Findings

Since the beginning of this week it was discussed should Sonzee require the PICC line for TPN that she would also have a video fluoroscopy and sigmoidostomy while under anesthesia.  The purpose of these two procedures was to see if there could be answer to the question of what is causing Sonzee’s issues.  We are aware that delayed gut motility can be a negative component of having a CDKL5 mutation, but there can be other factors such as an allergy to one of the ingredients in her formula, or the ketogenic diet by itself can have a negative impact on a child’s digestive system. 

It was determined after Wednesday night that it was necessary to give Sonzee nutrition and we entertained the NJ tube trial long enough without having any success with her absorbing the nutrients.  Our doctor had already filled our heads with the idea of a PICC line and potential TPN from the beginning so we would not be blindsided should it become a reality.  I am beyond appreciative for that, as by the time Thursday morning rolled around, after Sonzee was presenting concerning signs that she was extremely malnourished, I basically asked the day team what time the PICC line would be placed.  The procedure was finally set for 2:00 with the GI procedures following immediately after.  Sonzee’s blood sugar was very low indicating she was in acidosis from the ketogenic diet and her lack of nutrition so she was given sugar water to correct this prior to the procedure and her sugars came back up.

The PICC line was successfully placed and it was time for GI.  By 3:30, her GI doctor came out to us to discuss his findings.  I have to admit I honestly did not anticipate what he would tell us.  I really just assumed he would take the biopsies, send them off, and we would discuss the results after the lab performed tests on them.  What we learned was definitely unexpected.

When her doctor inserted the camera down her esophagus and into her stomach, he was met with resistance until he filled her stomach with air and noticed an obstruction.  The obstruction he soon learned was part of the PEG tube that had been placed back in March.  The PEG tube that was placed in order for Sonzee to receive supplemental nutrition to ensure she gained weight appropriately.  The PEG tube that Sam and I spent HOURS agonizing whether it was the right decision for her.  The PEG tube that was supposed to HELP her.  Instead, the PEG tube caused an obstruction that blocked the ability of contents entering her stomach to exit and enter into her intestine, and forced the contents to go back out the same way they entered.  The PEG tube has left her stomach and itself useless until it can be switched to the MIC-KEY in three more weeks after the tract is completely healed.  The tube is not solely responsible for the predicament Sonze is in, however, it is believed to have exaggerated the situation.  Her intestines were unable to pick up the entire responsibility we placed on them in terms of handling her feeds, but with the assistance of TPN, we will give them another chance.  We are hopeful that her stomach will be able to resume typical function following the MIC-KEY button placement and when she is ready to move back to stomach feeds.  

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Sonzee becomes a "Tubie"

Lately I have been throwing out names of feeding tubes as if they are common household appliances.  It is pretty amazing how only a year ago none of these terms were part of my typical every day speech and now they have become part of my "professional jargon".  Sometimes I forget how little I used to know and how different life used to be.  I honestly do not think I ever saw a feeding tube in a person's stomach until Sonzee's birthday party when a friend of ours brought her daughter.  I honestly had not even google imaged what they looked like after Sonzee's initial consultation with the surgeon and I do not even know why.  

There are so many types of feeding tubes and various reasons why families might choose one over another, so for the sake of this post, I will explain the type of tube we are doing for Sonzee and explain our rationale for the choice.  Please know there is no right or wrong choice when it comes to choosing the type of tube a person uses, it is all about what works for the family and the situation.  The most important thing to remember is that any way you can get your child to meet his/her nutritional requirements is the BEST way.

From the start, I was very clear that I did not want something that would potentially interfere with Sonzee's desire to eat.  An NG tube goes up the nose and down the back of the throat and ends up in the stomach.  Many families may choose this route if the feeding tube is assumed temporary, if they just want to give a tube a "trial run" so to speak, or because they do not want their child to undergo a surgery.  I personally was against this option because the tube being placed against the back of Sonzee's throat could be such a nuisance to her that she may decide she would rather not eat by mouth.  The reason we are doing a feeding tube for Sonzee is not that her swallowing muscles are weak, nor is she on any food restrictions as far as thickness of liquids or textures of food.  It is because of these reasons; we immediately ruled out the NG tube and settled on a tube that goes directly into the stomach (gtube).  

Originally, Sonzee was scheduled to have a MIC-KEY button.  After speaking with many families, we settled on the MIC-KEY button due to its ease of use as well as the fact that it was inserted directly into her stomach.  The MIC-KEY button does not have any extensions hanging out of the stomach that can be pulled on, and it is easy to clean and easy to change at home.  The problem in Sonzee's case with the MIC-KEY button is that this procedure requires interior staples that with her high dosage of steroids could potentially have problems healing, and she would have a higher risk of developing a post-operative infection.  While her neurologist felt the steroids were not worth putting off until after we rescheduled her surgery we all agreed an NG tube would be our temporary fix.  I was not ecstatic over this decision, but whatever is best for Sonze.  

After multiple back and forth phone calls between the interventional radiology team and Sonzee's AMAZING pediatrician, we were advised that a PEG tube could be our temporary solution.  The PEG tube is similar to the MIC-KEY button in that it is directly placed into the stomach, but there is an extension that hangs out a couple of inches.  This procedure does not require staples, and will give the insertion site ample time to heal before changing to the MIC-KEY button.  We feel this will be a great solution, and we have been advised by multiple medical doctors that the high dose of steroids she is currently taking should not pose a problem for this surgery.  

Her surgery will be Thursday, March 10 at 8am.  I am a little nervous due to our last experience, but March 10 is an auspicious day as Sonzee's Bubbie celebrates her birthday, so it is already looking to be a positive experience.  Please keep the bear and the rest of us in your prayers this week and especially on Thursday morning.

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