About our blogger: Hope
My name is Hope. I have amazing children that I'm lucky enough to spend my days with. I'm also a molecular biology major, which I really love. I'm a Neuroblastoma Stage IV survivor still dealing with the effects of that not only with myself, but my children as well. My youngest daughter is medically fragile. She has a metabolic disorder, airway problems, and many more issues. My blog has turned into a way to chronicle these problems, but I do pray for a day that she has no medical problems to write about! Welcome to my life!
March 31st, 2012
Ava finally got discharged yesterday. We learned what Red Man Syndrome is and that was interesting. It took a few tries to wean dextrose while on feeds, but she finally managed to maintain blood sugar levels. Got her GJ tube switched out. She’s lost about 4 pounds so her tube size is now 1.5 instead of 2. Ten days of Vanco and Zosyn. Her hemoglobin dropped and her CRP was elevated. She leaves us all confused. Her labs were off.
Her Genetics doctor asked for permission to present her case at the American Acadamy of Genetics meeting. Ava has more going on than just ETC - complex 1 (Mito). She has something else too. Endo is calling NIH and Mayo clinic for some ideas. Ava is a puzzle.
August 15th, 2011
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. Read the rest of this entry »
July 25th, 2011
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.
May 7th, 2011
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 fluids 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.
April 14th, 2011
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….
April 9th, 2011
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.
April 7th, 2011
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.
April 2nd, 2011
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. )
Read the rest of this entry »
March 3rd, 2011
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!
February 25th, 2011
This disease is terrible. So many children are suffering from it. Please pray for Maggie and Cooper as they fight end stage Mitochondrial disease.
