Christmas 2010

It actually started on the evening of the 23rd. After her shower Olivia came downstairs and was about to sit down on the floor in my bedroom with Tomas and I, when she gasped and stared at me, "I'm having a heart palpitation!"
Of course she had taken off her event monitor for her shower and forgot to put it back on, so upstairs we both go flying. Mike comes running out of his office thinking something is wrong with the baby. But we managed to get it one her and get 2 good recordings. What a crazy, mixed up blessing. She has been wearing that darned monitor for 5 weeks and could not wait to get it off. She was especially worried about wearing her Christmas dress with it on. So you see it really was a blessing that she had a palpitation.
We sent in her event to the cardiology center at the hospital and the montor has been "resting" in the drawer ever since. I'll take it back next week when Tomas goes in for a checkup.

On the 24th the girls sang in choir at a church pageant and then at mass. It was bittersweet for me. Earlier in the week Tomas saw hematology and immunology and among other things they reinforced that Tomas needs to stay away from crowds and germs. Last year Mike and I had to split up since he was sick, and this year we did the same. I went with the girls for the children's mass, and he went to midnight mass. It was this amazing, joyful, triumphant service, but I was alone (girls were with the choir), and missing my men.
Thankfully that feeling did not last long, and we had a lovely Nochebuena dinner once we were home. The girls started their own tradition a few years ago where they open their gifts to each other on Christmas eve. It is so beautiful to watch this blossom as they become more aware of the joy that comes from gift giving.

 and here is Tomas' gift to all of us!

Christmas morning I actually woke up before the girls. There is a first time for everything. We had a lovely morning filled with wrapping paper, chaos, and a baby who tossed and chased shiny bows across the floor all morning.

Christmas breakfast, Christmas dinner and The Nativity Story for Christmas evening. Really a wonderful day only mildly interspersed with medical stuff, which can wait until tomorrow.

There are a few things brewing and I am hoping I can make it through the rest of the year with no doctor's appts.  In the meantime:  

 

The King of Glory

I'll update all the medical stuff tomorrow after another appointment, but it is stable.
Now, I met the most amazing woman outside the hospital today. What a spirit lifter! She adopted 2 special needs kids, with the papers being finalized two days AFTER her husband died. Within 2 minutes of  talking to her I could tell she was doing exactly what God had called her to do. She just radiated it. I'm telling you, we were in the parking lot and she just blew me away. At the end of our beautiful talk we hugged like we had known each other for decades. I don't even know her name. I am so very, very grateful for that encounter.
The King of Glory comes
the nation rejoices.
Open the gates before Him,
lift up your voices.

The wait is nearly over, Advent in nearly finished, and here in this house we will be forever grateful for two tiny babies. The One who saved us all, and the other who, in his weakness, has opened my eyes to the majestic strength of the spirit that dwells within us all.

Making rash decisions

Sorry, couldn't resist the pun.
The rash is back- the interesting, unspecified, unidentified, not-going-away rash. Last week I took him in because it looked pretty bad and a few of the spots were weeping. The pedi said it was definitely NOT viral and gave me a steroid cream to put on it. This week, because the steroid cream was not working and it is spreading, I took him back in. The pedi got the other pedi to come take a look at it, and now it is DEFINITELY viral. So annoying, could someone just find out what it is! Last go round I had an appt. with a pediatric dermatologist but the wait was so long that by the time the appt came up the rash was gone and I cancelled it. Now I could kick myself, but c'est la vie. I will try again.
New developements:
1. His right pulmonary artery is slightly enlarged. This comes from the new pulminologist who finally looked at Tomas' x-rays. The newest x-ray is about 6 months old though so we have a follow up in early Jan with cardiology to check it out and try to avoid that pesky pulmonary hypertension that plagues so many of our DS babies.
2. After both my husband and daughter mentioned that Tomas' pee smelled like maple syrup I finally brought it up to the doctor. I had noticed but didn't realize it was that noticable or that it mattered much. Turns out there is such a thing as maple syrup urine disease. It is a METABOLIC disorder (no surprise there) that comes from the bodies inability to break down certain proteins. I have to wait for the next syrup-smelling pee (it is only intermittent) and then collect and freeze urine. This will be the second time I have frozen pee-pee in the back freezer. Just too weird.
3. One of his abdominal hernias is opening up again. This is brand new, as in I just noticed the ball on his belly last night. It is still small, about 1/2" across, and  I will just monitor it for growth. After the last surgery, Tomas' surgeon told me "the next time I go in I'm using mesh." I thought, "What do you mean next time?". Apparently he knew the fix was only temporary. Hopefully, we have a while before that needs to be addressed, but at least it is something we are pros at dealing with.
4. He has always slept poorly, but the last two weeks he has outdone himself, and caused me to fall down in the garage the other day out of exhaustion and dizzyness. So 2 nights ago, after checking with the pedi, I started giving him melatonin. The first night it did not help at all and on top of it he had a rather long and low central apnea episode that I had to stimulate him for to get his O2 levels back up. Not fun. The pedi said it could be from the melatonin as most of Tomas apnea is obstructive (he has very short central spells every once in a blue moon, but none like that night). So last night I gave it to him one more time to test it, and it went better. He slept like his previously poor-sleeper self, and no central apnea episodes. Which felt like a night at the spa for me. I'll keep using it and see if things continue to improve.

Other than that the regular stuff continues, blood counts low, immune system low, waiting on the metabolic tests to come back.
But you know I need to leave you with a smile (and a kiss):

I shop at Aldi's because...

there is only one kind of whatever it is you need/want. No choices, no decisions. It is this one or nothing. I love it. Here's why:
 Tomas started running a fever the night he got all those vaccinations. This paralyzed me. A while back the pediatrician told me not to give him Motrin/ibuprofen because of his platelet issues. Well, now can I give him Tylenol/acetaminophen due to his liver issues? In the end I went with the Tylenol - how bad can one dose be? But really, does everything have to be this involved?
By morning the fever was back, which opens up a whole other can of worms. It is called neutropenic precautions and fever protocol. I am supposed to call in every fever Tomas has to the CCBD (center for cancer and blood disorders) clinic, and if it is after hours I am supposed to bring him to ER for any sustained fever over 100.4 or any single temp over 101. Well his temp had been 100.5 for about 12 hours at that point but at least it was during office hours. The dilemma was that I was pretty sure it was from the vaccines and didn't feel like having the CCBD tell me he needed to come in for labwork and possible IV antibiotics. So here I sat for most of the morning fighting with myself about the fever. Is it from being sick, or is it from the vaccines, do I call, do I not call. I split the difference and called the pedi, who told me it probably was from the vaccines (whew!) BUT that I needed to call the CCBD anyway (why I didn't see that one coming I'll never know).
I called them around 11, and when I hung up the DME called and wanted to service Tomas' O2 compressor that afternoon. Fine, my husband was out that morning but would be back in the afternoon. I had to leave the house by noon to get to a 1pm GI appt. But it was now 10 mins till noon and no call back from CCBD yet, and Tomas still had a low fever and just looked under the weather. Another dilemma! Do I go, or not go. I really needed to talk to the GI and get things moving and sorted out. This time I decided to cancel since Tomas was tired and just feeling blah. By the time I called though it was 12 and the office was shut for lunch, we are the first appt. afer lunch and there is no way for me to cancel until 1. So I felt bad and decided to go. One problem, my husband wasn't back from his errands yet which meant the girls had to go with me. Called him on the way and told him the compressor guy was coming in the afternoon, met him at Walgreen's (getting flu shot) so I can give him the girls and then they needed to hurry back to the house. CCBD called while I was driving and said to monitor his fever and call if it didn't resolve, and to come in right away if his labs came back with an ANC under 800. He had labs drawn the day before but I didn't have the results yet.
Ok, girls were handed off and I went on to the GI office. The three of them got home just 5 minutes before the oxygen guy showed up, so that all worked out.
I am so glad I went.
He is such a conundrum of a doctor. Absolutely abhorrent at calling back, including calling other doctors back; but, oh so frank and straightforward at the office. I told him that the immunologist thought Tomas' illnesses were due to nutritional deficiencies. He said to simply look at Tomas tells you he is not malnourished. She is saying that because he is on a blended diet and it is an easy thing to blame, as opposed to digging deeper to find the problem. And by saying it is dietary, it kicks the ball back into GI's play and she is off the hook. Even if none of that is true, you've just got to love a doctor who can be that honest. I asked about the calcium results on Tomas' labwork (it has been low the last few draws). The immunologist thought he needed more calcium. I told the GI he was getting tons of calcium. He gets 5 grams per kilo and normal dietary guidelines are under 4. He said it is not from his diet it is because he is leaking antibodies out all over the place, after all his antibodies are only at 50% of normal. He said there is no way that Tomas' diet has anything to do with his health. If he were eating the things he gets through the tube by mouth no one would have anything to say about his diet. On a side note here, if he were only getting formula by mouth at two years old everyone would be up in arms at his diet. I fail to see how the method of delivery changes that, but it does. Very few doctors are supportive of a blended diet, mostly because they think parents can't get it right - but take a look at how your average oral-eating toddler eats - I don't see many docs ordering parents of those kids to ONLY give them Pediasure, as it is nutritionally balanced. But I digress...
Anyway, diet not an issue. Great, neither I nor the pedi thought so, now what? Turns out there is a new blood serum test to check for the disorder we all think Tomas has. The Mayo clinic in Rochester, NY runs it and I can just have him drawn here at my local lab. Awesome!!! It has a high false negative, so if it comes back as a no Tomas will still have to have the liver biopsy, but if it comes back positive, then we are done and do not have to go piercing organs (doctor's words). The GI said to leave him on J feeds for now, even though we are not sure if that is complicating getting a diagnosis. Honestly, no one knows how much Tomas having been on continuous feeds for most of his life is masking whatever is going on. We do know that for many varied reasons every time we try to bolus feed him we end up back on J feeds. He said that switching to G feeds, even continuous, would be too dangerous right now. The stomach doesn't empty constantly, even with normal motility, it fills and then dumps, rests and dumps, etc... We have no way of knowing if Tomas' hypoglycemia is from the stomach dumping and his body producing too much insulin, or if it is from his bodies inability to pull sugar stores from the liver during the times the stomach is not emptying. So crazy.
One other thing we discussed was the bloody discharge from Tomas' stomach and bloody seepage from around his stoma. The GI said it is probably granulation tissue in the stoma path and the only way to fix it is to remove the tube, cauterize the tissue, and replace the tube. I asked if we really needed to do that and he said, "Nope, we have much bigger monsters to chase, especially since he is not anemic." Unfortunately that was before I got the results of the previous day's labwork. Turns out Tomas is anemic and his hemaglobin is lower than it has been in the last 14 months. I'm not sure if I want to pursue it though. He is only slightly anemic and the whole removing the tube, replacing the tube thing is really traumatic for him. Luckily, he gets labs done often enough that I can keep and eye on it and pursue if necessary. And to finish off a very informative day his ANC was 1200!!! Woohoo - no add on trip to CCBD!!! His fever is still off and on, but the pedi said the vaccines could cause a low fever for up to a week, and with an ANC of 1200 I do not have to worry about him going septic in a matter of hours. What a relief.

Part of the beauty of this blog is it allows me a "mental unload". Typing it all out gets it out of my head and helps me put an order to all the information I get thrown at me. Before I find the time to sit and type all this stuff out my brain just sort of bounces all over the place. But seeing it here, and reading it in some sort of (I hope) cohesive manner helps so much. Thanks everyone for listening, and thanks even more if you can have the time to comment. I know how hard it can be to keep up with all our blogging families, so a very simple and heartfelt thankyou.

In a funk

I can't believe it has been two weeks since I posted. Now it seems so long I can't imagine how I'll catch up, but I was just needing a break, and in a rather great funk, mostly thanks to one doctor.
Two weeks ago Tomas was at his hematology follow up and that doctor wanted to go ahead and start the IvIg treatments to boost Tomas' immune system. She went through everything involved with me and just wanted to check with the immunologist to see if there was anything she wanted done before we started.
A few days later I get a call from the pediatrician who told me that the immunologist called and wanted Tomas to get certain vaccines and the flu booster, but that she DID NOT think the IvIg was necessary, since he never had a case of pneumonia.
This left me in such an awkward place. I have worked so hard to keep him from getting pneumonia. Breathing treatments every 3 hours, including through the night, rushed visits to doctors and ER's; antibiotics after antibiotics, oral steroids when the inhaled weren't cutting it, and now to find out, if I had let it go a few times and fester into pneumonia we could be getting some help for his immune system this winter. I have not met with the hematologist again so I don't know if her plan is to still go forward, but I fear that the immunologist's opinion carries a lot of weight.
Tomas is sick again, he got sick right before Thanksgiving and is just not getting better. Before the immunology comments I would have had Tomas at the doctor on Friday morning, but since I felt like being aggressive in his short term care was actually being detrimental to his LONG TERM care I waited. He just had his typical stuff through the weekend and early part of this week. Barking cough, cruddy nose, yellow goop from the G tube, and increased d-sats at night.
This morning though he started pulling in on his left side, and something snapped inside me. What the hell am I doing? I'm waiting for my kid to get really sick before I go and do something about it? It's like those (otherwise healthy) kids you read about that die from the flu. Fever of 104 for days and parents were just waiting to see if the kid got better, and you think, why did they wait so long? That was me, I was letting this doctor get to me so much that it was compromising my son's health. Ugh...So I snapped out of it and took him to the doctor. He has a sinus infection, the croup, and needs an x-ray to see if he has pneumonia (the doctor doesn't think so - but something is causing the increased labor in breathing - he said there was some extra tissue or something after one of the surgeries and maybe that was inflamed by the infection - not quite clear on that one yet). The doctor did say this was the second time he had the croup in about 5 weeks - could you pass that on to the immunologist please?
He was able to get the vaccines that the immunologist wanted, she will check his titers in mid-December to see how well his immune system reacts to the vaccine. Those 5 shots combined with a finger prick, a toe prick, a heal prick, and a blood draw added up to 9 sticks for him today and he was not happy about any of them!
Also gumming up the works around here are those pesky liver enzymes. They were very elevated, moderately elevated, normal, mildly elevated and now back to moderately elevated. The liver MRI came back normal, which is great in that there is no visible liver damage but not great in that it leads us nowhere. The liver biopsy, was on, then off, then on, then off, and after a call from GI yesterday asking to bring Tomas in, it is back on again; that appointment is tomorrow. His liver enzymes with today's draw were higher than the last draw so they are trending back up again. Add to that dehydration on the last two lab draws (even though he gets plenty of fluids) and low protein levels (even though he gets plenty of protein) and you have a big bag of who-the-hell-knows.
That also means he will need a specialized platelet test that can only be done in Dallas before he can have the biopsy done. I was in Dallas yesterday for Olivia and could have had it done then, but the GI called yesterday while we were away. So one more trip back to the Big-D. And why, you may ask, was I in Dallas Children's for Olivia? Because she needs to have a cardiac ablation.
She has had heart palpitations on and off all her life (she's 10). About a month ago she had one that was pretty severe and lasted almost 40 minutes and would not resolve with any vagal maneuvers that she had been taught. Her heart was racing over 230bpm for most of that time (thank you Tomas' pulse ox). Mike and I were about to call EMS when it resolved. After a visit to the pediatrician and a pediatric cardiologist, we were referred to a pediatric electrophysiologist. Never heard of such a thing? Me neither. She is a specialized cardiologist who deals with the electrical impulses of the heart. Cute huh? Anyhow, the running theory is Olivia has SVT (supra ventricular tachycardia). She is on a halter monitor until we get an episode recorded and then it is off to the cath lab for her.  For a variety of reasons medication is not an option for Olivia, and the doctor's are all saying that the procedure is very safe. She will have to stay the night, because we live far from the Dallas hospital, just for monitoring. That makes me feel better anyway, I'd rather her be monitored that first night. I suppose if the starts align themselves properly we can have Tomas' biopsy and Olivia's ablation at the same time - a two for one night at the hospital. Stranger things have happened I'm sure.
It hasn't all been medical here though, we have managed to live a little. We are becomming regulars at a local motorcross park, and are getting pretty good at setting up a day camp. Overnight camp is definately in our future, we just need to work out the oxygen, charging the pump, and wait for it to not be 25 degrees at night before we give it a go.
So that is what I'll leave you with, some great pictures of a great day with a great family, who has a great God to keep them going when things aren't so great: