Landon is doing great, here at home, and I love being a SAHM! It feels like I should be working on progress notes or calling clients, but the meds and feedings are certainly keeping me busy enough! Which, by the way, is all going well except for intermittent oopsies. Our little man is finally get hungry which is different for us. Last night, something happened with the feeding tube and from 8:30pm until 11:30pm, he got nothing and we had no idea. I walked in to a screaming baby (screaming as if he was in severe pain!) and couldn't figure out what was wrong. I noticed that half of his formula was in the farrell vent (bag for venting) and the other was in the feeding bag. Ahhh... Nothing we did consoled him until Jimmy gave him a sippy cup with some formula (ta da!!). It did the trick and he went back to sleep. Jimmy are pretty amazed at what we, with no medical background, are trusted to do! His meds are pretty complicated (it feels like it at least!) and between those and the feedings and using the tube (oh my!). I am getting more comfortable with it, but still.. Most of the time, however, he is his silly self! He is amused when he is connected to the feeding tube, during the day, and I follow him around with the stand. He crawls quickly and laughs as if I am chasing him! My plan to entertain him with Elmo or books (so that he is not mobile) has not really worked! It's okay though.. I don't mind chasing him around with the stand as long as it makes him smile!
One of my least favorite, of all of this, is the diaper rash! My poor baby never had diaper rash but now has very loose stools so his bum seems like it is always red! No sooner do we clear it up, I change his diaper and there it goes again. Ahh.. poor baby! I hope this ends soon!
I got a call from the geneticist that we met in the hospital. She's a great lady who is Lebanese, from Beruit, and has a great accent! I am part Lebanese for those who did not know.. very small part, but I love it! Anyway, the first test they did to discover where the mutation lies indicated that deletion (which from what I read, is the most common mutation) was not present. From what I gather, from reading, there are over 80 different mutations that can cause cystinosis. The most common (approximately 50%) being from deletion which means that a large part of the CTNS gene (sometimes referred to as the 57-kb deletion) is deleted, resulting in the complete loss of cystinosin. This is not the case for Landon so now they are looking into other mutations and we should know the results within the next 2 months when we go to our first appointment with the geneticist. We will get a lot of information about the disease and what the mutation means at his appointment. I wish I had more knowledge about genetics but it seems so complicated! I hope that I am understanding all of this because otherwise, this last paragraph was just some things that I made up! I apologize if that's the case and promise to give you information as it becomes available. So, we still do not know if Jimmy and I are both carriers or if one of us is a carrier and something crazy happened in utero (don't you love the use of my scientific terminology?!). They will offer to have us go through genetic testing and I really hope to do that, but I don't know how expensive it will be and if insurance covers it. **Fingers crossed!** I have to say that it kind of sounded like they saw something on one side and not the other but it may be a result of my misinterpretation and wishful thinking! And she certainly did not say that it's probable that only one of us is a carrier. Actually it is pretty rare to only have one carrier. But, Cystinosis is also very rare so who knows???
I hope you got something out of this post (anything?!) and it wasn't just rambling! =0)
Wednesday, June 29, 2011
Sunday, June 26, 2011
Honey- We're Home!
Saturday morning we found out, after 25 days in the hospital, that we could go home. As excited as I was to go home (I hadn't left the hospital since admission eithe) was as anxious as I felt too. Our son has different needs than he did when we walked through the door and, he wasn't remarkably better. He was still vomiting and actually threw up minutes before we were leaving. The doctor still allowed us to go. I really think that the renal doctors were considering our mental well being when sending us home. They are awesome people!
The minute we stepped in the door and Jimmy showed Landon his favorite toy, his race track, he lit up and looked like the Landon that we've known for 14 months. His aunts, uncles, cousin and grandpap came to visit and he happily crawled around, smiled and listened as he and Jillian were read books. Going home really was the best medicine!
The home nurse came to show Jimmy and I how to use the feeding tube equipment. Between the equipment and all of the medicine we were feeling a bit overwhelmed. Landon is taking 6 different medicines currently which include Cystagon (for the Cystinosis), Prevacid (to reduce the acid and protect his belly, a couple of suplements, and Enrythromicin (for the delayed gastric emptying. He gets, what are called, bulson feeds 4 times throughout the day which involves a larger quantitiy of formula given over 1- 1 1/2 hours and then recieves continuous feeds over night. Unforutunately his feeding tube will only hold half a night's worth of formula and he has to take his Cystagon every 6 hours so a full night sleep is not going be in the picture for this mommy. I guess I will have to learn to nap when Landon does during the day!
The night involved Landon screaming bloody murder several times, nasty diaper rash from intermittent diarahea, a vomiting episode- although not much considering his mommy mixed up the units on the feeding machine and gave him a large quanitity of formula too quickly and a disconnected tube that shot out formula. So all in all, not a great night! However, today was a new day and it went MUCH smoother. We have the feedings and meds scheduled throughout the day and it all seems manageable.
I have to brag that we are a pretty good team! I know that we will have good days and bad days, but my bet is that most of them will be pertty good!
The minute we stepped in the door and Jimmy showed Landon his favorite toy, his race track, he lit up and looked like the Landon that we've known for 14 months. His aunts, uncles, cousin and grandpap came to visit and he happily crawled around, smiled and listened as he and Jillian were read books. Going home really was the best medicine!
The home nurse came to show Jimmy and I how to use the feeding tube equipment. Between the equipment and all of the medicine we were feeling a bit overwhelmed. Landon is taking 6 different medicines currently which include Cystagon (for the Cystinosis), Prevacid (to reduce the acid and protect his belly, a couple of suplements, and Enrythromicin (for the delayed gastric emptying. He gets, what are called, bulson feeds 4 times throughout the day which involves a larger quantitiy of formula given over 1- 1 1/2 hours and then recieves continuous feeds over night. Unforutunately his feeding tube will only hold half a night's worth of formula and he has to take his Cystagon every 6 hours so a full night sleep is not going be in the picture for this mommy. I guess I will have to learn to nap when Landon does during the day!
The night involved Landon screaming bloody murder several times, nasty diaper rash from intermittent diarahea, a vomiting episode- although not much considering his mommy mixed up the units on the feeding machine and gave him a large quanitity of formula too quickly and a disconnected tube that shot out formula. So all in all, not a great night! However, today was a new day and it went MUCH smoother. We have the feedings and meds scheduled throughout the day and it all seems manageable.
I have to brag that we are a pretty good team! I know that we will have good days and bad days, but my bet is that most of them will be pertty good!
Friday, June 24, 2011
Keepin it simple..
Well the G tube is in! The surgery was quick (about 50 minutes) and Landon did well! He is sore and was cranky but we got a few smiles and babbled before he went to bed tonight. We just might get home this weekend! Ohh I can't wait!! We adore the staff here on 7a, and we will miss them, but we have to get out of here...
Oh, and should add a note of thankfulness tonight. Thanks to the Internet, we have been able to connect with so many families who have been touched by this disease. The feeling cannot be described! I feel an immediate connection and so supported by people who I know very little about. On that note, good night!
Oh, and should add a note of thankfulness tonight. Thanks to the Internet, we have been able to connect with so many families who have been touched by this disease. The feeling cannot be described! I feel an immediate connection and so supported by people who I know very little about. On that note, good night!
Thursday, June 23, 2011
Give me a G! G.. You got your G.. You got your G!
G tube that is!! I am kind of afraid to get excited, but I guess I don't hesitate to get upset so this is a better feeling. Landon hasn't been on continuous feeds today. Usually he is getting 50cc per hour but today he gets a break for about 3 hours and gets double-almost triple that for 1.5 hours. He is given the opportunity to drink 5oz of formula and then it is put in the feeding tube. The formula is gross so he has only drank half of an ounce each time.
Since he is doing well, they feel that a G tube is the best option. Yayyyy!!!! He is on the schedule for tomorrow, and he begin feeds through the new tube right away. Sooo... there should be no reason why we can't go home this weekend and at the very latest, Monday. Surgery is going to perform the procedure and will put a camera into his belly and change the tube so the recovery shouldn't be bad. The miracle of modern science, right? I just hope we continue to get good news!
Since he is doing well, they feel that a G tube is the best option. Yayyyy!!!! He is on the schedule for tomorrow, and he begin feeds through the new tube right away. Sooo... there should be no reason why we can't go home this weekend and at the very latest, Monday. Surgery is going to perform the procedure and will put a camera into his belly and change the tube so the recovery shouldn't be bad. The miracle of modern science, right? I just hope we continue to get good news!
Wednesday, June 22, 2011
How to Help
Once we get through the next couple of months which include getting things figured out with Landon and some very special events, we plan to begin fundraising. If you know Jimmy and I, and our incredible families and friends, you know that we can't just sit back. We are joining a fight to cure Cystinosis and to raise awareness about this rare disease that has changed Landon's life, and ours.
You can check out the website www.natalieswish.org to learn more, watch a video about people dealing with this disorder, learn about current research (it's really exciting), fundraising and donations.
Thank you so much for caring so much and for the prayers and thoughts! We love you and are so grateful!
Jimmy, Lauren and Landon Hartz
You can check out the website www.natalieswish.org to learn more, watch a video about people dealing with this disorder, learn about current research (it's really exciting), fundraising and donations.
Thank you so much for caring so much and for the prayers and thoughts! We love you and are so grateful!
Jimmy, Lauren and Landon Hartz
The Waiting Game
I didn't post earlier because I didn't have a good idea of the next step and was on what Jimmy would call an "emotional roller coaster." I don't know if I have ever felt so sad and so elated, all in the same day.
I was thrilled at the idea of placing a G tube rather than a GJ tube. "If he tolerates having the feeds go to his stomach, then we will place a real G tube..." So, imagine how we felt as Landon threw up after drinking water last night. Then he threw up 3 more times...
What's the big deal about a GJ tube? Nothing at first.. But then the surgeon who placed this current tube (which moved out of place) told me that a G tube would be ideal because GJs move and come out easily. They not typically like to place them in a child who is so active. The thought of making a regular appearance because of this tube was, well is, more than I can handle.
I can easily make this post incredibly long but will get to the point. Landon is currently getting fed 24 hours per day at 50cc. His formula has 30 calories per ounce so he is getting 1200 calories per day (yes, more than he needs!). This could be the reason why he is vomiting. They switched his formula to one that provides 24 calories per day and will do continuous feeds for 12 hours a day (so will cut his intake in half) and we will offer him food orally. He often doesn't eat much but that is no surprise since he is getting so much through the tube. Aside from 12 hours of continuous feeds and oral intake, he will get short bursts of formula through the feeding tube to supplement any additional calories that he needs. I cant quite wrap my head around what this will look like but we shall see tomorrow. If he tolerates this then it sounds like he will get a real G tube rather than a dysfunctional GJ, but if not, he will get a new and improved GJ. We are praying that he tolerates this tomorrow. In an ideal word (I can't believe what our new ideal world looks like!).. Landon will tolerate these feeds tomorrow, get a G tube on Friday and go home Friday night or Saturday morning. Prayers! Prayers! Prayers!!!
I was thrilled at the idea of placing a G tube rather than a GJ tube. "If he tolerates having the feeds go to his stomach, then we will place a real G tube..." So, imagine how we felt as Landon threw up after drinking water last night. Then he threw up 3 more times...
What's the big deal about a GJ tube? Nothing at first.. But then the surgeon who placed this current tube (which moved out of place) told me that a G tube would be ideal because GJs move and come out easily. They not typically like to place them in a child who is so active. The thought of making a regular appearance because of this tube was, well is, more than I can handle.
I can easily make this post incredibly long but will get to the point. Landon is currently getting fed 24 hours per day at 50cc. His formula has 30 calories per ounce so he is getting 1200 calories per day (yes, more than he needs!). This could be the reason why he is vomiting. They switched his formula to one that provides 24 calories per day and will do continuous feeds for 12 hours a day (so will cut his intake in half) and we will offer him food orally. He often doesn't eat much but that is no surprise since he is getting so much through the tube. Aside from 12 hours of continuous feeds and oral intake, he will get short bursts of formula through the feeding tube to supplement any additional calories that he needs. I cant quite wrap my head around what this will look like but we shall see tomorrow. If he tolerates this then it sounds like he will get a real G tube rather than a dysfunctional GJ, but if not, he will get a new and improved GJ. We are praying that he tolerates this tomorrow. In an ideal word (I can't believe what our new ideal world looks like!).. Landon will tolerate these feeds tomorrow, get a G tube on Friday and go home Friday night or Saturday morning. Prayers! Prayers! Prayers!!!
Tuesday, June 21, 2011
We have graduated...
To free parking, that is... After 21 days as an inpatient you get free parking at Children's. How depressing! It has been quite a journey. We are definitely moving in the right direction though.
The plan of using the G part of the tube is going well so far! No vomiting! I'm afraid to even type those words. The surgeon stopped by and said that he will be thrilled if Landon continues to do well with feeding to the stomach because it is much easier to manage a G tube especially with such an active little guy (active to say the least!). It sounds like they want to test the G tube until Friday and if all goes well then he will have a real G tube placed. He said that it would not be a difficult surgery and that the site is still fresh and looks good. I hate the idea of doing this again but knowing that there really wasn't a way around it and that the G tube will be much less of a headache is comforting. Plus it will allow his body to do what it naturally should do- instead of bypassing the stomach.
So, it is all good news so far except that we aren't going home yet. Who know? Maybe there is another perk for being here for 4 weeks. :0)
The plan of using the G part of the tube is going well so far! No vomiting! I'm afraid to even type those words. The surgeon stopped by and said that he will be thrilled if Landon continues to do well with feeding to the stomach because it is much easier to manage a G tube especially with such an active little guy (active to say the least!). It sounds like they want to test the G tube until Friday and if all goes well then he will have a real G tube placed. He said that it would not be a difficult surgery and that the site is still fresh and looks good. I hate the idea of doing this again but knowing that there really wasn't a way around it and that the G tube will be much less of a headache is comforting. Plus it will allow his body to do what it naturally should do- instead of bypassing the stomach.
So, it is all good news so far except that we aren't going home yet. Who know? Maybe there is another perk for being here for 4 weeks. :0)
Monday, June 20, 2011
Prayers and Crossed Fingers
After MUCH discussion, we decided not to have the GJ tube "fixed" and allow it to function as a G tube. Landon was vomiting as a result of the delayed gastric emptying but has done well keeping fluids down lately. We are starting off very slow, at only 5cc and raising it another 5cc every four hours. He is currently getting fluids from an IV so as he begins to take more formula, the amount of fluids will decrease. Our goal is to get back up to 50cc. If his stomach can't handle the feeds, meaning that he begins to vomit, then he has to have the tube fixed and we are here several more days!
He spent the first half of the day not drinking because we were expecting that he would have to go in for a "procedure" at any time to manipulate the tube. This meant that mommy spent the first half of the day walking the halls and attempting to distract a very agitated little boy. When he was finally given the ok to drink I wanted to kiss and strangle someone. :0)
He spent the remainder of the day in a great mood. He is such a silly little boy. He is a mixture of myself and his daddy. He loves to laugh and does so easily (like me) and he likes to make others laugh (like Jimmy). You see why we work so well?!?!
I am praying and crossing fingers and toes that his little belly is ready to handle feeds, meds, food and liquids! We are so ready to go home. God bless the families who spend months in the hospital!
He spent the first half of the day not drinking because we were expecting that he would have to go in for a "procedure" at any time to manipulate the tube. This meant that mommy spent the first half of the day walking the halls and attempting to distract a very agitated little boy. When he was finally given the ok to drink I wanted to kiss and strangle someone. :0)
He spent the remainder of the day in a great mood. He is such a silly little boy. He is a mixture of myself and his daddy. He loves to laugh and does so easily (like me) and he likes to make others laugh (like Jimmy). You see why we work so well?!?!
I am praying and crossing fingers and toes that his little belly is ready to handle feeds, meds, food and liquids! We are so ready to go home. God bless the families who spend months in the hospital!
Sunday, June 19, 2011
Set backs
Happy Father's Day to all of the amazing daddies out there! Landon certainly has an amazing daddy and so do Jimmy and I!
Yesterday morning, Landon had an Xray done to make sure that the GJ tube was still in place. Unfortunately it is not and those who can fix that do not work on the weekends. The stomach portion looks good but the tip that goes to the Jejunum is not. We were hoping to start a small dose of feeds yesterday but no such luck. He was finally cleared to take liquids yesterday during the late afternoon but no food until the tube is in place. Landon is not a happy camper when he can't drink water or juice from his cup!
We are also still waiting for his Cystagon (the drug that treats the Cystanosis. It was supposed to come Friday but the insurance company keeps requesting more information. Plus it s the weekend so things slow down significantly. We are shooting for Monday, and at this point, hope to go home on Wed or Thurs.
We are all remaining positive (most of the time! Hehe). Some things are out of our control. Plus, Landon's mood is improving and we are getting some smiles.
We will continue to update everyone!
Yesterday morning, Landon had an Xray done to make sure that the GJ tube was still in place. Unfortunately it is not and those who can fix that do not work on the weekends. The stomach portion looks good but the tip that goes to the Jejunum is not. We were hoping to start a small dose of feeds yesterday but no such luck. He was finally cleared to take liquids yesterday during the late afternoon but no food until the tube is in place. Landon is not a happy camper when he can't drink water or juice from his cup!
We are also still waiting for his Cystagon (the drug that treats the Cystanosis. It was supposed to come Friday but the insurance company keeps requesting more information. Plus it s the weekend so things slow down significantly. We are shooting for Monday, and at this point, hope to go home on Wed or Thurs.
We are all remaining positive (most of the time! Hehe). Some things are out of our control. Plus, Landon's mood is improving and we are getting some smiles.
We will continue to update everyone!
Friday, June 17, 2011
Busy Day...
Landon went into surgery today to have a GJ tube inserted. To learn more about GJ tubes, go here: http://www.cincinnatichildrens.org/health/info/abdomen/home/gastro-jejuno-tube.htm.
The GJ tube will be used for feeding, while Landon requires a feeding tube, and for administering the medication and providing fluids in the event he becomes dehydrated. It will make life much easier, but certainly not what a parent prefers to see. I'm glad the surgery is over and we pray the there are no issues with the tube and it stays put.
From what we understand, the medicine that Landon will be taking tastes terrible. And medicine compliance is essential so rather than chance his refusal to take it or him throwing it up, we chose the GJ tube. I just pray that Jimmy, the doctors and myself make the best decisions for him. Luckily, there are excellent doctors treating him and we absolutely trust them to guide us.
He was supposed to begin taking the medication today but the insurance company is being picky and keeps requesting additional paperwork. Thank God that the nurses are handling this for us. We also applied for Medacaid to receive additional assistance for the drug and co-pays. What a great support!
We hope to go home on Monday but are prepared to stay until he is good and ready to come home! We want to go home and stay home! We are ready to move forward and get back to our new normal. We have water tables to play with, our dog to chase around the house, play dates to look forward to, and baths in the big ducky!
The GJ tube will be used for feeding, while Landon requires a feeding tube, and for administering the medication and providing fluids in the event he becomes dehydrated. It will make life much easier, but certainly not what a parent prefers to see. I'm glad the surgery is over and we pray the there are no issues with the tube and it stays put.
From what we understand, the medicine that Landon will be taking tastes terrible. And medicine compliance is essential so rather than chance his refusal to take it or him throwing it up, we chose the GJ tube. I just pray that Jimmy, the doctors and myself make the best decisions for him. Luckily, there are excellent doctors treating him and we absolutely trust them to guide us.
He was supposed to begin taking the medication today but the insurance company is being picky and keeps requesting additional paperwork. Thank God that the nurses are handling this for us. We also applied for Medacaid to receive additional assistance for the drug and co-pays. What a great support!
We hope to go home on Monday but are prepared to stay until he is good and ready to come home! We want to go home and stay home! We are ready to move forward and get back to our new normal. We have water tables to play with, our dog to chase around the house, play dates to look forward to, and baths in the big ducky!
Thursday, June 16, 2011
Be Thankful
I don't want to lose sight of all the things in which I have to be thankful. There are plenty!
1. One of the top hospitals with incredible nurses, doctors and support staff. They have become extended family and although we can't wait to go home, they make our days better.
2. An incredible support system (grandparents, aunts, uncles, cousins, friends, my April BDC etc).. They have kept our bellies full, our room cheerful and have given hugs, tears and reassurance. What would we do without you? They run back and forth daily, take care of our dog, allow us to vent, and allow us to focus on our baby.
3. An incredible husband and father who will do ANYTHING to make his little boy laugh (and who entertains the nurses from the minute he steps on 7a... He is my rock! Everything seems just little better when he is here. I am so glad that Landon has his father's charm (and looks!).
4. My resilient little fighter... He charms the pants off of everyone he comes into contact with, has the biggest smile and the most infectious laugh but don't cross him! The poor souls who take blood and perform procedures won't get it without dealing with screaming and kicking. Don't ever stop fighting, my baby!
5. Forgiveness. We hold him down and make him go through some pretty crappy things but once it is over, the smile is back and so are the cuddles.
6. A treatable disease that won't stop Landon from reaching his fullest potential.
7. Milestones... Being in the hospital has not stopped Landon from performing new tricks. He now sticks his tongue out on cue, points at people and objects (I know- two wonderful habits!), and shakes his head No when he does not want something...
There are plenty more but I want to save them for a day when I'm not feeling so blessed...
1. One of the top hospitals with incredible nurses, doctors and support staff. They have become extended family and although we can't wait to go home, they make our days better.
2. An incredible support system (grandparents, aunts, uncles, cousins, friends, my April BDC etc).. They have kept our bellies full, our room cheerful and have given hugs, tears and reassurance. What would we do without you? They run back and forth daily, take care of our dog, allow us to vent, and allow us to focus on our baby.
3. An incredible husband and father who will do ANYTHING to make his little boy laugh (and who entertains the nurses from the minute he steps on 7a... He is my rock! Everything seems just little better when he is here. I am so glad that Landon has his father's charm (and looks!).
4. My resilient little fighter... He charms the pants off of everyone he comes into contact with, has the biggest smile and the most infectious laugh but don't cross him! The poor souls who take blood and perform procedures won't get it without dealing with screaming and kicking. Don't ever stop fighting, my baby!
5. Forgiveness. We hold him down and make him go through some pretty crappy things but once it is over, the smile is back and so are the cuddles.
6. A treatable disease that won't stop Landon from reaching his fullest potential.
7. Milestones... Being in the hospital has not stopped Landon from performing new tricks. He now sticks his tongue out on cue, points at people and objects (I know- two wonderful habits!), and shakes his head No when he does not want something...
There are plenty more but I want to save them for a day when I'm not feeling so blessed...
Wednesday, June 15, 2011
It's in the genes
I will not always post multiple times a day but this really helps me to retain everything. I met with a geneticist today. We met with her last week before Landon was diagnosed just to make sure renal wasn't missing anything. Today I learned that one of two things happened that resulted in Landon getting this disorder. Either Jimmy and I are both carriers which means that we have a 25 percent chance of having another child with Cystinosis and a 50 percent chance of having a child who is a carrier, or one of us is a carrier and the other is not but that it occurred in utero. If that's the case then our chances of having another child with Cystinosis is 3-5 percent.
The more I learn about this disorder, the more unbelievable it seems.
The more I learn about this disorder, the more unbelievable it seems.
What does all of this mean?
Landon was diagnosed early. The youngest diagnosed here in Pittsburgh was 22 months old. We are so grateful for very proactive doctors! Landon will begin taking the only medicine that treats this, Cysteamine. He will take this medication 4 times a day and it works by removing cystine from his body. They Cystine presents in the form of crystals, all throughout his body including in the eyes. Landon does not yet have crystals in his eyes, but when they do form, he will begin using eye drops 6 times per day. He will probably have a high sensitivity to sunlight when they develop. At some point in his life, probably in is teens, he will need a kidney transplant. We have been told that availability of a kidney is generally good and that often times parents can donate a kidney. Recovery time is often easier for the recipient than the donor! It will probably require about a week in the hospital.
We were told that Landon will have a normal life with some bumps in the road. Medicine compliance is essential and really determine his quality of life, how the disorder will progress and longevity of his life. He currently has a feeding tube called a NJ. It goes through his nose to his intestines and bypasses his stomach. He was vomiting so this ensured that he would keep down the formula. This is temporary. We just need to get him to his fighting weight! On Friday, he will have a surgical procedure to insert a GJ Tube. This will go into his belly to the intestine. He will only receive feeds for the next month or so but the purpose of this is to ensure medicine compliance. They said that the liquid form of the medicine tastes terrible. He could refuse to take it or vomit as soon as he does. It will also help to keep him hydrated. Kids with Cystinosis drink a lot and urinate a ton. If he needs fluid then it will be easy to get them to him. I read that most children urinate about every 3 hours, kids with Cystinosis go every hour to hour and a half.
Despite this being a rare disorder, there is a lot of research and support available, from what we have been told. It is treatable, not curable, but our baby is only a year old. They have plenty of time to figure it out!!
We were told that Landon will have a normal life with some bumps in the road. Medicine compliance is essential and really determine his quality of life, how the disorder will progress and longevity of his life. He currently has a feeding tube called a NJ. It goes through his nose to his intestines and bypasses his stomach. He was vomiting so this ensured that he would keep down the formula. This is temporary. We just need to get him to his fighting weight! On Friday, he will have a surgical procedure to insert a GJ Tube. This will go into his belly to the intestine. He will only receive feeds for the next month or so but the purpose of this is to ensure medicine compliance. They said that the liquid form of the medicine tastes terrible. He could refuse to take it or vomit as soon as he does. It will also help to keep him hydrated. Kids with Cystinosis drink a lot and urinate a ton. If he needs fluid then it will be easy to get them to him. I read that most children urinate about every 3 hours, kids with Cystinosis go every hour to hour and a half.
Despite this being a rare disorder, there is a lot of research and support available, from what we have been told. It is treatable, not curable, but our baby is only a year old. They have plenty of time to figure it out!!
Cysti what?!
We were confused when a renal doctor entered Landon's room. "GI consulted us because of a urinalysis that was done..." We learned later that renal almost never is consulted when a kiddo is diagnosed as failure to thrive (this diagnosis was a whole other pill for me to swallow, by the way). There are a million reasons why a child is diagnosed as FTT and the kidneys usually have nothing to do with it. We were then told that Landon has what is called Fanconi Syndrome. This means that certain substances that are usually absorbed through the bloodstream are passed through the urine. This is why Landon was not growing. He was missing out on getting a lot of those important substances. We learned that with supplements, for his life time, this issue could be addressed. Then we learned that FS does not usually stand on it's own. It is usually an indicator of another kidney disorder. The one that made sense was Cystinosis. He was a poster child for Cystinosis. The biggest indicator, aside from the FS already diagnosed, was our son's beautiful blue eyes, blond hair and fair complexion. It was unbelievable! Our families have no history of kidney problems, poor growth and most likely both parents are carriers.. Yeah right? What are the odds?! Well, on June 13, 2010 our beautiful blond hair, blue eyed little boy was diagnosed with Cystinosis.
Just as a side note, Landon was also diagnosed with severe gastric emptying which means that his stomach takes too long to empty it's contents. It was functioning at a 2 percent and should have been at 50 percent. This probably occurred because of malnourishment. And guess what, our little boy is already beginning to take food by mouth better despite recording all of the calories he needs from the feeding tube.
Just as a side note, Landon was also diagnosed with severe gastric emptying which means that his stomach takes too long to empty it's contents. It was functioning at a 2 percent and should have been at 50 percent. This probably occurred because of malnourishment. And guess what, our little boy is already beginning to take food by mouth better despite recording all of the calories he needs from the feeding tube.
Once upon a time...
Our perfect little boy came into this world on April 8, 2010. It was an easy delivery for me, whatever that means! He was beautiful and absolutely perfect. Months went by as his dad, Jimmy, and I adjusted to life as new parents. He was such a pleasant baby. When he cried, he needed something and that wasn't all that often. He nursed wonderfully but around 4 months, when he was given a bottle exclusively, we realized that he wasn't a big eater. He took to solids well but seemed to want less formula. His pediatrician told us at his 6 month appointment that she wanted to keep an eye on his weight and instructed us as to how much formula he should be taking. This began our journey.
Landon continued to maintain his weight so completely fell off the weight and length charts. Some don't hold these charts to a high standard, and to each their own, but we knew something wasn't right. His head circumference however did increase. I joked that he had the body of a 6 month old and head of a 9 month old. He saw a GI doctor, dietician, a feeding team which included a dietician, occupational therapist and speech therapist. He had an upper GI test done, a sweat test for CF and a scope all which indicated that he was perfectly healthy. "There seems to be no medical reason for him not to gain weight" we were told. It made sense, it must be behavioral. We tried EVERYTHING and could not get him to eat and in turn, not gain weight. I felt like such a failure. Jimmy called me every day from work and would ask me if he ate first and then how our day was going... It consumed our lives.
Following a negative scope, the GI doctor chose to admit him at Childrens Hospital in Pittsburgh for an intense feeding therapy. When she called me to give me the details, she told me that there were traces of sugar the urine that was collected when the scope was being performed. "It is probably a fluke" she said but asked them to collect another urine sample once he was admitted. This is when we began to get some answers..
Landon continued to maintain his weight so completely fell off the weight and length charts. Some don't hold these charts to a high standard, and to each their own, but we knew something wasn't right. His head circumference however did increase. I joked that he had the body of a 6 month old and head of a 9 month old. He saw a GI doctor, dietician, a feeding team which included a dietician, occupational therapist and speech therapist. He had an upper GI test done, a sweat test for CF and a scope all which indicated that he was perfectly healthy. "There seems to be no medical reason for him not to gain weight" we were told. It made sense, it must be behavioral. We tried EVERYTHING and could not get him to eat and in turn, not gain weight. I felt like such a failure. Jimmy called me every day from work and would ask me if he ate first and then how our day was going... It consumed our lives.
Following a negative scope, the GI doctor chose to admit him at Childrens Hospital in Pittsburgh for an intense feeding therapy. When she called me to give me the details, she told me that there were traces of sugar the urine that was collected when the scope was being performed. "It is probably a fluke" she said but asked them to collect another urine sample once he was admitted. This is when we began to get some answers..
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