Re: 5 yr old with SICCA SJOGREN SYNDROME?

Posted By Barb on October 09, 1998 at 22:43:44:

In Reply to: 5 yr old with SICCA SJOGREN SYNDROME? posted by Barb on October 08, 1998 at 23:39:10:






My daughter was born 4 months premature, as a result she has CP.
Some time before she was 5 months old, she had a cereral stroke.
She has poor balance, and is non verbal except for sounds.  She is ataxic,
has low vision(ROP) had ATNR and scoliousis.( last two resolved).
She had her first seizure at 5 months, and at that time she weighed a little over 3 #.  She was off Tegratol for two years when I started to notice what I called shudders, the EEG showed seizure activity and now she is on Depakote Sprinkle caps, 125MG  3x a day.  We recently found a catarach
(spelling?)  She also has an immune deficency to the point that she is home-bound schooled.  The Neuro thinks that all of her different symthoms,
aside from the obvious CP, looks like Marineseo Sjogren, which I have found is also called Sicca Sjogren Syndrome.  I have not been able to find out anything specific on this syndrome.  Everything is about Sjogren Syndrome only and this does not have the symtoms (symptoms) that HE attributed to the condition.  Oh yes, she is also haveing very high blood pressures(113/110) and she has swelling in her feet and her ears turn beat red for no reason(unless it is her BP).  Can you tell me what Sicca Sjogren is?  I must tell you that even though her prognosis was NIL at five months
and I was told she would be a veggie, she is walking, unaided, can tumble and finaslly jump on her trampoline(still a work in progress).  She has her own computer and uses it, in Kindergarten and doing 1st grade work.  In other words, she is a wonder of therapy and a lot of love,  Sorry, just had to brag.  She has come so very far and already has had one set-back and had to relearn evrything, I need to keep abreast of everything medically related to her in order to help her to keep growing.  Any help you can give would really be appreciated....Thanks....Barb
Dear Sir, Thank you for responding so fast.  Some of you questions are hard for me to answer but I will give it a try.  My daughter is adopted and came to me when she was 5 months old.  Four days later, she had a grand mal seizure in the doctors office.  During the subsequent hospitalization a scan showed what the doctors called a hole in her brain.  When I asked about it, no one could really explain it.  It was not until 31/2 yrs later that she had an MRI and a repeat that the hole turned out to be an infarction in the left side of the cerebelum.  By this time we also had a new neuro who really cared.  He says the loss of balance and the inability to talk both come from the stroke.  We do not know when the stroke occured.  It even could have been inuterol.  
Next question- The cataract may be a result of lazer surgery performed at 2 months for ROP.  There was a lot of bleeding and detached retinas in both eyes.  I have been told that this type of surgery can have the side effect of cataracts, but I was not expecting it quite so soon.  It is hard to get answers to questions sometimes, especially in a clinic situation.  To answer your question, I have not been told what caused the cataract.
Again, you pose a question I am unable to answer.  What type of immune deficiency does she have?  Being born at 23 weeks left her with a lot of defidiencies, sight, hearing, etc.  From the time she came into my home, I restricted people who were sick or even had sick children from entering the house.  Her ped. recomended exposing her to out side gerrms on a gradual basis.  She started to school when she was 3 and everything went downhill from there.  She stayed sick for 8 months, going from one thing to another.  The worst part was that she started to regress.  She went from being able to take about ten steps on her own to not being able to even crawl.  The ataxia became so bad she had difficulty standing, even when holding on to something.  Our neuro jumped right on it and started running all sorts of test he confered with other neuros on staff at Barrows and other hospitals.  He even tested for Maple Syrup Syndrome.  He could not find the reason for the regression.  The final conclusion was that neurologicly she sits on the edge of a cliff, when she becomes physically sick, it dumps her off the cliff.
We worked very hard, going back over past therapies and she finally regained all she had lost and has procressed way beyond.  All I know is, if she is exposed to an illness of any kind, it takes about 48 hours and she will have it ten fold.  She is taking maintainence anti-biotics now in the second month.
You have to explain what IVH is regarding her CP.  All I know is that when she was born along with her twin sister at 23 weeks, they were not expected to live.  I was told at 5 months that her brain damage was severe and involved more than 40 percent.  She was for the most part blind, could not track  did not respond to sounds, nor stimuli.  
The scoliosis was diagnosed with blood work and the fact that she looked like a pretzel.  She had more curves in her spine than a downhill ski run.  Believe me when I say it took hours and hours of therapy to get her straight. The ATNF persisted for about a year, the therapy was ongoing with all the other therapies.  She was placed in AFOs and a back brace, weights on arms and ankles.
We do not know the cause of the hypertension.  It has just been confirmed 2 weeks ago.  I now have a BP set-up and check it at different times during the day.  Trying to come up with a time it occurs most often, like after heavy exercise, certain foods, etc.  The doc. has done blood work and has the results but I have not been informed as of yet.  I am really afraid that she is going to have another stroke and lose evrything again,
No the Sjogren's Syndrome is not confirmed yet.  The testing has just started, right now I think the doctor is more worried about the BP.
My daughter has a habit of not fitting most standard molds.  Like cataracts at age 5 or high Bp.  She is an amazing child with a will to live and a stubborness that will not let her give up on anything.  If she can't do it the normal way, believe me when I say she will find another way to do it.  
She has such great potential and I thank you for taking the time  and the interest in her.   Barb