place at home. They are going marginally better than either infusion did in the hospital. It looks like a lot of prep work but it's really not...

Right now, we are doing weekly infusions on Wednesdays. At some point, we will slowly transition the boys over to weekend infusions that way it won't interfere with school during the week.

And for now, that's it because I have about 10 orders to go work on - look for another post on Thursday afternoon :)

IVIG

My lone post for the month of March touched lightly on our IVIG journey. I have to say that so far it's going very well. Brice has completed 4 infusions and Pierce has completed 1. Brice presented with an ear infection at this 1st infusion --- He also got sick about a week after infusion #3 (double ear infection and red, swollen throat - Rx'd a z-pak) --- IVIG #1 = migraine 24 hrs post; IVIG #2-4 = headache 48hrs post. Pierce's first infusion went a bit more smoothly than Brice's but it lasted much longer. And poor Pierce had a migraine for an entire day, starting a little less than 24hrs after. He was miserable. We will see how they continue to do - they may need a small dose of steroids at the 24hr-post window or they may need to switch to a different brand. They are presently on G@mmagard.

Infusion day has gotten REALLY tough with Brice. He knows what's going to happen... he knows he's getting an IV... and he knows he doesn't like it. His first IV went okay - the second was a disaster. By the third, they knew what to expect so they brought in the anesthesia team to do his IV. They were prepped to take him down to a procedure room to give him some nitrous, do his IV and bring him back to the floor. Thankfully, they didn't have to... but the kid has a reputation now. For infusion #4, in walked an anesthesiologist to do his IV. He isn't allowed to eat for at least an hour before in case they have to give him any nitrous.

Our immunologist called in an Rx of Syner@ for us. Our first time using them will be this coming Wednesday. It's basically a lidocaine/tetracaine patch that we will put on as we are leaving the house. It needs about 45 minutes to take full effect. Fingers crossed this helps reduce some, if not all, of the pain they experience during the IV process.

Last month, I spoke to the immunologist [that we see each month for IVIG] about switching to sub-q. I knew that we would have to wait because it was Pierce's first infusion but I wanted to know more about and what his thoughts were regarding it. He thinks it's a very reasonable option for both of the boys. He prefers to do in-hospital infusions at first just to see their reaction to the immunoglobulin and to have that one-on-one time with the parents. He also thinks that we will be able to find a suitable area for the sub-q infusions somewhere in the boys' flank area. As much as I don't want to me the "mean guy" having to stick my kids, I will gladly welcome it because it's a teeny needle and we get to do it in the comfort of our own home. Basically, we insert a thin needle, under the skin/into fatty tissue (sub-cutaneous), tape it down and begin the infusion process. It will take about 1.5 hours and we will do it once a week. The pump is similar to an insulin pump but a little bit larger. Home health would come in and teach us what to do, etc. The side effects are much less with sub-q because they are receiving a smaller amount of it each week. The purpose of any immunoglobulin infusion is to administer enough into the body to last for "x" amount of time. In the hospital, they are given a 4 week dose... at home, they would be given a 1 week dose.... so we may be able to completely eliminate the migraines/headaches! I plan on bringing it up again after our infusion this coming Wednesday. I would love to be able to start this route in June. The other good thing is that if sub-q doesn't work out, we can switch back to in-hospital infusions without it messing anything up! I would say expect an update next week but I make no promises because my life has the ability to go from very calm to insane in a matter of seconds... but for now, here are some pictures chronicling our infusions.

IVIG #1

IVIG #2

IVIG #3

IVIG #4 / IVIG #1