This isn't a terribly exciting post, but it has lots of important information that I want to document for myself. Feel free to scroll down to the cute pics at the bottom if you get bored!
It has been a pretty successful, productive and satisfying month in terms of all of Audra's follow up appointments with specialists and such.
I usually leave her various appointments feeling somewhat defeated...
because she is so complex,
and doesn't fit into a neat little diagnosis box,
and never follows the textbook in terms of progress!
But this month things have been different. Maybe because the doctors have known Audra for longer now, and have been able watch they way she has progressed over time. They also have the benefit of looking back upon all that has been done so far, and seeing what has helped and what hasn't. She continues to be a medical mystery to almost all of her specialists, but little by little we are chipping away, putting puzzles pieces together and figuring out how to best help her. We are
STILL waiting on her whole exome gene sequencing results which many of her specialists hope will help uncover THE diagnosis.
GI:
Audra continues to grow on her own curve, which is waaayyy below the 0 percentile, but one thing that has improved is her BMI. It is the first and only measurement that has ever plotted above the 0 percentile. We know that she has fat malabsorption, pancreatic insufficiency, high caloric needs, high energy expenditure and slow growth...but we don't know why. This month's appointment focused on her fat malabsorption and the impact it may be having not only in her physical growth but also her cognitive development. Can I get an AMEN?!?! This has been my concern since day one. I get that the doctors wanted to give her the time to see if a good 'ole American diet would fatten her up...but it was clear to me from nearly day one that no amount of food she consumed would be enough. This girl can eat, and DOES eat...anything, anywhere, anytime!!!
So what's next?
We have added a new supplement to her overnight tube feedings. It is composed of MCT oil which they believe will be better absorbed by her finicky GI system. It's called Liquigen and looks like a thick white milk. An 8oz bottle has 1125 calories and 125g of fat!! Oh my!
We are adding a little bit to her feeds each night and increasing the amount we give her every week.
We will also be doing a Dexa scan to check her bone density, an X-ray to check her bone age and more labs to check her essential fat levels. Fattening up her 'lil body can only mean good things for her physical health and development growth. Progress!
Enough calories to make her GI doctor and Nutritionist proud!
Immunology:
Overall, since starting the immune globulin infusion therapy in December, Audra has done very well. But she did have one hospitalization for a fever in March, a couple of ear infections and her IgG level is still not as high as they would like it to be, so they increased her dose of immune globulin replacement by 50%. We are still managing to infuse the dose with just one needle site, and we have started to use her belly for injections instead of alternating between her legs. But with the next dose increase we will probably need to start using two simultaneous injection sites. She has good infusion days and bad infusion days. Sometimes she lets me put the needle in by myself, but other times we still have to hold her down to get it in. Sometimes she breezes through the hour infusion without any trouble, and other times she gets quite uncomfortable. Haven't quite figured it out yet. But less infections and less antibiotics = Progress!
Audra rockin' her infusion
Neurology
I love Audra's neurologist. She gets how hard it is to be living without a true diagnosis, and is genuinely empathetic and concerned about the effect this has on our family as a whole. She is honest but encouraging about Audra's potential.
Audra was quite delayed developmentally when we brought her home. And while we all had hoped for some eventual "catch up" development, that just isn't happening. She has been home for a little over two years now and has only made about 4-6 months of developmental progress. Two years ago, at the age of 5.5 years old she was at a 20-22 month old level. And now, 2 years later she is at approximately a 26-28 month old level. The reality is that Audra's developmental quotient is (and probably always will be) 30% of her chronological age. So right now at the age of seven an a half we should only expect her to a 27 month old developmentally. At the age of 10years we should expect her to be a 3 year old developmentally. And at the age of 21 years we should expect her to be a 7 year old developmentally. Although this was shocking to hear at first, it has helped me to reset my expectations for her. She will not have any "catch up" development. We are beyond that window for that. But she is making slow and steady progress along her own curve (does that sound familiar?...at least she is consistent with following her own curve for both growth AND development!)
There is one little glimmer of hope though...we still haven't solved the mystery of her abnormal EEG. The abnormal brain waves are located near the language center, and the specific type of abnormal brain waves that she has are associated with language delays. She is scheduled for another overnight Video EEG in July and my hope continues to be that they will figure out what is causing her abnormal EEG, and/or do something to minimize the abnormal brain waves so that maybe, just MAYBE she will gain some language skills. Hopeful for progress to come!
IEP
This may have very well been the most dreaded "appointment" I had for Audra this month. After two years in pre-school Audra has become familiar with the routines there and comfortable with the staff. Moving her to a whole new school, with NO familiar faces will likely be traumatic for Audra. And my mama heart fears this transition. But it is necessary. She will be 8 years old in September...we can't keep her in pre-school forever! The problem is she still hasn't accomplished pre-school skills. And they can't complete any standardized testing on her because of her very limited language skills. Their best estimates put her in the "severely cognitively impaired" category. Whatever that means. I, of course, think my girl is wicked smart.
Next year Audra will attend a full-day Life Skills class with a 1:1 aide. And she will have some exposure to the regular education kindergarten classroom. Not at all what I have hoped and dreamed of for her, but this is what is best for her.
I am not an IEP meeting rookie...I have been to battle at IEP meetings many, many times over the years for Riley. But this meeting, and this team was different. When I cried (yes, I cry at
every IEP meeting!) they listened and seemed to genuinely care. They were willing to think outside of the box for ways to make Audra's transition to kindergarten the most successful it can be. Someone suggested making a photo book for Audra with pictures of her new school, playground, classroom, teachers and even the principle. Sure caught them off guard as they weren't prepared for a photo shoot at an IEP meeting! This will be a wonderful tool for preparing Audra this summer...similar to the photo album we sent to China before we traveled to meet her. It gave her the opportunity to become familiar with our faces and pictures of her new home before her entire little world was uprooted.
Another unexpected blessing was that they included 30 minutes of nursing for her every school day. This will be helpful in making sure she gets her medications everyday, and the extra fluid she needs through her G-tube on the days when she gets her immunoglobulin infusion. Didn't even ask for that, but so thankful it is being provided!
So, overall, it was the most successful IEP meeting I have been to...EVER! I am still a nervous wreck about her transition to kindergarten and the effect that uprooting her "little school world as she knows" it will have on her. But I couldn't be more comfortable with plan and her IEP team.
It's the best it could be, which is something I could not have imagined just a year ago. So I'll call that progress too!
More pics just because she is so adorable...
