Taking A Deep Breath

wednesday update

2012-01-25T19:28:00.000-05:00

Things have settled down here a little. Glucose is stable on dextrose. Neurometabolics came in earlier and they want Ava to see Dr Cohen. He is a Mitochondrial specialist in Akron. They are going to fax medical records over. I am actually happy about this. I love Cincinnati Childrens but its time for Ava to see a Mito doctor. Her primary ped knows Dr Cohen personally and they share a few patients. ava will be getting her GJ replaced in the next couple days. She had a nice run on g feeds but we need to switch to the J again. We are getting D10 and an IV pump lined up for home. Ava can get fluids at home and avoid being admitted. Thank God for her port. For now we are seeing favorite nurses and RTs. Ava has invited the lady that cleans the room over to our house to "play toys.". Haha She's known these people for 2 years now. Not sure how long she will be here.

In the hospital

2012-01-25T01:50:00.002-05:00

Ava was admitted today for hypoglycemia and feeding intolerance. Its really been a terrible day. Its almost 2 And we're still in the ER waiting for a room. She's finally stable enough to go upstairs. I really wish residents would listen more and realize they are still learning. Ava will never be textbook and to treat her like that is a huge mistake.

Going private

2012-01-22T19:36:00.002-05:00

Someone has stolen some pictures of Ava and is using them without permission. For this reason I am making my blog private. I'll leave this up for a few days so that everyone who would like to continue reading it has given me their email to be invited.

I am just sick over this and can't imagine why someone would do this. I can only guess for money or sympathy. Since anyone who knows Ava KNOWS she doesn't needs sympathy or money........I am sorry but I have to protect my family.

DME and rehab

2012-01-18T17:27:00.003-05:00

I'm not a DME (durable medical equipment) guru. When Ava's doctors say she needs something, we get it. Simple enough. She has more medical equipment than I know what to do with. I'm not talking about that right now. I'm talking about adaptive equipment. She has a wheelchair (TiLite) and DAFO's. I donated her gait trainer and walker. Her physical therapist started the process of getting her new carsear.

The special tomato is what we agreed on. It reclines, wipes clean, has lateral support and has some cute color options. Ava needs a seat that will recline. Her trunk isn't strong enough to support her in a typical booster seat.

I've been casually looking at bed options for a while now. She's currently in a toddler bed with the mattress propped up. There are rails on the sides. She has a difficult time getting in/out of her bed. I made an appointment with her rehabilitation Dr to talk about beds. Her rehab Dr is sweet and she has a daughter with Mitochondrial disease. Her daughter is 16 and doing fairly well. I like talking to this Dr, obviously! As far as beds go, she wants to go for the Sleepsafe Bed. http://www.sleepsafebed.com/ They are nice, have many options and don't look "hospital-ish". We're going for it! The classic with Hi-Low. So hopefully by the end of Summer she will have a new carseat and bed!

She said Ava's tibias are turning in. This is caused by her hypotonia. This is one of the reasons she falls so much. Her entire foot rotates in. There's nothing we can do, aside from surgery on both legs. This problem will be watched for a year or so before we even discuss that option.

Nursing is another issue altogether. I've always heard of children waiting months inpatient while waiting for nursing. I thought that was crazy. Ha....Here we are about 5 weeks into it and we still don't have it. Most home nurses are LPNs and since Ava has a port, she needs an RN. Cincinnati Children's Home Care has had a few nurses quit recently and have no part time nurses available. Her peds office is submitting to a 2nd nursing company to see what they have. I'm honestly not that upset (Shhh!). Still not sold on the idea.

Ava has been feeling better today. She's down to 1/2 lpm and finally starting to play. The steroids have been a blessing. The lowest her glucose has been is 62. If only she could use steroids with every illness!

More Inspiration Through Art pics

2012-01-17T21:43:00.006-05:00

2012-01-16T12:40:00.002-05:00

Ava is sick. She's on 2 lpm and on steroids. Some sort of respiratory virus. The temperature just dropped for the first time this Winter, so I will blame that. For those that know prednisone, you know it caused hyperglycemia, which has been the best side effect ever for Ava!! On this steroid, while sick, her blood sugar is 62. This is amazing! Without this medication, her blood sugar would be in the 20's or 30's. A hidden blessing!

She sees rehabilitaion this week to discuss medical beds. I'm nervous about this process, but people tell me they are so worth it. :)

Dakota's endoscopy results are in. She has IBS and esophogitis. The IBS is a chronic issue that she will need to live with. We're not sure what caused the esophogitis. It's not "EE" so we're watching this.

2011-12-14T12:28:00.004-05:00

Ava did great and is now home. Her adnoids were huge and obstructing her airway so hopefully this will belp her apnea when she sleeps. Her tonsils are at a 2-3 station (of 4), so we are waiting to have them removed. She may need a supraglottoplasty as well. I don't love that idea and will mention that at her follow-up in a month. She is not in any pain from surgery. :)

We're supposed to meet with her physical therapist and DME soon to go over car seat options. Her PT doesn't feel she has enough muscle tone for a normal booster. She's recommending an adaptive carseat. Since they cost over $1000.00 we are submitting it to insurance.

Home nursing is being worked out. I really don't know how I feel about this. I have mixed feelings on this. I have heard horror stories about private nurses. I don't love the idea of someone else caring for my daughter. But, I am the only person who does any of her home care and some days I'm exhauted and have no time for my other children. I'm on the fence about it. We are asking for her to get 4-6 hrs a day and see how it goes. I think I'm more nervous about interviewing nurses that they would be.

Dakota is having an endoscopy on Jan 5th. It will be outpatient - Imagine that! They want to test her for Celiac disease. Her stomach hurts every time she eats. She's otherwise healthy and rarely gets sick. She never complains about pain until the last year with her belly pain. We've watched and waited for months and GI thinks it's time to do a scope.

All the kids are excited about Christmas and this month is flying by. I'm trying to enjoy the season as much as possible.

In the big house

2011-12-11T10:12:00.003-05:00

Ava is inpatient this week for a few procedures. She's checking in at noon on Sunday for some fluids (D10). She'll accompany Dr Cotton to the operating room on Monday for an ML&B, adnoidectomy, possible tonsilectomy and PE tubes. She'll remain inpatient until ??

Thanksgiving

2011-11-23T17:25:00.003-05:00

Ava and Dr Cotton

This year I'm thankful for a stable 3 year old. She's really been doing well. Even when she's having problems, we fix them and keep going. No admissions. It does seem like whenever she's not feeling well her blood sugar tanks. We have a plan with her endocrine doc to keep her outpatient when this happens. This year is so much better than last year as far as hospitalizations and illness.

Of course, now that I said that.....Ava will be admitted on Dec 11 for a few days. Dr Cotton wants to get another look at her airway, so ML&B it is! She's also getting tubes in her ears and adnoids removed. Her tonsils are pretty big, but he wants to hold off on that until he sees what her airway looks like. She will go in on the 11th, surgery on the 12th. I have to say I really love that doctor. He's is the nicest man.

We are honestly enjoying family and friends. Christian is 7 years old! What a little man he's becoming. I love him to pieces. He's crazy and so funny. :) We are all looking forward to Christmas.

2011-11-13T16:55:00.010-05:00

Her first trip to Build a Bear:)

Her first time performing surgery. She did a great job!

Some of our fellow area Mito fighters at Cinci Children's Cincinnati Walks for Kids 5K. Yes, she can walk, but she was exhausted.

Organization, or attempting it;)

What to do with all the medical supplies? This doesn't include Zevex bags or Farrell bags or Formula!

Ava up to her usual self.

Not a whole lotta new stuff going on, more of the same. My baby girl is still up to her tricks. Mitochondrial disease is a chronic disease that will last her whole life. I've been reminded that 80% of children diagnosed with Mito before their 5th birthday will NOT make it through puberty. Since Ava is only 3, I am holding onto all the hope I have that a cure (or reliable treatment option) will change that statistic.

Ava is still having her hypoblycemia. There have been multiple "data meetings" between her doctors and they have a list of things they want to do. Endocrine has failed to show up, which I'm told is very common for them. It ticks me off royally. Neurology and Gentics have a list.... She will be getting a continuous glucose monitor put in. This is similar to an insulin pump or IV. This will check her blood sugar over 200 times a day. The liver biopsy has been put on hold simply because I refuse invasive testing right now. Water deprivation testing still needs done, as well as a few other things.

The fresh muscle biopsy to do more extensive Mitochondrial testing (MNGIE and a few hundred others) have also been put on hold by me. Let me explain why. We know she has complex 1 due to a positive muscle biopsy that was sent to Cleveland and Baylor. There is no cure for ANY Mitochondrial diseases. None of them. If I took her to get this new biopsy, she tested positive for complex IV or MNGIE or whatever...Then what? There is no magic pill for any of these diseases. No treatment options. No trials. Ava doesn't have Leighs, so she can't do the EPI trial. I will have consented to another surgery that will fix nothing. I am biding my time, waiting for a better, less invasive test. I refuse to put her through more pain to appease my curiousity. We know she has an Electron Transport Chain deficiency and that's enough right now.

Ava is happy and mostly stable right now. She's playful and she loves life. She loves school!

2011-10-07T23:27:00.002-04:00

Ava and I will be walking (and riding) in the Cincinnati Walks for Kids for Cincinnati Children's hospital next Saturday. We will be supporting the Center for Infants and Children with Special Needs. They are Ava's primary care pediatricians. They care for the sickest kids at CCHMC. We would LOVE it if you'd visit Ava's page and leave a message. If you can, donations are most welcome too!

http://giving.cincinnatichildrens.org/NetCommunity/Page.aspx?pid=1164&frsid=38147

Summer medical update

2011-08-14T18:48:00.002-04:00

I thought it was time to do a medical update, keeping in mind that we don't know more than we know. Here's what we know right now:

We know she has a Mitochondrial disease (ETC1) and many doctors feel she has multiple Mitochondrial deficiencies, including MNGIE. Her diagnosis was made with a muscle biopsy. A liver biopsy and lumbar puncture are being planned for this Fall.

She has Eosinophilic Esophagitis/Colitis. Right now it's mild.

She has severe hypoglycemia. Her blood sugar will drop while on continuous feeds (via feeding tube/infinity pump). She does not respond to glucagon, acarbose, corn starch (or polycose). She will drop below 25 with no symptoms.

She does get acidotic and does have ketones.

Her liver is enlarged. Labs are elevated. She has a substrate defiency, but which one?

Her bowels are an issue (no big surprise). I could write so much more on this..... Dysmotility and malabsorption.

Neuropathy is a chronic problem. Her feet and legs hurt every single day. So does her head.

The valves in her heart are starting to thicken, she now sees the cardiomyopathy clinic.

Tracheomalacia/Laryngomalacia is still a concern. She still requires oxygen. She also has RAD.

Growth hormone defiency. She is -.05% for height. Yes, negative point zero fifth percentile. She was taken off Growth hormone therapy because of increases pressure in her head. On a positive, she is gaining perfectly thanks to her feeding tube!

She is being tested for Diabetes Insipidis as soon as we can think of a safe way to test her. A water deprivation test isn't a good idea for a child that can't fast. She will drink hundreds of ounces a day....and pee even more.

She has chronic fevers (is it autonomic or cyclic?) and chronic Neutrapenia. Not at the same time. She's anemic. Her amylase is high. She tested negative for SDS......

Her motor skills are status quo. She still requires AFOs. Her physical (gross motor) tests at 19-24 months. Left side is much weaker. No unassisted stairs, kicking, jumping, etc.... Very wobbly at times. Balance is an issue.

Seizures are still there. Absense seizures and possible drop seizures.

Sleeping 18-20 hrs a day (at least 2-3 days a week), worsening eyesight, GERD, temperature controlling problems (too low), and a few other things round out her struggles.

Let's face it, Mito is the cause of most of her problems. There is no cure and no real treatment. We treat the symptoms with meds like Neurontin. It's sad that my 3 year old needs these medications, but they help her feel better. She's a happy little girl, she rarely complains, and (by Mito standards) she's doing pretty good.

updates on everyone

2011-08-13T09:22:00.005-04:00

We are getting ready for school here. Lots of school shopping.:) I rarely update on all my children (biological and step!) since this blog has turned into Ava's health blog. So...here goes....

*Dakota is going into the sixth grade, her last year in elementary school. She's into cheerleading and boys. She's a total drama queen, which is exhausting.

*Christian is now a first grader. He's my wild child. Not happy unless he's outside. A real daredevil. Scares me on a daily basis.

*Skylar is a sophmore. She is 16 and almost a driver. Sometime this Winter she'll get her first car. She is devoted to church (and some of the boys there....ahem.....).

*Michael is a junior. He's the typical football-player-dating-the cheerleader and getting into all kinds of teenage mischief.

*Ellie is now a Mommy. She came home from LSU pregnant (she had a full soccer scholarship). She transferred to a local college to be closer to family. Her fiance is a young soldier who has already done one tour in Iraq, got severly injured by an IED, had hip replacement surgery and is going back to the war in a couple months. He'll be gone for at least 9 months. Baby Grayson (pictured above) was born 7 weeks early, spent some time in the NICU and is now doing great.

*Ava is looking forward to preschool. She will go 2 days a week, health permitting. She's a happy little thing.

Inspiration Through Art

2011-07-26T10:15:00.008-04:00

Ava had her Inspiration Through Art (Littlest Heros Project) photoshoot. We had schedules it 4-5 times and everytime we had to cancel due to being inpatient. It was worth the wait and the photographer was a sweetheart. Here's the sneakpeek pictures (we are waiting for the disc with all the pics).

Long time no post

2011-07-24T21:19:00.002-04:00

I took some time off blogging, but I'm back:)

Ava's medport went in just fine. With the exception of strep colonization at the port site, all is well. She doesn't love being accessed at all, but she's getting less terrified. Her Mitchondrial disease is starting to progress. Her liver is enlarged, her motility is terrible, she's acidotic, etc.... She's now on neurontin for chronic nerve pain (neuropathy).

She's also sweet, happy and spoiled rotten.

Her heart is showing some effects, mainly thickening of the valves. Cardiology wants her tested for Danon disease (Lamp-2). GI wants a liver biopsy. Neurology wants a lumbar puncture. I'm sure I don't need to tell anyone my thoughts on these tests. We are having a big appt with multiple specialties next month to get a game plan. If they all decide these tests are needed, they WILL be done at one time and they will get everything needed because they will NOT repeat these tests again.

Catch up

2011-05-06T07:45:00.028-04:00

A lot has happened since my last post. Ava has been inpatient twice in the last month for cyclic fever and hypoglycemia. She also had to fast for an echo (she's been having tachycardia), which didn't go well. She can't fast with her blood sugar plummeting. Pedialyte doesn't help, it doesn't have enough sugar in it to maintain glucose levels. We checked into cardiology already low so the cardiologist did the echo himself without sedation and she was sent to the ED for critical labs and a glucose of 38. She has to be followed by the cardiomyopothy clinic.

Fast forward 2 weeks .... She was discharged last night and will go back in on Tuesday for fluids and port placement on Wednesday. She can't fast for surgery, so fluid are needed. She doesn't have access anymore. It literally takes hours to get an iv in and it's very painful for her. They have to dig, try multiple places, etc... A port will help with this.

Dakota Brooke

2011-04-13T08:12:00.000-04:00

Happy Birthday to my beautiful, smart Dakota. What a joy you are. You are growing into an amazing young lady. A perfectly understanding sister to a medically fragile young child. A perfectly bossy sister to a younger brother. And a perfectly annoying sister to all your older siblings. The way it should be. You have been on the honor roll every quarter since you started school. You take pride in your perfect attendance (a minor miracle in this house!). My retail therapy partner! Boy crazy and a bit (a LOT) melodramatic, but we love you for that. Never a dull moment....

2011-04-08T14:10:00.000-04:00

Ava was discharged from CCHMC:) Her EKG showed sinus tachycardia and she now has to go to the cardiomyopathy clinic regularly and get echos every 6 months. She has to have an echo within the next week or so. The cardiologist explained that since she has Mitochondrial disease, she has to be followed closely by the cardiomyopathy clinic. Mito causes heart weakness (and many other heart related issues). I also found out that her last echo showed a small VSD. I didn't know about that!

She's home and still not feeling well, but hopefully she'll start perking up. Her oxygen needs have increased. Her doctors said that she handles any illness a lot worse than "typical" children. The smallest things really set her back.

We met a sweet 12 year old girl with EB from New Jersey. Childlife asked if we could show her and her Mom Ava's feeding tube. Ava is very proud of her tube and was happy to show it off. Ava was very impressed with this girl's bandages (she has EB and wears bandages all over her body) and asked her nurse for some of her own. Today the girl is getting her feeding tube put in. I hope she does well.

childrens

2011-04-06T14:56:00.000-04:00

Ava is back in the hospital. She's febrile and tachycardic. We're waiting for an ekg to see if we can find a reason for her high heart rate, which is high even when her fever is down. She's on feeds and maintenance fluids (D10 H.N. saline). Blood sugar is good, for once. Hopefully we can get her fever to stay down, her tachycardia figured out and she can go home within the next day or two. We are really trying to keep her outpatient as much as possible.

My baby is 3

2011-04-01T19:23:00.000-04:00

Yesterday Ava turned 3 years old! It's crazy to think about all she's been through since she's been born. What an amazing girl she is... (I hope you don't mind but I left out all picu photos. )

Band-aids

2011-03-02T17:14:00.000-05:00

I've been sitting in this chair holding my sleeping toddler for 2 hours. I can't help but looks down at her long eyelashes. Such long eyelashes.... For the past few days I've been really struggling. Maggie lost her battle with Mitochondrial disease. This was a huge blow. Reading about Eithene, Cooper and Samantha also fighting so hard. This is the same disease my baby has. I can't wrap my mind around this.

I check Ava's blood sugar at least 6-8 times a day. Sometimes more. She never cries. Never whines. Never whimpers. All she asks for is a band-aid. Yes, there have been many times all her fingers were covered in band-aids. She doesn't mind. She's very proud of her band-aids. She will happily tell you how she got them. "Mommy checked my blood sugar". How brave she is. How I wish a band-aid would heal her Mito!

I hate this

2011-02-24T17:12:00.000-05:00

This disease is terrible. So many children are suffering from it. Please pray for Maggie and Cooper as they fight end stage Mitochondrial disease.

2011-02-17T18:49:00.000-05:00

Ava has been getting a veeg since Tuesday. I havent seen any episodes yet. She had a fever all day yesterday, vomiting last night. Fever is gone today, but hypoglycemia is an issue today. The neurologist came in today to tell me there are times her EEG slows considerably. This is also when she has low blood glucose. This isnt usually seen with hypoglycemia. Ava is writting her own book. Endocrine thinks she has dumping syndrome. We are going to try acarbose and a few other things. Hopefully outpatient!

Neurology floor

2011-02-16T14:51:00.000-05:00

It's always interesting to see how different each floor is. We are on the neurology floor, we checked in yesterday. A few of our 6th floor respiratory therapists and nurses came to visit this morning. We love them bunches. Ava has been getting fevers today and just being sleepy. I hope it's nothing that will interfere with her stable blood glucose. I don't need anything to keep her here longer than we have to be.

Valentines Day Video

2011-02-11T14:44:00.000-05:00

Here's a sweet video Cincinnati Childrens did for Valentines Day. Ava is in it towards the end (around 3:16, I think). I love this hospital and all the small things they do to brighten the days of their patients.

2011-02-05T12:23:00.000-05:00

I've been a terrible blogger lately. I'm sorry.

Ava had a week of appointments this week. We have successfully restarted G tube feeds again. She is continuous 21 hrs a day with one mini bolus. The trick is keeping her blood glucose up and we are still walking a fine line with that.

Some of her GI biopsies came back. Ava has been diagnosed with Eosinophilic Esophagitis. She has eosinophils in her colon as well. After her special needs and gastro drs discussed it, they decided not to treat it right now. We are watching it. Our first priority is glucose levels. She's on peptamin Jr, which is a good EE formula anyway. We can't afford to lose the complex carbs.

I had a heart to heart with genetics. They fully believe there is something more than her Mitochondrial disease going on with Ava. They have voiced this several times. She is constantly being poked and proded. Her results are always "weird". I've asked them to put further testing on hold for 6 months. Ava needs a break.

The weather has been terrible, so we've been in the house as much as possible. I hope everyone is enjoying their Winter:) I am ready for Spring!!

updates

2011-01-24T20:33:00.001-05:00

It's amazing what you become gratefull for when you have a medically complex child. I was against (and terrified of!) G-tubes for over a year. I didn't think I'd be able to figure out the feeding pump, etc.....

I can say now with all honestly that it was one of the best decisions in my life to sign those surgery papers! The feeding pump? A piece of cake. She's now continuous GJ tube fed and her weight is great!

She continues to have low glucose all the time. 40-60 is her norm. Occasionally she will shoot to 300 with no real cause. This is a new problem. She also drops LOW (below 20). All these can happen on or off feeds. There's no rhyme or reason to it. She keeps me on my toes.

Her mitochondrial disease is slightly effecting her body's ability to regulate her temperature. This is new to us. I've learned that things can change so very fasy with this disease.

I've decided to put her in a toddler gymnastics class. I know she won't keep up with the other 2 year olds, but this disease can be devastating with no warning. I don't know when she'll progress, so I want her to enjoy being a toddler while she can be. Making friends! Playing! She can wear her backback with her pump in it. I will be with her the whole time. I'm so excited to see how she does!

Dress up

2011-01-21T15:08:00.000-05:00

Updates

2011-01-11T09:40:00.000-05:00

Ava spent 9 days in the hospital and is finally home. She had an MRI of her brain, which showed some scarring from past brain injury and delayed myelination. We knew about the brain injury, obviously. There are a couple concerns there with changes due to her Mitochondrial disease.

She had an endoscopy, colonoscopy and biopsies done. We're waiting for pathology results. Her doctors and I are working on a plan to keep her outpatient for longer periods of time. She's spent almost 6 wks in the hospital just in the last 3 months. She's averaging half her life at Childrens and that's not okay in my opinion. This is time I'm also away from my other children. I'm hopeful that the changes we've made will keep us home more.

Once again

2011-01-03T14:30:00.000-05:00

Ava was admitted last night for the usual problems. I'll update more when they actually tell me something....

Christmas is over

2010-12-30T13:17:00.000-05:00

Now that Christmas is over, we are so ready for Summer!!

Dakota and Christian are loving their Christmas break, playing video games all day. They are both doing great! They keep me grounded when I need to be. :)

Ava is doing ok. We're battling hypoglycemia and energy problems lately. She's going to start going to the Neurometabolic clinic. I'm hopeful that they will have some answers. Some of her symptoms are not usually seen with the mitochondrial disease she has. They are thinking she may have another Mito disease called MNGIE. She's having another MRI of her brain on the 11th of January. She's also having some increased seizures and will be getting a 3-5 day VEEG. She's always had constant retching, but we've been venting her Gtube with a Farrell bag. She remains on 24/7 Jtube feeding to maintain blood sugar levels. She takes zofran daily, which is a godsend! :)

You can tell from her picture that she really has happy days, goofy days!!

2010-12-27T15:31:00.001-05:00

We had a great Christmas here. Dakota and Christian are loving all things video game related (wii, xbox 360)... Ava is still recovering from Christmas. We've noticed that her body stresses easily. Long drives, vacations, a lot of excitement, etc... totally through her for a loop. It takes days for her to recover from anything. I assume this is Mitochondrial disease related?

The fact is that we're a little depressed right now. Our baby isn't doing the best and her longterm prognosis isn't great (as with all Mito kids). But there is plenty of hope and praying and soul searching...

2010-12-21T14:41:00.000-05:00

Over the Summer our pediatrician asked me to find Ava a new primary care Dr. She felt Ava's needs were too complex for her. This was a huge blow as we really loved and trusted her. Ava was actually the most medically complex child she's ever treated. I respect her for being honest with me. She was basically getting status updates from residents when we were in-patient with nothing to add. They were doing tests she had never heard of. She remains Dakota and Christian's pediatrician.

I spoke at length with Ava's genetic doctor about this. He had taken over all aspects of her in-patient care long before this happened. I'm told this is out of the norm for genetics doctors to do what he has done for Ava. He really is an amazing man. We sat down and discussed what we needed in a pediatrician for Ava, other than the norm.

We needed someone experienced with Mitochondrial and Metabolic diseases.
Who is closely affiliated with Cincinnati Childrens and hopefully had worked with many of the specialists closely.
Would and could handle all aspects of her healthcare and be an advocate for her.
Handle toggleing g-tube, j-tube and TPN feeds.
Could be reached 24/7 easily and quickly.

What we needed was a miracle and her genetics Dr knew who to call. Dr Levin at the The Center for Infants and Children with Special Needs. They are a practice that only treates medically complex children. There are 4 doctors and there is always someone available. They take over everything. Thankfully they overlooked the year long wait list and picked up Ava as a patient within a week. Ava fits in well with them. They have many other patients with Mito, which is also helpful.

You can click it to make it larger and read about them. Ava is on the bottom left. :)

Ava got out of the hospital on Saturday. We stll have no real answer to her severe hypoglycemia. We know it's probably partially related to her Mito, but they feel there's something else that they're missing. She doesn't respond to glucagon, polycose, acarbose or any of the other conventional methods to treat it. The only thing that will work is D12 and I obviously can't do that at home. We know it's probably liver related since she doesn't have glycose stores she should have. She remains on 24 hour J feeds with added cornstarch.

Wednesday

2010-12-15T10:35:00.000-05:00

I can't believe Christmas is 11 days away and I haven't even startes Christmas shopping. My sweetie is still in the hospital. I never leave while she's here. I miss my other children and hope Ava gets discharged soon.

Ava's stay on the endocrine/CF floor lasted less than 24 hours. Her blood sugar dropped to 26 and they got most of the needed labs. Her genetics Dr and endocrine docs are seriously ticked because the nurses failed to give Ava glucagon before a D12 bolus. Glucagon would've told us how her liver is doing or if she has a wierd varient of glycogen storage disease. They are drawing more liver function labs today. We've been discussing liver biopsies for a few months and if we don't get some answers soon she'll have to have it done.

While her glucose levels were 26 Ava had no symptoms. This is not a great finding. This means her body is used to being that low. She's obviously low more often than we realize and just not symptomatic. Not the greatest news I've heard all day.

Test results are slowly coming in. She's spilling ketones, her growth hormone remains extremely low (it should raise when she's hypoglycemic). Being on growth hormone shots should've prevented drops in her blood sugar.

They are considering trialing her on a new drug that isn't typically used in children and isn't used for hypoglycemia. It would have to be started here in the hospital since there are some bad side effects. Her special needs doctors have never used it on a patient so this will be uncharted waters for them too. There's a small chance that it might help her. We shall see....

Ava has been asleep for the last 15 hours and will probably sleep until this afternoon. She's having a Mito kind of day.

Saturday

2010-12-11T15:59:00.000-05:00

Ava had a picc line put yesterday. She's lost all peripheral iv access and we were told the chances of getting another picc in after this is very low. They tried for 2 hours to get the picc in. She has terrible picc access too, this was a blow. If we can't keep her stable then she'll need a central line.

They moved her up to the endocrine/CF floor today. They want to fast her and when her blood sugar drops then do a lot of labs. They are going to look at everything and try to pinpoint a reason for her hypoglycemia. We are weaning down J tube feeds by 10mls/30 minutes until she's on nothing. I'm curious to see what the results are.

square one

2010-12-08T11:50:00.001-05:00

Were still here at childrens. Ava has everyone at a real loss, she always has. She was admitted with blood sugar at 32 on Monday. Yesterday she became febrile with head and back pain. Blood sugar is everywhere. Today she has no fever, head /back pain continue. The doctors feel terrible with over a year of no real answers and worsening symptoms. GI is being brought in again. On Friday the head of neurology (who specializes in neurometabolic diseases) and her genetics dr are having "clinic" with endocrine in Avas room. Her growth hormone injections have been stopped due to brain pressure problems. I'm hoping to take her home over the weekend and keep her outpatient through the holidays.

hospital

2010-12-06T19:15:00.000-05:00

ava is back in the hospital with crashing glucose again. I hope this is a fast admission.

Ava's new ride

2010-11-22T15:19:00.000-05:00

Today was the first day of speech therapy, which went well. It is such a beautiful day, so I snapped some pictures outside of her new wheelchair.

I'm still waiting for the oxygen tank holder and IV pole attachment, but the chair is really nice. Very easy to push. Ava is learning how to make herself go.

Oh...and of course it matches her glasses and braces (not pictured). :)

Hospital pictures

2010-11-15T08:07:00.000-05:00

Just to show you that being in the hospital for 3 wks isn't all bad for her.
(taken by my cell camera)

Painting:

Playing in a box:

Temperature taking:

Fishing:

Walks:

Wagon rides:

And whatever else:

Most importantly, making new friends!

our newest normal

2010-11-13T18:45:00.000-05:00

I'm sure most parents of medically complex kids know that their "normal" is always changing. Always. We are home! Ava is on a hectic schedule of meds and glucose checks, and injections, and more meds and breathing treatments and................... I am just keeping up. This is our new normal - for now. I'll post some more pictures in the coming days. This is right before we left the hospital. Ava and Derby (Ava's new wheelchair is in the background).

missing home

2010-11-11T22:48:00.000-05:00

Ava is still inpatient and and has been having a grand ol' time here. She got her wheelchair today (pics coming!). She's meeting new friends and is turning into a very social girl. She'll even hug a couple of her nurses! She and her friend Derby have become the girls of A6 south! They've been neighbors for a couple weeks now.

I'm glad Ava is handling this admission well, but I'm not. I miss my other children so much. I want to go home.

We're trying to decide if we can handle Ava's GI problems outpatient or not. She's having some malabsorption problems. I've been doing her hormone shots nightly without any problems. Her blood glucose has been stable for a few days. She hasn't needed any oxygen in days, which is a great surprise to us all:)

A long day

2010-11-04T21:04:00.001-04:00

Ava had her GJ tube surgery today. The reason the switch was done in surgery under GA is because she had a peg tube with an enternal disc. She didn't have a balloon style gtube. The surgery actually took over an hour because they couldn't get the peg tube out and they pulled several times very hard. In the end, they had to pull it through her mouth to get it out. She still has a long tube, so still no Mic Key button.

We're currently waiting for the I.V. Team to come and put in another line since her 4th one infiltrated today. The port keeps looking like a better idea everyday.

surgery

2010-11-03T20:20:00.001-04:00

Tomorrow Ava will get her peg tube taken out and get a GJ tube placed. This tube gets placed in her intestines and bypasses her stomach. The GJ tube should get rid of the daily retching. She started COQ10 and carnitore for her mito disease a few days ago and is doing well with them. Ava met a new friend and fellow airway baby, who she's been playing with. They are neighbors and take turns walking by each others rooms;) They are having playdates in the activity room - super cute!

updates

2010-11-01T11:51:00.001-04:00

It is hard to update because I don't have my laptop here with me so I have to use my phone. Ava crashed to 30 while on feeds on Friday. We are trying to find a formula with the most carbs to maintain her glucose levels without as much volume going through her gtube because she isn't tolerating her feeds. If this doesn't work, she will have her gtube replaced with a gj tube. Her hypoglycemia is getting worse over time. Nobody has answers. All I know is that I don't like seeing Ava turn gray and become unresponsive.

Inpatient

2010-10-28T13:23:00.001-04:00

Ava was rushed to trauma upon arrival with a blood glucose of 30. After being stabilized she was admitted yesterday. Her ped and I had a long talk about her mitochondrial disease today and we can't put her on the ketogenic diet because she needs too many carbs to maintain her blood glucose.Carnitine might be started. There is a bunch more that I will update on when I can.They are testing for pancreatitis today.

2010-10-27T08:17:00.000-04:00

Ava is getting sick again. She woke up this morning throwing up and chilling. Poor baby. I don't know whether to start her on a slow pedialyte drip through her gtube or not. I'm going to call her doctor and hope she can remain outpatient.

This picture was taken yesterday during the rare time she was awake. I should've seen this one coming.

Muscle biopsy results

2010-10-20T17:03:00.001-04:00

We got some of the muscle biopsy results back from Cleveland. Ava has Mitochondrial complex 1. We'll know more and have an official "results appointment" in about a month when the rest of the tests come back. We're now getting some answers as to what is going on with our little girl. There is no cure for this. I need to learn more about this disease, but from what I've read this explains so much about Ava.

Sweet Little Lamb

2010-10-17T13:18:00.000-04:00

Freshly rested from my blogging hiatus, I'd like to introduce you to my sweet lamb. Isn't she sweet?

Our new lawn boy found her.

(He's a sweetie too!)

She's still hospital free! The change of weather is effecting her airway, so she's on her oxygen about 60% of the time. Prednisone, Xopenex and pulmocort are back for the winter. Love the steroid cheeks:) We've also added protonix and miralax to her meds. Her HG is a super low 7.9, so we've upped her iron. ANC is holding steady, Yay!!!!

Feedings are going slowly. Her goal is 50/hr 20 hrs a day. She's holding steady at 30/hr. If we up it, her Farrell valve quickly fills. We have a few more "hiccups", but we're managing them at home with the help of Ava's new pediatrician (another post!)...

We're learning how to conserve Ava's energy for the times she really needs it. She's very low energy and would love to lay around most of the time, even to play. I'm sure her hemoglobin has something to do with this!

I decided last night to make g-tube pads. These are my first attemps:

Ha! I really hope practice makes perfect.

Status quo

2010-10-04T19:14:00.000-04:00

We've been balancing our life, or trying to. It's Fall, beautiful weather. Ava is home and doing well. Do we take advantage of this time and do family activities that have been on hold? Museums. Pumpkin patch. Park. Football games (My sister is a college cheerleader). Etc... Life with an immuno-suppressed child can be difficult.

Christian is doing great in school. He's playing soccer. I LOVE having a little boy! He gets to be in the Homecoming parade tomorrow.

Dakota is my typical GATE child. She tested into the gifted program and has flown since. She amazes
all of us.

Have you gotton your child a flu shot this year yet? Why or why not?

Home again

2010-09-23T21:34:00.001-04:00

We are home again after almost 2 weeks and an emergency surgery.

We went from going to the Cancer day at the zoo:

To a very dangerous situation:

in 12 hours. Thank God for amazing doctors and surgeons!

Good news

2010-09-20T21:16:00.001-04:00

Ava tolerated 10 ml/hr through her g-tube today with no leaking. She's still on TPN and lipids through her picc. The Dr ordered an O2 sat study tonight while she's healthy (from a respiratory standpoint) and feeling well. This will give them a good baseline to compare to. Her fear of nurses is much better now. She helps them do her vitals and even tries to do picc and gtube care. IV iron is being discussed again as an option in the future.

Dare I mention the "H" word for sometime this week? I hope so!!

2010-09-19T19:14:00.000-04:00

Ava has been on IV antibiotics for 8 days and remains on TPN. There isn't any changes since my last update. Tomorrow we will try feeds again through her g-tube and see what happens. She is now allowed in the big girl play room instead of the infant/toddler room. Much fun:) :)

Still in-patient

2010-09-16T20:59:00.000-04:00

Ava is now on TPN and doing okay with that. I spoke with her surgeon today and we will try to feed through her g-tube again on Monday. If this doesn't work we will have to talk about more surgery. Hopefully her belly is healed by Monday! I don't want to think about 3 surgeries and a picc placement in less than 3 weeks for Ava.

So...we're in the hospital for a while longer and trying to make the best of it. She's been through so much in the last year alone and still has a smile. Her newest thing is having the colored wrap around her picc line changed to different colors as often as she can. Fun times. Ha!

picc line

2010-09-15T11:12:00.000-04:00

Ava has no iv access and needs some nutrition. For both these reasons she'll have a picc line put in today. TPN will be started too. Other than that, there's been no changes. Thankfully this floor has a free parent laundry room. :) We're hanging in there.

Monday

2010-09-13T18:54:00.000-04:00

Ava is feeling much better today. They tried to start slow feeds today at 5 pm, but it was coming right out through the stoma. She needs to heal for a couple more days before we try to introduce feeds again. We are going for lots of wagon rides and watching lots of Dora.

unexpected complications

2010-09-12T18:00:00.000-04:00

Ava was admitted last night and had surgery this morning. The disc was too tight on her g tube, causing restricted blood flow and death to the wall of her stomach. The internal disc migrated out of the dead stomach tissue and into the abdominal wall. She has an infection from this also. Surgery this morning was successful in saving most of the stoma and repairing the damage. She's on a few different antibiotics and is on GI rest. Nothing in the belly for a couple days to help healing.

Ironically, yesterday was the cancer survivors' picnic at the zoo. We went for a while. Ava was very tender and started running a fever of 103, so we left. I called the GI Dr on call and took her in. I had no idea that Ava was so sick at that time.

I don't know how long she'll be in patient, but she's doing well right now. She's trying to play while laying down. Progress.

Snapshot Saturday

2010-09-05T18:29:00.001-04:00

recovering

2010-09-01T20:59:00.000-04:00

Surgery is over and she's on the GI floor recovering. Her gtube and muscle biopsy sites look good. She had some pre-op hypoglycemia that required some fast action. She's on morphine every 2 hours for pain. They are going to start her on continuous feeding 24 hrs a day for a month, which I wasn't expecting. Thankfully she wears her backpack with no problems.

That's about it tonight. She's doing well, so I'm happy.

2010-08-30T20:03:00.000-04:00

Tomorrow my little boy starts Kindergarden. When did he get so big? I know tomorrow morning will be filled with picture taking and tears (mine!).

Neuroblastoma can be hereditary. It's rare, but happens. Ava has some symptoms of mature NB and will have some work-ups done later this week while she's in-patient for surgery. Catacholamines will be tested. Her CT scan was reassuring, as it only showed some lung issues. Do I think she has NB? No, I do not. It's better to be safe, though.

2010-08-27T18:07:00.001-04:00

I know I've been MIA lately. Things are busy with back to school for Dakota and Christian. Ava's appointment schedule is tiring. Today was hemotology clinic. This specific doctor was one of the doctors that cared for me when I was Ava's age with Neuroblastoma. She's treating Ava for her ANC counts and amemia. She's considering IV iron for Ava. We don't know what's causing the neutopenia. If it's Mitochondrial disorder related the treatment will be tricky. A bone marrow aspiration will be done the next time her ANC drops below 500. We need to know if the problem is her bone marrow or not.

Ava isn't feeling great. G tube and muscle biopsy surgery is Wednesday. She's sleeping way more than she should. She plays for a few minutes, then wants held for an hour. 4-6 hour naps everyday combined with smaller "cat naps". She also sleeps 12+ hours at night.

I'm trying to keep caught up in blogland, but it's difficult right now. I hope I'm not hurting anyone's feelings. Everyone is in my thoughts.

I Love You too, Mom

2010-08-23T16:21:00.001-04:00

Photo and video editing at www.OneTrueMedia.com

2010-08-07T21:45:00.001-04:00

We are home again. She's adjusting to life with an ng tube as well as any toddler can. We are using a kangaroo joey pump - super easy to use. She also has a backpack to use. She'll keep this tube until the 1st of September. Her ANC was 610 this morning, so she's still immune suppressed and will use masks in public until she doubles that number. Weekly labs are now a part of our schedule as well as a few new doctors to add to the list. Hemotology and Immunology.

Cincinnati Childrens has been amazing. Ava has them all baffled. Something is wrong with her and they are doing everything to find out what. Her CT scans showed no masses.

Ava has her own Dr House. (He looks just like him in person. haha)
This is one of the genetics doctors working with Ava. Please excuse the fit that she's throwing.

Trying to flush her own tube.

2010-08-05T13:45:00.000-04:00

Today has been hectic. Her NG tube was placed earlier and she will keep this tube until her g-tube surgery on the 1st. Her counts are rising a bit, but still remain low (ANC was 250 late last night), so she's still in isolation. Her fever has been gone for almost 48 hours. Things are improving, just very slowly.

Her surgery on Sept 1st will consist of g-tube placement, muscle biopsy, bone marrow biopsy, and GI biopsies.

All the different specialties have ordered labs and as of this morning there were over 30 blood draw orders. Ava is anemic (RBC is 8), so she cannot have this much blood taken. They are prioritizing the orders.

Thank you for all the T&P's. They are helping her.

All the best doctors

2010-08-04T18:00:00.000-04:00

It's been a whirlwind the past couple days. A normal absolute neutrophil count (ANC) is between 1,500 - 7,000. Neutrophils are the body's defense against infections. ANC below 500 is very serious due to the risks of infection. Ava's ANC is 124. She has to wear a mask when she goes for testing. Nobody is even allowed in the same elevator as her, besides her nurse and me. Gowns and masks are obviously required in her room.

We have the best doctors trying to figure out what's wrong with her. Everyday the following specialties see her:

general pediatrics
Genetics
Rheumatology
Infectious Disease
Gastrointerology
Immunology
Hemotology
Dermotology

It's been crazy busy. The good news in all this is that her airway is handling it perfectly! No oxygen, thankyouverymuch:)

A new day, a different plan

2010-08-04T10:02:00.000-04:00

The plan for today was to get a lumbar puncture and picc line done, and start TPN. That's not happening anymore. Her white count keeps dropping and is now 1.6.It's supposed to be 4500. Her ANC is extremely low also. She's at a high risk for infections and is now in isolation. Hematology is now getting involved. Lumbar puncture and picc were canceled. She's now having liver and pancreas ultrasounds today at 1:30. That's about all I know right now.

unchanged

2010-08-03T11:11:00.001-04:00

Today starts a battery of new testing with the focus on her immune system. She's still very sleepy. Not much has changed since yesterday other than calling in Rheumatology. Genetics and infectious disease have been on board. She's looking puffy and miserable.

Yesterday Christian had his very first soccer practice, which I missed. I really hate that.

Back in the hospital

2010-08-02T10:13:00.000-04:00

My girl was admitted again last night. Fever of 105, very high heartrate, dysmotility, hypoglycemia, low blood counts, etc... They are worried about blood infections, so IV antibiotics were started. There is some discussion of a possible blood transfusion. This morning her fever is down thanks to motrin/tylenol and she's sleeping most of the time. She feels terrible.

I'll try to update the blog, but the internet here is unpredictable.

hypoglycemia

2010-07-30T14:44:00.000-04:00

We just got home from another stay at Childrens. Blood glucose levels were 41, so she was admitted for a few days. Genetics say this how how Mito effects some children - dysmotility and hypoglycemia. She's having the big muscle biopsy at the same time as her gtube placement. We'll find out more when the results of that biopsy are back in a few months (it takes 2+ months to get results!)

We're back

2010-07-26T12:13:00.000-04:00

We had a good time in the OBX. It's such a pretty area. The kids had so much fun playing on the beach everyday. Ava isn't a beach gal yet. She was terrified of the sand. Really. Terrified. I went to the store and bought her a baby pool, put it on the deck and enjoyed the view with a happy toddler. We needed that. I'll post pictures in a day or two.

G-tube surgery is September 1st. In the meantime, we are using periactin to induce hunger. Appts start back up tomorrow. I'll find out on Wednesday if a thigh muscle biopsy will be added to the list on Sept 1st.

vacation

2010-07-15T18:23:00.001-04:00

We saw GI today and he's on board with the other doctors on getting the ball rolling with the G-tube. She'll get a PEG tube at first, then go to a low profile (AMT) button 12 wks after PEG placement. She'll stay in patient for 4 days or so. I'll let everyone know when I have the date, but it will be within the next month. Her liver enzymes have been elevated and continue to stay that way. This is something concerning. What is causing this? This means more GI visits.

Good news is that her SMA test is negative. And we leave for the Outer Banks on Saturday for a last minute vacation before her next surgery. We need this and are stoked!!

Park pictures

2010-07-06T18:22:00.002-04:00

We spent some time at the park yesterday. Here's some pics:)

Confusing

2010-07-02T20:24:00.000-04:00

I'm sorry if this gets confusing:

Since Ava's initial surgeon consultation for a g-tube was referred by her pulmonologist, it's taken a little longer to have everything going. We met with the surgeon, who agrees with the pulmonary doc. Ava's GI Dr (who will assist on the PEG tube placement) would like to see her before taking her to the OR. I agree! He probably feels like he's out of the loop (or pissed!) since Pulmonary, Neurology, Surgeon, Genetics, and Rehabilitation have consulted each other about this.

This GI doc is part of the Feeding Team that Ava sees. This is part of Dr C's Aero team that specializes in airway kiddos. They are always packed and hard to get an appt in a timely manner. Ava's appt is the 15th. He usually sees 9 patients in clinic a day, but Ava makes number 16 as an addon. It will be a long day!

The nurse told be to expect PEG placement in early August.

Other than that, everyone is doing well. I hope everyone is having a great Summer!

Neurology

2010-06-22T18:09:00.000-04:00

Ava saw her neurology doctor today. We discussed everything going on. She wants Ava to be tested for SMA (Spinal Muscular Atrophy) before we do a muscle biopsy for Mito disease. She also wants a few other tests done, and honestly she lost me after she mentioned SMA. That threw me for a loop. She's calling Ava's Genetics Drs and rehab doctor to get them on board. She wants a "master list" of labs and tests they all want to do, so that we don't have to keep bouncing all over with 10 different ideas from 10 different doctors. They will have a plan. She's supposed to call me Thursday and let me know this plan.

Other than that, we've been trying to enjoy the outdoors as much as possible. Not as many germs:)

I'm done

2010-06-16T10:57:00.000-04:00

In less than a week I've had my 2 year old casted for larger leg braces, measured for her wheelchair, ordered the wheelchair, was told she has to stay on oxygen , wants to trial her on Bipap next time she's in the hospital, and is being referred to a general surgeon for her g-tube.

I'm done.

I love her team of doctors at Cinci Childrens. They're the best of the best. But I'm annoyed with them right now. My plan was to go to her Aero appt yesterday, be told her sleep study was great, her weight was picking up and she could come off O2 and bypass more surgery.

That didn't happen.

They took her vitals. She lost a couple ounces. She was sating at 85% . Not the best start, right?

She's not oxygenating well. They feel more airway surgery will do more harm than good. Next time she's sick in the hospital they will get a blood gas to get Bipap approved. They said when she's sick, she breathes very shallow and her co2 levels aren't where they should be. This will hopefully be a "use when sick" piece of equipment.

As far as the dreaded g-tube. Its no secret I've been fighting this for a year. They are now taking it to their roundtable meeting this week and discussing referring her to the general surgeon. I'm been told to expect it to happen.

I'm done.

Mobility

2010-06-10T19:05:00.000-04:00

This week was dedicated to Ava's mobility needs. Today we met with 2 doctors, 3 therapists and the vender at the seating clinic to pick out my daughter's first wheelchair. Hot pink, of course. Last night she was casted for her new DAFOs. I'd be lying if I said I wasn't disappointed. Can she walk? Yes. She never has much energy. She gets so shaky. The Dr says that is Mito disease. There's a good chance she'll be started on Coenzyme Q10 soon. I hope that helps her.

Good News

2010-05-28T18:03:00.000-04:00

I have some good news :)
Dakota is finishing 4th grade with straight A's and she tested into the GATE program for next year. I'm giving her the choice on that since she would take seperate classes from all her friends. I'm very proud of her!

While Ava does need more reconstruction on her foot, we can wait a few months to even schedule it! We will go back and talk about it in December! Yay!

Some answers

2010-05-26T18:39:00.002-04:00

When Ava was in-house last month, Genetics did some preliminary Mitochondrial workups. They even sent some blood to the Mayo Clinic. Some of these tests have given up some answers. It looks like Ava has a Mitochondrial disorder (the Dr is 99% positive). They have ordered a muscle biopsy to be performed this Summer to help pinpoint which disorder she has. Some of these Mito disorders are degenerative, some are not.

She is also back down to 22 pounds and talks of a g-tube are back on the table. Other than that, She's doing well. She's a goofy little thing and is happy most of the time. And oh, so stubborn!

Sleep study

2010-05-24T16:12:00.000-04:00

Ava had another sleep study, which could tell us so much. Does she breathe well enough to wean night O2? Does she need CPAP or BiPAP? More surgery? Can you tell I'm nervous? It doesn't help when the Aerodigestive nurse calls today and says "Dr B definitely needs to see her." Dr B is her pulmonologist. She will see him and Dr. ENT in 2 weeks in a shared clinic appt. Other important appts are coming up too. Genetics (on Wednesday), Orthopaedic surgeon (on Friday) and her wheelchair fitting appt (2 wks).

Christian and I have been having much fun lately since he's already on Summer break. He, Ava, and I have been spending most of our free time at home in our yard playing and planting flowers.

Ava's new blankie!

2010-05-13T18:35:00.000-04:00

I'm sorry I'm out of the loop this week. Ava is sick again and we're trying (and failing) to avoid the hospital again. This Spring is being so rough on her, worse than Winter. My girl is struggling lately. I did want to thank Lacey at Jaxson's Blankies For Babes for her beautiful blankie they made her. It's precious. Ava loves it!

I do have good news, her echo didn't show any heart defects! That's such a relief. She took her new blankie with her to get the echo, so maybe it's good luck!!

I

Wordless Wednesday .... almost

2010-05-12T09:42:00.000-04:00

Christian picked these and surprised me with them. The best flowers ever.

Blooms

2010-05-03T15:59:00.000-04:00

How to balance

2010-05-02T13:39:00.000-04:00

I may have spoken prematurely about getting Ava out to enjoy the Spring weather. After being in the hospital last week and now having a hard time with croup. My daughter's body just isn't ready yet. She's retracting, DSATing, and has crazy stridor between bark-like coughs. I'm sad for her and for the older kids. How do I find the balance? How do I pack Ava up and take them somewhere, knowing she'll likely get sick from it? How do I keep them home, knowing their missing out on fun? Either way I feel guilty. They are such great siblings to Ava, very understanding and patient. How long will that last?

Happily home

2010-04-26T20:57:00.001-04:00

Ava spent the weekend in-patient again. We spent some time with Endocrinology and Genetics. Genetics actually spent anout 90 minutes talking with me today. Hypoglycemia and blood glucose checks every 2 hrs left her miserable.

They are now worried that Ava has a Mitochondria disorder due to so many different health problems. It was explained to me that Mito is more widespread than a metabolic disease, which fits Ava. They took blood and sent it to the Mayo clinic for some initial testing. She will also get a muscle biopsy. They said it's a large, invasive biopsy that will require surgery and another hospital stay. A sedated Echo is in the near future, sleep study, as well as meeting the folks in the Neuromuscular clinic. Just what we needed is more doctors, right? They are the doctors that will order her muscle biopsy.

I'm done with the negative update.

Guess what you get when a 2 year old has 2 bags of dextrose bolused in a short time? Give up? A hyper toddler that feels much better. :) Here's proof!

Make an on-line slideshow at www.OneTrueMedia.com

Busy week

2010-04-22T19:25:00.000-04:00

I love Spring. Love. It. This year we are enjoying the sunshine. Ava hasn't had a real chance to play outside and enjoy the outdoors. This year I hope to introduce her to nature. I don't care how much equipment we have to lug around with us. Ava will feel the breeze on her little face this year!

Dakota turned 10 last week. I can't believe my girl is 10! How crazy is that? I love this girl more than I could ever say.

We've added two new people to Ava's healthcare team. A new PT (her usual PT is pregnant via IVF with twins and on bedrest!) and a new rehabilitation Dr, who I really like. She works closely with all the other doctors, but looks at "the big picture" instead of one small part of Ava's problems. She puts everything together. She feels Ava needs to be tested for Mitochondrial disorders and Neuromuscular disorders. (Infectious disease has already started testing for the neuromuscular disorders also). She said she's almost 100% certain we will get a definite diagnosis within those two disorder catagories. Ava will have to get a muscle biopsy to get tested for the Mito disorders and I'm not in a huge hurry for that since she also has some subglottic stenosis (narrowing in her throat).

Ava has a wheelchair clinic appointment with the vendor to actually be fitted and pick out what will work for her on June 10th. I've been told that the wheelchair process is very slow and she won't actually get her wheelchair for probably 6 months.

Party pictures

2010-04-12T09:31:00.001-04:00

Here's some pictures from yesterday's party. I'll add more when I go through the rest. It turned out to be a beautiful day.

(If only I could get her to eat the cupcake!)

learning to walk again

2010-04-02T19:24:00.000-04:00

Ava is learning to walk all over again. She's doing very well, don't you think?

Photo and video editing at www.OneTrueMedia.com

Today was her 2 year check up with her pediatrician. We are so blessed to have this doctor on our team. She's simply amazing and we love her.

I hope everyone has a great Easter. It's my favorite holiday ever!

Happy Birthday, sweet girl

2010-03-31T09:26:00.001-04:00

A two year old is so many things.

A tiny discoverer of butterfly wings.

A hugger of teddies, a sweet sleepy head.

And someone to dream for in bright years ahead.

A special new person who right from the start,

has a place in the family and in your heart.

And just when you think you've learned all the things,

that your dear daughter is,and the joy that she brings,

a hug or a grin comes with such a sweet surprise,

that love finds you smiling with tears in your eyes.

I love you so much!

cast removal

2010-03-30T21:06:00.000-04:00

Ava had her cast removed yesterday, finally! The surgeon isn't thrilled with how the actual toes are laying so she goes back in 2 months to talk about more reconstruction surgery. The Dr referred Ava to the seating clinic to look at wheelchair options. Here's some pictures of our day at the orthopeadic surgeon's office. :)

not knowing

2010-03-16T15:26:00.001-04:00

I haven't done a healthy summary lately because it seems whenever we get answers, we always end up with more questions. Ava sees Infectious Disease on Thursday for her cyclic fevers/connective tissue disorder. She also battles her usual things like a metabolic disorder, chronic Neutropenia, anemia, hypoglycemia, OSA, GERD, central apnea, breath holding spells, cyanosis, seizures (lessening!), FTT, temors, CP, laryngomalacia, tracheomalacia, hypotonia, wierd rashes, developmental delay, macrocephaly, etc... The newest is subglottic stenosis from her multiple intubations, which is being watched. She's been intubated 11 times that I can remember off the top of my head. She has at least two more surgeries in the future (foot and airway). We now need to watch the SGS and weigh pros/cons for upcoming procedures.

I've heard so many possibilities.... I'd love for some answers. I'd love it! But would a diagnosis change anything that I'm doing? No, it wouldn't. She'd still be my sweet, beautiful, perfect in every way, little girl. She'd still be the goofy baby that loves to laugh at her big brother. The same toddler who loves to borrow (ahem..take) her sister's Nintendo DS.

I'm really struggling to find a happy medium. I want to know what's wrong with my child. I want to know there is nothing else wrong with her that we don't know about. I don't want any more little diagnosises. Will it change anything to know? No. But I do feel like I'm waiting for the other shoe to drop always.

What I want to know is if the above things are the actual diagnosises or are they the symptoms of something much bigger (and meaner)?

No words

2010-03-07T17:59:00.000-05:00

Books

2010-03-01T12:10:00.000-05:00

Ava loves books. She loves being read to. Whenever she's stressed, sick, or upset reading to her calmes her. I read to her so much when she was on the vent in the PICU, I just couldn't stop. Her heartrate was even better when I was reading. She would try to breathe over the vent more often, letting us know she was getting stronger. I love the time she and I have when I read to her. Love it.

I have some sweet memories of Ava (and all my children) and books.

Recovering from this last surgery is no exception. We've read a lot of books in the last few days.

She's doing much better, less pain.

The year of scooting on her bottom has paid off. She's figured out how to use her good leg and scoot herself sideways all over the place. She looks like a crab. They are resilient, aren't they? Her cast comes off on March 29th. Two days before her birthday. :)

Another surgery down

2010-02-24T21:43:00.000-05:00

After 36 hrs on the hospital, we are home. She has an incision down her entire foot and had 4 bones and a toe removed. She also had some reconstruction done. She's in a lot of pain. Her breathing isn't great. I have to reposition her every 2 hours to prevent skin breakdown, which sucks. She is not allowed to bear weight for 4-6 weeks. She's not in a walking cast, obviously. Her leg swelled and she had to be recasted today. This could happen a couple times in the next month. Hopefully she'll be in less pain soon. All she does is cry "ow". She's breaking my heart.

surgery

2010-02-22T11:11:00.000-05:00

Tomorrow is surgery day. Ava will have her complex polysyndactyly removed as well as extra metatarsal. She is having 3-4 bones removed from her left foot. She'll be in-house for who knows how long (since she's an airway baby) and in a cast for a month. Please pray her airway handles the intubation without swelling too much and pain meds don't cause breathing problems. No PICU, please! Here's an xray of her feet. If you can read xrays, you'll see the bones needing removed.

Feeding appt update

2010-02-19T12:21:00.001-05:00

Ava weighed in at 20 pounds yesterday, so she's still losing weight. Her growth chart is plunging and everyone is concerned. She isn't getting taller either. Her entire growth is stunted right now. We only saw ENT and nutrition, but I think they had some great ideas. We are really trying to avoid a g-tube. They think this might be possible because Ava does take bottles. If we can get enough calories through her bottle.... We're trialing Boost 1.5 and Boost Breeze. They put her on multi-vitamins and iron. Also doing things like adding whipping cream to all her bottles, which is 50 calories per tablespoon. High calorie formula is another thing we're doing. If she does lose any more weight we'll have to try an NG tube while she recovers from surgery and go from there. They think this is directly related to whatever metabolic disorder she has. She'll be watched more closely by them now. She'll be scoped again to check her airway in late Spring.

Things are a changin'

2010-02-16T12:21:00.000-05:00

I'm annoyed today. I don't think some people realize how difficult it is to get GI, Genetics, ENT, a nutritionist and a speech pathology at the same appointment, at the same time. It takes a while. This was to be a very important appt. We've gotten so much snow the last 48 yrs that the roads weren't clear. Wrecks everywhere. Obviously this appointment wasn't happening today. I called this morning to reschedule, they said they were already into June for just GI and Nutritionist alone but they wanted to get her in sooner. I got a call back 5 minutes later with an appt with ENT and the nutritionist this Thursday. (insert cringing here) They will get the Full Team appt much sooner for us than the receptionist. This ENT that Ava is seeing is the same one that did her FEES test and is the director of the Feeding Team. (Not Dr Cotton, who does her airway surgeries.) I'm almost certain with Ava's weight loss and metabolic issues he's going to schedule another scope. I was told there was a very high chance she'd need more airway surgery, but I hope to avoid it as long as possible. On a positive note, we won't have to worry about seeing GI and the possibility of a G-tube until the full team appt, right? More time to try to bulk this baby up! The silver lining:) :)

This time next week Ava and I will be in house again at Childrens recovering from more surgery.

rambling

2010-02-11T21:28:00.001-05:00

This post might be all over the place tonight.
Christian had his sleep study on Sunday night. I don't know the results of that yet, but he did very well. I honestly don't foresee any bad results from it. Just precautionary due to his little sister's health problems.

The next 2 weeks are going to be stressful. Ava has her GI appt, Genetics appt, and Feeding Team appt next week. Add her usual therapies and surgery the following week. Busy, Busy, Busy. I weighed her today and she's at 22 pounds, 3 ounces. Not the best news...

Dakota and I decided to see what Ava's hair looks like curly, so we braided it tonight after bathtime. I'll have to take some pictures tomorrow. ;)

The girls decided to play with sparklies tonight and Christian felt left out. haha Blackmail pictures!

That's my boy!

glowy toes

Can I just say that I'm loving these numbers! I rarely see that 3 digit number at the top so I'm thrilled to post a picture of it. She's been doing much better keeping her sats up while awake without her oxygen on. I hope we can wean her off it while she's awake and only use it at night. Just might be possible after this next surgery!

Mom! Ava needs help!

2010-02-05T16:14:00.001-05:00

Not the words I want to hear while folding laundry this morning. I run into the living room to find that indeed, Ava needed help clearing her airway. Ava has another virus and needed her airway suctioned. She can't cough hard enough to clear it herself. This usually involves laying her flat on her back, tilting her head back to open the airway and suctioning through the mouth into the throat. I will also suction her nose This has to be done fast, as suctioning can remove all the gases from the lungs. I have a depth which I will not go past when using the actual long, thin catheter. I asked the Respiratory Therapist to actually mark the "stop point" on the tube. I'm always terrified of going in too deep and damaging something. Ava is usually freaking out when I have to suction her like this. She's not able to breathe due to the plug (or secretions in her throat) and will panic. These are the things I use on Ava. The first two are for her mouth and throat. The last picture picture are nasal suckers.

I don't know how mother (and fathers!) of children with tracheostomies suction hourly like it's second nature. It makes me a nervous wreck to deep suction her. It always has and I've been doing it for over a year. I guess it's like everything else, you do what you have to for your children.

I often wonder how Dakota and Christian feel about all this. They both know too much about these things. They have both been amazing helpers. They've both missed out on various things because of their little sister's health. I won't lie, I feel so guilty for it. I try very hard to make up for it. I don't want them to look back on their childhoods and only remember Ava's medical problems. I'm terrified they'll resent her (or me). I want them all to have fun, happy, carefree childhoods. I wonder if they can have that while yelling for me to "Help Ava!" or visiting their sister in the hospital again? I hope to find a happy medium somewhere.

Oh boy

2010-02-02T18:33:00.007-05:00

I know some of you wonder why I don't post a lot of pictures of Christian. I couldn't tell you.;)

Taking A Deep Breath

wednesday update

In the hospital

Going private

DME and rehab

More Inspiration Through Art pics

In the big house

Thanksgiving

Summer medical update

updates on everyone

Inspiration Through Art

Long time no post

Catch up

Dakota Brooke

childrens

My baby is 3

Band-aids

I hate this

Neurology floor

Valentines Day Video

updates

Dress up

Updates

Once again

Christmas is over

Wednesday

Saturday

square one

hospital

Ava's new ride

Hospital pictures

our newest normal

missing home

More answers

A long day

surgery

updates

Inpatient

Muscle biopsy results

Sweet Little Lamb

Status quo

Home again

Good news

Still in-patient

picc line

Monday

unexpected complications

Snapshot Saturday

recovering

I Love You too, Mom

All the best doctors

A new day, a different plan

unchanged

Back in the hospital

hypoglycemia

We're back

vacation

Park pictures

Confusing

Neurology

I'm done

Mobility

Good News

Some answers

Sleep study

Ava's new blankie!

Wordless Wednesday .... almost

Blooms

How to balance

Happily home

Busy week

Party pictures

learning to walk again

Happy Birthday, sweet girl

cast removal

not knowing

No words

Books

Another surgery down

surgery