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<< The hypertonia is very much improved now that he is getting his PROM on a

regular basis. >>

Carol,

Thanks for writing. What is PROM? I haven't heard this term before.

Joanne, mom to , 1 year, New York

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I want to thank everyone for the warm welcome. I have 42 emails to read

through. I'm sorry I didn't respond to much during the week. I work 15 hour

shifts and just collapse when I come home. I don't get very much sleep and

hardly any time to do the things I want to do, like read all that email.

The baby I care for is a 7 month old boy named Mark. For obvious reasons, I

can't give more information than that. I am copying emails and taking the

information to Marks parents. They are very excited to learn of the support

network you have set up. The only problem is they don't have a computer with a

modem. They are working on it, so maybe they will be online soon. I know you

could help them a great deal. They so badly need to hear some of the positives

about your children. Mark was not expected to live a week. He was left lying

in the hospital and they parents said they felt like the medical staff was just

waiting for him to die. See, I can do a little nurse bashing myself! You

really don't have to worry about that with me. There are good and bad in every

profession.

Mark does not have a specific diagnosis other than PMG in general. We don't

know the extent of the damage, and I really don't think his doctor does either.

They said if he lives, he would be blind, never have teeth, walk, crawl,

interact, etc

The first week Mark was home from the hospital was a tough one. He had not been

held or handled at all in the hospital. He had severe hypertonia of the lower

extremities and his hands were already contractured into tight little fists.

The poor baby was not use to the human touch and was very agitated when held. I

took care of that as quickly as possible. After a few 15 hour shifts of

holding, rocking, singing, etc, Mark began to relax and within the week, was

relaxed and seemed to be use to being held. Now at 7 months, he loves it being

cuddled and rocked. He doesn't even mind my bad singing!

Mark does not have a hx of seizure activity, but...................he had 2

seizures about a month ago. One was on my shift and it was an irretractible

seizure. The parents like to think it was tremors from the albuterol, but I was

not able to stop it. It lasted about 45 seconds. It also happened to the other

nurse on the case. An EEG was done and the parents were told it was unclusive

because his EEG read abnormal, but that was to be expected with pmg. If it were

tremors, we would have been able to stop it by holdling him. I just hope there

is no more. His fontanel was bulging at the beginning of the week, but is back

to normal now.

Back to the things the doc said would never happen.......Mark has 3 teeth and

is cutting his 4th. The doc said he should be blind but I found him to just

shut down with too much stimuli. If I do his visual therapy through the night,

he always responds and is now responding at times during the day. I turn out

all the lights in the room, put him in a vibrating infant seat (iittle help with

the neb/cpt) and turn on the cartoon channel with no sound. With all other

stimuli removed, Mark will look at the tv for about 20 minutes at a time. He

turns his head toward the lights. He definitely can see, he just can't handle

a lot of stimuli at once.

Today I held him facing me and he was trying to smile. We are just starting to

see a little interaction once in a while. He was looking right at my face and

I'm sure he was trying to smile. He just can't lift up those chubby little

cheeks yet. We are working on head control at this point, and Mark will work

very nicely with us. He is making subtle progress

I mentioned that his hands were contractured when he came home from the hospital

at 4 months, 3 days old. His hands are now loose and relaxed. The hypertonia

is very much improved now that he is getting his PROM on a regular basis.

Mark also has bilateral hip dislocation, scoliosis, tachycardia, bradycardia at

times, a rt inguinal hernia. He is on a respirator and continuous pulse

oximetry.

I'm probably rambling here, I am exhausted but get to stay home tonight so I

didn't sleep at all today.

The parents were advised to visit Dr. Dobyns in Chicago so they were excited

when I took the information that one of you sent me about Dr. Dobyns and the

research he is doing.

I can't begin to tell you how much you have all helped this mother already.

Just with the few emails I printed and took her, she is very happy. Being a

rare disorder, she didn't think anybody could really understand what they are

going through. She is anxious to read about your experiences with your kids and

hopes to learn more about her own child.

Mark is a beautiful little boy. I wish I could put a picture of him up, but

that would be a big confidentiality issue. I know once the mom is online, she

will be happy to share Marks picture with you.

I really must get some sleep, but will try to go through all the email before I

do so. Thanks again to everyone for letting me in the group as Marks nurse. I

know this is where all the REAL information is at. I hope to hear more from

all of you.

Carol

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