Long awaited doctor visit

A couple months back we found out that a specialist at the University of Minnesota was willing to meet with us about Bridget.  We were thrilled at this news as we had yet to meet with a doctor that knew much about her diagnosis (other than everything we had already found ourselves while scouring the internet).  Unfortunately, the trip was less than impressive, tedious, and to be honest…a huge mental letdown.


           On day 1 of our trip, the only information we learned was that Bridget's heart is working wonderfully (she previously had a minor heart repair done), so this was great to hear.  Other than that, we had various questions that we were previously told to bring with us, that unfortunately did not come with any real answers.  Day 2 ended on a positive note as we met with another doctor, whom we could tell read through all of our notes and paperwork that we sent in prior, and we had a very open and honest conversation.  He listened, was patient, and gave us genuine advice.  I also appreciated that he confirmed that we have already done everything possible to get Bridget set up with the best care and support systems available back home.

           I always worry that we (I) haven’t done enough for Bridget.  Is there something else we could try?  Is there a therapy or medication that would be beneficial for her?  In the end, we found out that Bridget is cognitively around the age of 1.  This is a decline from what we had previously heard, but not a shock to us at all.  As far as medicine goes, there is no data to support that any medical interventions have significantly helped any other Sanfilippo children. 

           One of our most important questions was about an approximate timeline of Bridget’s life.  I understand that to many, this is a morbid question.  However, we have been on this ride for so long now, that it is crucial we know as much as possible to enjoy whatever time we have available with her.  We know that she developed relatively normal until the age of 1 ½ or 2.  Since then she plateaued for years before beginning a downward trend.  I have observed other Sanfilippo children making progress far surpassing what Bridget was ever capable of.  I asked if her early lack of development correlated with length of life…and he confirmed that yes, he does believe that is true. Sanfilippo is a neurodegenerative disease and unfortunately, there’s no more making gains or progress from here on out. 


            Our final day in Minnesota was filled with A LOT of waiting around (times were given to us incorrectly).  Luckily, Bridget was a trooper through all of this. The doctor came in and discussed the procedures that he would be performing that day which included: a brain MRI, MRI of the abdomen, and also a spinal tap.  I can’t even count how many times we’ve put Bridget under anesthesia throughout her short life, but it never gets easier.  Of those assessments, we found out that her spinal fluid is normal (clarifying that she does not suffer from normal pressure hydrocephalus), but also that she has “severe atrophy of the brain”.  I will never forget the lump in my throat as her scans filled the screen. So much damage has already been done. Her scans don't portray the brain of a sweet, happy-go-lucky 6 1/2 year old, but that of a person struggling with Alzheimer's for quite some time.  

           There is nothing worse than being told that there is absolutely nothing you can do to help your own child.  Just to take them home, and love them.  To focus on making memories, and prioritize “quality of life”.  To begin the process with palliative care… 


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