New tube

Here is a picture(albeit fuzzy camera phone pic) of Marissa's new GJ-tube. Im LOVING it so far! She hasnt thrown up even ONCE in 5days...WOW!
The top "tube" is her Broviac Central Line and the GJ-tube is the bottom. You can see two "extension" tubes that come off the button. One screws into the G port and one into the J port. We give meds and elecare in the J port(18hours per day) and drain and vent out of the G port(24hrs per day). GREAT system!

Its been a long time

Since Ive updated this blog. Like with my other blog I think its important that I continue to blog here because if anything I am able to track my own progress. I can read back through old posts and just see my knowledge grow, and sometimes laugh at myself for my "overreactiveness" to certain things and lack of understanding and knowledge of others. The truth is I was woefully unprepared to deal with all involved in Mari's care. How can one prepare anyway? I was about as prepared as I would have been had I given birth to her. I was someone who wanted a child and had the love in my heart, its that love and commitment to my child that has gotten me through. We go through so much everyday and learn all the time. Its always something new with her, just when we think we've got things figured out everything changes.




Since my last update we've had one hospitalization, for Gingivistomatitis(herpes simplex 1). It was a frustrating experience as they really had no idea what was wrong with her. She had high fevers and negative blood cultures but as long as she had a fever they had to assume line infection and they kept her admitted and on IV antibiotics. They were not effective and I was losing my patience. I couldn't understand why they weren't doing more to figure out what was wrong, it was obviously not a line infection but that's what they continued to treat her for! Finally, my friend Raechelle and I took her other symptoms(red swollen gums and sores on her cheeks) and punched them into google and we can up with high fever+red swollen gums+sores= herpes simplex 1(stomatitis) it fit so we took all the information to the Drs. They also had a light bulb moment and started anti virals, she was home 2days later.




During her 10day hospital stay she was obviously not as active as she usually is. That prevented her from being able to tolerate her full feeds. When we got home the trend continued. She went from tolerating 24ounces per day(her highest) down to only about 6ounces per day 12 tops. She couldn't seem to really recover. On the 17th we had a GJ-tube placed(replacing her G-tube via the same stoma) we are now doing continuous feeds for 18hrs per day. We started very slowly at 3ml/hr and are now up to 5ml/hr. We have a current goal of 15ml/hr and will go up from there as tolerated. The other thing that has changed is I am now venting/draining her out of the Gport and feeding her into her Jport. She is draining about 200mls of bile per day and as a result not vomiting, that is SO nice for a change. Her diapers are also more pleasant to changed without that added bile. They still aren't pretty and the addition of Elecare(new formula) means they are super smelly....but so far we have a good thing going. I hope things continue on this path.




That's our update!

No more nurse

I'm done. We've tried 3 nurses and I'm really just done. The truth is LVNs don't have the training or knowledge base to handle my kiddo and the ones they've sent me don't seem to have the ability to understand the seriousness of it all. I'm not sure I even blogged the full reasons we let nurse #2 go but suffice to say her actions(or lack there of) put my child in jeopardy not once but twice and what I discovered yesterday with nurse #3 is inexcusable. These are life and death issues and I mean that quite literally. Its not really completely their fault, they don't have the formal training/knowledge to handle it. I was told RNs in this area don't do home health/shift work so I could have an LVN. The LVN could do supportive work for me IE her laundry, clean her room, bleach out her personal fridge, stock and organise supplies, do weekly supply order, give her g-tube feeds and medications, attend/assist at therapy appointments, bathing/changing. I was also told this person could attend school with her, and stay home with her when I had errands appointments I needed to be at without her. The problem is this, stuff happens all the time that requires observation and intervention that relate to her line and important fluid and electrolite issues. Her pumps alarm(FOR A REASON) simply TURNING THEM OFF and not calling me is a HUGE HUGE HUGE problem. Casually mentioning it HOURS after the fact is beyond inexcusable. TPN is quite literally life support for my child. Turning off her pump and not clamping her lines, not flushing her lines means blood can backflow into the hub and she can develope an infection which could lead to DEATH. Her line could clot off which could also lead to infection and necessitate the need for major surgery! On top of that I discovered the fact that now twice in the past few days the nurse improperly used our gastric pressure relief bags. I am using them to vent her tummy over night and then if she is about to throw up I am dropping the bag letting some of the bile/vomit flow into the bag. Just enough to take some of the pressure off. Once she stops retching I clamp off the bag. I have explained this repeatedly to the nurse and several times I have found out she let the bags fill to bursting without notifing me in the moment or replacing any of the fluids. If you take it out you need to PUT IT BACK save causing serious imbalances. So not only have we been dealing with this but several times I have found her laundry not put away after the nurse leaves, or syringes left unwashed, supply orders just not done or supply orders filled out saying we need items we dont and not indicating items we do need. On top of all of this Marissa has gone down hill fast. It gets worse everyday instead of better. After the first week with nurse #3 she started having a lot of anxiety. She would begin to cry(scream/thrash/hit herself) the moment the nurse arrived in the morning. At first it was just when the nurse was doing care for her and then progressed to ANYONE doing anything for her but me. This includes Shana and Raechelle who are both primary(secondary?) caregivers for her, beascially they are both also as good as mommy--or WERE. To the point that she will cry if I leave the room or anyone else even talks to her. She stopped sleeping at night crying for me all night long. The amount of food she has been able to tollerate has decreased drastically and her vomitting has increased from not at all to daily to twice daily plus. Basically my life is hell right now. Im not getting any sleep and the illusion of help is just f*cking with me because it is without a doubt nothing more than a hinderance and disapointment. SOOOOOOOOOO starting Monday I will no longer have a nurse. Im going back to doing it mostly on my own. THIS time I am hiring help in other ways. Our current house keeper is moving out of state so I will be replacing her with a part time nanny/housekeeper. I will hire someone to come in two days per week. This person will be responsible for ALL school transportation( 3 kids 3 different schools to and from) and watching Mackenzie while I take Marissa to PT and OT. She will also be responsible for cleaning(all 3)bathrooms, vacuuming the whole house, dusting and mopping the kitchen floor. Insurance wont pay for this person like it would pay for a nurse....But my sanity and my daughters LIFE means it will be worth it.

A New Line!

Unfortunately Marissa's line was dislodged Tuesday morning. We don't know how and at first we weren't even sure if that's actually what had happened. There was some bleeding and drainage around the site and it looked pulled out slightly, not more than 1/8inch. After further testing it was confirmed that it was indeed dislodged. We have a new nurse and while I do so appreciate her and all the wonderful help she gives me...I'm a control freak and knowing she discovered this and not me and not knowing if she knows more than she let on...well it makes me sorta panic a little ok ok a lot. She's home now. Surgery went well. I thought Id have more to say...but I guess I need to process a bit more before I do.

Suzi

I don't have a pattern per se but I do have detailed pictures of it laying flat. I will upload them soon. It's what we used to create our own pattern, it really was simple to make.

16 ounces per day!

How awesome is my mom?

I found this product online today called a summers.lifeguard. Wow what a great concept! I knew right away it was something we could really REALLY use with Marissa! I immediately called the company to find out more info. I really love what they have done. Unfortunately, I don't have an extra 375$ right now (which is about how much 3would cost without shipping) and 3 is really the minimum number that we would need. I was pretty bummed. I called my mom and she said she'd bring her sewing machine over and we would see what we could come up with. I had a few yards of some flannel Id bought to make night gowns for the girls and my mom picked up a few yards of velcro. I am so so so thrilled with what my mom was able to do! I still really want to order a summers.lifeguard when I can afford it as I like to support small businesses and their inspiration is such a blessing to us.

The things that excite me

well for one my daughter has not thrown up for over a month...not ONCE. This is the child who used to puke daily and after nearly every feeding. I am nervous to be blogging this...I hope I don't jinx us. Second, this twin size bed...oh I want this bed. I am waiting to hear from the company how much it costs. I found some pointers on the site for getting insurance to pay for it. I will definitely pursue that. But in the meantime I may just cough up the money--IF possible. I am thinking this could cost several grand...but I hope not. Marissa has decided she wants to climb out of her crib. I cant sleep I am a nervous wreck. I am so afraid she will pull out her line or at the least break the iv tubing in the process. I thought about putting her into a twin bed but there is noway she would stay in it. Off she would go and she'd just keep on pulling until her line snapped and she was free. Ask me how I know *smirk*

Success!

Since her last appointment Marissa has gained ANOTHER 1.4pounds and grown a whopping 3inches! Shes grown so well her GI agreed we can reduce her TPN from 20 to 18 hours per day!!!!!!!!!!! This is VERY exciting news! So that gut of hers is obviously absorbing---I give SO MUCH credit to those digestive enzymes! *happy dance*

How to change a Mic-Key g-button

This is what I found this morning.....niiiiiiice ehh? Whats a mom to do? Why grab the camera and create a tutorial ofcourse*grin*

Breakfast tutorial

When "hooking up"....has nothing to do with the opposit sex

I stole that quote off of a member at a support board Im a new member of. So many machines for such a small little person to carry.


g'nite

Our routine

is ever changing. Here is an example of our current feeding/med schedule:



7am catch puke and administer glycerine suppository
8am blend breakfast 2ounces banana with 2ounces sweet potatoes 2capsules of digestive enzymes, .6ml iron and a large splash of almond milk...zevex infinity at a rate of 30mls per hour for four hours.
12noon meds actigal and flagyl via g-tube unhook enteral feed.
4pm unhook TPN and hook up enteral feed(similar to breakfast but with veggies)30mls per hour over four hours.
6pm meds actigal and flagyl via g-tube.
730pm prepare TPN and lipids.
8pm hook up TPN and unhook enteral feed.
12am meds actigal and flagyl via g-tube.

ER

I'm pretty sure this is our 6th visit to the ER since Christmas, but who's counting? We discovered this morning that her bag of TPN was leaking. Somehow it was cut---though I cant for the life of me figure out how(it was inside her backpack) So I paged the nurse and she paged the Dr who said in come in for a blood culture and some prophylactic antibiotics. Hours in a germy ER. Now imagine how UN FUN that might be, then double it. That's about how fun it was. I am so tired I cant function. Im stressed and tired. I decided not to drive the hour to our children's hospital but to go to the ER near me. What a huge mistake. I'm warning you that what you are about to read is shocking, I can imagine that it would be shocking to most lay persons who have no medical experience whats-so-ever and even more shocking to those who do.

Upon arrival our RN came in to do an assessment. She asked me what had happen. I explained that her TPN may have been contaminated. As we talk I am changing Marissa's diaper and nurse notices her g-tube. She then asks me...wait...are you ready for it? she asked me if I gave Marissa her TPN via her G-TUBE. I'm kidding right? I mean really a REGISTERED nurse would SURELY know the difference between ENTERNAL and PARENTERAL nutrition right?? Nope.

Next comes Dr and I ask him to do blood cultures and for some antibiotics. He starts to write a script. I mean surely this DOCTOR read the chart and her medical history right? How does some liquid bubble gum flavored stuff sound to you? I mean she doesn't swallow or take anything by mouth, and she doesn't ABSORB in the 2cm of small intestine and no colon she has(doesn't have)...No Mr smarty pants Dr we would like a shot--thank you very much. Then he asked me which one to give her---hello who's the fucking Dr?

OK moving on.

Blood cultures. In walks the barely 18yr fresh out of mail order trade school to do blood cultures on my daughter---who has a CVL. Me: you cant do that. Her: I assure you ma'am I can its my job and she shows me her badge. Me: yes I'm sure the 6weeks of school you got qualifies you to do this. Sorry girly you'll have to get an RN(OK I didn't really say that, I was nice..turns out she had no intention to do them out of her line she was going to do it via a peripheral vein...um mm yeah.

So in the meantime while we wait for RN to come in. I can hear her talking with the pt in the next bed. He fell on his skate board and broke his wrist--well really they think he may have broken it so they split it and she gives him his discharge orders--and she says...again I am not joking, she says: Make sure you follow up with an orthoDONTIST about this arm...HIS ARM. an orthodontist. I was dying.

Then she comes into our room and proceeds to do the blood culture---no gloves didn't even see her wash her hands. I stopped her right there and said oh no. For one you didn't wash your hands, for two you aren't wearing gloves(and they need to be sterile) and you don't have all of your supplies. She looked at me like: are you kidding? I wasn't. I then told her I didn't have an extra clave with me and Id need for her to get one. She didn't know what a clave was...I show her she calls it a cap. fine whatever. So then she comes in with the clave and gloves. Puts the gloves on THEN opens the other supplies and goes right in to do the clave change. I almost screamed at her. First of all if she does the clave change now she will almost certainly contaminate the culture and for two she touched everything, wasn't sterile and needed to start over. She walked out and came back with another nurse. Because you know I'm a crazy mom. Then she did the culture(though I'm not convinced she didn't contaminate it) and went to do the clave/cap change without cleaning it or priming the new clave. I just asked her to walk away and I did it myself.

They were all talking shit about me behind the curtain--saying have you ever called it a clave? what the hell is a clave? and saying: oh your not sterile and then laughing. Whatever whatever whatever. They were all about 22 at the most and its anyones guess how that nurse passed her state boards. I do remember in school our teachers saying there are great nurses and there are awful nurses. Today I met an awful nurse. Even the Dr was clueless. I wont ever again try and take the easy way out with my daughter again by going to a local er. I learned my lesson.
Does this post show how truly tired I am?

Where do I even start?

I'm tired so I'm not sure this is the best time for a blog post but I'm afraid if I don't get this stuff down it might just as well be gone for good. It seems I do have some resilience after all, in the case of me in comes in the form of selective memory. One of the past three days--why yes you see the selective memory is already kicking in!!....I went to change Mari and noticed her shirt was wet. I looked down and what do you know? She had no g-tube in place...just a nice fat juicy hole in her abdomen, yeah. Totally traumatic. This all might be old hat to many of the parents who Ive begun to associate with but for me? Not so much. I wasn't sure what to do next. I had after all, no clue how long it had been out. I started to instruct each of my children to hunt for it and soon it was found. Then some common sense popped in and I decided it wouldn't be the best idea to reinsert the same mi-c key so I began to search for the extra I had on hand. It was quite literally 10inches away from my face yet it took me about 10minutes to find it. Once I did I lubed it up(with water, because its all I had) and popped it right in without incident. It was amazingly simple and drama free, who knew? This morning I went in to wake her and the back of her shirt was soaked. I tape her CVL over her shoulder to keep her curious fingers off the connecting parts. Sure enough her line was broken, damnit. So I called the tpn nurse and she had another tpn nurse meet me at the hospital(an hour away) to repair it. I wasn't home until 5pm so it took all day and she got two shots of antibiotics. She vomited on the drive home and then again before bed. I only had to do one dressing change thankfully. This brings me to a conversation I had with her specialty team today(over the phone mind you as I couldn't attend clinic because we were repairing her line) Ive been confused as hell. I'm keeping a journal of everything that goes in and out. I couldn't for the LIFE of me figure out why we can go a week without any vomiting or diarrhea and then out of the blue(when nothing has changed) she will suddenly vomit etc. Well one member of the team asked me if I noticed a correlation with the flagyl. Sure enough she didn't vomit at all last week when she was on it and has had several bouts with vomiting this week(her week off) SO we are going to try out constant tx and see what happens. I hope this is it. Though I am sure it will only be one in a long line of "its" over this child's lifetime. I found out today that this is the longest Marissa has ever gone without a line infection. Today is my 120th day as her mom. Seems like YEARS. We've had 6trips to the hospital in those 120days with 3hospitalizations, thankfully none of them due to a line infection. That's it I'm so tired I cant see anymore.

Friday March 28, 2008

breakfast: 2ounces prunes and pears

lunch: 2ounces applesauce

Poop!

dinner: 2ounces banana

snack: 2ounces kefir(yah its back)

DX

First I took Marissa to an appointment with her Endocrinologist. I am proud to say that my daughter is ON the growth chart for the first time in her LIFE. In 3months she has grown 1.5inches and gained 2.5pounds. I wasn't expecting the strong reaction I got(very positive) Not that I was expecting a negative reaction but lets just say I get LOTS of pats on the back, tears and hugs from the Dr's who have been caring for her since birth. I left feeling recharged and accomplished with the progress we are making. After a quick stop for lunch we headed to regional center for her evaluation with the Neurologist. We did get the CP diagnosis that I was hoping for, but somehow I wasn't glad to get it once we did. She expressed that she felt that Marissa may be able to walk at some point, with support of braces and canes or walkers....something about her special stroller and how we can get away with that for a few more years before she needs a wheel chair. Spastic hemiplegia I believe is the DX. I left devastated and inconsolable. I don't feel like I am strong enough to handle all of this. As parents we all have dreams for our children, for the type of life we hope for them to have. I not saying she wont have a GREAT life, its just not going to look how I pictured it. That doesn't make it a bad life, just different. Letting go sounds easy enough in theory. I just feel like its one thing after another with her and I just wonder when its gonna end? When is this kid going to get a break? When we got the call about her we were told that she "used to be a tpn baby" that she had a g-tube and wasn't walking yet--but on the verge of and had some developmental delays due to lack of stimulation and deprivation. That she had improved to the point that she needed to be out of the medical facility and to be placed with a family. What they didn't tell me was that she only had a g-tube to get meds that she was currently on tpn and would be for the rest of her life(that she didnt talk, or swallow, or take any foods by mouth). That she had a potentially terminal condition and would be facing multi visceral organ transplant. That she had chronic organ failure, chronic anemia, left sided weakness(that is now DX as CP) That she's never formed a secure attachment to anyone person in her life. That she's spent more time in the hospital than out. But by the time I learned all that she was already mine. I wish I was a bigger person, if I was I would say I would have agreed to take her knowing all of this upfront. But honestly had I been given all of this information upfront I would have said a prayer that the right family was found for her and moved on without ever having met her. Of course now as her mother I know how blessed I am to have her as my daughter. I never imagined in a million years that I would be raising a child who is facing so many challenges, I mean what parent does really? I guess no one. I know I'm not only parent out there, so why do I feel so alone?

Friday March 21, 2008

AM: No poop

Breakfast: 1/2 a banana pureed and blended with 2ounces formula

Thursday March 20, 2008

AM--No poop.

breakfast: pureed prunes and pears 2ounces(50cals) diluted with 2ounces formula(40cals)

poop!

snack: 2ounce green beans(20cals) diulted with 2ounce formula(40cals)

lunch: 2ounce steamed quinoa and brocoli(100cals) pureed and diluted with 2ounce formula(40cals)

poop!

The following is vitamins/minerals and where they are absorbed

DUODENUM:
Riboflavin (B2)
Niacin
Calcium
Iron(iron absorbs better when taken with Vit C)
Protein
Carbs
Starches
Magnesium
Thiamine (B1)

JEJUNUM:
Sodium
Electrolytes
Glycerol
Iron
Calcium
Fatty acids
Cholesterol
Carbs
Peptides
Starches
Amino Acids
Water: 85% is absorbed in jejunum
Folic Acid
Thiamine (B1)
Bile Salts (small amount)
Nicotinic Acid

ILEUM:
L-Absorbic Acid
Vit. C: proximal ileum (iron absorbs better when taken with Vit C)
B12- terminal ileum
Fats
Bile Salts: Mostly in ileum (terminal)
Fat Soluable Vitamins (A, D, E, K): Ileum

Residual Water: Colon

And, here is a list of tests and what they are for (a special list
for people with malabsorption issues):

A: Fat Absorption:
1) Fecal fat microscopy - normal results are less than 100 globules
2) Quantitive Fecal Fat Analysis (one of the best tests for fat
absorption) - normal is 3-5 gm fat/day excreted. More than 6 gm/day
is significant
3) Serum Carotene - carotene level falls in fat malabsorption
4) Serum cholesterol - cholesterol is decreased in malabsorption
5) Fat Breath Tests

B: Carbohydrate absorption:
1) D-xylose absorption test: monosaccaride absorbed in jejunum.
Decreased level seen in jejunal mucosal disease and bacterial
overgrowth syndrome (breakdown of xylose)
2) Glucose tolerance test: results in a flat curve on test
3) Lactose tolerance test: shows an intolerance in infants due to
lactase deficiency or in adults with lactase deficiency
4) hydrogen breath test: disaccharidase deficiency and bacertial
overgrowth. Increased H2 (hydrogen) in breath if dissacharidase
level is low due too increased sugar in gut

C: Protein absorption:
1) fecal microscopy - shows animal skeletal muscle fibre if positive
2) fecal nitrogen - Normal is 2 to 2.5 gm/day.
3) protein losing enteropathy - increased fecal clearance of alpha-1-
antitrypsin. There is increased protein leakage in the intestine
when positive.
4) serum albumin - albumin levels are decreased

D: Vitamin Absorption:
1) B12 deficiency - Schilling Test
2) Serum folate - indicator of jejunal dysfunction (not enough folate)
3) Prothrombin time - Vit K decreased

E: Bile Salt Reabsorption
1) Bile Acid Breath Test: glycine is used. Bile acid glycocholate is
formed. In ileal dysfunction, jejunal bacterial overgrowth, short
bowel syndrome, there is labelled bile acid breakdown in colon

F: Mineral Absorption:
Serum calcium, phosphorus, magnesium, iron and TIBC

Pancreatic Function Tests:
Secretin test: duodenal contents are assayed for enzymes
Lundh test: Duodenal juice is analyzed for tryspin activity

Other Tests:
Duodenal aspirate: microscopy - girdia, culture- bacterial overgrowth