Do not fear the tube

Every time we make a decision, we wonder if said decision will result in a positive outcome.  There have been so many times along this journey that the decisions have resulted in a domino game effect with literally no end in sight.  Along the way we have almost become numb to the negative outcomes, I say almost, because even though we say we are numb, we are simultaneously saddened and frustrated with the choice that led to the undesired outcome.  Despite the fact that even though I can assure and reassure myself "we made the best decision with the information that was presented at the time" it never quite seems to soften the blow.  However, there are definitely rare times throughout this adventure that we happened to make a decision that 100% was unarguably the best decision ever. 

Usually the results of our choices do not present themselves immediately because in the land of CDKL5 you can be assured that the term patience will hold an entirely new meaning.  Then one day something will occur and it will dawn on you that "yes indeed that was the best decision ever".  The decision for us to give Sonzee a feeding tube was never one we entered into lightly.  In summary, after months of little bear not gaining adequate weight we had a consultation for a feeding tube to be placed in her stomach.  Prior to her surgery she had a negative reaction to IV fluids that were ran too quickly and almost killed her.  During that hospitalization, she was diagnosed with an abnormal background on her EEG and Infantile Spasms that resulted in high dose steroids and so the surgery was postponed.  After many days of back and forth between many medical professionals, it was decided that Sonzee should have a PEG tube placed.  After the PEG tube was placed, we were uncertain if this was actually the best decision for the bear.  She was not gaining weight, she was having severe reflux, increasing irritability, and difficulty with not vomiting up everything she consumed.  Finally, in May we hit our breaking point and she was hospitalized for a month resulting in us leaving with an intestinal feeding tube (NJ tube).  

Sonzee has been discharged from the hospital for one month and 17 days.  She has grown at least 2 inches and has gained at least 4 pounds.  Finally, our little bear is getting adequate nutrition and she is beginning to thrive in so many areas.  I look back on my concerns regarding this feeding tube adventure and fear was ranked high, followed closely by wondering if a tube would even help or how this would negatively impact her quality of life.  So many parents have asked me if we think we made the right decision in pursuing a feeding tube for Sonzee, and I used to waver in my replies.  If you ask me today how we feel about Sonzee not eating by mouth and having all of her nutrients be given solely through a feeding tube, we will answer "Undeniably the BEST decision we have made.  Do NOT fear the tube."

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Warnings

When you study a specific subject matter for a lengthy amount of time, you become extremely knowledgeable on that topic.  The same can be said in regards to dealing with certain situations for an increasing length of time; you become so well versed on that specific subject matter that you know its ins and outs.  When you spend your days caring for a child who has a specific genetic condition, even without any prior formal medical training, over time, you become an expert in this specific field.  That is why from the very beginning of this journey when other parents in the CDKL5 parent support group shared their experiences with seizure treatments, the best types of therapies, vaccinations, overall health difficulties, etc., we have listened and we have trusted.  

Every wobbly step Sam and I have taken on this journey has been done while holding the virtual hands of other parents with kiddos with CDKL5.  All of these parents love Sonzee as much as we do.  They want only the best for her.  They share their experiences both successful and those that turned out unfavorable for us to use as tools for guidance.  The successes they share to not only instill hope, but also with an extreme desire that this specific recommendation might result with the same positive outcome for Sonzee.  They share the unfortunate experiences not to take away hope, but more as a warning; a message to keep in the back of our heads that says, "Hey, this could happen" or "be sure to keep a lookout for....”   

As we all know there is no specific cookie cutter route for dealing with all of the situations that could potentially arise while having a child who has a CDKL5 mutation.  Each child has his/her own specific path to take, dealing with his/her health and developmental obstacles.  There are certain key elements that are present in the majority of the kiddos, but how each child responds to specific treatments, therapies, vaccinations, etc. varies.  The only option we have is to take the advice of other parents, combine it with the information shared from our medical professionals, follow our wavering instincts, and simply gamble.

I personally am not a fan of gambling, it is not a thrill of mine.  Maybe it is because the negative feelings of losing outweigh any potential feeling of joy that could occur if I were to win.  Maybe it is because I am not an inherently lucky person and I figure with the odds against me that there is no point.  No matter the possible reasons as to why, I just really dislike gambling.  This is an extremely difficult thing to dislike when essentially EVERY decision we will ever have to make for Sonze is based off some sort of gamble.  A gamble that no matter what the result is essentially life changing in all interpretations of that term.  From the very beginning, we have lived with only seeing the consequences of our choices in Hindsight.  

These past three weeks we have seen the consequences of our choices for Sonzee play out in a manner that has left me way down at the bottom of the coaster.  I do not and cannot regret any of the decisions we have made, I know we are only doing our best with the situation we have been given.  HOWEVER that does not change the guilt, anger, and sadness that follows when you see your child suffer based on the decisions you made with the main goal being to help her.  It is so hard to say if her current predicament is a result of the ketogenic diet, the gtube placement, the high dose steroids, or a combination of all of the above.  Was this issue inevitable regardless of any decision we made just because of her specific body and the effects of her specific CDKL5 mutation?  Regardless of the cause, I cannot say we were not warned.

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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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Rolling with the punches

Life is all about rolling with the punches.  Lately, punches are exactly what have been coming at us.  Sam and I were so nervous about Sonzee's g-tube procedure that when things went South on Friday we felt like we had lost our abilities to breathe.  We had been back and forth with our decision to get the gtube all week long.  I honestly almost cancelled it two times during that week alone.  I have been comparing the experience with a flight cancellation.  When you miss your plane or your flight is cancelled and you are placed on another, there is that brief moment where you might find yourself wondering, "which one of these flights is going to crash?”  Maybe you are not as neurotic as I am, but I have definitely had that thought when my flight plans have changed.  The thought that both Sam and I wondered was, "Is this G-d's way of saying we shouldn't be getting a gtube for Sonzee?"  Did we just dodge a bullet or is it more of the message, "Now isn't the time".

One of the twists of our weekend in the hospital was that we finally got Sonzee to have her EEG that we have been essentially begging for since she weaned from Topamax.  I am pretty sure her epileptologist was holding off giving the Ketogenic diet more of a chance to do its thing prior to looking at her brain's background.  We all had an idea that she had hypsarrhythmia, I had sent some videos of her most recent spasm episodes over to her doctor last week, and it was decided on Thursday that following the surgery, she would have an EEG, and then after the surgery site was healed she would most likely be starting the prednisolone.  Either way, this road was going to be traveled.  The order just threw us off our game.  

I have been emailing with her epileptologist since Sunday and as soon as the bear is over her little cold, she will be starting 40mg a day of prednisolone for two weeks.  After the two weeks, she will have a repeat EEG and if the hypsarrhythmia is gone, she will begin a four week wean.  If there is still hypsarrhythmia then she will continue another two weeks at 60mg of prednisolone a day and then have the four week wean.  Our team has decided it is more imperative that we treat the hypsarrhythmia at this point vs waiting until after a gtube has been placed and healed.  Due to the dose of the prednisolone, we are unable to do the gtube simultaneously with the prednisolone as her immune system will be depressed and healing would be challenging.  We are working on scheduling an NG tube placement for the duration of the steroid treatment, and then we will reschedule the gtube placement afterwards.

She will remain on the ketogenic diet; we just will not be as concerned about her ketone levels needing to be high.  We will also be receiving some stage one food recipes so we hope it will help with the anticipated bear-like appetite, as well as administering her steroids.  I am sure the punches will continue to roll in as we deal with some of the ugly potential side effects of the high dose of steroids.  

Sam and I are at peace with our teams decision.  We feel that we have done our best with all of the medical decisions we have made for the bear along the way, and even though we were unable to "outsmart" the hypsarrhythmia, we gave it our all.  Some things in life are just unavoidable no matter how much you try your best.  

We are praying that she only has to complete this treatment once, and that we will successfully rid her of these ugly spasms.  We are praying that she will have as few side effects as possible from the treatment.  We are praying that she will have a honeymoon period from all of her seizures during or after the treatment.  We are praying that this experience will turn out to be one that we praise and would do all over again if we were asked.

Thank you all for your unwavering support and love, and thank you for helping us continue to roll with the punches.

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What nightmares are made of

On Friday we arrived at the hospital at 9am to check-in for the bear’s surgery.  Sonzee was visibly upset due to not eating, so Sam was walking around with her while I took care of her admission.  We went up the fourth floor and by 10:30, we were back in the pre-op room.  They took her vitals; she received Osito a tiny red beanie baby bear.  Things were going smoothly and we were just waiting around.  Sonzee had a little stint of spasms around 10:45, and then again around 11:20.  We were told around 11:30 that the surgeon was running behind and it would be at least another hour, so we asked if it would be possible to get some food into her.  After speaking with the anesthesiologist, it was decided we could start her on IV fluids.

It always takes a minimum of three attempts to get into Sonzee’s veins; it is just how it goes.  After multiple attempts, and two nurses, she was finally set up at 12pm.  I made sure multiple times that they were going to administer straight sodium chloride vs the dextrose 5 bag, as she is on the ketogenic diet.  Our neurology team made it clear that even if I tell the nurses about the ketogenic diet, and they act like they understand,  I shouldn’t trust anyone’s word, and so I peaked at the bag myself to ensure it was the correct one.  Around 12:05, they began to administer the fluids.  I was holding Sonzee at this point to attempt to calm her down because she was clearly uncomfortable, but we decided shortly after I took her that it would be better if Sam held her since I just stopped pumping and she could probably still smell breastmilk.

At 12:10pm, she started to cough.  She looked panicked, she was clearly having problems breathing, but I was assured it was nothing, and it was because she was upset.  (She was not that upset to cause this type of coughing).  Another 2 minutes of her coughing goes by and she is now having large amounts of secretions come out of her mouth.  She suctioned her mouth.  I am visibly upset, the nurse is staying right next to us, and she tells Sam to place her on the bed.  He does.  I ask her if we can turn off the IV, and she reassures me it is just water and that this is not due to the IV.  I was doing my best not to be combative, but I made it clear this was not normal behavior, and while I was not arguing to the fact that it was straight water, clearly something was wrong with Sonzee.

Thankfully, an air pocket was found in the IV tube, so the pump began to ring and the nurse was forced to work out the kink.  Over a couple of minutes, the IV was turned off, and she started to calm down, and had less coughing.  Sam decided it was a good time to run downstairs to grab a quick snack as we had not eaten and were waiting for her to be taken back.  He left, the kink was fixed, and the nurse turned the IV pump back on.  Within a couple of seconds her coughing began, I was repeatedly telling the nurse this is not normal, she continued to reassure me, and as a peace offering, decided to put a pulse ox on Sonzee’s foot so we could see the amount of oxygen in her body as well as her heart rate.  After a couple of minutes her oxygen dropped to the 70s, there was foam coming out of Sonzee’s mouth and I am pretty sure I screamed to the nurse, "this is not normal, you need to do something".  I ended up walking out of the room as the oxygen bag was taken out and I am pretty sure the “code blue pre-op room 1” alarm must have sounded because within 1 second there were 20 people standing in and outside her room.  I tried to text Sam and give him a warning that they were taking the oxygen out, but he did not read the text and when he walked through the pre-op doors, all he saw was the chaos.

Everyone was trying to blame her current state on a seizure.  I was adamant this was not a seizure.  I was continuously telling everyone that I was not trying to argue, but while they might be new to Sonya and seizures, I knew this was NOT at all seizure related.  Her stomach was distended, her lungs sounded tight, they rushed in a chest x-ray, and shoved an NG tube down her nose into her stomach to release the air/pressure that was inside.  That was an immediate help, but at this point, she was just lying there.  There was no movement.

Her surgeon was the only person willing to listen to me that this was not due to any seizure activity.  I had spoken with my dad who is a doctor and he asked how many cc’s of fluid were given, I was not sure at the time, but he told me if it was less than 100cc then it would be fine.  I went back to the nurse and asked, and they said it was 150cc’s.  I said, I think that is the problem, and I was met with all 20 people telling me this was appropriate for a baby Sonzee’s size based on a calculation (which I will find out which calculation they used because it makes zero sense with what I know her weight is and what my dad says is the recommended dosage).  No one listened to me.

We were transferred to the ER, and she just laid still on the gurney.  Her body was beyond pale, and her lips were a greyish/blue tinged color.  I get it now what people are referring to when they ask if lip color has changed.  She looked so awful; I took a picture and could not bear to look at it.  It made me sick.  We got into the ER and the ER doctor comes in and talks to me as if this is my first go around with seizures.  He starts to tell me that it is possible Sonzee is having sub-clinical seizures that we might not be able to see, I retort back with “You mean status?”  I think he was taken back when I said those words.  He said, “Yes”.  He then continued to tell me that he just spoke with neuro and they want to give her Ativan to take her out of the seizure.  At this point, Sam almost punched him in the face, I told him to sit down and be quiet or get out.  I told the doctor, we were not doing that, but I would be more than happy for her to have an EEG and if it showed she was actually in status, he could give it to her.  He said, “Why are you against the Ativan”, I said, “Because she isn’t having subclinical seizures”.  He said, “Well I can’t just order an EEG, she would need to go to the ICU for constant monitoring”.  To which I replied, “Okay, then put her in the ICU”.  He goes onto to explain that usually Ativan is just administered so as not to waste the time it would take to get her hooked up to the EEG.  I said, “Well until you show me she is having a seizure, you aren’t giving her anything.”  I am pretty sure he did not realize I know a thing or two about seizures; he walked away, I am sure to make a phone call. 

When he came back, he listed all of the tests he would need to perform since I was not willing to give her the Ativan.  I am pretty sure he was not so excited her blood work indicated she was sick, not saying he wanted her to be sick, but it was not helping his case.  He had them do urine cultures with catheters, he had them drawing blood out of her arms, they put in another IV, she continued to lay there.  We were adamant this was related to the IV and the fluid administration; everyone else was blaming it on a seizure, or they would simply say, “Well, we just don’t know what happened, it’s hard to say”.  I know they have to do that to protect themselves, but it can be quite frustrating to deal with.  We wanted to try to feed her; the poor girl still had not eaten.  I kept asking for an NG tube if they were not going to let me feed her by mouth.  We were told for that they would need to do more x-rays of her stomach and lungs in order to make sure we could place the NG tube.  Neither of those tests was ever done.  Finally, around 3:15pm after all of the poking and prodding, the bear began to come back to life.  We begged to give her a bottle, by 3:30pm, she was finally taking one.

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What is best for Sonzee

For those of you who have been following our journey from the beginning, you are probably aware that we have been battling weight issues since Sonzee turned 5/6 months old.  This issue, like many that present themselves with CDKL5, became apparent gradually.  It was only after she had multiple trips to different doctors for various reasons that it dawned on me that her weight was dropping from the previous visit or remaining stagnant.  Unfortunately, when your child is seen my various medical professionals, it becomes more challenging to keep track of these "smaller" ticket items.  During Sonzee's 10 month checkup, I mentioned to her pediatrician that the previous week her weight was only 13lb 9oz at the neurologist, and I could have sworn it was higher at the appointment she had just a couple of weeks before that one.  The neurologist’s office chalked it up to being weighed inaccurately, but I was uneasy because I knew she had been weighed each time with her clothing off.  It was then that her pediatrician was on board with there actually being a concern.

As many of you also know, Sonzee is a breastfed baby.  I have breastfed all four of my children for various lengths.  I introduced solids to each of them at different times during their "babyhood" as well.  For Sonzee, Sam and I decided early on that we would not like to play the seizure/food lottery (as we are typically only winners of the less desirable lotteries), and so we found a nutritionist willing to work with us, and we began to incorporate foods that were low in carbs/sugar in addition to her breastmilk intake.  For all of my children, we use the first year to be primarily breastmilk and the solids to be more of food introduction and to gain oral motor skills (the speech therapist in me cannot help herself).

We decided with our neurologist as soon as their staffed nutritionist was able to take on Sonzee as a new patient we would transition into the ketogenic diet.  For us, this would be surest way to not aggravate the seizures.  We have been feeding Sonzee avocado, hummus, olive dip, and asparagus for a good while now.  We have had some successful weigh-ins since October, with her weight remaining at least stagnant and not dropping, and her most positive being just 3 weeks ago with Sonzee weighing 14lbs 10oz.  In my mind, we had a gtube consult back in December to just discuss the possibility of the gtube, but not to actually ever have to need it.  

During our visit, the surgeon, who was recommended by a dear friend here in Phoenix re: personal experience, was very helpful in answering our questions.  We decided we would not be interested in the ngtube, as those can be very irritating to the throat, and if we wanted to preserve her desire to be fed by mouth; this route was not recommended for long-term use.  We instead figured when/if the time came, we would prefer to start with a gtube, as this would be better for the long haul.  I think I can include Sam in my current statement, that when we left his office, we were still undecided about whether Sonzee would actually need the gtube.  The doctor stated that she was on the cusp of failure to thrive, but to us, she still had time to show everyone she could gain weight on her own.  I figured by the time of our ketogenic diet consultation, we would have a good idea of whether the gtube would be beneficial for her.

I was even more determined following that appointment to get her filled with foods.  I even mentioned multiple times that if at her next weigh-in, if she had not gained weight at least I would know I had given it my all.  Any chance we had, we would force food down her throat.  I woke up every time she shifted in the middle of the night to encourage her to eat.  I really, truthfully did the best I could in that department.  I know she ate whenever she was awake and willing.  I tried nearly every suggestion that was shared with me to increase her calories, but it was also the seizure factor.  When she has a seizure she will sleep for hours following it, and there is no amount of encouragement I could offer to change that.

Last Wednesday we had our meeting with consultation team for the ketogenic diet.  We are to be starting it any day now.  Her lab work has been completed.  She has a couple more tests/scans she needs to undergo, and we are just waiting on the email from the nutritionist.  Her medication has been changed to pills, so they can be cut, crushed, and added into her diet without added sugars from liquids.  They weighed her at this appointment, and sadly, she is only 14lbs 8oz.  We decided that the gtube is what is best for Sonzee.  

She had her video fluoroscopy this morning and the results show she is a great candidate.  Her stomach empties appropriately, and her anatomy of both the esophagus and stomach are great.  We are now just waiting to hear from the surgeon as to the surgery date.  

We know we did the best we could to help her without the gtube.  We understand and know the risks of having a surgery; we know and understand there will now be an increased risk of infections.  We are aware that with any procedure there are always negatives as possibilities.  We are doing what is best for the bear.  We have HER front and center with all of her care decisions.  This is what is BEST for Sonzee.  

Maybe if I say that last line enough times, I will be "okay" with this outcome.

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