Created another site

I wanted to say more, with a little more privacy, so I've created a Caring Bridge site for Peanut. If you'd like to continue following our journey there, go to http://www.caringbridge.org/visit/kctwins. It's currently set to private. So you'll need to register and log in, then I can add you. I've already added most of the emails I had for our family and friends. I hope you'll check things out over there.

I may lose it soon!!

Well, I’m finally coming close to losing it! I don’t know when, but I can see me getting really frustrated and angry with someone soon. So take this as fair warning. I’m tired of it all! I’m sick of the decisions, lack of information, inability to know what we should do, and trying to maintain some sense of normalcy in the middle of it all.

Last night we realized why Dr. A. and the CAMP team differ. According to her CAMP doctors, we’re supposed to work on this ladder system. When he CO2 goes up too high or down to low, we make vent adjustments. However, we weren’t really told what to do when we reach the end of that ladder. And that happened last night. So when we contacted them, or I should say her nurse did, they didn’t really give much advice. They just pointed out that they’re to be consults to Peanut’s regular pulminologist. The problem being, Dr. A. doesn’t use the ladder system. He doesn’t believe in adjusting the vent for every little issue. And now I’m starting to see why.

Last night her CO2 levels were in the mid-20’s. I’ve always had this backwards, but apparently, it’s more dangerous to not have enough CO2 instead of too much. When you have too much, your body can blow it off and will get rid of it. But your body also needs enough for your brain to function. So if it’s not getting enough, then you’re risking brain damage. This is what we were dealing with last night. So while Dr. A. doesn’t like making the adjustments, he certainly told her nurse to lower her breath rate. Within 15-20 minutes, her CO2 was in the mid to upper-30’s, which is so much better. And even her 02 levels, which initially dropped to lower 90’s, came back to level off in the mid to upper-90’s.

We’re leaving the settings as they are for the weekend now. Since the CAMP team, or at least the one doctor we spoke with, felt that she would be OK like this for the weekend, then they could review things further. Umm… no! I can’t handle the thought of her having possible brain damage, or worse, cuz someone thinks that’s OK.

We’re dealing with two different sets of thoughts and two different protocols. One of them is much more research based, in the hopes of getting more answers to help Peanut, and other kids, further down the road. The other one is based more on real life. That one is trying to look at her quality of life and making sure that she’s not getting into dangerous zones. In fact, Dr. A. told me today that if we wanted to continue using the ladder protocol, which dropped her CO2 levels that low, then he feels that it would be unethical of him to continue advising us on much more. He wouldn’t want to be involved in decisions that could cause serious harm. Of course I can't help but wonder if we need to choose between quality of life and quantity of life!?!?! With no guarantee that we'll have both!

Everyday I think that we’re getting more answers, and instead we’re getting more questions. When we left the CAMP program last week I thought that we were heading in the right direction. And even after our initial issues between them and Dr. A., things were going OK. But now we realize that there’s a lot more that we haven’t thought of. Not only can we risk serious damage, we could end up pushing her body to become completely vent dependent. Since the lungs only need so much pressure in them to work, they work fine when that’s there. But then the vent adds more pressure, which over time creates scar tissue. And eventually, that scar tissue can only be expanded by the pressure from a vent.

So… are we helping her the best way with having her on the vent 24/7 when we know that she CAN breathe on her own? Or are we risking creating a completely vent dependent 8 year old by keeping her on it all the time? We’re really lost with all of this. At times it seems so obvious, and other times, I feel like I’m in the fog.

We’re really hitting the wall here. We thought that having this diagnosis would really be great, that it would send us on a new, and better, path. But we’re finding that’s not really true. We see that there’s just so much more that we don’t know! And we just can’t seem to get any real clear pictures for most of her problems. And it doesn’t help that Hubby and I have had about 30 minutes in the past 10 days to really talk about this. With Nutty Buddy being in and out of school (sick days, days off, weekends), and then since he can’t come to the hospital anymore, we don’t have any more than a few minutes together at the hospital, when we could sit down and talk with the doctors more thoroughly.

It feels as though we’re back to square one on so many things! Ok, maybe square 3, but still, just very little concrete info to work with anymore… So, if I get angry or short with anybody in the coming weeks, just bear with me. The holidays are coming up and my little girl just wants to be in her house for them. As it is, we doubt that she’ll be discharged before Thanksgiving, but she will be given a 1-day pass to be at home for the day to celebrate. Actually, for that Saturday, so hubby’s whole family and my parents will all come to have dinner. But I’m tired of dealing with a lot of this shit! I just want us to be in our house, taking care of her, and working on getting her better. If only we knew how to do that!

Met with our doctors here today

I wanted to give some of the latest info that we're dealing with now. We met with Dr. A. today, Peanut's pulminologist. He actually stopped in to see hubby briefly last night. So today we both talked to him. He had some reservations and concerns about what kind of life we were giving Peanut. Being on a ventilator 24/7 is extremely difficult to care for. It takes a real toll on you, the child, the family, and everyday life.

It's not that he thinks we shouldn't do that. He wants us to be aware of what we're in for. I think he wanted us to know this since we know that Peanut CAN be without a vent. She won't immediately suffer fatal consequences if her vent fails her. There are some kids he treats that this is the case. So he just wanted us to think about things. Especially since the ROHHAD team is doing things mostly as a study. Not that it's experimental or anything like that. But there aren't any real answers yet. We don't have a cause or a cure right now.

But, after talking with him today, he told us that we should certainly follow the CMH team's recommendations. He basically wanted to lift off the burden of us choosing. He didn't want us to struggle between two different medical thoughts. And it's not as though what they're doing for the ROHHAD team is hurting her in any way at all. He just knows that caring for a vented child, 24/7, is very hard on families. Which I totally understand that.

We also talked about the vent we chose when we were at CMH last week. We chose one that we thought would be best. However, we've since learned that most hospitals and pediatric respiratory units don't use it. They use the LTV. So, we've decided to go with that one instead. If something happened while she was on the Newport, and we came into the ER, whether it was here at LGH or at CMH, the staff there would almost certainly not know the Newport. Whereas the LTV is the standard. So knowing that, we knew that we couldn't take that chance.

The days here are becoming longer it seems. We're all getting bored of being in here. Plus she's still in isolation. The tests ate CMH came back negative, but then they wanted to double check here. So hopefully, tomorrow we can be out of isolation. Then we can start taking walks again. She's done hardly anything since getting back here.

I did want to say thank you to Kathy, Marianne, Mike, Nicole, and Joe for all stopping by over the past 3 days. She really loves to have visitors. Even if she doesn't seem like it when you're here, she does like to see new people. She doesn't need gifts, cards, books, or toys, just a person to talk to.

I'm sure you can only imagine what it's like, we've been here for 2 months now!! We still don't have any date for possible discharge. And since we changed vents today, they'll need to get us trained in that. But that's easy. And they're still waiting for the Medicaid waiver to get through, plus the home nursing agency needs to get the staff we need to get started. So while we really WANT to get home, we know there's a lot to take care of.

I think that's it for now...

OK, so here's the realllyyy.... LONG update!!

Well, I guess it’s time to sit down and write this all out, to explain it to everyone. Peanut has been diagnosed with ROHHAD. This stands for Rapid-Onset Obesity with Hypothalamic Dysfunction, Hypoventilation, and Autonomic Dysregulation, presenting in childhood. This was the syndrome that we have been looking into for a few months now, and somewhat expected them to give us this result. So while it wasn’t completely out of the blue, it’s not something that’s easy to hear.

What does this mean? In basic terms, Peanut’s body has no idea when it’s not breathing. She can look completely normal to everyone around her, but in fact her O2 saturations are at 80%. Normally, we are all around 95-100% for our saturations. In addition, she would often drop down to 50% sats. Now obviously, this is a bad thing. It also means that for several months, or year(s), she has not been getting a lot of oxygen many times.

She has been put back onto the ventilator 24/7 for right now. She’s maintaining oxygen sats anywhere from 95-100 pretty much all the time. Plus, she hasn’t needed to have actual oxygen pushed in as well. The vent is what helps her breath. It assists her in making sure she’s always breathing, plus it helps her take good, deep breaths. We found that she had atelectasis in her lungs. That has to do with the lack of enough oxygen getting into her lungs. There are small alveoli that don’t get filled with oxygen, so they can collapse. Hopefully, now that she has the vent, which helps her take these bigger, deeper breaths, this will resolve itself.

She also underwent about 3 different trach changes. They ended up using one that does have a cuff. But this isn’t inflated until she goes to sleep at night. This helps to prevent any air leaks. She can talk with the vent on still, although it’s a little bit harder. Plus, when she’s trying to talk, she’s actually putting opposing pressure on the vent. So the alarms on that go off all the time.

This is all the respiratory stuff! Then we have the autonomic dysfunctions, which we’re still learning more about. Plus the obvious weight issue. There isn’t anything that they can figure out on what to do with that. This entire syndrome is still unknown. They’re trying to figure it out, research it, and come up with what genetic code will show them when it’s present. But it all takes time. So, we still need to watch her diet, get her more exercise, and stay on top of all that.

They did test her neurocognitive as well. We weren’t really surprised when they told us that she’s in the above average range. She definitely tested at the top of kids with ROHHAD, as well as kids in general. We’ve always known that she’s smart though. She does struggle with certain tasks (i.e. starting a project, adapting to changes, or working with her peers), but those are things that can still be worked on. Plus, once she returns to school, we’ll be able to work these issues into her IEP (Individualized Education Plan). The doctors at CMH really want her to get back into school as soon as possible! They said as soon as she’s home, settled back in, and ready to go, she should get back into that environment.

Of course, we’ll be asked what the prognosis is and is there any cure. The answer is we don’t know, and no. The prognosis is something that we can’t say for sure. The good thing is that since she’s already ahead of the curve when it comes to her intelligence, and we’ve been diagnosed somewhat young, we can preserve all of that. But for cures, there’s nothing to “cure” it right now. It’s just using the tools that are already there to treat the various components of it. Like the vent for her breathing, dieting for the weight, etc… It’s not the best answer, but it’s all we’ve got for right now.

There are some positive things though. We live in the city where the experts are! There are only 2 places in the world that study and research ROHHAD, one is in France and the other is 10 miles from our house! So, we know just how lucky we are in that aspect. Most of the patients that make it there are from out of state, and some from out of the country. The other good thing is that we got in to see them and that she’s been diagnosed. Now we can manage her care and get her to live at the top of her game. Give her the best treatment we can. Plus, we’re willing to be very involved in the program at CMH. I haven’t mentioned this part yet, but Peanut became either patient number 53 or 54 that they know of with ROHHAD, and that’s in the world!! Soo… we’re dealing with something that is so far beyond rare it’s not even funny. But, that also means that we, as advocates, can have our voice heard as well.

Now, we’re back at LGH until probably next week. We’ll definitely have a lot of nursing care when we get back home. All of that is still being worked out though. She would love to have visitors, as long as you’re 18 and older. They’re not allowing anyone under the age of 18 on the floor now, cuz of the flu. So even her brother hasn’t seen her in a week. And she’s in isolation, but hopefully the tests they ran come back negative tomorrow. It’s just a precaution.

I think that’s it for now… I’m sure I’ll have more to add later. But this gives you the major points of it all! Keep in mind she was tested and monitored for 72 hours straight and hubby and I spent about 4-5 hours/day in offices with doctors. So there’s a lot to take in.

I will eventually update more

But not right now! I need to get it all thought out and get all the info straight in my head. It's a lot to take it. We are back at LGH though, which makes it much more comfortable to deal with. But it's still ot home and it'll still be a LOT to go through!! Anyway, I should have more info up here within the next day or two.