Friday, October 14, 2011

Down Syndrome Awareness - two years later

Here we are; a year after I wrote the story. I am sitting in the hospital next to Tomas' crib while he sleeps. He has been inpatient for 7 days in order to have some procedures done and some tests run. This makes 4 hospital stays in 4 months for a total of 20 admissions in his 32 months of life. Tomas has had a rough few months; 2 line infections, one that was pretty severe, degenerating gut motility, progressive liver damage from the tpn, re-discovery of the ASD in his heart, a recommendation from one of his specialists that he be evaluated for a mitochondiral disorder, chronic neutropenia which prompted another bone marrow biopsy and some neutrophil testing, and another fasting study to get to the bottom of the metabolic disease causing his hypoglecemia. The goal of this hospital stay was to be able to safely reduce the number of hours his TPN runs in a day, in the hopes that we can slow the liver disease and possibly reverse it. In order to do this he has to be able to fast as he can not take in enough volume by tube feedings anymore to reliably count on enteral feeds as a source of glucose. Before admit he was fasting 4 hours and we are going home tomorrow with a plan for fasting 6 hours.
What is glorious about that? Grand, beautiful, splendid? Nothing. I miss my girls, I miss my husband, I haven't unpacked my house, I know hospital life inside and out. I have minor anxiety attacks when I see a NICU unit, and had a full fledged PTSD episode when Tomas was sent to ICU even though it was just for some testing and he was healthy. How is it that I can laugh away an afternoon in a windowless room, hold my moaning, near comatose hypoglycemic child in the evening and then smile again with the sunrise? The answer is so easy.
Tomas came into this life with no guarantees. I never had an expectation that he would live a long healthy life. I just needed to get past the first 3 days and the first surgery. Then his liver stopped working when he was a week old and again my husband and I just waited. Next came the blue spells which left me thinking that this time for sure I was holding my blue, limp, dead baby. After that came that horrible, terrible, angelic night that he coded with an angel by his crib.  Many more life threathening events followed, waxing and waning with his given health status, up until I got a fresh reminder in August with the sepsis and another blue spell. For my girls I fully expect that barring a tragic accident they will grow up and grow old; it is just not the same for Tomas. I have no idea if an acute illness will kill him, a chronic illness will kill him, or barring any tragic accidents he will grow up and grow old.
Point being that every sunrise is something to give thanks for, even the ones that happen here in the hospital. Each day is a gift, filled with gratitude for the continued existence of my son, my daughters, my spouse, my life. I do not ignore the difficulties my family faces, or sweep them aside, or make light of them - I couldn't if I wanted to as they encompass every aspect of it. Everything - right down to needing toilet paper but being unable to just run to the grocery store for it - that kind of everything. Instead I wrap them around me, build them into my being and then take a step. Somedays it is just one step, getting out of bed, those are usually the days after I've been notified of something else gone wrong. Other days, the glorious ones, the ones that I flashback to at the crackle of a leaf, or the scent of the ocean, or the warm sun hitting my cheek, those days my God I swear I can soar. Most days fall in between; the drudgery of day to day living. But all those days snap together to make the picture I see. And the picure is good and happy and at peace in this moment.

Sunday, August 14, 2011

Recap

Friday 8/5 - We close on our home in NY

Saturday 8/5 - While cleaning the house in order to move in the next day, Tomas gets grumpy, spikes a temp, and has trouble moving air well. Call 911 and are brought to local ER.

Saturday evening - Unable to find an easy solution to his issues we are transported down to New York Presbyterian. 2 doses of morphine need to control his pain. IV antibiotics started.

Saturday to Sunday 8/6 - In ER at NYP testing everything imaginable, morphine as needed, bowel surgery likely. Rest of family moves out of hotel into house.

Sunday evening - Moved up to room under GI care. Round the clock pain meds and antibiotics, antifungals added.

Monday 8/7 - perking up a bit, sitting up and watching VeggieTales. Line infection comes back positive for bacteria, putting bowel surgery on back burner as it is not the source of Tomas' troubles. Mike and girls come to visit.

Tuesday 8/9 - Line infection not clearing but under control, possibility of surgery to remove port.

Wednesday 8/10 - Moving truck unloads at home. Line infection now showing yeast as well, port has to be removed. Breakthrough fevers and pain, sliding downhill fast.

Thursday 8/11 - Unresponsive and lethargic all day. White blood cell counts and platelets are very low. Surgery to remove port.

Friday 8/12 - Worsening of condition, everyone worried, tests ordered to look for hidden yeast in the body. Heart and kidneys are clear but the eye doctor found swelling of the fluid around the brain, putting pressure on his occular nerve. An emergency CT scan of the brain is orderd. No masses found. He is very, very sick.

Saturday 8/13 - My anniversary. Day surgery ordered open, OR nurses called in from home, and the lumbar puncture is done. He is still so tuned out to the world. Initial fluid tests show no meningitis but spinal fluid pressure is too high.

Saturday night - A new pain med is ordered that is also a strong anti-inflammitory and Tomas perks up. He sits up in his crib, sings along to Veggietales and gives me little smiles. And then he sleeps, almost all night, only waking twice. Such a blessing.

Sunday 8/14 - My husband is coming down for the day and I will go see my house for the first time since we have moved in.

Tomas has 4 issues going on:
1. The bacterial and fungal infections in his blood - they are being treated with IV antibiotics and antifungals. The bacterial infection is cleared and he has been moved to maintenence antibiotics on that, yeast is still growing, but slower than before so we are headed in the right direction. He will need this IV therapy for weeks, but it can be continued at home.
2. GI issues - We have intermittently tried to feed into his J but so far no luck, even at just 7 mls/h (a little more than a teaspoon an hour). There is also the abnormal section of bowel and he will need a new central line placed before we can go home.
3. Blood counts - Hematology hasn't weighed in yet, but most likely it is suppression in the bone marrow due to being so ill, and his counts should climb once he starts feeling better.
4. Due to the increased pressure in his skull and the increased pressure in his spinal cord fluid Neuro is being brought in. So far all we know is there are no blatant masses and meningitis is unlikely (there is still a chance the fluid could grow something).

He is on a ton of meds. Besides his regular ones, all the infection therapy, he is also getting constant anti-nausea and antiinflammitory meds. He has two IVs (one in each hand), and so far has only blown one (for a total of 3 sticks). That is great for him.
The best guess anyone can give is he will be here for another week, or more depending on what neuro finds and if the spinal fluid growths anything.
As always, the community of people surrounding us has lifted and cared for my family. Family, friends, and strangers contribute a lot or a little to make something beautiful happen. It is one of the gifts that Tomas brings, that any sick little one brings. The number of kindnesses that would go undone without these least of us among us would make for a much darker world. Thank you.

Thursday, August 11, 2011

I'm not an inspiration, I'm a mom!

It has been a rough week, a really, really rough week. Way to many high stress things going on at the same time, and there is not an early end in sight. Tomas is not well, and this line needs to come out ASAP so he can get better.
But something bigger is on my mind this morning. So many of you have said such lovely things to me ABOUT me.
How do you do it? I could never survive. You're amazing. You're such an inspiration.
Well listen up - STOP IT! I am nothing more than you, nothing better than you. I am exactly what you would be if you were here.

Do my words inspire you? Thank you, but they are not mine. They are pulled from the collective body of knowledge of men, from the great conversations that have taken place through history. Great books, great stories, great speeches all jumbled together in my brain; words rolling and bumping around waiting to be pulled at the appropriate time.
Does my strength inspire you? Hah, it's SO not mine. It is pulled from the Most Holy Trinity, the vastness of space and the awesomness of creation. It is a limitless supply and I go back for more many, many, many times.
Does my faith inspire you? This one is harder, because it was a gift given at a horrible price. Formed from watching people I care about trudge through misery. Those who had faith made it through to share laughter and radiate joy despite the pain. Those who did not hold tight carry with them a great pain from which they find no relief. Early on I had to choose which path I would want to look back upon at the end of my days.

To any of  you who think you could not do this, that you would collapse in a corner, Just ask yourself one question. Are you a mother?
The question isn't - do you have a child? It is - are you a mother? Every time your child frustrates you, or angers you, or causes you pain, do you choose to walk towards the problem rather than away? Do you make mistakes and get things wrong, but then go back and apologize? Then you would make it through a trial like this as well. You would rise and rally beyond anything you ever thought yourself capable of, because your child needed you to.
Because it is not about how high the bar is set, it is about whether or not you choose to jump it.

Monday, August 8, 2011

This little piggie

Tomas is admitted to the hospital again, but for something beyond anything he has ever dealt with before. Intense attacks of abdominal pain. After 2 ambulance rides, 2 ERs, and 30 hours he was finally moved up to a room and allowed to rest.
His toes are still this morning. He is sleeping and his toes are as still and peaceful as the rest of him. I prayed for quiet toes many times over the last 2 days. Just recalling the vision of him in pain makes my throat shut, my heart race, and my breaths come with effort. Watching was unbearable. It happened 3 times, each one worse than the last, and longer than the last, requiring more morphine than the last. He would roll to his side and bend his body like a tight "V", rigid and trembling with pain. No crying, just a grunting moan, his hands clasped tightly together and his eyes shut. His eyes were not shut tight which I find comfort in. I pray it means he wasn't there, that he escaped, that while his body raged his soul was safe in the extracorporeal world of The One who loves him more.
While I laid my head next to his back and rubbed his tremoring body and waited for the morphine to work I  found myself staring intently at his toes. His feet would shake and his toes would spasm independently, his whole body was in pain, and I realized that Our Lady must have seen Jesus' toes like this too. That she watched his toes spasm, and I couldn't decide what was the greater burden; the dying for the sins of the world, or the watching of your son's toes dying for the sins of the world. To not turn away, not collapse, but to watch and smile gently when your child's eyes met yours in pain; to have that vision burned in front of your own eyes forever is a burden most heavy and enigmatic. Somehow, this great weight strengthens me. It is so heavy there is no room for the petty things, it is so rough it scrapes the meanness and harshness away. It is The Burden of Perspective and it is what the Lord meant. Only through Him can the yoke be tightened and still feel easy, and only through Him can the burden be loaded and still feel light.
Today doctors will round, and tests will be run, and plans will be made. Tomas is on complete gut rest so with any luck the only thing his toes will be doing are eating roast beef and going to market.

Friday, July 29, 2011

Tests

We have been in our 1 bedroom hotel suite (with a tiny kitchen) for 34 days. 5 people, 2 dogs, 27 pounds of Special Needs eqipment and 34 days. 'nough said about that! Delay after delay keeps popping up on the closing of our NY house. We were supposed to close on the 15th, at this point the earliest will be the middle of next week.
Our belongings have been trucked up from storage in TX only to be moved to a storage in CT; just waiting on an ok to unload. My husband may have an aneurysm soon, he took off work last Fri. to do a walk through on the house. It only took the morning but he was so stressed he just took a long weekend. That NEVER happens. Having a super stressed out husband hanging around you for 2 days, let alone 3, is the opposite of fun.
Luckily, I have an amazing friend here who is keeping me sane, and letting me use her home as needed when our space gets too cramped. I also met some moms from our new homeschool group last night and everyone was very friendly, so super glad about that.

I knew moving to NY and starting over with new doctors would mean lots of tests for Tomas. He has seen endocrinology, GI, ENT, and Hem/Onc so far.
The endocrine doctor took away his lovely synthroid compund because she says it is not stable and they don't use it at the hospital. His thyroid levels were off otherwise I may have fought her over it, but instead I am back to crushing the pill and chasing the mixture of water/med all over the bowl with a syringe to make sure I have drawn it all up. I love me a compounded med with a syringe top bottle! Sigh...She is waiting on his medical records to come up from Dallas to proceed with the Growth hormone/hypoglycemia testing, but for now is sticking to the strict no fasting orders for him.
GI: This could be it's own post so I'll try to summarize.He ordered a tube study, barium enema, upper GI, endoscopy and weekly/monthly lab work. The tube study showed his GJ perfectly placed (in case the bile/formula reflux from the J into the G was due to the tube being out of place). Sorry guy - no easy answer there. The barium enema showed no microcolon - again no answer. Yesterday Tomas had the upper GI with small bowel follow through. The radiologist puts barium into the stomach and then takes x-rays at timed intervals afterwards to see how quickly it moves through the GI tract into the colon. After 5 hours it was still in his stomach. Score! Hoping for some good info to come out of that one.
ENT: He ordered a sleep study, swallow study, laryngoscopy, and bronchoscopy. He doesn't think Tomas' desats are due to true obstructive sleep apnea, he wants to look for a laryngeal cleft. Also, one of Tomas' ear tubes is out so the dr. will clean out both ears while he is under, do the hearing test, and then replace them. I'm curious to see where his hearing is without the tubes in. He did tell me that any vagus nerve damage that is causing the GI issues would not be affecting Tomas' swallow. He still has so much difficulty swallowing without aspirating. That's where the cleft comes in and possibly that right subclavian artery that is out of place.
Hem/Onc: She just reminded me of the risk of TMD babies transistioning to AML by the age of 5 (Tomas has enough friends who have gone through this that it is always in my mind). His neutropenia requires more testing so she tagged on to the GI's lab orders. Since his platelet count is usually normal and his platelet function is always normal she feels like the petechia are most likely due to weak blood vessels. Not sure what the means for Tomas. I was absorbing too much other info and will have to ask at the next visit. At the end of the appt. she brought in her nurse to get blood from Tomas for labs. This is almost comical. He has a port - it should be a breeze to get blood. But blood return is not my friend. The nurse was fantastic; she hooked the syringe up to his needle but alas, no blood return. Then she started cajoling Tomas into making sounds which would cause him to inhale and exhale deeper giving her just a drop of blood every time he did. For about 15 minutes she worked with him to get enough blood so she didn't have to stick him. The doctor was undecided about what to do about the port. Since we could at some point get blood from it, the problem could just be positional with how the needle is placed in the port, but because this happens even after needle changes, she also wants to do an x-ray and dye study to see if that is definitely the case. I'm a little aprehensive about that because it could mean surgery to replace the port. Not a huge surgery, but still.

Tomas is doing ok. A couble of small antics but on the whole the TPN is working and keeping him home. He was dehydrated on last weeks labs due to an increase in G drainage, so the volume on his tpn was increased. A few months ago that would have meant an admit, but now he is stable and  hydrated, allowing me to not stress too much over the continuing GI issues.

Saturday, June 25, 2011

My next job will be

An air traffic controller at Dulles International Airport, and it will feel like being on spring break.
The logistical mountain that needed to be coordinated and moved to get Tomas to New York was overwhelming.
BUT, we are here, it is done, and it went off without a hitch. He saw his new pediatrician the day after we got here, that pedi drew the TPN labs, and the new infusion DME has been in contact with the dr. and me, a nurse from the new skilled nursing company came out this morning and changed his port needle, which was overnighted to my hotel room by the old infusion DME along with enough TPN to get us through the new lab results and new written orders from the new doctor. Tomas' first specialist appointment is on July 7th, with 10 more to follow in the next 3 months.
I am so grateful for the TPN because I am pretty sure he is in the middle of another shutdown, and is barely tolerating the tiny drip of J enteral feeds we have running. I am certain he would be in the hospital, but instead he is here with his family and gaining weight every day. It is a gift, short and simple.
We drove here, it took 4 days because we didn't push the kids to go 10+ hours a day. I loved watching my girls faces as the hot, drought ridden, cracked earth landscape around north TX gave way to the green rolling hills of TN. Then the woods got thicker and the people got sparser as we headed through northern VA, NJ and finally into NY. I grew up here, but my kids have only known the flat pancake that is FL, and the small hills of TX. Climbing the large hills of the Hudson Valley and having them look out the car window and look DOWN on the villages below took their breath away, and that gave me such pleasure, and such admiration for the creator and the variety he bestowed upon us. In all things there is this incredible variety, a newness waiting for the next newcomer to discover.
I wonder what this new place will make of the variety my family brings with us. The local newspaper had an ad for an OB/GYN office that headlined with "Genetic Counseling; Nuchal Translucency Screening; Choronic Villus Sampling; and Genetic Amniocentisis". What will this tiny part of the world make of my boy who "failed" all these tests? We left the relative safety of a very conservative area and once again God is holding His hand tight to His chest.
How much more I would prefer to offer my faith up as a single gift, on a silver platter, one time, and be done with it. That is not my path though, my path  is the Hansel and Gretel path. The one where God leaves little breadcrumbs for me to find my way, and while I am aware when I find a large enough crumb to propel me forward I shudder at the disheartening thought of how many smaller ones I have missed. So I wait, because God knows I am dense, and stubborn, and prideful, and He knows that every once in a while I need a whole stinkin' loaf of bread to find my way.

Sunday, June 12, 2011

The Long Hello

I have been debating about whether to try to catch up on the blog or just let it go, but in the end I decided I missed it too much. That leaves me with a tremendous amount of catch up, and trying to sum up the last few months in a few short paragraphs.
Tomas began a downward GI slide in March. He was not tolerating his J feeds and would retch and back arch and stomach crunch and cry to the point where he needed to be hospitalized in order to run IV fluids and give his gut a break.
The episodes became closer and closer together and by early June he had been hospitalized 6 times and spent a total of 45 days in the hospital. It got to the point he couldn't be home for even a week. As soon as we started his feeds back up within the next few days the same things started happening.
Finally, in late May the doctors switched gears from trying to figure out what was wrong (I'll get to that in a minute) to trying to find a way for me to keep him home. An IVAD (he got the under the skin kind) and I was given protocol on when to switch from J feeds to IV fluids and how long he could stay on fluids before he would need to be admitted. That worked for about 2 weeks, and then I couldn't get him off fluids. His gut just didn't want to work. Plus, he continued to lose weight at an alarming rate. He went from 25lbs 13oz to 22lbs 2oz in 3 months.
After a roundtable discussion with Tomas' GI, pediatrician, nutritionist, and myself, the decision was made to place him on TPN. So one more trip to the hospital, another week long stay, another protocol for TPN labs and weight checks, and he has been home for 10 days now. Doesn't seem like much but it is the longest he has been home since early April.
It is going well, a few ER runs for clotted lines, and phone calls for pump issues, but he is gaining weight, and looks so much healthier. He still has a very slow J feed running to prevent cell atrophy and to help mitigate the negative health issues that come with prolonged TPN usage.
Well, what the heck is wrong with him anyway? In all the hospital stays he has had a different attending GI every time (they do 2 week rotations at our children's hospital). Each one had a different theory, the first was he had a metabolic disorder, but metabolics said no. The second was he was on the wrong formula, 3 formulas later and that was kicked aside. Another thought he needed different motility agents, after 4 different meds that was given up. The running theory at this point is that his Vagus nerve was damaged during the last fundo surgery and that the pacemaker area of the stomach has been affected. That area controls the impulse to contract the stomach as well as the small intestines. If that turns out to be the case then there are not many options available, keep him on TPN, or have a stomach pacemaker implanted. There are only a few doctors in the country that do it and so far the youngest patient I've found is 5. So we have a ways to go for that. But first someone would need to prove that is what's wrong. The medical community in Dallas is unable to do that. They are out of tests, and ideas, and are just working in a palliative care mode. Good thing we are moving to NY.
Yes, we sold our house in only 14 days. I was in the hospital when  the offer came in. We have a house picked out in NY that is beautiful and are waiting to get to contract on it. The movers come tomorrow. They will be packing for 3 days and loading for 2, and on Friday we say goodbye to this house! It happened so fast we are still spinning. A few kinks need to be worked out with both sets of contracts but I am keeping faith it will all work out. The logistics of moving Tomas have qualified me to be an air traffic controller x2. That is it in a nutshell.  Actually, that is definitely the shelled out version. In the future look for the fluff. An ambulance ride, a nurse's tale, and a shopping cart are all coming to mind at the moment.
If you are still out there and still reading :God bless.

Friday, April 15, 2011

Stuck in Traffic

We had Tomas' appt. with the new GI and it was a good one. He is a man who likes the puzzle kids, and he and Tomas' oncologist share a few of the more challenging kids at the hospital, so I really think it will be a good team for him.

Another huge plus is that this GI nurse actually calls you back, and has been tremendous at keeping contact going between the doctor and myself during the last week. Tomas is having slow to no motility again and I and his home health nurse have been sidewinding our way through trying to keep him home. It has always been, "Well, do what you can for him at home, and when you can no longer feed him enough to keep his sugars stable or he gets dehydrated bring him in." So frustrating.

He had 4 fabulous days last week where he had zero bile output and he tolerated his feeds great. That ended on Sunday and has been progressively sliding downhill, to the point where yesterday was an on the fence kind of day. Problem is, Mike is gone, and I KNOW if I bring him to ER he will get admitted. So we struggle on here, hoping that the next day is better than the last. As of right now he hasn't hit his calorie goal since Sunday, but I am doing my best to use a mix of pedialyte and formula to keep his GI tract running. Oh, and I need to add that the little stinker is now smart enough and coordinated enough to yank off his extension set. He knows it hurts when he eats, so he just wiggles and wiggles it until it comes loose. He has managed to free himself a few times now and make giant messes!

He had a sleep study done on Friday and I should have the results at the next pulm appt. I hope they were able to get some good data as I don't really know how well people sleep at those things. I did find my miracle - make your baby sleep - cure though. It is a farrell bag. It hooks up to his feeding set and is a way for the stomach (or in his case intestines) to release gas pressure. Basically, the bag is an outlet for the tube set, if the pressure inside is greater than the pressure in the bag, then the formula will flow up into the farrell bag until the pressure equalizes, then slowly drips back in. We tried it when he was an infant but it has so much tubing he was never strong enough to push it all the way through. Now, it is amazing to see the difference in how he sleeps at night. If I leave the bag open he sleeps like a normal kid. Falls asleep, moves a bit in the night, but stays asleep without a sound. The problem is that he is not getting all his food, so if too much starts to back into the bag I have to clamp it shut so he can eat and not become hypoglycemic. He does not like this and within 30 minutes of the clamp is moaning and whining in his sleep just like before. So we do the farrell dance through the night, but all in all, he is sleeping so much better.

Due to all the bile output, we are having to monitor his electrolytes closely. Last Monday his sodium/potassium/chloride were low, and they were still low on Wednesday. This Tuesday we had them drawn again and the only one still low was potassium. This was after the good 4 days and before yesterday though so I am thinking his next check (Monday) won't be as pretty. Yesterday was tough. The girls had a field trip.

It sounds so simple doesn't it? It's not, and it has nothing to with the amount of gear or prep involved. That is not it at all. It is the absolute lack of consistency in Tomas' state of being. It is never knowing if he will make it through. I can't say if it is the GI problems or the hypoglycemia that are more the culprit here. If he could eat fine then we wouldn't have issues, but then again, if he wasn't so severely hypoglycemic, it wouldn't be as dire a situation when he is not tolerating his feeds. You and I, and Victoria and Olivia, and most other people on the planet can skip a meal. You eat breakfast, and for whatever reason something comes up and you miss lunch. Before you know it, you look at your watch and it has been 8 hours since you ate. Maybe you had breakfast at 7, and now it's 3 and you're starving. You eat and all is well. Not so. He can't do it, if he were to go 8 hours or 7 or 6 or even 5 without food there is a very, very good chance he'd be in a coma. It is the single most difficult thing we are dealing with right now because it means he HAS to go to the hospital when he can't eat.

Back to yesterday, It started out pretty darned good. It was a progressive field trip through historical markers in a nearby town. The first two stops were perfect, he was happy and playful and just regular old Tomas. It was early, and not time for his feeds to start. The third, and final stop was the long one, were we would spend the rest of the day, so his nurse got him all hooked up and everything running and sat with him while I bounced back and forth between the two girls' groups. A little into it things went downhill and I was calling the doctor to tell him if Tomas didn't wake up from his nap better then when he went to sleep I would be bringing him into Dallas.

 Their plan was to try to get him through to his already scheduled surgery appt. in just a few hours and see if the surgeon had any ideas on what was going on with the bile output. Tomas did wake up a bit better and we did make it to the appt. Unfortunately, the surgeon didn't have anything to add, and it was more of the same;. bring him to ER when it gets bad enough. I asked if there were any tests we could run to see what was causing the bile reflux, or if it could be fixed with any different motility meds. Nope and nope. Just a long talk on how dangerous it would be to do any kind of surgery on his bowels given what they had already been through, and to call him any time. Thanks; and we walked out just as close to an admitting as we went in.

As we were leaving the hospital pavillion I thought I'd pop into the blood disorders clinic and see if his hematologist was there. I've been mildly worried since Tomas' pathology report came in from his last bone marrow biopsy; not too much because I figured his doctor would've called if anything terrible was going on. But I still had some questions and had been playing phone tag with the dr. for a few days. Luckily, she was in and close to finishing so we hung out in the waiting room for a few minutes. She came out, saw his bile drainage, and pretty much became as stressed out as I was over the whole thing. She spent a very long time talking with me and going over EVERYTHING that has been happening with Tomas in the last month. She wants a CT of the abdomen and brain (for unrelated autonomic issues).  When we left she was going to call the GI and then call me back today.

That means that within an hours time I had two wildly different opinions on what could be done for my son and on how to proceed with his care and maintenance. This is the stuff that drives special needs' mommies insane! Truely, who do you listen to? In this case it was easy, since it was do nothing and hope it gets better or let's do a CT and see what we can see. But it is not always that clear cut a choice.

The bright and shining spot not to be lost in the mist of all this GI junk is that his bone marrow was normal! Even though he has blasts they are the "friendly" kind and she is not concerened at all by his report. That made yesterday get a whole lot better.

Last week when Tomas and I were driving home form the new GI appt. it was rush hour and we were stuck in traffic the whole way out of Dallas. It very much struck me that caring for him and navigating the medical world is very much the same. Slowly, so so slowly, we trudge forward. Every once in a while we switch lanes hoping to move at a faster pace, all the while watching those around us do the same exact thing. But the most amazing thing happened while I was stuck mentally and physically in traffic on Wednesday. Tomas happened. He was fascinated by the cars all around him. Every time one would pass by the window he would kick and scream with a JOY that almost popped him out of his carseat; and I laughed. Right there in the middle of six lanes of traffic moving at a snails pace, we laughed and laughed and laughed. A tuck would roll up and he would explode in shouts of sheer delight. Oh my word how much fun we had. And thats it really, that's the big fat secret to our lives. He brings us joy beyond measure.


Tuesday, April 5, 2011

Where the heck have I been?

Well, first off, thanks for asking. Makes a girl feel loved, lol.

Let's see. Mostly doctor's appts, as usual. Only now we have the whole buying/selling/moving thing thrown in. My husband came home on Sat. and we have been working hard to get the house ready to list. Sprucing up the outside and shoveling out my middle child's bedroom come to mind. Outside is done, bedroom is still a work in progress. We are trying to list the house this week though so hope to get it done soon.

Of course, Tomas has not made this easy. He got out of the hospital on the 18th and started running one of his cyclic fevers on the 23rd, prefectly timed so that I would have to cancel his swallow study (I think he knew).

A pulmonolgy appt. for all 3 brought 7 new tests smattered among the little darlings (with bad lung genes).

Last week Tomas' nurse fell and sprained/tore tendons in her ankle. Replacement nurse came the next day, but then missed the next two due to a doctor's appt. and an ER visit on her part for a severe sinus infection. Do I want another replacement nurse? Um, no thanks, they are dropping like flies. Regular nurse will be back next week.

This week brought a pedi visit for that pesky fever. It doesn't get over 100.2, but has been around for almost 2 weeks now. A failed cath attempt and blood draw attempt brought a trip to the children's hospital for round two of torture the baby, but it worked. Still waiting on culture results.

Today was a waste of time and money at a dermatology appt. for him. Only thing worth even mentioning is that Tomas' cold sore isn't your typical cold sore (not really worth mentioning as I could have had the doctor pay me to tell him that) and is the kind you see in chronically ill kids. Smack me on the forehead, I didn't see that one coming.

Tomorrow brings a semi-big appt. with a new GI. It is the attending from his last hospital stay. The doctor was fabulous at the hospital and I hope that trend continues as Tomas has been draining up to 6 oz of bile  day from his stomach. His latest labs showed low electrolytes because of it. I have been giving him pedialyte but I can't replace 6 oz on top of what volume he is already getting for his feeds.

Thursday is a surgery consult to discuss the bile output as well. There is a chance that some stump of gallbladder remnant or some other weird (and rare - make me laugh) post gallbladder removal thing is going on with his bile.

Friday is a sleep study. Sweet, sweet sleep. Some good data from this study would be awesome, but even more awesomer would be a way to help him sleep better!

Now, of course my life isn't all medical. Well ok it is, but it doesn't really matter because the living still happens. Sleep not so much, but living  - hell yeah!







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.
Endocrinology.
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

Progress!

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

Admitted

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.

Friday, February 25, 2011

GI issues :(

First - I love having a nurse for Tomas. It is so reassuring to have another pair of eyes on him. She has worked with him for 7 days now and only had one uneventful write-up. She is definitely getting lots of multi-system experience. I even left her with Tomas and ran the girls over to the library the other day. It was a quick trip, only about 40 minutes, but it was just so nice to be able to focus my attention on them. Bonus, we started school back up this week and managed to finish everything on their lesson plan, and went to go see a children's concert at the Dallas Symphony Center yesterday (with nurse and Tomas in tow).

So Tomas: Tomas, Tomas, Tomas...
He is having GI issues. This past weekend he started retching again. A few minor episodes, and then on Monday a fairly large one. He had an appt. with his surgeon anyway to look at a mass by one of his scars, so it worked out well because I am concerned that the hiatal hernia repair may be giving way. The surgeon looked at the mass and said it could be scar tissue or could be the abdominal hernia opening up again. He would put orders in the computer for the GI to check it out when Tomas has the endoscopy. As far as the retching, the surgeon checked an x-ray from January that showed the repair in place. That's all great - but he wasn't retching in January. For now he thinks it could be a stomach virus.

As the week progressed, the GI issues worsened. By Wednesday he cried after being on the J feeds for only half and hour. I can't switch to pedialyte or take him off feeds because of the hypoglycemia. It is J feeds with formula or ER for IV fluids. With the nurses help we were able to reduce the rate and figure out how much sugar to run through to keep his glucose levels stable. Slowly increasing I was able to get him back to normal by yesterday afternoon. His nutritionists were out this morning and he has lost 7 ounces in the last 2 weeks. He has not slept in 3 days, and not slept well in about 2 weeks. He is actually napping right now, and I pray he sleeps for several hours, but it hasn't happened in a few days. The longest time he's slept for since Friday is 1 hour and 40 minutes. He moans in his sleep and is clearly uncomfortable. Poor baby, if this is just a virus it is taking its toll.

My husband has started hi job in NJ, and will be travelling back and forth, which makes me all the more grateful for the nurse. I tried calling the new hospital in NY to get the ball rolling with transfer of care. I was transferred 4 times and then disconnected. This is going to be a long road. And if somebody doesn't start sleeping soon it is also going to be uphill!

Wednesday, February 16, 2011

Faith, Hope, and Pixie Dust

Faith
Olivia continues to do amazingly well. Just a palm sized bruise that only bothers her during sharp movements, but other than that she is fine. I can not express how wonderful that feels, for her as well I'm sure!
Tomas saw the urologist today. His renal scan was clean but there was some bladder wall thickening that needs to be watched. The urologist felt that since the bacteria Tomas grew was so rare that is just couldn't be true. He wants to catch it again but with a cath. This means having him cathed every time he has a fever, or if we have any suspicious urine output here at home. This makes having a home health nurse such a blessing. That is the silver lining in a rather disappointing morning! Some days the whole "tiny pieces of the puzzle" thing just really grates on me and it takes a whole lot of prayer to just get out of my cozy chair. Just once I'd like to walk into a doctor's office and have the doctor know what is wrong and how to fix it. It often happens that investigating one thing causes us to to trip over another. The bacteria was found while checking for a urine metabolic disorder, and the bladder wall issue was found while checking on the bacteria. Plus, I feel like every time a test result comes back normal we get further and further away from a common diagnosis, or maybe further and further away from a diagnosis at all. But somehow God moves me forward, and as every mother of every SN child out there knows, somehow we rally and rise again.

Hope
The nurse is working out very well. She is young and new to nursing, having only worked in a hospital for a few months. Tomas is her first home health patient. At first this sounds rather scary, but I like that she is not coming in filled with a preconcieved notion of what kids are/aren't supposed to do. She is, of course, quite competent in the basic nursing stuff - BP, HR, O2 - all that, but otherwise rather the blank page. That is ok though, because she watches, listens, has a quick learning curve, and I get it all done my way because she has no other experience to draw on. She works 3 days a week with Tomas and I am very much looking forward to the options this will open up for me and the girls once we are all comfortable.

Pixie Dust
As some of you already know, my family is moving (again - it's what we do, lol). Mike accepted a promotion and will be working in NJ. He worked there for a while before (it is his company's headquarters) and we lived just accross the state line in NY. That is the same area we will be looking at this time. The best part of this whole deal is no travel. He will have a regular office job and with the excepetion of a few meetings a year, be home every night. That is such a burden off my mind I almost had to put this in the faith section. Some nights I have a hard time falling asleep just wondering how I will manage if Tomas needs to go to the hospital while he is away. We have no family close by, and really - who else do you call at 3am to come and watch your kids? But it goes in the Pixie Dust section because that is what it will take to make this happen. The thought of buying/selling a home and transferring medical care just about paralyzes me! Not to mention a very difficult transition period where Mike will already be working from NJ before we are all moved up there. He will be gone for 2 weeks at a time. Again, the nursing was truely a blessing. I have meetings with the admit team the week of march 7th, and Mike starts the new postition on March 14th. Like I said - lots and lots of Pixie Dust!!!

Sunday, February 13, 2011

Sunshine and Lollipops

We had a fantastic week with my mom. The kids all had a blast with her. They made a snowbaby together, did Valentine's crafts, made fudge (my favorite part!) and stayed up late watching movies. The week went by too fast!

It was not all sunshine and lollipops though, on second thought it was. Olivia had her cardiac ablation on Wednesday, and because the weather was supposed to be terrible early Wed. morning and she needed to be there at 6:15, she and I drove down to Dallas the night before to stay at a hotel. This is the first time since she had any sibling that she and I have been alone at night together. We had a blast. My husband is a frequent traveler so the hotel chain sent up some free ice cream and sodas, and we had access to the concierge lounge with even more goodies. We stayed up playing cards, eating ice cream (until she was NPO), and just having some wonderful time together. The next morning we made it to the hospital just before the weather turned really bad. She took her goofy juice and was just hysterical to be around. My sweet, sweet angel. What a soft spot is in your heart for your firstborn! They took her back around 9 and were done by 12:30. That was pretty good considering they had warned me it could take around 5 hours. The doctor was able to freeze the bad spot, and told me it was for sure an AV Nodal Re-entrant Tachycardia. If you are going to have heart palpitations those are the ones to have! So all good news. She recovered just fine, with a small scare to a resident overnight with some funky heart rhythms, but the cardiologist put us all at ease in the morning. All in all quite a successful and uneventful hospital stay. I must say I spent a large part of the time waiting for the other shoe to drop. It was just so surreal to have everything happen the way it is supposed to.  She is recovering perfectly with just some leftover soreness. I couldn't be happier!

Tomas is as Tomas does. I think that will be my new catch phrase for him. My mom was here for 8 days and we had three doctor visits, two Rocephin injections, one lab draw, one new issue pop up requiring another -ologist, and one ECI evaluation with 7 people at my kitchen table.  He is stable, and got an all clear for the UTI from a few weeks ago, but with the last urinalysis some ketones were in his urine. I'm grateful for this, I have been testing him at home and have been telling the doctors he's ketotic, but now I have clinical evidence. The pedi is trying to get the endo consult moved up. In the meantime Tomas has a kidney ultrasound on Wednesday followed by a meeting with his new ologist - the urologist.  He actually had one until he was 5 months old, but then was discharged. So not sure if it counts as new or not.

Yesterday the weather finally warmed enough to take him outside. See if you think he enjoyed it:





Forgot to add that the nurse couldn't make it on Wed or Thurs because of the weather, but everyone muddled through just fine. On my way out the door I asked my husband if he knew what to do if the button should come out. He said just one word.
Panic.

Monday, February 7, 2011

My dear heart

Olivia is having her cardiac ablation on Wednesday. Up until now I have been pretty nonchalant about it, but as the hours creep closer, the panic grows larger. Did I make the right choice for her? Ugh.. that is the curse of parenting, did we do it right? She can't take any cardiac meds because of her asthma, and she hates the SVT episodes and voted for the procedure when I asked how she felt about all this. I hate doubt!
She will be under for about 5 hours, the doctor maps the heart and burns any bad areas. A cardiac surgeon is on standby - just in case, blech. I am also worried about her asthma doing things to her breathing during and after the sedation. She and I are driving down to Dallas on Tuesday night, as a large snowstorm is supposed to head our way on Wed. morning. I'm glad we will have some special time at a hotel on Tue. night before she goes in. She has to stay the night, but if all goes well we will be home on Thursday.

Tomas had to go back to the pedi this morning for a re-draw of that pesky urine infection, and still needs follow up with the urologist. We are waiting on scheduling for the kidney ultrasound and VCUG. He also needed a shot of Rocephin today because of a pesky sinus infection that wont go away even after two different antibiotics. So he got an injection and a cath today, not a happy camper. Well, ok now he is, but at the time he was pretty ticked off!

In other news, his nurse comes out tomorrow for training. She will be here T,W,R - the days my husband usually travels, from 9:30-5. To say I am excited will be an understatement for sure. My girls aren't going to be to happy because I have now decided to take full advantage and go to a 3 day school week, pretty much matching the nursing hours. That means longer hours those 3 days, but off on Mon and Fri. It all worked out so well though, because now a nurse will be here with my husband and Tomas for the days that I am at the hospital with Olivia. Thank you, God for your perfect timing!

Friday, February 4, 2011

8 years ago


The world became more intense. Most of the world doesn't know this yet, because most of the world doesn't know Victoria Maria. But you will, she is not the type to go unnoticed. Beware, because she has exacting standards of justice and fairness; but do not fear for she is also filled with mercy and compassion to nearly overflowing. Lucky for you world she is not ready yet. Much needs to be honed and shaped still, but this I know - she will meet you head on and she will live in you with an energy you rarely see. You will be better for it.

Sunday, January 30, 2011

So glad this week is over!

It was a long one! The two ER visits last weekend, GI on Monday where he told me he wasn't the right doctor to help Tomas, Tuesday brought a pedi visit for the girls who have been battling this virus for two weeks, and then Thursday.
I applied for a medicaid waiver for Tomas, he was approved, and in order to start nursing services I had to bring him to a nursing home overnight.  I went to the center on Thursday morning and filled out all the paperwork. Then we went to our two big appts., then at 11:45pm I had to bring Tomas back to the home for his "stay". It was very easy, but such a long day. My husband was out of town so I had to bring the girls with me. We got home around 1am. Thank goodness for homeschool and not having to wake up early the next morning!
But it is done. He has medicaid!! The nursing agency is sending someone out on Monday and we will get a schedule set up. Also, my husband's company switched to co-insurance this year so having the secondary insurance will be such a blessing.
Now for the appointments. We met first with the hem/onc and she wants to go ahead and repeat the bone marrow biopsy. Then on to the GI across the hall. He spent a ton of time with us and went over everything. I had typed up a GI history for the visit and we went over the whole thing. We then went back across the hall to hem/onc to finish up with her. All total we were there 4 hours! My girls were so good, and we still had the nursing home to do that night!
So what's the plan? Well, the GI wants to proceed with the endoscopy, and he wants a full metabolic workup done in the hospital, and wants to get the endocrinologist to do another fasting study. He thinks the elevated liver enzymes are not related to the liver. They are referred to as liver enzymes but they are also present in skeletal and cardiac muscle. Basically, an elevation in the enzymes only occurs when either the liver or muscles are damaged and the enzymes "leak" out into the blood stream. The doctor thinks a type of muscle breakdown disease may be the culprit. That makes my choices so far some kind of liver disease or some kind of muscular dystrophy. As I told a friend the other night - I'll take door #3 please!
I am not ready to throw the liver disorder out the window yet though. It explains so many of his symptoms that a muscular myopathy wouldn't; I can't dismiss it yet. Unfortunately, the only definitive way to check for it is the biopsy. So for now it gets pushed aside. First comes the admit.
The only catch to all this is that Tomas tested positive for parainfluenza at the ER last weekend. He now has an upper respiratory infection and the croup. So no sedation for six weeks.
At this point my week really should have been over but it was not to be. Friday came with a late morning for the girls, followed by a pedi visit for Olivia and Tomas. Olivia has severe asthma and had been blowing in her yellow zone for 4 days no matter what treatments we tried, and Tomas is, well, Tomas. So prednisone for both to get those lungs back to normal!
Ok, Friday evening, everything should be settled down by now right? Wrong!
I was cleaning up a bit in my bedroom and the kids were all in the living room watching TV when I hear a panicked "Mom!" coming form Olivia. Tomas' GJ tube came out. The balloon ruptured and the whole darn thing just slipped right out. So I grabbed him and put an old G button in to keep the stoma open. Great, that's the emergency part, keeping the stoma viable so no repeat surgery will be necessary. Now what? Can  I feed him into his stomach? Give him pedialyte? What about his hypoglycemia, and dumping? It's a Friday night, no IR doctor is going to be at the hospital, if I take him they are just going to start and IV and make me wait until morning. And my husband was still gone! A few phone calls back and forth between Tomas' pediatrician and the GI doctor on call at the hospital and we came up with a plan to keep him home and keep him safe. It involved lots of blood sugar checks on my part, but he made it through perfectly. Saturday, we drove down to Dallas to get the tube replaced while a friend watched the girls. The GI on call doctor never put the orders in so it did take a really long time, but other than that all was well.
I am so ready to start a new week, and shove this one to the deep recesses of my mind, preferably to a black it out area! Tomas is still not feeling great and very week. He played a bit on the floor this morning, but really has just wanted to sit in his little recliner chair that PT brought out for us.  I hope to see him gradually improve over the next few days. It has been a rough go for him lately!
This Wednesday I meet with the endocrinologist. The GI would really like to bring him on board. Up until now, it has been a challenge to get any endo group to pay attention. In the words of our old GI, "If your kid doesn't have diabetes, you're not going to get anywhere with endocrinology." Hopefully, the notes from the GI clinic will help to propel things forward, although the GI did say he would pick up any slack left behind by endo. He must be used to them.

I am just grateful that we are moving forward. I've felt stuck since summer and would really like to make some headway in the diagnosis game!

Tuesday, January 25, 2011

Big Neon Signs

Friday morning Tomas had another suspected hypoglycemic seizure. I actually forgot to check his sugar because I was so in the middle of fixing him and getting sugar into his body. Again, this is the mystery metabolic disorder we are still hunting for. Friday night he spiked a fever and Saturday morning I had to take him to ER to get his ANC checked. That is his infection fighting blood cells, and because he runs chronically low any time he has a fever her needs to get checked. Since the only place to do that on a  Sat. morning is ER - off we went.
His ANC was only midly low so he was able to just get IV antibiotics; however (damn that word) his overall WBC was very low, and his platelets were low also. This bought a call to the hematologist on for the weekend. Any guesses on who it was? The doctor whom I  dismissed last week. Sigh. He sent us home, following ANC protocol. I think that is my biggest issue with him, he is a competent doctor, but I have never, not once, seen him step outside the box. Anyway, home we went.
Sunday Tomas still had a fever of 102, then in the early evening when I vented his stomach it had so much more blood in it then ever before. He wasn't gushing or anything - just so much more than usual. I called my husband in and even he thought it was a lot of blood. I debated about taking him in again, but then remembered the low platelets, so off we went again. The ER was packed that evening and it took almost an hour to get triaged. Once that happened though we got right in. The nurse said we trumped everybody out there so we went to the front of the line - kind of a strange perk but a most welcome one at the time.
The ER doctor said he would most likely be admitted, they ran in some IV Zantac to stop the bleeding, and drew labs. His platelets were up and his WBC was slightly up, but his ANC was slightly down; so another call to the hematologist on duty (yes, still the same doc) and he said it was ok to discharge him. I told the ER doc I was not comfortable taking him home with the GI bleed, fever, and funky blood numbers. She called the GI on duty and ran through everything, and he also discharged Tomas, with orders to see GI the next day because as it turns out his liver enzymes were very elevated and his liver was enlarged. He also doubled Tomas' dose of prevacid, which the nurse tried to give him by mouth (that is why I can NEVER leave Tomas' bed - just too many things can go wrong). I told the ER doc I didn't want to give him the med because he already had hardly any stomach fluid and I didn't want to make it worse. So I had to refuse it, and we went home, at 3am!!! Crazy.
Now I was supposed to follow up with GI for the elevated liver enzymes on Monday. I knew it would be a battle because of the difficulty I had getting Tomas switched over to the hospital clinic in the first place. First thing Monday morning Tomas' pedi called to find out what had happened over the weekend and he was just shocked that Tomas wasn't admitted. Me too. Anyway, he said to try the clinic and if I had difficulty to call him and he would run interference for me. I called the clinic and told them what was going on, she said she would call me back in a few minutes with an appt. 2 hours go by and the pedi calls to find out how things are going. I had to hang up with him though because the GI clinic was calling in. The woman on the phone said that the doctor said until Tomas is an established patient that I needed to follow up with the current GI. His new patient appt. was on Thursday, and I had been seen at his hospital. I called the pedi back and he called out to the old GI and got me an appt. that afternoon. In the meanwhile I called the hem/onc clinic and got an appt. for Tuesday morning with his regular doctor  to follow up on the cytopenia issues.
Tomas' bleed was still under control and I went to the GI appt. and met with the doctor, whom I like very much. We were leaving because the oncologist wanted to do the whole "team admit" thing and work closely with the GI at the children's hospital. Anyway, the current GI walked in and spent an hour firing me. He was so nice and compassionate and just fully admitted that Tomas' care was beyond the scope of what his practice could offer. He though it was a great idea to team up out at children's. He said the best thing for Tomas would be to have a medical student assigned to him who could spend the hours of research necessary. He also admitted that he ordered the wrong test when he ordered the metabolic study for the glycogen storage disease. Turns out we waited 6 weeks to find out Tomas does not have a chronic inflammitory disorder.
As I was leaving the office, already feeling quite dejected, a voice mail came in from the hem/onc clinic saying that the appt. for Tues. morning had been cancelled and that Tomas did not need to be seen. To say I was at a low point was an understatement. What is it going to take to get someone to pay attention to this baby? Here he is with a GI bleed that has been going on so long it has depleted almost all the iron stores in his body, he is hypoglycemic like clockwork about 4 hours into a fast, he has chronic neutropenia which no one can explain, and an immune system that is so suppressed he is growing a bacteria in his urine that the pedi had to go look up because he had never heard of it.
So I got in the car and started talking to God. Clearly, this was not the path I was supposed to have Tomas on, but if He could please show me the right path in the next, oh let's say, 12.5 seconds  I would greatly appreciate it. Do I let Children's try this team admit? Do I pack him and the girls up and head to Houston, do I wait it out until the summer when I can take him to NYC? I called a friend and she commiserated with me (thank you friend), and said she would pray that God send me a sign.
I hung up with her and not 10 minutes later Tomas' oncologist called. This is the doctor who was determined to help Tomas at our last appt. and wanted to get the team together to do the admit and testing. She talked with me for almost an hour, and we went over everything. She had heard through the pedi that I wasn't particularly comfortable with the GI switch over. I told her I wasn't because that doctor wanted to give me an appointment for March 21st, even after I told them that they were not seeing Tomas for feeding issues, but the GI bleed and elevated liver enzymes. It took the pedi calling for me to get the appt. on the 27th. She said that didn't really make any sense because she had met with him for an hour about Tomas and he was very intrigued. She said it sounded like a front office issue and that she bet me if she spoke with the doctor he would not know any of this had gone on. It turns out I misunderstood the pedi, he didn't call the GI to get the appt. moved up, he called her, and SHE called the clinic and told them to get Tomas in asap. She also told me that one of the swabs from Sunday' Er trip came back positive for parainfluenza, which could explain the WBC suppression and platelets, but is not a virus associated with elevated liver enzymes., that is all Tomas. She asked me to please give this a try. She cancelled the appt. for Tuesday because she wanted to see me the same day as the GI doc at the same location and save me the drive to Dallas. So come Thursday I meet with them both.
That evening the pedi called again to check on how things went at the GI.  I told him everything that had happened and he was almost as relieved as I was. He was shocked that the GI had dismissed us, but agreed that the doctor was limited in what he could help us with. He also told me that any future ER visits should be down in Dallas. I like to use the satellite campus because it is so much closer. But he told me that he had never heard of giving someone IV Zantac to stop a GI bleed and then sending them home. He was mad that Tomas wasn't admitted (he doesn't have rights out there) and said that if he were in Dallas at the teaching hospital it would never have happened. He has been Tomas' strongest advocate and I am so appreciative of his efforts.
Sometimes God sends me signs so unobtrusive I don't recognize them until well after the fact, other times things happen that just nudge you along, and then there are days like these, when big neon signs hit you square on the head. And although it wasn't EXACTLY 12.5 seconds I am grateful that I was not left at that low place for long. I couldn't have stayed there anyway - Tomas is still sick, and so are both girls!

Thursday, January 20, 2011

Where's his birthday post??????

His birthday was on the 16th, today is the 20th - what am I waiting for?
Just for putting my mind together.
Of course there is more medical stuff going on but I wanted to get the birthday post in before I relay any of that, and really it is just more questions and no more answers; too annoying to write about. I can't really find a word for how I am feeling these days. I just know one thing; I do not want a do-over. 

We had a quiet day, it was too cold to go outside, and he is too immune compromised to go anywhere inside, but we had a special day anyway. Lots of love, lots of attention, lots of singing, playing, reading favorite books, and watching favorite shows. The girls and I made him a special cake.


 
I think he liked it (could only taste the frosting):


 Wrapping paper is the bestest ever!

 Just love that cute little face.

Right now we are waiting on a bunch of doctors to come together and get some testing done. Waiting is hard, and fatiguing, and frustrating; but giving up would be harder, more fatiguing, and more frustrating. Instead we wait - in joyful hope, because as for me and my house we will serve the Lord. and who wouldn't hold out joyful hope for this:

Thursday, January 13, 2011

Tiny whispers

I had a first today for me - I had to fire a doctor. It felt horrible, but it needed to be done. I love Tomas' hematologist, but not one of the other doctors in the clinic. We have seen this other doctor several times, and although he is kind he has a very laissez faire approach to patient care.
I called Tomas' CCBD clinic two weeks ago and asked for an appointment because I was concerened about his labwork trend. This doctor saw us (our regular was not in clinic that week), and he dismissed it. For today's appt. I really needed someone to listen to my concerns, and it was supposed to be with his regular doctor.
However, Dr. X walked in the room. My heart sank, I asked if we weren't supposed to see Dr. Y. He said she was around, and asked what was going on with Tomas. I told him everything and that I was worried. He said it sounded like it was a follow up for GI. I said I was having trouble getting a timely response from GI and getting Tomas' care moved over to the GI clinic at the hospital also would take a while. He said that would be ok.
I took a deep breath and said, "I'm sorry, but does this mean we are not seeing Dr. Y?"
He asked if I wanted to see her, I told him I did and that for continuity of care for Tomas I thought it would be better if I stuck with her.
He told me he had seen Tomas a few times already. I told him I'd really like to stick with one doctor throughout this diagnosis. A few more words (none unkind) and he was gone. That was so hard!!! At that point we were called back to lab.
Later, Dr. Y walked in and I don't think she even knew Dr. X was in because the first things out of her mouth were, what is going on with your baby, we need to check this, and this, and that.
She looked over the spreadsheet I put together and was very concerned about the low calcium and low protein. She said his body has very little muscle mass and is using his nutrition to rebuild lost tissue it is burning for energy. He is unable to build any significant new tissue and his nails are showing signs of chronic calcium loss. The she said very firmly, "This is not diet related!"
She also said that as this point she thinks he has two separate syndromes going on (not including Down syndrome - we are so far from that now it is not even funny). One syndrome to explain the neutropenia, liver and hypoglycemia; and another for the inability of his body to metabolize food properly. His labs showed a huge increase in iron, which she said was because I had just given him a dose a few hours earlier. However, the iron binding proteins were the highest she's seen in anyone in a while, which tells her his body is absorbing the iron but unable to use it properly.
She asked if I had though about moving GI care back to the hospital clinic, and  I told her that the scheduler said I needed to have records sent over, the doctor would need to review, and then Tomas would need to get a new patient appt. She told me to wait a minute, left the office, came back a few minutes later and told me she and the GI were going to meet this evening about Tomas!
There was so much more but honestly my head is swimming. What it boiled down to was she thought it was time to be very aggressive in getting some metabolic testing done.
She already knows GI wanted to do the scope and liver biopsy, and she wants a repeat bone marrow biopsy. So, the plan right now is after tonight's meeting, and hammering out of details, and possible bringing in of endocrinolgy, Tomas will get admitted. I don't know when, but am assuming sooner rather than later since the GI bleed still needs to be addressed.
I am on pins and needles. My biggest fear right now is that something will fall apart and the admit won't happen, or that someone will tell me that there is nothing wrong with Tomas, and to stop worrying.
I hope that I finally have their attention and can keep it this time long enough to get a diagnosis, and a treatment plan. His PT was out this afternoon, and since Tomas had not had any floor time this morning, and had taken a nice afternoon nap I was hopeful for a good session. He played a bit on the floor, and then tried to pull up to stand (to get my computer - turkey!) and then about 25 minutes into the session he scooted over to me and grabbed at me until I picked him up. Then he laid his head on my shoulder, panting from the hard work, and rested.
She wrote in her note that he was having difficulty acheiving tasks he was performing well just a few weeks ago, and that she was bringing a feeder chair next visit for supportive sitting when he is worn out. Watching the progressive weakening has been the hardest part. It is by no means severe, but I have noticed it for a while, and now the therpists are starting to see it. I pray once we get the iron under control that will resolve most of the stamina issues.
Thank you to all who keep him in your prayers. They are powerful indeed. They came in like a thousand tiny whispers and pushed me forward this morning to advocate for my son.