Friday, July 29, 2011

Funky town ...

Sometimes, we all get in a funk. I'm in one now, up to my shoulders. The easiest thing to do is hide myself away in the house, soak up the misery, and wait it out. Unfortunately, Emily is very high needs and soaking in misery is not an option. Rather, I am running around all day doing her medical care and trying to sprinkle some play and love and cuddles in, while splashing in the misery puddle.

I know it's not easy having a special needs child, and I know it's not something to take for granted. There are a million amazing wonderful fantastic things I have gained by being Emily's mom.

But sometimes it's just HARD. It's summer, and everyone around us is running around and doing fun things. The fair is here, but Emily wouldn't like it. She can't handle the heat, she doesn't eat the delicious and disgusting fried foods, she hates the smell of animals, she wouldn't be able to ride hardly any of the rides. She gets bored and grumpy very easy. We live mere miles from the beach, but there's no way we can go there. Emily hates sand, she hates water, not to mention that she has a central line that would NOT be good to get sand/water in. We would have to just sit on a blanket (after carrying here and all her connected accessories because her chair can't roll on sand). She would be miserable, and in turn so would we.

The biggest frustration is that we can't just pick up and go. Most people with kids can, even though they might think they can't. You grab a diaper bag or a bag of snacks and you go. In my case, I would have to pull up meds for every few hours, make sure her TPN is running okay, get her IV bag ready for the afternoon, and prep the next round of TPN. Not to mention figure out where to change her diaper/clothes when she has an explosion, and how to clean her up if she retches all over herself while we are out.

I'm just frustrated. I know that I am incredibly lucky to be Emily's mom, and I wouldn't change it for the world. Please don't tell me how blessed I am. I know. I am also tired, sad, frustrated, and bored at home.

And if you are one of our friends in real life and reading this, PLEASE do not be upset or offended by this. It happens every day, whether you invite us somewhere or not. PLEASE do not stop inviting us to every thing. The rare times that I *CAN* get out and go do things with you, are worth all of the "No, sorry" replies. ((love))

Thursday, July 28, 2011

Knock on wood ....

I spoke to the GI on the phone yesterday morning and we decided to wean off the Baclofen that was started to help with Emily's retching. I told her that we hadn't noticed a difference. Then my husband reminds me that Emily didn't wake up retching yesterday morning. She just woke up. It was such a NORMAL thing, that it didn't strike me as to how abnormal for her that was! This morning, no retching.

Could it be? I need the most gigantic piece of wood ever to knock on as I say that two mornings in a row she hasn't retched ..... KNOCK KNOCK KNOCK.

I just emailed the GI again to let her know that I am not weaning the Baclofen after all and perhaps it's working!!

We are going to try a gj-tube again for Emily. It will be placed by the GI herself during an endoscopy. This is a last ditch effort to get meds in Emily. Please cross your fingers, pray, turn in circles, and hope it works!

Tuesday, July 26, 2011

We went on a little vacation!

We decided to go out of town last weekend, to visit friends and support them with a fundraiser. They had a second annual bbq fundraiser to raise money for the Foundation for Children with Microcephaly. Emily doesn't like being away from home and was a grump most of the trip, but it was SO nice to get out of town for a few days! We visited the Charles Schulz (Charlie Brown creator) museum, and Jacob had his picture taken picking the nose of every statue we saw. :)

We are home now and settling in. Emily is doing the same, not very well. Her g-tube is draining 24 hours a day, except for right after giving meds. She is still retching and vomiting, and crying. I emailed the GI this morning but haven't heard back from them yet.

Something has to give, this girl needs a break!!!

And for all of you who live local to us, mark your calendars - October 15th is going to be Emily's giant 8th birthday bash! :)

Thursday, July 21, 2011

Where we go from here

We had TPN clinic yesterday, and I think I left equally frustrated as I arrived. It went beyond the fact that we weren't seen until 3:45, and our appointment time was 2:40. I make appointments for as early in the day as possible when we go to UCLA, because the traffic coming home gets horrid. It took me over 2 hours to get home, and it's only 42 miles.

The good news is that Emily weighed 15.9 kg, which is officially 35 pounds! So her nutritional status is good on TPN, at least it's doing one good important thing.

Her retching and vomiting however are uncontrolled. We discussed a few things, and for now we are going to try Baclofen. The thought is that it will relax the muscles in her stomach, and hopefully ease the retching. As a bonus, it should relax the spasicity and clonus in her feet/ankles. The downside is that it will further relax the rest of her body, which is already low tone. He said that if her drooling gets worse and she's choking more on secretions, we will increase her robinol. So we add a med, which will make us increase another med ... and then he has the audacity to say "She's sure on a lot of meds, we should see what we can discontinue soon". Seriously? Ugh.

If the baclofen doesn't work, we will consider a seperate j-tube. She doesn't tolerate gj-tubes, she vomits bile and screams with j-tube feeds. The thought behind doing it is that at least we could get her meds in her, without them being retched up. We leave her g-tube open to gravity 24 hours a day, except for right after giving meds. We're supposed to clamp it for 30 minutes to let them absorb, but I wait 60 minutes. Even at 60 minutes, a large portion of them drains out.

We go back in 2 weeks. I am crossing my fingers that the Baclofen works, without having too much detrimental effect.

Wednesday, July 20, 2011

TPN Clinic today

Emily has an appointment at the TPN clinic today. I am nervous. She is in a bad way again. Retching all morning, throwing up meds, grumpy most of the day. I hate this for my girl!!!

I am fearing that they will want to admit her again. I am going to be strong and tell them NO. We have a 4 day trip planned, we are leaving Friday through Monday. We are going to visit some friends and have a relaxing weekend. They can admit Emily as soon as we return if they want.

And then I realize how insane it is that I talk about admits like this ... but I just counted up on her medical sheet that I printed for the TPN appointment, and she has been in the hospital 16 times since January. Please cross your fingers and pray that they listen to my wishes!

Sunday, July 17, 2011

Slow and steady

We are doing good at home so far, KNOCK ON WOOD! :) Emily retches every morning for a while but it's not too bad. She is her normal happy self otherwise! As long as Fresh Beat Band plays over and over (thank heavens for DVR) she is content. She is playing with her toys and happy. It's so nice to be back at our "new kind of perfect". :)

I am in the midst of a huge organizing stretch. This house has gotten out of control while we were in and out of the hospital for the last 4 months. The first thing I tackled was Emily's medical supplies. You have no idea how happy this shelving unit makes me. I am such a supply geek!

Friday, July 15, 2011

Home again!

I'm sorry I haven't updated since the last post, which was quite a crisis time. She stabilized that night, and hasn't had another episode like that. Thank goodness! The metabolic geneticist came in the next day and talked to us in depth. He feels that the episodes were not true metabolic crisis but rather a reaction to pain. It makes a lot of sense, when we review it looking back. Her lactic acid was high, but that is expected when you are in pain or stress. This was the first time we had met the geneticist at this hospital, and he had a lot of good information for us.

He truly believes that Emily has some sort of maternally inherited mitochondrial disorder. I have reflux, migraines, fibromyalgia. Jacob has migraines and reflux. Emily has everything under the sun. We are waiting on authorization to do a mitochondrial gene array, and a whole exome study. He is also referring us to a different metabolic Dr, who is the leading mitochondrial specialist on the West Coast. His waiting list is about 12 months long, but hopefully he will get us in sooner when he sees how fast Emily has been going downhill.

GI wise, things are the same. She is on TPN for 22 hours a day. She retches a lot in the morning, but it's not too bad during the day. She manages to keep most meds down now. They started her on Rifaximin for bacterial overgrowth. It's a nasty, thick, orange medication. As long as we give it at a different time than her other meds, she usually keeps most of it down. As a side effect, her poop is bright orange colored which is interesting.

We go to GI clinic on Thursday, and then we are going out of town for four days. We are going to visit some friends we haven't seen in a while. It will be SO nice to get away for a bit! I can't wait. We have told Emily that she is NOT allowed to relapse, as we have vacation plans! :)

Saturday, July 9, 2011

Back in and not so well

Emily was re-admitted to the hospital on Thursday. She is back to retching, gagging, screaming. Her g-tube is draining so much that she is on an IV of fluids in addition to her TPN. Today she has had three horrendous episodes of what appear to be metabolic crisis. She screams hysterically, cramps up, her legs and arms get bent and stuck, she shakes and trembles, and drools and gags. The last time she did it today the nurse called the Dr's in. They immediately ordered stat labs to check her metabolic function and found her lactic acid to be high. They said it wasn't quite "acute" but was elevated enough to be concerning. It shows that she is verging on metabolic acidosis. She is stable and asleep for the night, and we are going to see the genetic/metabolic/mitochondrial team in the morning. Please pray for some answers. The neuro and GI still think that everything that is going on is a progression of her underlying disease. Without knowing what that disease is, it's hard to know how to treat or how far to push her. Please pray for answers and guidance!