Tuesday, March 22, 2011

Discharge catch up

Tomas was discharged on Thursday. It was a crazy scramble because I had to leave to pick up the girls from their Spring Break day camp, and the hospital staff was trying to make it easy for me so I didn't have to come back and get Tomas. It was just crazy and I still ended up being 10 minutes late for pick up. Almost enough stress to make me want to get them a cell phone. ALMOST.
He has been doing well. Not great and not just ok, somewhere inbetween. Every once in a while he acts like he is in pain, and this afternoon he ran a fever. It broke by late evening, and I hope that is that. Unfortunately it means I had to cancel our swallow study for tomorrow ( I had to call and cancel before closing and that was before the fever broke), so who knows when that will get rescheduled for.
All 3 have a pulmonary appt. on Friday and I am so anxious to go over Tomas and Olivia's plan of care. Both need adjustments, so I'll be glad for that appt.
I got THE phone call from the metabolics clinic and his appt. is on April 21st. I'm thrilled it is not 4 months away! I also heard back from the endocrine clinic on his growth hormone deficiency and they are going to do some growth vector chart plotting or something like that. Not really sure how it is different from regular growth charts, but I guess I'll find out. Turns out the growth hormone he is deficient in is not just for stature (in which he is doing great), but also for muscle development (in which he is not doing great). Very interesting...
Still waiting on the call back from the GI clinic, and Tomas still has blood in his stomach. Such a chronic issue, I'd really like to see it get fixed.
I am beginning the logistical nightmare of The Move but I will have help. The absolute best part is my husband's company pays for the movers to pack the house for us. We've had it done many times and it is awesome. They come in like a swarm of bees and in 2 days have your entire house packed. He seriously told me this time we should pack ourselves. I seriously told him I didn't think so!

Wednesday, March 16, 2011

Day 6

Tomas is doing well. After several start-ups with feeds that increased too fast we are going the really slow approach. So far it is working, but it means an extra few days in here. The doctors (and there are lots) have all rounded. Tomas' stomach biopsy came back with chronic gastritis, and the liver biopsy came back with inflammation around the bile ducts. As usual it doesn't point them to any on diagnosis or direction, but this time around he is being followed by an incredibly caring GI doc. He said Tomas will go home with an appt. from metabolics (by-passing a possible 4 month wait), and he is going to call and/or email all his other doctors to get everyone on the same page, and that the time has come to stop the specialist approach and start managing him as a team. He will also be going home on a new medicine to try to keep his motility going and prevent any further psuedo obstructions. It has a very hit and miss reputation, so let's hope for a hit!

As you all know my husband started a new job on the 14th. It has been incredibly difficult, for him and for me. He was so torn when it was time to leave. I know he wanted to stay, and I know he needed to go. But God has covered us, and surrounded us with people who treat their neighbor as they treat themselves. In particular two people have helped a tremendous amount. Tomas' nurse came and stayed with him the first night Mike had to leave. She came off the clock and left her two small boys overnight to stay so Tomas would not be alone. It turned out to be his roughest night and she had to make a lot of decisions in caring for him, and she did wonderful. The next day my neighbor volunteered to pick the girls up from their day camp, keep them overnight, and bring them to camp this morning. This allowed me to stay all day yesterday and last night. I have friends and family who would be here in a heartbeat if they didn't live over 1000 miles away. Then I have all of you, this community of women. You beautiful, beautiful women. You who pray for my son, who send messages of compassion, hope and good cheer.
Words like thank you and grateful are so insufficient. Whether you know it or not, the glory of the Lord shines through each and every one of you and your kindnesses; and it shines unto this tiny person whose body does not work the way it is supposed to. This tiny person who does not have periods of illness, but periods of wellness, who has fought for so long, and been poked, tested, held down, and woken up more times than I care to think about. And what does he do with this light you all shine on him?
He shines it right back:

Monday, March 14, 2011

Day 4

Ok- small bowel series=same torture as upper GI series. Can't anyone come up with a kinder gentler version? Sheesh. Lots of new petechiae over that one!

It showed no blockage, which is a double edged sword. A blockage meant a bowel clean out and perhaps surgery depending on what was found. So it is great that is off the table, however (HATE that word) it complicates things. Why did his GI tract shut down? It has happened in the past but because I didn't know about his ketone issue, I would just try to keep his sugars up at home. The doctor is thinking the psuedo obstruction path may be what is going on. He said the key would be to find the one "syndrome" that ties all this stuff together. Hah - just a great big "Duh!" to that one.

Anyway - we are going to try feeding him, since there is no reason he can't have food, and his bile drainage has slowed down quite a bit. He will be starting on pedialyte 10 ml/hour and then work his way up to his goal rate of 62 ml/h. If all goes well he can go home tomorrow!

That would be awesome as I have major logistic issues starting tomorrow afternoon and would have had to leave him overnight tomorrow - which would have just killed me.

So prayers he tolerates his feeds, and we get out tomorrow. Then follow up prayers for the upcoming metabolic clinic appt. for wisdom so docs can start piecing this all together!

Saturday, March 12, 2011

Back in the saddle again (re-admitted)

Tomas is back in the hospital again. It has only been 6 days since we were on this floor .
Yesterday he had OT at 9, and he was tired so sitting in his high chair. About halfway through the hour he looked over at me and just started crying, always an indicator that something is big time wrong with him. You've all seen that smile.
I ran and grabbed his bile drain and hooked it up to his G port. Sure enough about an ounce of bile came out. Ok - that is not a completely unheard of occurrence. But a half hour later the same thing happened, and again a bit after that. At that point I left his bile drain open and he wanted nothing more than to sit in his seat inside his crib and watch VeggieTales. I would have loved to have been able to switch his feeds over to pedialyte, but because of the hypoglycemia, I can't. It doesn't have enough sugar to sustain him. His heart rate climbed steadily and around 4 pm he began running a fever. I had called pedi and GI earlier and they both said the same thing. If I couldn't maintain him at home to bring him to Dallas. Around 4:30 I called it.
There was a pretty scary part there in traffic when his heart rate was 180 and his O2 was 93 that I thought I waited too long, but we made it. I got rushed through triage and brought right back to an exam room.  Several tests, labs, cultures, and Iv meds later, he is admitted back onto the GI floor.
He is on full gut rest and still having significant bile output through his G. Docs rounded this morning and are ordering full rest for two days and then a small bowels series on Monday morning to look for obstructions.
Tomas is quite the bit perkier today. After switching from feeding him to giving him IV fluid he slowly became his happy go lucky self again. This morning he had a brief relapse after his morning meds were pushed through to his belly and intestines, but some anti-nausea IV meds and leaving him alone after that helped a lot.
So he either has a tummy bug and can go home on Tuesday if he tolerates his feeds, or he has some kind of blockage, and can go home goodness knows when.
Fun times - but at least I have my husband to keep me amused!

Saturday, March 5, 2011

Ongoing glucose utilization in the absence of an ability to use ketones as an energy source

I'll get to the title of this post in a bit but first:
We are home! He is doing fabulous!

 GI rounded on him this morning and said he had a sever case of gastritis. He is going back on Carafate, taking Mylanta 3 times a day and his Prevacid was doubled. All of that should heal his stomach in 2-3 weeks. The cause is still under investigation, and the biopsies of several sites in the stomach should help us out there. Appt. with them on Tuesday.
Hematology ordered some more bloodwork this morning and he is neutropenic again. That was a great two days without neutropenic precautions in the hospital! Appt. with them on Monday.
This is where it gets complicated. Your amazing body has 3 separate processes for getting the energy it needs. First, sugar that comes in through the food you eat provides the primary source of energy. Any excess sugar gets stored in the liver, and when liver stores are sufficient, as fat. Second, when you have burned through all readily available sugar your body releases glycogen (the stored sugar) from your liver. In a normal person these stores can last 18-36 hours. When the stores are critically low, the brain signals the liver to save what's left and move to phase three. The third phase is using fat as fuel. Your body brakes down fat into energy, particularly a by product of fat metabolism called ketones.
When you enter this phase you are in ketosis, your body uses those ketones for energy and your blood sugars stabilize. This is the state that people on the Atkins diet are aiming for. You burn through fat stores and can remain in this state as long as you have/and or consume a lot of fat. (If you are still with me - God bless you - and we trod onward!)
Tomas has two large issues going on. He is moving into ketosis way too fast, after only 2-3 hours of the second phase. Then, he does not use what ketones his body is making (the absence of an ability to use ketones as an energy source), so he keeps burning through those glycogen stores (ongoing glucose utilization) and his blood sugar keeps dropping, within 4 hours he is in a severely hypoglycemic state. His body wants that to stop ASAP so it keeps making ketones. If left untreated, his blood sugar would hit zero, he would make way too many ketones (which are an organic acid) causing his blood Ph to change, his brain would be damaged from the lack of sugar, and eventually he would die.
It is a metabolic disorder (ps - one of the causes of gastritis is a metabolic disorder) called a Ketone Utilization Defect and the doctor told me this morning it is "really really rare". She is going to do some research and then I meet with the Endo team on Wednesday. She gave me strict orders that Tomas is not to go more than 4 hours without food, it could be a very dangerous situation for him.  She also told me that she is not aware of a "cure", other than to make absolutely certain the body has readily available sugar for energy.
I am thanking God that Tomas got a feeding tube at 2 days old making it a breeze to control his feeding. I am thanking God that he has a GJ tube, so when tummy bugs hit (or gastritis for that matter) and he doesn't want to eat, I can still get food into him via his intestines. If any of that should fail he would need to get to a hospital for IV glucose.
There are a few different kinds of ketone utilization defects and they are going to try to figure out which one he has, and if any dietary changes will help alleviate his issues. None of this bothers me as much as you would think. I have known for so long that something was wrong, and to have people pay attention and want to help is a huge relief.
Thanks for tagging along on this crazy roller coaster ride!

Friday, March 4, 2011


What a long day! First Tomas went downstairs for a liver ultrasound to mark the positioning for the liver biopsy. Then we came back up to his room and waited for OR to call us down. Since it was a rather boring wait Tomas threw some fun at us by dropping his blood sugars while ON the IV fluids. Scramble, scramble, and an increased sugar solution IV was ordered and kept him stabilized. Eventually we were called down.

What can I say about that kiss goodbye? Nature may abhor a vacuum but that kiss does not. Placing your lips on your little ones forehead as they are about to be taken from you creates a nothingness, an abyss that pulsates its emptiness through to your soul. It recedes only after the massive exhale you take when you first see your child post-op.

The first doctor to come out was GI. He was able to get the liver sample with absolutely no problem, and no bleeding, praise God. HE should have the result by my appt. with him on Tues. Now onto the endoscopy. These were his exact words, "Tomas' stomach is very unhappy. It has blood oozing from everywhere." A feeling of giddiness washed over me. I wanted to do the happy dance all over the corridor. Something concrete? An answer to why he has had blood coming from his G port for 18 months? Yes!
He is treating it with some IV zantac and then doubling his prevacid for the long term. Possible causes are metabolic issues or motility issues, both which will be investigated.

Speaking of investigating metabolic disorders: While I was waiting for the oncologist to do the bone marrow biopsy two endocrine doctors came and got me out of the waiting room. We went to a conference room to discuss what has been happening with Tomas' sugars. What a relief it was to have the attention of the right people to help him. We went through everything and they assured me they would get to the bottom of what is going on. I was flying high when I left that conference and bumped into the hem/onc doctor coming to find me. The biopsy went textbook perfect and they will have the preliminary results for Monday's appt.

I went into PACU happier then I have been in a very long time. Tomas was sleeping and came out of the anesthesia perfectly, again praise God. We wheeled him up to his room where he woke up very unhappy from all the prodding in his stomach, began retching, desatting, turning blue, stomach arching, etc, etc... I ran and grabbed his stomach vent and between myself and two nurses, some zofran and loratab where able to get him settled about 45 minutes later. He was fairly touchy the rest of the evening, but has finally fallen asleep, and is on room air!
I am so exhausted, but finally, finally feel like we are making forward progress. He should get to go home tomorrow and then next week is follow up with all three clinics.  Thank you all for your prayers and well wishes. If you have been through it then you know how much it means, and if you haven't, well, it just means a whole, whole lot!

Record breaker ; broken record

Well he broke his old record set back in August. He made it from start time to end time on the fasting study in 5.5 hours. Last one was 6. His blood sugars hit 41, labs drawn, rescue med given, and now he sleeps. Anyone who needs a play by play can read this post from the August fasting study: Fasting Studies 101
At least this time we were able to go straight to the D10 and not try the glucagon.
I'm kind of mad sitting here tonight. In August after the first fasting study we were finally starting to get somewhere, but then another doctor stepped in. He ordered a second fasting study, during which Tomas never dropped his blood sugars. The doctor then discounted the first study saying something went wrong with it, and that the second study was the accurate one. Yet, here I am, at a different hospital, with a different doctor's group, going through the same test with the same results, feeling very much like a broken record. I'm thinking at this point the test where his sugars stayed stable was the fluke, and I am also trying to pray away some serious darkness concerning that doctor at the other hospital.
What's next? God willing, not another fasting study! Tomorrow (today actually) brings an endoscopy, bone marrow biopsy, and liver biopsy. All these tests have been waiting in the wings for so long. I am nervous. Nervous that they won't show anything, nervous that they will show something, nervous about anesthesia. Just the It's 3am and I can't sleep so I'll put my thoughts on paper nervous.
Luckily, this past Sunday's entrance hymn and Gospel were chosen specifically for me, so that 4 days after the fact I could call them up from the "Help me Lord" section of my brain. The entrance hymn was

Be Not Afraid
You shall cross the barren desert,
but you shall not die of thirst.
You shall wander far in safety,
though you do not know the way.
You shall speak your words in foreign lands,
and all will understand,
You shall see the face of God and live.

Be not afraid,
I go before you always,
Come follow Me,
and I shall give you rest.

If you pass through raging waters
in the sea, you shall not drown.
If you walk amidst the burning flames,
you shall not be harmed.
If you stand before the pow’r of hell
and death is at your side,
know that I am with you, through it all

Be not afraid,
I go before you always,
Come follow Me,
and I shall give you rest.

Blessed are your poor,
for the Kingdom shall be theirs
Blest are you that weep and mourn,
for one day you shall laugh.
And if wicked men insult and hate you, all because of Me,
blessed, blessed are you!

Be not afraid,
I go before you always,
Come follow Me,
and I shall give you rest.

The Gospel was Matthew 6:25 -

"No one can serve two masters. He will either hate one and love the other, or be devoted to one and despise the other. You cannot serve God and mammon. "Therefore I tell you, do not worry about your life, what you will eat (or drink), or about your body, what you will wear. Is not life more than food and the body more than clothing? Look at the birds in the sky; they do not sow or reap, they gather nothing into barns, yet your heavenly Father feeds them. Are not you more important than they? Can any of you by worrying add a single moment to your life-span? Why are you anxious about clothes? Learn from the way the wild flowers grow. They do not work or spin. But I tell you that not even Solomon in all his splendor was clothed like one of them. If God so clothes the grass of the field, which grows today and is thrown into the oven tomorrow, will he not much more provide for you, O you of little faith? So do not worry and say, 'What are we to eat?' or 'What are we to drink?' or 'What are we to wear?' All these things the pagans seek. Your heavenly Father knows that you need them all. But seek first the kingdom (of God) and his righteousness, and all these things will be given you besides. Do not worry about tomorrow; tomorrow will take care of itself. Sufficient for a day is its own evil.

So tonight I rest in Him, and tomorrow I kiss my baby goodbye again. For the terrible, awful, hopeful walk down the corridor to the OR. If 10,000 angels can fit on the head of a pin how many do you think can fit on a laptop's keyboard?

Thursday, March 3, 2011


Yesterday afternoon, while at a playdate with my homeschool group Tomas went into respiratory distress and his nurse called EMS. He stabilizied pretty quick and we had a relatively non-urgent ambulance ride to the hospital. His J tube had clogged a few minutes before the event, but I was able to rock the syringe and get it clear. Then he began acting like he was in pain and the distress came shortly after.
We were taken down to Dallas as the EMS thought his situation was too much for the satellite campus, and were in the ER for 12 hours before being moved up to the GI floor.
Several x-rays, fluoroscopies, labs, and tests later we don't know much, except he doesn't have a UTI, his tube placement is good, and he doesn't have strep. He did get a new tube placed today because the J valve was leaky, but that is unrelated.
The plan right now is to try to get him co-ordinated for an endoscopy, bone marrow biopsy, fasting study, and metabolic testing all before the weekend. It would really stink to have to come home just to go back and do all that testing!
He is on full gut rest right now and eventually we will run pedilayte and then formula through. He was incredibly distended and had lots of bile leaking out of his J port yesterday. Today, after a day of no feeds, he is back to normal.
As always, I am praying that this is the time something shows up on their radar screen.