Going to see if I can (semi) quickly sum up the latest events. LOTS of medical info but I’ll try to make it concise yet understandable.
Manny is not doing well on the TPN. It’s not something horrible, but lots of subtle signs that are relatively easy to overlook unless you’re his Mama. I’ve been noticing them and asking questions and commenting on these signs for months now but I also don’t KNOW that I’m right so I keep researching and learning. My personal take? He's not metabolizing it well.
History lesson … Manny was with birthfamily from birth until 8 months. Arnold Palmer for a month where he had extensive GI stuff done. Went home with us 9 months. Has had troubles the whole time. We were with GI group A and they didn’t get it at all. I was admitted in the hospital with him in severe pain and problem when they were going to discharge us without even doing any testing, etc. It was the final straw with this group and I “fired” them. My first and only time of firing a doctor.
An hour later, GI group B came in. They did tests and by that afternoon, had determined his stomach had completely stopped working. They have been our GIs ever since.
During the time with them, however, we were advised to go to GI group C for a consultation and we did. They ended up admitting us and we were there for almost a month. He got so weak and sick that they had to put him on TPN. At that time, they thought it would be only a few weeks to “Get him out of nutritional deficiency.” But they SAW the horrible reactions he had to feeds.
So Group C wrote the TPN and Group B did all the rest.
Well, months went by and they still didn’t have any new plans. So I devised a new plan to go to GI Boston for motility study and see if they could see what was going on. I finally got there last September. Now it had been almost a year by this time that we were with Group C and this group is 3 hours from us.
They had originally started out doing relatively well with the TPN but towards the end, someone who had no clue what they were doing was writing the TPN and it got BAD. Bad enough he ended up in the hospital!! So I figured, might as well have GI group B write his TPN as well. I figured he’d be starting feeds per the Boston study and we might as well have all his GI care in one place (the closer place).
Uh … mistake
So Group B started taking over the TPN and initially it was better. That was in September. But it was never quite right. Something is “off” in the TPN. Missing an enzyme? Vitamins? Something. But darnit, I don’t know enough about this to even speculate!
Within this group are two doctors. We saw Dr. A. This doctor told me point blank, “I will not babysit TPN”. And he was being truthful with me. He did not. He was pushing for a surgery that wasn’t going to work and Manny would not likely survive. ALL of my doctors thought it was a bad idea.
Eventually I got our Palliative care doctor to call the GIs and work out a solution for me. I would be switched from Dr. A to Dr. C.
Thankfully, Dr. C agreed to take on our care even though it’s complex and messy. I haven’t seen Dr. A since November. That’s right, a child who should be seen every 2-4 weeks hasn’t been seen by a GI in 3 months.
All that so I could tell you the current situation.
Manny is less stable now than ever. I’m very concerned about several things going on. Weekly he gets labs and they are trending in a bad way. But I am POSITIVE I am the only looking at the labs from a GI perspective. Why? Because Dr A thinks Dr C is handling and vice versa.
Feb 5 I write a TPN guru at Chiidren’s Boston. She is the one I talked to back in September and shared my frustrations and she ran some labs and determined, yes, he was having troubles. I remembered she said there was an outpatient TPN guy who might be able to help. I couldn’t figure out how to get hold of him so I emailed her directly. Never heard back from her and figured it was a dead end.
Feb 18 Labs are drawn and I get them Feb 19. There are some things on there that are “low” that are NEVER low for Manny. And I start to digging. It all points to kidneys having a hard time. Meanwhile, he stops urinating. Completely stops. He went 36 hours without any pee. Now we’re 72 hours in and only one diaper. His potassium is low and dropping (more about that in a second). And I have not seen GI! Finally, he has a ridiculous diaper area issue … the pee is scalding his skin. Looks like a chemical burn. This has been happening since Mid December when I noticed certain things start to go awry. EVERY treatment under the sun has been tried and we were referred to a wound care specialist. Yes, it’s that bad. His anion gap is 21 (normally it’s single digits.)
Feb 19 I go to bed asking for prayers on FB. He’s a sick little dude.
Feb 20, I wake up in the middle of the night to discover the TPN doc from Boston has emailed me back. Turns out she went on Maternity leave on Feb 5 and just got my email. She suggested I go to see them ASAP!
Feb 20 I have our first appointment with Dr. C (Our new GI whom we know but has never been in charge of our case until now.)
Now here’s the cool part … I brought her the Quest magazine where we are the cover, a Mighty Manny book and a nice bag of chocolate. I want this meeting to go well! Ha. Before we even get started I thank her profusely for taking over our case. And she stops and admits that he is way over her head. I tell her that he’s over EVERYONE’s head but I thanked her for not giving up on us!! That was HUGE.
I also told her of my middle of the night email from Boston offering to take over his TPN management if/when we can get back up there. She said YES. And basically asked how quickly we could get there! She will work with them on coordinating local care.
As we proceeded with the visit, she had a lot of concerns about his current health and heard all of my concerns. She felt there are a lot of adjustments that need to be made as well as a lot of tests that need to be run. There are also tons of things she wants Boston to sort out. Specialized tests. So we might be in Boston for a while if/when we go.
All of this was a direct answer to prayer. THIS is EXACTLY the solution I wanted. A local doc who gives a care and a TPN team who knows their stuff. J
Meanwhile … remember those symptoms I told you about earlier? They are very concerning and contacted my pediatrician on 2/20 and she agreed. She got me in to a nephrologist on 2/21. As I write this current part, we haven’t been to that appointment yet … it’s in a few hours. But his constellation of symptoms is concerning.
For example, his potassium: Should be 3.9-5. He used to live around 4.5 which is basically perfect. For the whole time he was on TPN he was stable with potassium. He was getting 20 meqs per day. Then in about September, his potassium level started to drop below normal or hover around 3.9. So they added more potassium in the TPN. Repeat. Repeat. And now he’s below normal with 80meqs per day. Why the increased demand for potassium?!? And if you’re not really paying attention to the behind the scenes it’s easy to miss that. Like Monday’s labs? His potassium was 3.9, the lowest it can be and still be normal. If you were to just glance at those labs, you’d see it was fine. But what you miss is … what all did it take to get that barely in range? And did that cause his system to become too acidotic? Now he’s breathing weird and his Co2 level is low. He’s dehydrated. His anion gap, Etc.
Finally, he’s needing oxygen last night. He NEVER needs oxygen.
Oh Manny … you love to be complicated don’t you?!?