Liam's one year Tubieversary

adjusting to his new backpack

right before surgery 1 year ago

Dear Liam,

It wasn't an easy decision for us to lay you in the hands of a surgeon one year ago. This surgery was brought up to us several times in the past but it wasn't until you went through your PH probe study (remember when you had that giant tube down your nose for 24 hours) that we realized we didn't have much a choice, that at some point you could start aspirated on your acidic reflux which would lead us down an entirely different road. It's hard to believe what you've been through this past 20 months and your infectious smile is what gets us through it all. It seems like ages ago that you had your 1st feeding tube placed, but it has been exactly one year today. In this last year you have undergone 7 surgeries, 2 long hospitalizations, ER visits, dozens of specialists visits, and have had a lot of testing done. Your current Jejunostomy (J-tube), which because it bypasses your stomach and goes straight into your intestine, has given you the ability to eat without pain and suffering and vomiting, has been a lifesaver to us. Unfortunately because this is a surgically placed feeding tube you have to have it replaced in surgery every 3 months, we aren's sure at this point if that will end for you anytime in the near future.

We (your parents) are in a different place than we were one year ago, we know more about feeding tubes, medications, the digestive tract, and many other things than I'm pretty sure either of us have ever wanted to know. It's a funny thing feeding you through a feeding tube, it takes a lot of mental work for us to wrap our head around it and become ok with it. Feeding you solely through a feeding tube really goes against all of our parental instincts, especially those of  me (your mother). As soon as you were born it was my natural instinct to start feeding you immediately and for the several months that followed. When I couldn't  provide that for you a big piece of me felt broken.  As your getting older you know whats going on around you and withholding food from you is becoming harder and harder and sadder and sadder.  I know that you want to eat but we have to protect you from hours of screaming in pain or vomiting your food up, and that's not an easy job for us. It breaks our hearts everyday not letting you eat.

 For the first 6-8 weeks after your Fundoplication surgery you were doing so well, gaining weight, and were no longer throwing up. We naively thought this was it, that we had finally done something with such positive results. Then one day you starting vomiting again. When we went into your GI specialist we were expecting to get a date to take out your feeding tube but instead we had it changed out and were told that you were going to have to keep for "a while". In our mind we were thinking great another 6 weeks or so, we can totally do that, but 6 weeks turned into 6 months which now has turned into we just simply don't know if you will ever be able to be without it. 

In the process of learning how to balance feeding you through a feeding tube, bolus feeding or continuous feeding, G-tube feeding vs J-tube feeding, when to vent or flush or when we should continuously vent and feed, with what I might add was very little training on (thank goodness I was already a nurse because we would have been completely lost), which pump we should use, etc. etc. After we found out at that you had something called gastroparesis or delayed gastric emptying, which basically means your stomach doesn't work properly, we had questions as to why this happening to you, we saw neurologists and metabolic specialists all of whom couldn't give us a complete picture as to what is happening to your little body. They would tell us your lab work is abnormal but doesn't point to anything definitive. Let me just tell you that this was probably the most frustrating part of this year was going in for  test after test all coming back abnormal but not showing anything definitive. We still don't have a lot of answers, except that your stomach is essentially paralyzed and doesn't contract and move food through your GI system as it should. Unfortunately this is one of the main systems in your body that needs to all work properly for you to survive.

 In this past year we have learned to cherish the times when everything is going well and when you are happy, but we have also learned that that typically won't last long. We have learned to let go of things we have no control over and accept help when it is offered even though our pride is injured in the process. The truth is that we cannot do this by ourselves,  we would not have survived this last year had we not learned to accept the help when offered. Our entire family dynamic has changed and it is difficult to find a balance when you have an older brother and sister who are perfectly healthy and need us more at their age now than they ever did before. We are spread thin at times, if you have a procedure, hospitalization, speciality appointment, or therapy we are often split between home with your brother and sister and the hospital or appointments with you. I thank God everyday that you have the most wonderful older brother and sister who have taken this all in without question and whom love you to the moon and back.

One of the hardest parts about this, for you and your parents,  is living in a society in which everything we do if food related. Parties, holidays, play dates, birthdays all involve a snack or meal of some sort. When we go somewhere we have to follow you around everywhere making sure you don't pick something up off the floor or that someone doesn't feed you something you cannot have. Your not quite old enough to understand that part yet. Our family dinners have turned into your brother and sister eating, usually by themselves, while we entertain you in the other room. If we eat dinner it is typically way later in the evening after everyone has gone to bed, or hiding in a corner somewhere trying to jam as much food in as we can before you find us. We have a hard time not feeling guilty eating in front of you when you can't have what we are having.

Our routine is medications, new feeding bag, cleaning your stoma, new extensions, setting up your pump and farrell bag to drain whatever is leftover in your stomach from the day. Doing this every single morning and night along with all your routine medications throughout the day is overwhelming at times, exhausting at times, ok at times, and sometimes we just cry through it all because we cannot possibly do this again, not one more time. When your pump beeps all night long (which happens more than it doesn't), when you vomit in your sleep, or you unplug your ports (which has become your favorite thing to do again) at night leading to midnight baths and bed changes, or screaming continuously for hours on end, the nights can be long and daunting. For you and us both I'm sure.

For now we will continue to feed you 18 hours a day through your J-tube to make sure you are the getting the calories you need to grow and thrive. Feeding you formula out of a box has been a hard thing for us to accept, especially when it includes 44 ingredients. But for now your on the best kind available with the most whole foods we can have where it is still safe to feed you into your intestines. You bypass many stages of the food breakdown process when your don't eat through your mouth. You've been especially happy now that you can have some pureed baby food and even some dissolvable baby puffs. I see the light in your eyes when you are able to eat just that small amount of food. I wish we could give you more but for now we will have to rejoice in the fact that you can even eat this small amount.

If there is one thing I have no doubt about, it is the fact that you were made for this. You never miss a beat and you have the most uplifting personality. You are classic in many things you do, and always bring a smile to peoples faces. While you had some challenges developmentally when you were younger, you have not let any of this slow you down. You are the one thing, universally, that makes everything that is going on OK. Knowing that you are the happy kid you are makes everyday ok, no matter how tough it is on these parents of yours. Happy tubieversary (pronounced to-be-versary your one year anniversary of having a feeding tube).


We love you,

Mama & Dada


Next week we go see the Motility specialists at UCLA to have them evaluate Liam.

Liam's year at a glance:

2nd hotpitalization for GJ tube placement

EEG

Snuggles

G-tube button

ER visit

Recovering from his 1st surgery

Super tubie is right!

Mom this thing is ridiculous, get it off my back!

Liam's Original G-tube button

our new large GJ tube

Finally got our nicer smaller infinity pump

Upper GI series

why oh why does my pump beep all night

so happy to see brother and sister

Happy boy, as usual

before your fundoplication

all your medications for the day

good by pump

cruising the hospital making lots of friends

before GJ tube surgery

our old really bulky Joey pump, so glad we got rid of this
before you had to start carrying it around with you!

I love my sports

He likes to show off his tube

I love outside

beach time

happy boy

I love versed

I LOVE to play with my tubie!

Six Great Weeks

I think this is the first time since before his big surgeries last November that we have had a full six weeks of no ER visits, unexpected appointments, or hospital stays. It was great timing since the kids were home for summer break and it allowed to get out and about more than we have really been able to. The three big changes we made in Liam's plan of care I feel are responsible for him feeling so good lately. I had hesitation changing him to gastrostomy-jejunostomy (GJ tube) feeding tube knowing this is a more permanent tube and will require a lot of ongoing care and procedures but it has really changed his life. He was able to get all the nutrition he needed and in response has gained a significant amount of weight and has given him the energy to live how a normal 17 month old should. We also started draining his stomach with a farrell bag which has helped relieve some of his symptoms as basically his stomach is always empty when it is attached.  This has been an ongoing challenge with Liam because he is quite curious about all these tubes and loves to disconnect them, in which we are left with bile all over his bed, our floor, his clothes, and even our friends. We have tried everything to keep the ports on his tube closed but he has figured out a way to remove all of them! We also started giving all his medications straight into his jejunostomy port for them to be better absorbed. This was a difficult transition at first from giving them orally all day long and Liam had a hard time understanding why he couldn't take his medication in his mouth anymore. But after several weeks I think we all have the hang of it and are in a good routine of medication giving. He still receives three medications given four times a day. Because he has been feeling so well lately we have been able to start giving him about 3oz of pureed baby food a day, usually most of it drains out his stomach after several hours but for the most part it has made him so happy to be able to orally eat something. Our eventual goal will be to give him more and more little bits of food so we can cut down on tube feeding but this will be a very long process (literally as I am typing this I just walked in the kitchen because Liam was making a strange noise and he just threw up all over the kitchen floor...). 

Liam's GJ tube

Oh how I like to unhook
myself

How we feel at some point
 on most days

As the summer came to an end Liam's appointments started creeping back up again. In the middle of August we saw Liam's neurologist and had a really good appointment with him. He showed me Liam's brain MRI and went over the images with me, he said everything looks good except the few abnormalities that he has, we will repeat his MRI when he is about 2 years old. He reviewed Liam's high metabolic blood work, he has an elevated CK and elevated isoenzymes of his CK as well as a lot of other abnormal metabolics that won't make any sense to you because they barely make sense to me. Going forward his neurologist would like to do some muscle biopsies to check for a few things but for right now we are going to hold off on doing that until he is a little bit older. He has already been through so much and any information we would find out from that won't change his course of treatment that much. He also brought up something called intestinal pseudo-obstruction (which basically means your intestines think there is blockage when there actually isn't one and they basically shut down until they decide the blockage is clear) which is something I have researched a lot as well and does fit Liam's pattern. Sometime in the next year we will probably be seeing some specialists at UCLA and CHLA, which his neurologist also brought up and will be speaking to Liam's GI specialist about it. They have a more specialized center and some of the top motility specialists in the world. We also saw a cardiologist to review Liam's MRA (magnetic imaging angiogram) to check on whatever is compressing his esophagus (which they discovered during an endoscopy) and they wanted to do an 12 lead EKG, an echocardiogram, and blood pressures on all four extremities. Needless to say it was a miserable appointment Liam was so upset he threw up three times and they weren't able to finish any of the tests they wanted him to do. But the cardiologist agreed that because his MRA looked good that he did not need to complete his tests he wanted (thank goodness) and after and hour and forty-five minutes we left exhausted.

Cardiologist Visit

This week I met with the Orange County Regional Center and they evaluated Liam for some therapy. Back in early August I think Liam was officially discharged from his occupational therapy center but the regional center is suggesting Liam continue with therapy. He will also be seeing a early intervention speech pathologist as well as a behavioral specialist. They all work together and will help Liam work on his sensory processing disorder as well as learning how to better communicate and hopefully help him with his "medical trauma". The regional center has been awesome to work with, they came to our home for Liam's initial evaluation and while I will have to take him in for his therapy they were extremely helpful and got us an appointment for next week. As much as I'm not looking forward to weekly therapy again I really feel this will be good for him.

Having some fun!

when our tummy hurts

 So although Liam is medically stable we continue to have our daily ups and downs, but I couldn't be more happy and grateful that what we have done so far has kept him his happy self. I hate seeing him in pain, I hate that he has to be kept alive by a feeding tube and pump and a ton of medications, and I really hate seeing him so traumatized by everything but seeing his progress over the last 2 months has really helped me put aside my anger, fear, guilt, and anxiety over it all. If over the last year we hadn't put our trust in our medical team, handed over our baby for several surgeries, or given his small little body dose after dose of potentially harmful medication this is not the baby you would be seeing today. I am so thankful that we live in an era in which medical interventions and technology are available to us so that we have the opportunity to continue and watch our precious baby boy grow and develop.

August is Gastroparesis awareness month, as you may have seen on Liam's Facebook page https://www.facebook.com/littlebuddyliam?ref=hl. This is an awful disease that many people are fighting every day of their lives, there is no cure and no good treatment options. Just in these last two months I know of 3 people that were in their 30's that have passed away from this disease. If not adequately managed it will slowly kill you. Please visit the G-Pact website to find out more information or if your interested in donating they have fantastic and cute merchandise for sale and the profits go to support research for gastroparesis. https://www.g-pact.org

Maneuvering through the world of insurance

Dealing with insurance companies has probably been one of our most frustrating struggles through this whole ordeal. We were lucky that Matt landed a new job in September after his company closed down but with that it lead to a change of our insurance plan in the middle of one of Liam's hospital stays.  We started out with Kaiser which was OK until we needed intervention for Liam, they are more of a wait and see organization, which don't get me wrong is a great way to look at medical problems in certain cases. By the time Liam was 5 months old I had repeatedly gone to his pediatrician saying that something was not right, he wasn't growing like he should, he was losing weight, he cried in pain all the time, he constantly vomited.  It was that mom gut of something is just not right. She patiently listened and tried some different medications for Liam but nothing really worked. Right after he turned 6 months old he had another checkup and this time she was concerned because he again lost more weight. She ordered some specialized blood work and sent us the hospital that morning to have it done. As I was arriving home she called and said she had spoke with the pediatric specialist at the hospital and they want Liam to be admitted to the hospital right now. So we went from me constantly telling his pediatrician that something wasn't right to hospitalization. I was in shock and I really felt like if she had listened to me sooner we could have avoided this drastic of a measure. We were there for 6 days until October 1st when our insurance changed and out we went with a not so great plan but they were done with us we were no longer paying for their services. We got our medical records (which was more than 500 pages from Liam's birth to 6 months old) and moved on.

Moving from Kaiser to an outside HMO is like being hit by a truck. At least with Kaiser everything is internal and it is way easier to maneuver,  however you have to stay within their internal circle without the ability to get second opinions. At least before we were kicked out of the hospital our GI doctor reached out to a another GI doctor, that he knew well, so he could see us within a few days of us being discharged from the hospital.  We had already found a new pediatrician knowing that our insurance was changing so the hospital scheduled us an appointment with her for day after we were discharged. Talking to a new pediatrician that didn't have our medical records or know our history was exhausting. She had to put in 4 urgent referrals because at that time Liam was also seeing a neurologist, having OT and PT, and needed to see a GI doctor. Our very first bad experience with our new Cigna HMO and our pediatrician's office (which has since proven to be extremely difficult to work with) was when our GI appointment was cancelled because our new insurance did not authorize the visit. We were discharged on a Wednesday and we were suppose to see our new GI doctor on that following Friday. On Thursday I had this nagging feeling that I should call the GI office and make sure they received all of our insurance information and to verify our appointment, well it turns out they didn't and they had cancelled our appointment because they never received authorization from our insurance company to see this doctor (keep in mind this was all urgent since we left the hospital without a good plan in place). Well after I of course talked to the office manager (and frankly told her they had to see my son that day) they rescheduled my appointment for later that Friday. I have to say that this GI specialist was amazing.

I quickly learned that anything that was done- referrals, appointments made, prescriptions, insurance authorizations etc. etc. needed at least 5 follow up calls each to make sure that people were actually doing their jobs and making this happen. It is extremely frustrating and extremely time consuming which is really difficult when I'm trying to also care for Liam. After only about 6 weeks with our new GI doctor it was determined that Liam was a "medication failure" and needed surgery, which basically means no medication could manage his symptoms effectively. We were referred to a great surgeon at Miller Children's Hospital so we felt as comfortable as we could letting our 8 month old have surgery. A few days before we were going in to meet this surgeon and schedule surgery our GI doctor (himself) called and said that our insurance denied us to have surgery with this surgeon and we would have to find a new one on our own. This was on a Friday afternoon and was absolutely devastating. Knowing that me fighting the insurance over this would lead to months of appeals and phone calls and postpone much needed care for my son and being the "demanding" mother that I am I couldn't accept this and I found a new surgeon that afternoon and had an appointment scheduled for Monday afternoon. We were happy with our choice but we had to wait 4-6 weeks to get the authorization from insurance (standard time frame) then he was booked out 5-6 weeks so we were looking at 2-3 months before he would be able to have surgery. Our GI doctor was not OK with this he personally called the surgeon who then switched our surgery to urgent and we got in within the next week. Like I said our GI doctor was amazing.

From here everything went OK (minus the several long phone calls a week I made to keep everything moving along) until Matt's company decided to change insurance companies. Starting in January we had to switch from our Cigna HMO to Blue shield HMO which was equally if not more frustrating and difficult to deal with. Our primary pediatrician (who you have to go through for all referrals) only accepted blue shield under one medical group so we had to switch to that medical group within Blue Shield which was and absolute disaster for us. We could no longer see our beloved GI doctor who had cared so much for Liam, and after calling, writing letters, having our pediatrician call and explain the medical necessity of him needing to stay with this GI specialist they denied all our claims and said that because they could offer "equivalent" services we would have to change specialists. This was a huge nightmare for me as our GI doctor couldn't lean us in the direction of a good new doctor we were going in blindly and I was really disappointed. So like we have done so many times before we picked ourselves up again and researched every doctor available.  Our pediatrician had to again put in referral after referral because at this point Liam was seeing 6 different specialists. Which then I had to call her office every day to make sure the referrals were done correctly and sent to my insurance. So we saw all our new specialists which took about 6-8 weeks since that was the first available appointments. I finally found a GI doctor I sort of like, we have been hospitalized, had tests, blood work, MRI's, EGD's, EEG's, a GJ placed, had lots of new medical supplies, go to OT and PT weekly, switched formula's and medications and all with me fighting our insurance company tooth and nail.

Our current battle is trying to figure out how much of our out of pocket max we have met (which I have calculated myself but I need an "official" number for insurance) which we are almost there since everything for Liam is so expensive. This has proven to be difficult since our medical group will not release to me our claims summary and explanation of benefits and right now they won't release that information to Blue shield as well which I just cannot wrap my head around so we are in an appeals process for that. For now we are just going to wait it out until we can switch to a PPO, which I know will also have its battles but at least we will be free to see the doctors I want to see that will care for my son to the best of their ability. Right now we are just a number in a giant organization that does not specialize in Liam's condition and they don't have any other insight except to just tackle each of his symptoms individually. I am so glad that I am a nurse and understand the medical community as well as I do.  I feel really badly for the families who don't and don't understand how to fight the insurance companies with the appropriate terminology and how to maneuver getting what you need for your sick child. It is a specialized art form, one that I have become very good at because I like a lot of other mothers out there with sick children will not allow insurance companies to push me aside and not give me the best possible care for my child.

Liam's Surgery Today

Happy Boy

being silly on versed

Waiting to go in

buy buy big GJ tube!

This morning Liam underwent his 5th time under general anesthesia to have surgery to get a new low profile transgastro-jejunal mic-key button. His peg GJ was irritating his stoma and he has already had one infection from it. He also has been having an "ileus" episode in which part of his intestines shut down making him not able to tolerate any of his feeds.  No one knows why this happened but everything was basically traveling backwards through his GI system so he had bile coming out his stomach which shouldn't happen. This new button will be so much easier to manage and so much better for his small body. We arrived at the hospital this morning at 6:30 and we were home before noon which was a nice change to the normal 3-6 day hospital stays. He has been having a lot of pain this afternoon but has finally settled into a nap. He of course was his happy smiley self for almost the entire time (except when anyone medical tried to touch him lol) which made it all the more bearable.

love from daddy

finally napping at home

sleepy after surgery

You would think by the 5th time of going through general anesthesia it would get easier, but it doesn't and in a way it almost gets harder because the more you do it the more you know. You can foresee every step before it even happens; the waiting room filled with anxious parents, the pre-op area where  they hook him up to machines and shove a needle in his arm, to watching them wheel him down a long hallway until you can no longer see him, to becoming one of those anxious parents waiting in the waiting room to find out how everything went. Every time it makes you a little bit stronger and gives you the ability to love and fight for your child a little bit more. We had an exceptional pre-op nurse who was so interested in Liam's case she asked all kinds of questions which was so refreshing and such a change from the normal medical staff not knowing what to say or do for us because a lot of Liam's issues are still a mystery. She was laughing because under his list of diagnosis it has one that says "unknown" and she was like well that must not be frustrating at all! The procedure and PACU nurses were also exceptional which always makes me happy since that's what I did/do for a living (whenever I'm able to work again lol). The PACU nurses have never seen a baby with a GJ tube so that was interesting but again they wanted to know why and asked so many questions which was great that they were so honest with us. They asked about our 'I love a tubie" bracelets and what they meant and they told us that Liam was lucky to have us as his parents. Not sure what our next steps will be, but for now we know that he is growing and thriving and we are hoping that he will tolerate his feeds with this new tube. 

GJ Button

Kaitlyn & Matthew

I have been thinking a lot about the role that Kaitlyn and Matthew have had to play in Liam's life. Over the 14 months that Liam has been alive they have shown him more love and adoration (sometimes too much lol) than any little brother could ask for. At a drop of a hat they have to had to be shuffled around to people's houses, sleep over places or stay home with other people, miss sports practices and events, they have had to adjust and change their daily life all because of the extra care their little brother requires. They have had to come to countless doctors appointments with him, have had to visit him in the hospital, seen him hooked up to machines and pumps, and have watched and participated in his daily routine everyday at home.  Matthew spent his 6th birthday in the hospital with his little brother because that's where he wanted to be. These are the most amazing 8 (almost 9) and 6 year old kids and I have the pleasure of calling them my own. They have never once hesitated or been afraid they worry for him and just want to be there for their little brother.

I forget sometimes that Kaitlyn is only 8 years old, she has handled this all with such grace. She helps me daily venting Liam's tube, getting his flushes ready, gets me the supplies I need whenever I forget something. She holds Liam when he is screaming and I just need a minute to get his meds or fix his pump. She wears her "I love a tubie" bracelet with such pride and tells all her friends about Liam and his struggles. She is truly inspirational and I hope that this whole experience will stick with her and continue to follow her as she grows up into the wonderful lady I know she will become.

Matthew has been equally as helpful (although not as motherly as Kaitlyn) and never batted an eye to what his little brother has had to endure. He stands right by him and entertains him when I need to clean his site and flush his tube or give him meds. He loves his brother so much that he rarely lets him be more than 2 feet away from him. Matthew being younger than Kaitlyn has done remarkably well being shuffled from one house to another while I have been hospitalized with Liam.

A huge reason the kids have done so well with this all is our large support group.  They are so familiar with our friends and their kids that going to their houses when needed is more of a treat than a burden for them. I cannot thank everyone enough who has taken time away from their own families to help watch out for Kaitlyn and Matthew while we haven't been able to.

I sometimes forget to take a minute and realize that what Liam has had to go through has affected them equally as much.  I couldn't have asked for more understanding, loving, kind, and generous kids and I need to tell them that much more often than I do.