[Cahill]

Hello Dear Folks, (reposted from intro forum)

I sit here scouring the internet for information that might help me to save my 9 year old daughter. I am home for the night after 26 days in hospital with my Emma.

My hope is that one of you will have information to help us.

We took Em to the Hospital for Sick Children in Toronto on Hallowe'en for rapid inspirations (gasping, air hunger) and increasing muscle weakness. Em has always fatigued easily but since September, she has felt like there are magnets drawing her limbs to the ground. She is brighter in the mornings and very weak by evening.

In Emergency, the consulting docs suggested Myasthenia Gravis or Guillan Barre Syndrome (comorbid?). Em has no lower reflexes but has not experienced ascending paralysis as in GBS. Em was given a dose of Mestinon (pyriostigmine) in the ER.

Admitted to the neurology dept, the testing began. No one could be sure if the Mestinon was working. Her pattern of bright in the morning, tired in the evening continued. Ptosis was more and more of a problem. IVIG was performed on day 3 and 4 with no obvious benefit noted.

On day 5, Emma didn't restore...she awoke tired after a night of obstructive sleeping and frequent desaturations. Her spirometry was 34, her ptosis didn't allow her eyes to open at all and she desaturated into the low 80's and her co2 was 84. Emma was moved to the ICU where a femeral line was inserted and plasmapheresis started. Docs started Prednisone 30 mg bid and Mestinon was increased to 30mg q 6 hrs. She was restrained to start bi-pap therapy to aid in blowing off her co2. Plasma exchange was done 5 times over a week. Emma was a new girl. By the second exchange, she had renewed strength, her eyes were open, she was funny, animated and interestingly, her Aspergers' Syndrome characteristics were GONE. She connected well with us and was more engaged than I've ever known her.

We graduated back to the Neurology unit on day 12. Emma's carnitine level test came back, low - level carnitine started. Lumbar puncture revealed elevated lactate in CSF. MRI (finally done!) revealed spots in the basal ganglia suggesting inflammation or possible mitochondrial effect. Mestinon increased again to include 90 mg slow release for nighttime.

Emma's weakness worsened. Bi-pap support all night with 2 hr breaks during the day. Spirometry results varying, co2 level no longer a problem. Low potassium resolved with one off supplements. Blood pressure and heart rate increase causing concern. PRN neFEDopine started and soon being used q 4 hrs.

OG tube insertion last weekend after bout of Norwalk Virus sent her into a tailspin. Breathing issues resurfaced when her tummy bloated and forced lung capacity to diminish further causing a great deal of pain especially on bi-pap.

Today, Emma's breathing is erratic with frequent desaturatons, she cannot open her eyes (we hold them open for her to communicate with docs, family), she has blood in her urine, Amlodopine added for blood pressure control. She cannot bear her weight and is confined to bed in a diaper.

Em has a muscle biopsy scheduled for Monday, the day after tomorrow. We've been fighting to have it done without general anaethetic as we've been warned that if she is intubated she may lose her ion drive and have to stay on a ventilator. We are not ready to never hear her voice again.

In a meeting yesterday the docs said that they think she has mitochondrial disorder comorbid with myasthenia gravis. There are no documented of this comorbidity although myasthenic effect has been seen.

We want plasmapheris again. How often can rounds of plasmapheresis be done? I was told today that she can't have it again until after the muscle biopsy because of the heparin needed for the plasma exchange.

Any advice, insight, resources would be appreciated.

Thanks so much for reading,
Christine

[Stellatum]

Christine,

I'm so sorry for what you and your little girl are suffering.

I don't have any help to offer for the big questions, but I have a small comment to keep in mind for the future. IVIG often doesn't take effect right away, and the plasmapheresis your daughter had took out all the immunoglobulins that the IVIG put in, so you won't get to see if it has any delayed effect this time around. Also, some of us have found that the first IVIG treatment, or the first few, doesn't do much, but then it starts really helping.

I mention this only to say that even though IVIG didn't help in the emergency situation, it still might be an option down the road for maintenance.

That is fascinating, about your daughter's Asperger's and the change in her after the plasmapheresis. I read this book a long time ago: http://www.amazon.com/WELCOME-SILENC...2571550&sr=8-1 It's about a woman who was cured of schizophrenia with a procedure "similar to dialysis." I wonder if it was plasmapheresis. I'm certainly not suggesting that Asperger's is related to schizophrenia, but both cases show how much we don't know yet.

Abby

[art chick]

Christine, Thank you for reaching out...I am very sad to read about what your family is going through and I hope that you get the help and resources you need very very soon.

My teen daughter was just diagnosed with MG this spring and someone mentioned a website to me: http://www.myasthenickids.org/

They are based in the UK but since they all have younger children, I am wondering if they may be of some help for you too?

I was told by my neuro that I would get plasmapheresis 5 days for the starting dose and then 1-2 days every week for maintenance until we could do it less often. (I am currently having ivig). It has been since day 5 of 26 since Emma had her dose, right? She definitely could be having more from what I understand.

debra

[jana]

Hi Christine!

First of all, Prednisone OFTEN makes MG WORSE before it makes it better. So, if your daughter's doctors don't know this, they need to do research:

http://www.uwo.ca/cns/resident/pocke...uscular/MG.pdf

This is by Dr. Michael Nicolle -- an eminent Canadian MG specialist. Note Prednisone information on pages 9 and 10.

It seems to me that Myasthenics are OFTEN allergic and chemically sensitive -- so, I wouldn't be at all surprised if your daughter has problems handling meds and/or treatments. They are "throwing" so much AT her right now to make her BETTER -- some of this may actually be CAUSING problems.

Hang in there -- it sounds like the doctors ARE looking and ARE concerned.

You and your daughter will be in my thoughts and prayers.

(((HUGS)))

[DesertFlower]

Christine, I am so sorry for what your daughter is going through. I want to offer some sort of advice but don't have any other than to don't stop looking for answers.

When I was 2, I also got very very sick and almost died, I spent a year in and out of the hospitals...my mom counted over 200 shots, all experimental treatments...I had so many shots that my muscles collapsed. The doctors told my parents that I was going to die. My parents finally went to another doctor. The conclusion was that I had an initial reaction to antibiotics, which caused a yeast infection that had gone untreated and undiagnosed for all that time, and all the treatments given to me in the hospital kept making me worse. This doctor gave me medicine for a yeast infection and I was mostly better within 2 weeks.

Don't give up hope, look into everything, even what might have triggered this in September. It might be a good idea to get copies of all the tests done and review them yourself, just in case something has been overlooked.

*hugs*