Thursday, April 28

So update email. Itís been a long time, but Iíve been sort of waiting to get everything together before I send anything out. The doctorsí appointments have been rapid-fire for the last month it seems, and I hate that.

Here goesÖ

About two weeks after Autumnís birthday, she asked to sleep with the bipap mask that covers her mouth and nose. She likes the nose mask, but her nose was stuffy, and the bigger mask feels more comfortable. Well, apparently she had a small Ďwet burp,í it stayed in the mask, and caused some issues, to say the least. She started running a temperature a few days after that, no coughs, but just acted very lethargic. On one day she wanted to be on bipap almost all the time she was awake, her oxygen was 93 (her norm is 99) and she slept 16 hours out of 24. I called the p​e​d​i​a​t​r​i​c​i​a​n​, was told hers wasnít there, and was instructed with her diagnosis to go to Childrenís Hospital and be evaluated for pneumonia. Well, I know Childrenís likes to admit, give oxygen, and antibiotics automatically, and are not very supportive of the whole CoughAssist/bipap thing, so I  opted to take Autumn to Oakwood Heritage hospital, which is smaller, thinking that if she couldnít be treated there, weíd have her transferred to the University of Michigan Mottís, where she is seen for pulmonary and where she had her tube placed.

Long story short, she had a chest x-ray, was diagnosed with early pneumonia, given an injection and some antibiotics to finish at home. We kept up with the CoughAssist, did Albuterol nebs and she was fine at the pediatrician for follow-up. Also noteóher O2 was only below 96 for one day, and she read 99 before she was diagnosed with the pneumonia a few hours later. I thought pneumonia made O2 levels go down as a rule, but I was wrong.

So the real issues started about a week after the pneumonia. Autumn has been on continuous tube feeds at night since she got the tube in 2008, getting 50ccs an hour for 12 hours. She began throwing up like clockwork 2 hours after feeding, every time. Of course that means we RUN into her room, quickly unplug the bipap, flush and unplug the tube, get her into the shower, wash her clothes, change the sheets, then before we redo everything she gets a CoughAssist treatment just in case she aspirated.

Now Iím going to be disgusting for a minute, but I want people to understand what exactly we go through when this happens. Autumn is unable to turn her head when she is lying down, and when she throws up, itís all overóall over her face, in her hair, on the sheets, pillow, everything. She also has food in her tummy, all liquid, itís pink and chunky and smelly, so itís a mess. And we have to HURRY, so she doesnít choke, so this crap isnít breathed in. Itís awful. And over a weekís time this happened every night. One Saturday it happened twice, and the next Sunday three times. I wanted to SCREAM, and my Autumn just looked at me and said, ďMommy, Iím sorry for making a mess.Ē I lost it. Iím never EVER cross with her about throwing up, Itís not her fault, Iím too scared to be angry, and all I think about is getting that crap out of her so she doesnít get sick again.

So thank God for Facebook, where I was able to figure out that Autumnís throwing up was due to gas. Some friends sent supplies to us so we were able to vent Autumn at night​ó​meaning hook up a long tube to her tummy button and connect the tube to an open syringe so the gas is released. NO MORE PUKES. However, sometimes Brian and I lay awake at night, listening to gas coming out of the tube. It sounds like water boiling. Itís bizarre and frightening. And she is sound asleep.

So instead of the night feeds, weíve been doing bolus feeds, 200ccs four times a day, a good glassful. We just hook up the tube, and pour the formula into the open syringe, and gravity pulls it into her tummy. Once when she wakes up, once after school, once before bed, and once before Brian & I go to bed while sheís asleep. Itís working fine, gas isnít a problem at all when sheís awake, but even with Tums and simethicone drops (baby gas drops) sheís still having issues. And at the well-child check at the p​e​d​i​a​t​r​i​c​i​a​n​í​s we discovered sheíd lost two pounds, most likely from all of the throwing up before we started bolus feeds. Pediatrician recommended going to see a GI doctor, so thatís where we were today. Left before 1 pm today, came home at 6:30. Yeah, this sucks.

So no concrete answers from the GI doctor. He said sometimes after an illness the body produces excess gas, nothing you can do about it, and it will eventually go away. The other theory is that as Autumn has grown the Ďendí of the tube in her stomach blocks the foodís access to the intestine while lying down, which causes gas. Waiting for a call next week to schedule an upper GI to check for that. Autumn will lie down, wearing her bipap, and the dye will be put through her tube than x-rayed to see where it goes. Pretty easy, Autumn had one before her g-tube surgery (she drank the stuff) and thankfully it is not painful, nor does it involve any anesthesia.

And we saw a nutritionist to figure out how to put some weight on Autumn. Drives me bonkers. Of course I got the ĎNo, Iíve never dealt with a child with SMA beforeĒ and ďWow, Iíve never had a child on Vivonex for tube feedings before.Ē Frustrates me. I brought in a copy of her feeding plan that was drawn up on Excel to show how much and what of she was getting. Didnít have the latest blood draw results but that was just done on Friday. (Her blood draws have always been spot on, except for last time when her vitamin D level was too high. Remedied that by only giving vitamin D every other day.) Went through the NO dairy NO meat NO milk-based protein shakes. Hard to explain to someone who isnít with Autumn every day, but whenever she has more than a bite or two of something with ANY animal protein in it, fish included, she gets sweaty and stinksóitís like her body has to work extra hard to burn off animal protein, and thus uses even more energy and calories. Kinda like Atkins, and thatís why we use Vivonex.  Vivonex contains amino acids, which are proteins broken down and easier for her body to digest.

So the extra calories will be coming from peanut butter, o​l​i​v​e​/​f​l​a​x​s​e​e​d oils, veggie burgers, olives, stuff like that. Sheís not in dire straits by any means, but the docs just want her to have more calories to catch up. Sheís so tall thoughó85​-​90th percentile for height, and seems to use the calories for that. Doc said to keep up with the bolus feedings and donít worry about continuous night feeds, which is fine. Weíre going to be reducing the amount of water in the formula and increase the amount of juice. Normally itís 500cc water/500ccs V8 Fusion juice, but weíre going to 250/750. That will help too, especially since eating for Autumn is hit or miss, she fatigues so quickly, never gets hungry, and has a laundry list of things she canít chew. Thank goodness she doesnít yet have any swallowing and choking issues. Thatís very common with SMA, and so many families struggle with that. Their kids canít eat, if they do they choke, throw up, and end up with aspiration pneumonia. Itís part of the disease, and almost universal in children with type I SMA. Scares the crap out of me, but thankfully we had the g-tube done, and even if that becomes an issue, we never have to worry about nutrition.

And Autumn is doing GREAT. She is so good with the machines, mouths off, is fiercely independent, fights with her brothers (well, just Aaron!), loves school, makes friends wherever she goes, and is an all-around awesome kid. This pneumonia was caught early, treated at home, and was the FIRST EVER, practically unheard of for a type II SMA kid. And now we have a Vest airway clearance system (Google that if youíre interested) and a suction machine for when she does get sick. Hoping we donít have to use the suction machine too muchósnots and boogies sick me out, and I have problem when Autumn spits after her brushing her teeth. But whatever. Weíll do what we have to.

This disease is taking SO MUCH TIME away from my family. Brian and I canít go out alone on Ďdate nightsí in case Autumn throws up. Vacations donít happen, too much crap to travel with, and Autumn only can be transported in her powerchair. I hate that SMA makes her so fatigued so bedtime prep starts at 6pm in order to get all of the treatments done. And this doctor stuff is crazy. I joke that I need to quit taking her since every time I do there are always two more appointments we need to make.

Again but whatever. We do what we have to do. Some families canít leave the house, some kids canít sit up without choking, some kids canít breathe well enough to talk, some kids spend more time in the hospitals than at home. But donít tell me ďI could never do that, I could never do what you do.Ē I HATE that. And you would. Youíd do whatever you could to keep your kid healthy and improve their quality of life. If you didnít youíd be a crappy parent who didnít deserve children in the first place since there are no guarantees. And you would also do whatever it took to keep your sanity intact so you are better able to navigate this whole mess. My daughter didnít ask for SMA, and my family didnít ask for all of the crazy shit that comes along with it. But I love her, I love all of them, and weíre managing quite well, thank you very much.


Friday, June 24

Here goes, another updateÖ

So school is done for 2/3 of the kids. Harrison will be in 6th grade next year, and Autumn will be going into 1st. It was a good year, and Iím so proud of how the kids did. Harrisonís overall grades were A- in English, Language Arts, Math, Science, and Social Studies and Art, a B+ in Reading, and As in Music, Gym, and Moral F​o​c​u​s​/​C​o​n​d​u​c​t​. For his progress report he scored in the 95th percentile for Math, 99th for Reading, 99th for Language Usage, 85th for General Science, and 84th for Concepts and Processes. He won awards for Honor Roll and Behavior.

Autumn did very well too. K ​i​n​d​e​r​g​a​r​t​e​n​e​r​s score on a scale from 1-4 rather than letter grades, and she got 3s in Basic Skills, Language Arts, Mathematics, Science, and Social Studies. She received a 3.5 in Art, a 2.5 in Reading, and 4s in Music, Gym, and Moral F​o​c​u​s​/​C​o​n​d​u​c​t​. Autumn also received awards for Behavior and improvement on tests.

Hereís why I LOVE this school, why their program works for my kids: Autumn tested in low, very low. She didnít have the best start with preschool, at the Taylor school district tried to strong arm me into putting her into a school for special needs kids. I knew that this would be a bad idea, so we ended up bouncing her around for awhile for preschool, and settled on the Child Development Center at the University of Michigan. Great program, but play-based and run more like a daycare than a preschool, so she was a little behind academically on the testing. In addition, she wasnít very good with a computer mouse since our computer isnít in a place where itís very accessible to her. So after a year in school and some practice on the computer here are her results. Took me a minute to pick my mouth off the floor. In Math she tested in at the 23rd percentile, but rose to the 92nd percentile. In Reading she tested in at the 1st percentile and rose to the 99th!!!!!!!! Now thatís some TEACHING! And needless to say I am 100% confident that Autumn wonít have any problem learning and that a Ďregularí classroom in a Ďregularí school is the most appropriate place for her.

And Mr. Aaron will be starting at a co-op preschool in the fall, going 3 days a week. Heís smart too, and I know heíll be a hit in kindergarten​.

Medical stuff now. Sigh. Autumn is still gassy, and must be vented at night when she sleeps. Her bipap is causing the gas since her pressures are so high, but she needs that to sleep. Brian & I canít feed her continuously with the feeding pump because she pukes, and we worry about her aspirating and getting pneumonia. So weíve been doing 200cc bolus feeds 4 times a dayódumping her food mix right into an open syringe and letting gravity pull it down the tube into her belly. Seems like a huge amount of liquid, but she handles it just fine. Sheís getting the calories and nutrition she needs just in spurts.

Of course with all the pukes/gas/weightloss we made an appointment with a gastroenterologist just  to make sure there wasnít anything wrong with the tube since sheís had it for almost 3 years. Our 1st appointment the guy was nice, but sent in this crazy nutritionist (allllll of them are crazy, and none too helpful) who didnít know a thing about SMA, never even heard of the formula we had Autumn on, and didnít listen to a damn thing I said. Autumn was 37 pounds, a little underweight, but not so much for a child with SMA since she has much less muscle mass than a Ďtypicalí child. She lost a pound from a year ago, not good, but when I tried to explain that this was a result of the pneumonia where she didnít feel well enough to eat orally and we couldnít tube feed her because of the pukes, it just fell on deaf ears. Anyway, Autumn was weighed in at 43 pounds yesterday, which was good, but thatís an awful lot of weight to gain so fast. Canít win. Sigh. I told the nurse not to bother sending in the nutritionist, since all  she wanted was to see numbers on the scale and didnít listen anyway. I handed her a copy of Autumnís most recent labs, which of course were perfect, and was told that she was fine. And her upper GI, which was performed with her bipap on, and barium pushed through the tube showed no abnormalities whatsover.  I was sent home with no explanation as to why she was so gassy, but given a pat on the back and the keep​-​doing-what-youíre-doing speech. Sigh. At least Autumn was a trooper, but Iím pissed that this appointment amounted to a 5 hour weight check. So I guess our deliveries of tube supplies wonít include feeding bags, and we can put our IV pole and pump away.

Tomorrow Autumn is going to MDA summer camp. Sheíll be gone from Saturday until Friday, and we wonít be staying with her. I managed to get all of her supplies in a big Rubbermaid tote and a large duffel bag. We have to drag along the CoughAssist and bipap, so weíll be using a wagon when we get to camp. All this stuff is heavy. And Iím not worried about Autumn at all, that girl is SO social and SO friendly. Sheíll cry when we pick her up. Fingers crossed that the equipment makes it home in one piece. The guy who does her braces has a daughter, Katie, who will be with her the whole time to help. Katie came over this week and met Autumn, and the two hit it off immediately. It helps that Katie knows how to make bracelets and likes to braid hair. Brian and I showed her the ropes on equipment, and today I spent some time writing little cheat sheets for her on routine and how to handle everything. Sheíll do fine though, and MDA staffs the camp with doctors, nurses, physical therapists, respiratory therapists, wheelchair repair people, etc. Sheíll be better taken care of there than at home.

And  here are some pictures. Enjoy. Iíll have more from camp, Iím sure, and for those on Facebook, more are posted there.