Yesterday was a very special day in our lives... Ari turned 4. I vividly remember the two weeks on the high-risk perinatal floor, the hours of labor... and then then panic.
Ari's heart rate rapidly dropped in utero. Larry noticed... 170, 120, 80, 50... As he ran to get the nurse, she was already on her way in with a team of nurses behind her. Ari's umbilical cord had prolapsed, or pinched, from the extra fluid and was cutting off his oxygen. The results of this can be fatal and quick. The nurses were running. One, Two, Three- they rocked me back and forth by the blanket beneath me and lifted me onto a stretcher as the IV ripped out of my arm. As we passed the doctor, she was yelling "I said STAT!!!" holding up her hands for the nurses to glove her.
As the nurses ran out of the room with the stretcher, my bed slammed into the nurses station like a scene from a movie. In seconds, I was in the OR and masked. The medicine made me lose my vision. I counted 20 shadows in the room, the most important one by my head. Larry managed to gown up quick enough to make it in time for Ari's birth.
The baby was out, but he wasn't crying. "Save my baby! I don't hear him!" I cried over and over again. There were so many people in the small OR, their voices merged together in a loud buzz. Larry insisted he heard a cry, but I didn't hear him. Finally, I heard his meek cry and I joined him, overwrought with emotion and so grateful for his life. Another shadow approached me- it was a nurse from the NICU team. She got close to my face and said loudly, "He has imperforate anus. DO you know what that means?"
"Yes," I answered, "I do."
Ari was brought to me for less than a second, then whisked away to the NICU.
We are amazed at what he has endured over the past 4 years. Through it all, he smiles and has the best attitude. I am proud to say that this weekend, Ari is having his first real birthday party... the kind where you invite the whole class, go to a place that specializes in kid parties and eat cupcakes. It's a big moment in normalcy for us.
BUT...
I would be dishonest to you, my readers, if I keep one very big, very new part of Ari's journey a secret. I can't get my arms around it... can't form the words to describe the heartache and dread this musters within us.
I brought Ari to see his GI yesterday, on his birthday. Ari loves this doctor and it is always a treat for them to see each other, they've grown close over the past 4 years. I had no idea that we'd be there for an hour and a half, no idea that we would once again be making life changing decisions in this office.
The "egg shaped" area I described in my last post was a bit of an understatement. The truth is, this stretched out area is about the size of his stomach. His peristalsis is not working at all in this area. Food is storing in it, randomly moving down to block the esophagus when it becomes loose, causing choking, regurgitating and the worst case scenario, aspiration.
He is now a severe aspiration risk and has been taken off solid foods. His diet is purees only and he doesn't understand at all, though he does know he's been choking more. He's angry, acting out, and sad. He's starting to "get it" and knows this isn't good. We are going to have to insert a G-tube again. This was our measure of success! Oh, the steps back we're taking.
He has started choking in his sleep, so he's now sleeping with us. We can keep an eye on him and re-adjust his position as needed, getting up often to make sure he's ok.
Please hear me loud an clear when I tell you there is no medical solution for this. I don't want my baby cut open front to back, all the way around his side- he's already had this done on both sides. I don't want to have to cut another section out of his esophagus out when he worked so hard to get to this point. And what for?!? So the same thing can happen again? There is no solution and I'm scared. I'm scared that he looks like he's getting worn out, that he'll give up. I am so scared that I can barely swallow past the knot in my throat. I feel paralyzed... unable to think about anything else.
As Larry and I try to go about our daily lives, the sadness in the air is noticeable. I see it in our faces and our posture. Someone asked me today what's next and I'm having trouble seeing past tomorrow, when we go to the endoscopy clinic to change out the faulty cecostomy button. Can you believe that he is going to have a button to assist with stooling, one for catching urine, and one for feeding? We will control his 3 primary bodily functions. That's a lot of responsibility for any of his caregivers.
I will get a grasp on this situation. We'll develop a plan and it will be our new normal. In the meantime, my wall is down, exposing the fragility that I try to hide with self-confidence and a smile. I have to get over this, and quick. I cannot expect Ari to be strong if I do not show strength myself. I cannot expect him to smile and be brave if I am showing sadness and fear.
Please pray for my Little Lion to gather his strength and be brave, and for us to lead by example, rising above the fear and doubt and focusing on the positive and finding the right course of treatment.
Inspiration . Courage . Roar. The story of Ari Shumbres and our life surrounding his 100+ intubated surgeries and procedures.
Thursday, January 26, 2012
Monday, January 23, 2012
An Esophagus Story
Although able to eat, Ari's food repertoire is limited. It can't be too fibrous, too chewy, too stringy, too sharp or too sticky... and we had to learn this all by trial and error. It's a lesson in irony for our family... our child born without an esophagus wants to eat everything and our healthy child has an extremely limited palate.
One of Ari's favorite foods is apples. I have tried every possible way of serving them to him. Outside of thin applesauce, the chunks got stuck. After the third emergency procedure to remove apple from his esophagus, his GI sternly told me "NO MORE APPLES." Point taken.
On the contrary, fluffy scrambled eggs are easy for Ari to swallow. The eggs that got stuck over the weekend were fried, not scrambled, and stored in the balloon-like section of his esophagus over the connection site, which narrows significantly. Think of it as a kitchen funnel. The first piece of food that is too big for the funnel blocks the opening and the rest of the food builds up on top of it.
To exacerbate the situation, Ari has something called a Nissen Fundoplication (Fundo). Since he did not have an esophageal sphincter, his stomach is wrapped around the esophagus and secured in place, like a loop on a roller coster. This keeps the food he swallows and his stomach acid from coming up. The problem with a Fundo is two-fold. First, Ari's peristalsis (the wave pattern of the esophagus during swallowing) is inconsistant and slow, if present at all. He has to chew, swallow and then drink with enough force to flush the food down. The second problem is that the Fundo limits the ability for him to throw-up.
When food gets stuck, Ari can make himself gag to regurgitate the food above the Fundo. Water also helps carry the food back up. He drinks and regurgitates water over and over again until the food dislodges and he swallows it or it comes out. It's exhausting for him.
If the food does not come up and he has gone too long without fluids or if he starts to show signs of aspiration, we have to go to the ER. Like all of his procedures, he has to be intubated on a ventilator while the surgeon picks out the food piece by piece, or as in this case, has to try to scoop it out.
One of Ari's favorite foods is apples. I have tried every possible way of serving them to him. Outside of thin applesauce, the chunks got stuck. After the third emergency procedure to remove apple from his esophagus, his GI sternly told me "NO MORE APPLES." Point taken.
On the contrary, fluffy scrambled eggs are easy for Ari to swallow. The eggs that got stuck over the weekend were fried, not scrambled, and stored in the balloon-like section of his esophagus over the connection site, which narrows significantly. Think of it as a kitchen funnel. The first piece of food that is too big for the funnel blocks the opening and the rest of the food builds up on top of it.
To exacerbate the situation, Ari has something called a Nissen Fundoplication (Fundo). Since he did not have an esophageal sphincter, his stomach is wrapped around the esophagus and secured in place, like a loop on a roller coster. This keeps the food he swallows and his stomach acid from coming up. The problem with a Fundo is two-fold. First, Ari's peristalsis (the wave pattern of the esophagus during swallowing) is inconsistant and slow, if present at all. He has to chew, swallow and then drink with enough force to flush the food down. The second problem is that the Fundo limits the ability for him to throw-up.
When food gets stuck, Ari can make himself gag to regurgitate the food above the Fundo. Water also helps carry the food back up. He drinks and regurgitates water over and over again until the food dislodges and he swallows it or it comes out. It's exhausting for him.
If the food does not come up and he has gone too long without fluids or if he starts to show signs of aspiration, we have to go to the ER. Like all of his procedures, he has to be intubated on a ventilator while the surgeon picks out the food piece by piece, or as in this case, has to try to scoop it out.
Below is some history to fill in the blanks on how we got to this point and what a true miracle it is that he can actually eat.
When Ari was born, he did not have an esophagus, a condition called Esophageal Atresia. Instead of a connection to the stomach, his esophagus ended in a balloon high in his throat. There are 7 variances of this condition, Ari's being the most severe and called Pure Long Gap Esophageal Atresia because of the distance between the pouch and his stomach.
From the moment he was born, he had a tube in his mouth that went into the pouch to suction his saliva. If he did not have that tube, he could fatally aspirate. At 1 day old, he had his first surgery- the placement of a G-Tube (gastric tube for feeding) and a colostomy.
For 11 weeks in the NICU, his surgeon came in daily to stretch his esophagus from the mouth with a red rubber catheter. I hate that catheter. To this day, every time I see one I cringe, flooded with the memories of seeing the tube stuck down my baby's throat, sharing space in his mouth with the suctioning tube and a breathing tube.
Ari's time in the NICU was unstable. Ideally it should have been wait and grow period, but not for Ari. His de-stat episodes were frequent- 20, 30, 40 + in a day. Alarms would sound and the nurses would rush over to help him breath again. Each time we wondered if "this was it".. the time we has to say goodbye forever. We would brace ourselves mentally, unable to take our eyes off the scene surrounding him. We prayed for the day his surgeon would tell us "It's time to make the connection between his esophagus and his stomach." When that moment finally came, we were ecstatic.
After 6 hours of surgery, I will never forget the feeling I had when his surgeon walked into the waiting room, surgical scrubs on, goggles resting on his head, asking us to meet with him in a private consultation room. Immediately assuring us that Ari was fine, he explained that there was no way Ari's esophagus was going to reach his stomach. Other surgeons joined him in the OR and they concurred. As I mentioned in my last post, our worst case scenario came true... Ari had to get a spit fistula.
A spit fistula is extremely rare. Most doctors have never seen one and most nurses have never heard of it. Ari's esophagus was literally pulled out the left side of his neck into a stoma (hole) to allow for drainage of his oral secretions. Every time he swallowed, spit would come spurting out of the hole. There is a video link on this blog called "Ari's Spit Fistula". Check it out and you can see what happens when he drank juice.
Larry and I made the decision to teach Ari how to eat and drink at the same time he received his G-Tube feeds in an effort to create the association of a full tummy and oral satisfaction. Oh boy, was it messy! I think this early training contributed significantly to Ari's ability to eat today.
Due to other anatomical defects, we did not have any options for Ari to ever have an esophagus. His surgeon will always be my hero for taking the risk to recreate a not-well-accepted procedure and make it work for Ari's anatomy. In June of 2009, Ari "grew" an esophagus... it was nothing short of a miracle.
Despite the new esophagus, Ari was still tube fed. His esophagus just wouldn't stay open for food to pass through. He was going to the hospital to get an esophageal dilatation every 1-3 weeks, having to be intubated every time. We ran out of options and discussed redoing the entire connection surgery again, this time removing the area that had narrowed. One of the surgeons came up with the idea of 2 dilatations per week for 12 weeks. Without any other options, we gave it a try... 24 times in the OR in 1 summer and no results.
Out of ideas, we scheduled the re-sectioning surgery for October 2010. About 7 days before the surgery, Ari told me he was hungry. Cautious, I fed him a snack. He asked for more and I obliged, carefully monitoring every bite. He was eating, and it was going down. The power of a Miracle, the strength of a Lion, the grace of God. Call it whatever you want... I call pure joy. We canceled the surgery and worked on introducing new foods and textures to Ari.
I can't paint a prettier picture than our actual reality, though. Food gets stuck, his peristalsis halts, and his esophagus randomly closes for no good reason. Episodes like this weekend are frequent. We continue to work on his chewing and swallowing and live with the fear that the choking could happen at a friend's house or at school, where the adults in charge will not be comfortable dealing with it.
And speaking of dealing with things... In the time it took me to proofread this post, Ari accidentally pulled out his button. Calmly, I gathered the gloves and surgical lube, made saline, had Larry hold him down and Liv hold his hand. I reinserted it, inflated the balloon inside his intestinal wall, and exhaled.
"Bed time kids!" It's as if it never happened. We certainly do live a different type of normal.
Despite the new esophagus, Ari was still tube fed. His esophagus just wouldn't stay open for food to pass through. He was going to the hospital to get an esophageal dilatation every 1-3 weeks, having to be intubated every time. We ran out of options and discussed redoing the entire connection surgery again, this time removing the area that had narrowed. One of the surgeons came up with the idea of 2 dilatations per week for 12 weeks. Without any other options, we gave it a try... 24 times in the OR in 1 summer and no results.
Out of ideas, we scheduled the re-sectioning surgery for October 2010. About 7 days before the surgery, Ari told me he was hungry. Cautious, I fed him a snack. He asked for more and I obliged, carefully monitoring every bite. He was eating, and it was going down. The power of a Miracle, the strength of a Lion, the grace of God. Call it whatever you want... I call pure joy. We canceled the surgery and worked on introducing new foods and textures to Ari.
I can't paint a prettier picture than our actual reality, though. Food gets stuck, his peristalsis halts, and his esophagus randomly closes for no good reason. Episodes like this weekend are frequent. We continue to work on his chewing and swallowing and live with the fear that the choking could happen at a friend's house or at school, where the adults in charge will not be comfortable dealing with it.
And speaking of dealing with things... In the time it took me to proofread this post, Ari accidentally pulled out his button. Calmly, I gathered the gloves and surgical lube, made saline, had Larry hold him down and Liv hold his hand. I reinserted it, inflated the balloon inside his intestinal wall, and exhaled.
"Bed time kids!" It's as if it never happened. We certainly do live a different type of normal.
Sunday, January 22, 2012
Ari in Surgery
Ari just went into the OR for an emergency procedure to remove food stuck in his esophagus. We had 2 ER visits, 2 IVs, and 1 admission in the past 24 hours. We've been up all night.
To the nurse who walked us down to surgery... I don't care about your cats, how many you have and what color they are. I also don't care that you like to read and knit, have a niece and nephew and whether they've lost their baby fat or not. Five minutes before my child who is scared out of his mind goes into surgery is not the time to inform him of the potential inaccuracies of computers.
I understand that you are trying to fill silence, but I wanted to have quiet time with my child. Sunday surgeries do not come with pre-op meds (different route in) and I was trying to calm him, pray and summon his brave roar.
Now that I got that off my chest...
There is a lot of food stuck in an odd egg-like sack above the site where his esophagus was connected. He had an esophagram (similar to a long, video x-ray) in radiology this morning to verify the impaction.
I started writing about his esophagus and will post it tonight. It should help explain why this happens and how blessed he is to have an esophagus at all. A true miracle.
I pray for a error free and safe surgery and that he will chew better and drink more when he eats in the future.
To the nurse who walked us down to surgery... I don't care about your cats, how many you have and what color they are. I also don't care that you like to read and knit, have a niece and nephew and whether they've lost their baby fat or not. Five minutes before my child who is scared out of his mind goes into surgery is not the time to inform him of the potential inaccuracies of computers.
I understand that you are trying to fill silence, but I wanted to have quiet time with my child. Sunday surgeries do not come with pre-op meds (different route in) and I was trying to calm him, pray and summon his brave roar.
Now that I got that off my chest...
There is a lot of food stuck in an odd egg-like sack above the site where his esophagus was connected. He had an esophagram (similar to a long, video x-ray) in radiology this morning to verify the impaction.
I started writing about his esophagus and will post it tonight. It should help explain why this happens and how blessed he is to have an esophagus at all. A true miracle.
I pray for a error free and safe surgery and that he will chew better and drink more when he eats in the future.
Thursday, January 19, 2012
Diagnosis
Some of you may remember when Ari was 3 months old and went into the OR to have his esophagus connected to his stomach. We were so excited. I wrote in our Care Page about 3 possible outcomes, mentioning a spit fistula as the worst case scenario followed by "but THAT will never happen". Of course we all know by now, that was precisely the outcome.
Did I not learn my lesson?! Going into yesterday's test, I had a similar list of possible outcomes, worst case being a diagnosis of 1- no chance of bladder continence, 2- signs of kidney issues, and 3- the need for catheterization.
My conversation with the urologist was long and medically detailed.
- Ari's bladder has impaired compliance, meaning it does not contract a lot. He's had this study done twice and both times it didn't contract at all.
- His bladder is enlarged, holding about 250 cc or over 8 oz of urine before it starts to leak out- something called overflow incontinence.
- The rest of his urine is retained in his bladder, causing bacteria and pressure.
- His urethra in extremely narrow, making it all the more difficult for the urine to release.
- He has urine refluxing in the left ureter, backing into the kidney. This can lead to long term kidney problems, especially if bacteria is present.
What we can do:
- Ari needs to get something called a Mitrofanoff. It is a discrete stoma, or hole, through his abdominal wall and into the bladder, using his appendix as a conduit.
- We will have to cath him through the Mitrofanoff every 3-4 hours.
- The doctor will laparoscopically correct the urethra and ureter.
- He has to stay on a daily antibiotic to fight the introduction of bacteria.
There's a problem... Ari has something called a cecostomy. It is a "button" in his intestines that we use to manage his bowels. Everyday, we make a saline mixture and flush the stool out of his bowels. Imagine a 3 year old sitting on a grown-up toilet for an hour each day. If we push too slow, the stool gets stuck, if we push too fast, he cramps up. We have to use just the right amount and inject it at just the right speed.
You may be wondering why this is an issue. Well, a year or so ago, when Ari first got a cecostomy, we had to make a choice regarding his appendix. His surgeon wanted it for the cecostomy (making it an apendecostomy to be exact) and had a discussion with the urologist who wanted to save it "just in case" he ever needed a Mitrofanoff. Larry and I made the decision and it went to the surgeon. Six months later, that apedecostomy was reversed because the button kept getting painfully caught on the waistbands of his pants and a true cecostomy was created. We ended up revising that too, but that's another story.
The surgeon tried to save as much of the appendix as he could, but we are not sure there is enough there for the urologist to use it. His plan "B" is to use part of his colon, but remember, that is deformed too.
All the surgical details aside... we're talking about having to cath him every 3-4 hours for as long as we can see into the future. That's a big commitment.
I hung up with the doctor and called Larry. We went over the details in a very matter of fact manner. The details are the most important part... emotion can come later.
As we both sat in our offices, a text conversation began. One may think it would be dramatic and emotional, but that's not how we deal with things. We pepper everything with a little humor, a little sarcasm, and an understanding that we're in this together. Here's how it went:
Me: The more I think about it, the more I want to puke, cry or hide. Our baby needs a break!!!
Larry: I feel like that too... we need to do what keeps him healthy. Our options are limited- 10 years from now he may be fine... with new surgeries, medicine, etc... I may be dead, but he will be fine.
Me: Lol. Don't leave me. I'd end up in the psych ward and the kids in DFCS.
Larry: I'm headed that way.
Me: You have no idea.
And that was it. Back to work, though a little harder to focus. When I picked Ari up today, we hugged for a long time. I was so happy to see him. Apparently he was happy to see me too, because he did not stop talking for 1 second between that moment and bedtime. It was a great reminder that he's a normal toddler and we try to cherish every minute of it. Although it would have been nice if he could leave his sister alone for just a few minutes so she could do her homework. :)
We're still going to wait for the spinal MRI to make any decisions on surgical timing, as it may have to take precedence. I took Ari to get new shoes last night, his right foot is an 8 and left foot is a 9 1/2... confirmation of the spinal cord starting to re-tether.
Only time, and an MRI, will tell.
Wednesday, January 18, 2012
What's in a Name?
Ever wonder where the name Roar Little Lion comes from? Watch this video I took today to find out... We do this every time Ari goes back for surgery, regardless of how well... or sick, he is that day.
http://www.youtube.com/watch?v=9q8kktGAGXo&feature=mfu_in_order&list=UL
http://www.youtube.com/watch?v=9q8kktGAGXo&feature=mfu_in_order&list=UL
The name Ari means Lion in Hebrew. We selected the name long before he was born, but it's fitting. Our strong Little Lion is full of fight and ready to roar his courage whenever it needs to be summoned.
Ari knows the routine in Day Surgery and loves the attention he gets from being a frequent flyer. Everyone from the registration staff to anesthesiologists know him by name. He knows when to step on the scale, which finger to hold out for the PulseOx monitor and how to sit still enough to get his blood pressure on the first try.
Selecting his DVDs (or as Ari calls them, DDDs) for each hospital trip is a treasured process for Ari. As soon as we get in a room, his DVD goes in and he's genuinely happy. That is, until the pre-op meds come. We've done this so often and he still fights the Versed, the medicine that make him relaxed and a little loopy. It's his indicator of what's next... the breathing treatment, the gown, and eventually the nurse knocking on the door, slowly opening it and peeking her head in, "Are you ready to go?"
I always act overly happy, assuring him everything will be fine. Before the nurse comes in to get him, but after the Versed kicks in, I always ask, "Let's hear you roar, Little Lion."
Sometimes his roars are big, like today, making both of us giggle. Other times they are so weak, he'll open his mouth and make a noise only audible to his angels. It's OK... I know they're listening and that roar is a sign to surround him with the comfort only they can bring.
As soon as the door opens, Ari puts on what I call his "brave face"- a huge frown, eyes full of fear, but refusing to cry. It breaks my heart. As they wheel him to the operating room, I hold his hand and tell him I love him and to be strong.
They doors to the OR corridor open and I listen carefully as they page the surgeon, making a mental note of the room number and saying a prayer that I do not hear the words "code blue, OR room ....." over the intercom. I play out in my head what I would do, then shake away the thought with the fear that thinking about it will somehow give fate permission to make it happen. Silly, I know, but true.
"Be brave! I love you!" I try to be cheery for him, but at this point, he doesn't speak. He just looks ahead as they wheel him in. I stand there, watching him, waiting for the doors to close behind his bed, and exhale. My shoulders slump, there's no need to pretend to be happy anymore. I walk back to the room and sit in silence.
This routine never gets easier, as I though it eventually would. I have grown more numb to the emotions, but they're still there. Every knock at the door makes my stomach rise in my throat for fear of bad news.
Today's procedure was a little different- we've done it before. He spends about 30 minutes in the OR, goes to the PACU to wake up a little, then gets transferred to fluoroscopy for the urodynamics study. I was with him doing my usual role... holding him down while he shakes from anesthesia and writhes and screams in pain.
I feel guilty that he has to go through this, Guilty that I made him this way, and Guilty that he knows real pain. I can't forget the look in his eyes, the big elephant tears, and the feeling of helplessness and fear. I try to channel the pain away from him, literally feeling it for him, but in the end this imagination game only comforts me, not him.
Back to our room and 2 popsicles later, the nurse removes his IV and we're ready to go. He got a present today- a plastic cup with CHOA's Logo on it. Ari is obsessed with the little boy and girl in their logo- Hope and Will, though he pronounces it "Hope and WEE-il", like a true Southerner. He dances all the way to car and even stops to sing happy birthday to his favorite security guard.
We arrived at 6 am, were home by noon and the events are now behind us. There was a mix up with Ari's Neurosurgeon's office coordinating his spinal MRI for today. He'll have to go back in for it, but I don't mind- it wasn't meant to be done today.
Thank you for following Ari's story- he is a true hero!
This routine never gets easier, as I though it eventually would. I have grown more numb to the emotions, but they're still there. Every knock at the door makes my stomach rise in my throat for fear of bad news.
Today's procedure was a little different- we've done it before. He spends about 30 minutes in the OR, goes to the PACU to wake up a little, then gets transferred to fluoroscopy for the urodynamics study. I was with him doing my usual role... holding him down while he shakes from anesthesia and writhes and screams in pain.
I feel guilty that he has to go through this, Guilty that I made him this way, and Guilty that he knows real pain. I can't forget the look in his eyes, the big elephant tears, and the feeling of helplessness and fear. I try to channel the pain away from him, literally feeling it for him, but in the end this imagination game only comforts me, not him.
Back to our room and 2 popsicles later, the nurse removes his IV and we're ready to go. He got a present today- a plastic cup with CHOA's Logo on it. Ari is obsessed with the little boy and girl in their logo- Hope and Will, though he pronounces it "Hope and WEE-il", like a true Southerner. He dances all the way to car and even stops to sing happy birthday to his favorite security guard.
We arrived at 6 am, were home by noon and the events are now behind us. There was a mix up with Ari's Neurosurgeon's office coordinating his spinal MRI for today. He'll have to go back in for it, but I don't mind- it wasn't meant to be done today.
Thank you for following Ari's story- he is a true hero!
Sunday, January 8, 2012
Ari in ER
I Thought we'd end up here today after Ari choked at lunch.
I didn't think it would be due to his cecostomy button coming out. I couldn't reinsert it- looks like the balloon has a leak. The hole is closing by the minute. Praying they can get a new button in or he'll be in surgery this week. It will be painful either way.
Poor kid cannot catch a break.
***UPDATE*** The hole closed significantly, so there was no time for meds or anesthesia. The doctor had to use 3 different sized rubber catheters to dilate it, then use a stylus to stretch and insert a new button. Ari was screaming in pain. I had to hold him down, whispering in his ear, "I'm so sorry, it's almost over, I'm so sorry, Baby." Though normally composed, I couldn't hold it together this time. It was awful.
When it was over, I rocked him in the chair and within 30 minutes, he was his normal, happy self- proud to show off his new button. One visit to fluoroscopy to check placement and we were home by 8:30.
He told me later that he does not want to have any more "button aches"... proud of his funny play on the words "tummy ache". I love this boy.
I didn't think it would be due to his cecostomy button coming out. I couldn't reinsert it- looks like the balloon has a leak. The hole is closing by the minute. Praying they can get a new button in or he'll be in surgery this week. It will be painful either way.
Poor kid cannot catch a break.
***UPDATE*** The hole closed significantly, so there was no time for meds or anesthesia. The doctor had to use 3 different sized rubber catheters to dilate it, then use a stylus to stretch and insert a new button. Ari was screaming in pain. I had to hold him down, whispering in his ear, "I'm so sorry, it's almost over, I'm so sorry, Baby." Though normally composed, I couldn't hold it together this time. It was awful.
When it was over, I rocked him in the chair and within 30 minutes, he was his normal, happy self- proud to show off his new button. One visit to fluoroscopy to check placement and we were home by 8:30.
He told me later that he does not want to have any more "button aches"... proud of his funny play on the words "tummy ache". I love this boy.
Thursday, January 5, 2012
Our focus today
If you asked me a week ago, I would have told you our latest challenge has been urinary track infections. A major issue for us of late, Ari has been on a daily antibiotic for 2 months straight. We scheduled a procedure called urodynamics for January 19th to explore his bladder and kidney functions internally.
I remember Ari receiving a battery of renal tests when he was in the NICU. The "R" in VATER stands for renal (the kidney or urinary system). We checked the "R" off the list, thankful to be spared one of the letters in the acronym.
Over the course of the past few years, Ari had a few bladder/urinary track infections. He's rarely symptomatic and never complains of any discomfort associated with them. For any of you who have ever experienced a UTI, you know that they can be extremely painful. I often wonder if he lives with a certain level of pain in this area every day as his "normal".
Within the past year, his frequency of infections increased dramatically and his lack of bladder control became evident as we attempted potty training. Ari is unable to control his flow of urine, a common result of a tethered spinal cord and sacral deformity. We started seeing a pediatric urologist and discovered that his bladder is retaining urine. This is a concern for multiple reasons.
1. He can not fully empty his bladder, causing the urine to sit and grow bacteria which leads to infection.
2. The bladder has to expand to accommodate the excess urine, which can be painful.
3. The urine can back up into his kidneys, causing kidney damage, stones or failure.
4. The urine can overflow into the testes causing epididymitis and possibly infertility.
So far, all of the above mentioned effects have happened except for #3, which we are watching closely.
With this being our focus, I couldn't help but wonder if something could be wrong where his spinal cord was tethered at birth and released by a neurosurgeon while still in the NICU, so I scheduled an appointment.
Unfortunately, my suspicions were confirmed. Ari's right foot is about an inch smaller than his left. Combine that with some right leg and lower back pain, right leg dragging when tired, and the increase in UTIs, and the symptoms point to a re-tethering.
Here's the problem... a tethered spinal cord is a degenerative defect, often referred to as a syndrome of its own. As he grows, the cord can re-tether at multiple times in his life. MRI technology cannot detect the tethering, so his symptoms dictate the next surgical step, which is extremely risky in the big picture of his surgical spectrum. If we wait, it can lead to progressive, irreversible spinal cord damage, such as loss of movement or feeling in his legs.
Since he'll be intubated on the 19th for the urodynamics, neurosurgery ordered a spinal MRI to rule out a spinal fluid leak or mass. We'll determine his next steps after we receive the results from these tests.
Though hard to imagine, our biggest issue today is not his bladder or his spinal cord. It's something I never thought I'd have to deal with... Ari developed a tic. It's a hard blink that last longer than a normal blink and is uncomfortable to watch. I brought him into the pediatrician's office yesterday and they drew a full series of labs. Ari's fight or flight instinct is ridiculously strong. I had to lay on top of him to hold him down while they drew vial after vial of blood, he stared at the needle, screaming. "I no yike that shot, Mom." he said to me as we left.
I don't like it either.. but for a different reason. The fear of the unknown. It could be a strep infection in the blood, but that's just 1 vial... what were the other 4 for? I didn't ask, knowing his doctor is using the "rule out" method we've become used to with Ari's many diagnoses.
It turns out it is not an infection, so we are left with 3 other options. It could be a nervous tic that goes away as quickly as it came, it could be a mass in his spinal cord compressing the optical nerve, or it could be his spinal cord re-tethering and pulling on an optical nerve.
For now... we live with the unknown, give Ari lots of love, and have faith in his strength. It's also important that our daughter Liv does not feel slighted while we deal with this new chapter, as she's had her own series of challenges lately.
At 7 years old, Liv is a strong athlete and competitive cheerleader. She sprained her left ankle a few times over the course of the season and was already wearing a support brace. During a practice prior to the holiday break, Liv tumbled INTO a trampoline, chipping 2 bones in her left knee. The ensuing hip to ankle immobilizer caused her to walk on her ankle at an awkward angle, which led to a ligament tear in her already weak left ankle, taking part of the growth plate in her heel with it for good measure. Her physical therapy appointments are keeping us... and my parents... very busy.
Ari dancing to Hi-5
YouTube video- Ari's spit fistula
Ari's spit fistula
If you haven't seen this, check it out! For about 18 months, Ari's esophagus was pulled through to the left side of his neck into a stoma that allowed for drainage of saliva.
We still taught him how to eat, drink and swallow with hope that one day he would have an esophagus and need these skills.
How it all began...
Like many pregnant woman, I felt huge when carrying Ari. I had been through this before with my daughter born in 2004, but this was different. By 30 weeks, I looked like I was ready to deliver. My breath felt condensed, pinched nerves sent pain through my arms and legs and I was all belly. I literally looked as if I grew by the day. It turns out, there was a good reason for all of this.
It took until 35 weeks gestation to officially receive a diagnosis. I called my OB daily, urging him to see me, intuitively knowing something was wrong.
"You look great." "Many women carry larger with their second." "Everything is fine, you're perfectly normal." The answers from the doctors and nurses I saw were repetitive.
I tried to suck it up, telling myself that lots of women have babies and I was not special. But I knew otherwise and was relentless in my calls and requests for urgent appointments. My nights were spent pacing the room, tears leaking at the pain in my numb arms, feet itching so bad that I scratched them to the point of bleeding.
At my 35 week appointment, I measured 42 weeks, or 2 weeks overdue. An immediate in-office ultrasound was ordered. This day plays over and over again in my head, like a haunting re-occurring dream. Part of me tries to push it out of my mind, and part tries to never forget. Thank God my husband was with me.
The ultrasound tech was chipper and chatty, proud to show us a preview of our perfect baby boy. Her temperament grew somber and beads of sweat formed on her brow. With a nervous quiver in her voice, she told us that it looked like I had polyhydramnios, or excess amniotic fluid. She was searching for his stomach, but couldn't find it. Excusing herself, we were left to wonder what what was wrong.
A different nurse came in and guided us back to an exam room where a doctor was waiting. Arrangements were made to see a perinatologist immediately. Ironically, it was the same practice I had been to for 2 prior ultrasounds of this same baby.
What we heard from the perinatologist was unexpected, scary, and changed so many lives forever.
Our baby was missing an esophagus.
My body was making amniotic fluid for the baby to swallow as nutrition, but without an esophagus, the fluid built up, causing me to visibly grow by the day. Our baby was receiving all of his nutrition via his umbilical cord, but that too was defective- having only 2 vessels instead of 3.
The severity of the birth defect was not able to be detected due to the excess fluid and the doctor mentioned the possibility of other birth defects that tend to travel in a pack with esophageal atresia, called VATER Syndrome or VACTERL Syndrome. We would not know about any other defects until after he was born.
We were paralyzed with both fear and reality. WHAT NOW??
We left the doctor's office in separate cars. Wracked with emotion, I called my parents... A conversation they are sure to remember forever. Within 24 hours they went from Pennsylvania to Atlanta, dedicating their time and lives to the comfort and routine of my daughter.
I tried to stay composed, but I just couldn't. Excusing myself from the family that evening, I remember sitting the my son's nursery, rocking in his glider and sobbing- big, choking sobs, yet it didn't help. My life was not going to change because I "cried it out". This my was first lesson in bearing the weight of fear, worry, and love and not being able to do anything about it.
I glanced around the room at the perfect blue, brown and cream stripes on the walls and at the wooden letters "A-R-I" that hung above the changing table, took a deep breath and vowed to rise above the pain. Ari did not have a choice in how he developed in utero, but I had a choice in how I was going to deal with it. From that point on, I became him champion, his voice, his advocate. Squaring off my shoulders, I joined reality again- and never looked back.
It took until 35 weeks gestation to officially receive a diagnosis. I called my OB daily, urging him to see me, intuitively knowing something was wrong.
"You look great." "Many women carry larger with their second." "Everything is fine, you're perfectly normal." The answers from the doctors and nurses I saw were repetitive.
I tried to suck it up, telling myself that lots of women have babies and I was not special. But I knew otherwise and was relentless in my calls and requests for urgent appointments. My nights were spent pacing the room, tears leaking at the pain in my numb arms, feet itching so bad that I scratched them to the point of bleeding.
At my 35 week appointment, I measured 42 weeks, or 2 weeks overdue. An immediate in-office ultrasound was ordered. This day plays over and over again in my head, like a haunting re-occurring dream. Part of me tries to push it out of my mind, and part tries to never forget. Thank God my husband was with me.
The ultrasound tech was chipper and chatty, proud to show us a preview of our perfect baby boy. Her temperament grew somber and beads of sweat formed on her brow. With a nervous quiver in her voice, she told us that it looked like I had polyhydramnios, or excess amniotic fluid. She was searching for his stomach, but couldn't find it. Excusing herself, we were left to wonder what what was wrong.
A different nurse came in and guided us back to an exam room where a doctor was waiting. Arrangements were made to see a perinatologist immediately. Ironically, it was the same practice I had been to for 2 prior ultrasounds of this same baby.
What we heard from the perinatologist was unexpected, scary, and changed so many lives forever.
Our baby was missing an esophagus.
My body was making amniotic fluid for the baby to swallow as nutrition, but without an esophagus, the fluid built up, causing me to visibly grow by the day. Our baby was receiving all of his nutrition via his umbilical cord, but that too was defective- having only 2 vessels instead of 3.
The severity of the birth defect was not able to be detected due to the excess fluid and the doctor mentioned the possibility of other birth defects that tend to travel in a pack with esophageal atresia, called VATER Syndrome or VACTERL Syndrome. We would not know about any other defects until after he was born.
We were paralyzed with both fear and reality. WHAT NOW??
We left the doctor's office in separate cars. Wracked with emotion, I called my parents... A conversation they are sure to remember forever. Within 24 hours they went from Pennsylvania to Atlanta, dedicating their time and lives to the comfort and routine of my daughter.
I tried to stay composed, but I just couldn't. Excusing myself from the family that evening, I remember sitting the my son's nursery, rocking in his glider and sobbing- big, choking sobs, yet it didn't help. My life was not going to change because I "cried it out". This my was first lesson in bearing the weight of fear, worry, and love and not being able to do anything about it.
I glanced around the room at the perfect blue, brown and cream stripes on the walls and at the wooden letters "A-R-I" that hung above the changing table, took a deep breath and vowed to rise above the pain. Ari did not have a choice in how he developed in utero, but I had a choice in how I was going to deal with it. From that point on, I became him champion, his voice, his advocate. Squaring off my shoulders, I joined reality again- and never looked back.
Wednesday, January 4, 2012
Ari's story
It's been 2 years to the day since I posted on Ari's Care Page. Some of you may have wondered what happened. No new is good news- right?
Well... that's all in the interpretation. At times there was so much going on, it was overwhelming to write. Other times things were going so well that I didn't want to revisit any pain or worry by opening up the door to my emotional state... I secretly feared that my words and fragility would collide and emulsify.
This site was set up as a way to celebrate our accomplishments, to share some of the heaviness of the unknown and seek comfort (not pity) for the sadness one can only get from seeing their child suffer.
If you don't know Ari yet, don't worry... you will soon. His story will inspire you, his smile will charm you, and his personality will capture your heart.
Over the course of the next few weeks, I will post some of my older blogs to get new followers up to date. I look forward to picking up where I left off and sharing Ari's amazing story with you.
I'm also proud to say that with the encouragement from many of you, I have started writing a book. It has been hard to deal with the emotions as they resurface. My husband asked me today if I was writing the book for others or as therapy for myself. A good question indeed... I mulled it over for quite some time. The answer is, for others. I truly believe that you will be inspired by our Lion's fight and the ups and downs of his journey. If some of the weight I carry on my chest gets lifted in the process, then I'd call it a win-win.
Please pass on this blog to everyone you know- the more followers, the better!
Well... that's all in the interpretation. At times there was so much going on, it was overwhelming to write. Other times things were going so well that I didn't want to revisit any pain or worry by opening up the door to my emotional state... I secretly feared that my words and fragility would collide and emulsify.
This site was set up as a way to celebrate our accomplishments, to share some of the heaviness of the unknown and seek comfort (not pity) for the sadness one can only get from seeing their child suffer.
If you don't know Ari yet, don't worry... you will soon. His story will inspire you, his smile will charm you, and his personality will capture your heart.
Over the course of the next few weeks, I will post some of my older blogs to get new followers up to date. I look forward to picking up where I left off and sharing Ari's amazing story with you.
I'm also proud to say that with the encouragement from many of you, I have started writing a book. It has been hard to deal with the emotions as they resurface. My husband asked me today if I was writing the book for others or as therapy for myself. A good question indeed... I mulled it over for quite some time. The answer is, for others. I truly believe that you will be inspired by our Lion's fight and the ups and downs of his journey. If some of the weight I carry on my chest gets lifted in the process, then I'd call it a win-win.
Please pass on this blog to everyone you know- the more followers, the better!
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