Feeding Evaluation

Today Landon and I ventured to the Children's Institute in Squirrel Hill. For those of you who are not from Pittsburgh, it is our local children's rehabilitation hospital. We have been receiving services through an early intervention program for feeding issues and physical therapy for about a year and for speech therapy for several months. He has made great progress with eating but we're a bit stumped and trying to figure out the next step. The feeding team consisted of a group of wonderful women- a nurse practitioner, an occupational therapist, a speech therapist, a psychologist and a nutritionist. The appointment lasted for about 3 hours so was incredible thorough. Landon didn't eat any of the food that I brought for him, which included his favorites, but he was much too distracted with all of the fun things in the room so I wasn't very surprised. The occupational therapist "played" with him during the evaluation which made it much easier for me to pay attention and to convey all of the information that I needed and wanted to. I put "play" in quotes because her evaluation was observation of various forms of play with him, for the most part. He really seemed to like her. She had some pretty cool toys that we don't have at home and was lots of fun to play with. Our main goal is to increase the volume of Landon's oral intake so that he has to rely less and less on the feeds. He was pocketing food a lot but that hasn't been so much of an issue lately. I think much of that was behavioral and a habit. First he wasn't eating at all, then he began to put food in his mouth and hold it there (which was okay with us because at least he was interested and willing to put food in his mouth). When he was doing this, we would try to help him move it to the middle of his mouth but when he wouldn't, we would take it from his mouth or tell him to spit it out. I realized a little while back that Landon was exerting a lot of control when it comes to eating. We never wanted to make it a bad experience for him so just followed his lead. It worked for a while, but we then realized that we had to take back some of that control. The psychologist agreed and praised me for what we have been doing and gave me a couple more recommendations. Meal time needs to start ending on Mommy and Daddy's terms- so allow some control but when we see that he is getting antsy at the table, tell him to take one more bite or one more drink before he gets down. She told us not to battle about it if he won't but then tell him that if he does not, then he needs to throw his plate in the sink. Then he sees that ultimately Mommy and Daddy are boss but that he has some choices in the process. Presently, Landon gets two bolus feeds (higher volume during a shorter time) during the day and one continuous feed (goes in his belly slowly throughout the entire night) while he sleeps at night. The nutritionist recommended that we work on eliminating the night time feed. Now, in my brain, I never cared if he had night feeds for another few years. He is asleep, who cares, right? Nope. If our goal is to get him eating on a normal schedule and routine then we have to depend on bolus feeds more than a continuous because they are more natural. We don't eat continuously for hours and hours. We eat, stop, get hungry, eat again, stop and we certainly don't eat while we sleep. So first we have to get Landon back on track with his feeds (7oz twice during the day and a continuous feed at a rate of 80 instead of 65 which is what we cut him down to when he vomited the other night). Once we get back on track, we are going to begin to cut back on the night feed and will add in a third bolus feed during the day. At first I was bummed to hear that (as was Jimmy), but now after thinking about it, I don't see why we couldn't do that third bolus feed right when he goes to bed and turn it off when it finishes in an hour or so. Then he could still be free of a feed after nap time but still get his last feed. Landon takes a medication that serves two purposes- it is an appetite stimulant and it helps to calm his belly so he doesn't experience as many GI issues. We give him this medicine at bed time because a side effect is that it makes you tired. Well, his appetite may have increased after taking it at 8pm, but it's night time and he is feeding through the night anyway so we'd never know the difference. It was recommended that we give it to him twice a day, at 9am and 4pm. Now we have to experiment a bit with this because I don't want him tired all throughout the day particularly on the days that he starts school at 9am. The speech therapist that we saw today thinks that he may have an issue with his esophagus. She didn't tell me the name but I imagine it will be in the report that is written up and sent to us. A researcher recently did a study about this particular problem in adults but she said that they are looking at it in children now and Landon fits the criteria very well. He has low muscle tone for one. The occupational therapist explained what this means and I actually learned that I probably have low muscle tone as well. Obviously I made it into adulthood with no problems which is certainly reassuring! Landon is very flexible. He can bend the tips of his fingers forward (go ahead, I know you are trying it right now)- so can I. She said that the usual way of holding a pencil is often difficult for people with low muscle tone so they tend to hold a pen/pencil using different fingers to make it more comfortable (yep- that's me too).. I read more about it that made me laugh because I also fit the low muscle tone definition. So, anyway, low muscle tone.. Landon chooses certain foods- foods that are "slippery" and go down his throat easily (i.e. salad with Ranch dressing, pasta with sauce) and although he sometimes eats dry foods (dry cereal, crackers etc)it is not his preferred food because it gets caught in his esophagus (if he has this particular problem). I reported that Landon has a bit of anxiety (it has gotten much better).. this is also an indicator of this "esophagus thing." She seemed pretty confident that he has it but you can only know for sure when a Cookie Swallow Test is done. We attempted this test in July and Landon refused. The treatment is fairly simplistic and would involve some exercises. It's not something to be incredibly concerned about but would explain some of the feeding issues. She wants to meet weekly with Landon to begin some of the exercises and wants to work on getting him comfortable enough to actually be able to perform the Cookie Swallow Test. I think a big issue for him is that lead vest that people have to wear. We'll have to work through the anxiety so we can get accurate results for this test. We will also meet with the nutritionist once a month and she will work on the feeds and how many calories he is getting and so forth... I am sure that I am missing quite a bit but those were certainly the main points. We are going to begin meeting with the speech therapist once a week and the nutritionist will be present at one of those sessions a month. Between all of the therapy he gets now, school two days a week and now going to the Children's Institute one day a week-we are really going to have to consider what to keep and what to put on hold because Landon and I certainly can't make all of those things happen each week. I feel good with the plan and goals, and I hope that he makes lots of progress in eating. It actually will be nice that eventually he will be off night time feeds particularly because we are going to transition him soon to a big boy bed. So, Happy Labor Day weekend. It's such an important holiday but it's also hard to celebrate the end of summer! I have to admit, I look forward to the fall, the Lots of Love for Landon Halloween Party (yes, we're doing it again!), the holidays and to meet Landon's little brother Jordan. This summer was great but I'm ready!

The Hard Part

At 3:30am we woke to the sound of Landon crying, hard... I went to his room to check on him and within a moment he threw up. He probably threw up his whole feed which started at 8pm. Jimmy rushed to his room and we took our positions- me undressing and comforting our boy and Jimmy grabbing towels and changing sheets. We can tell when the moment has passed because he stops screaming and crying and begins to talk to us. Jimmy put him back in bed and he said "nigh nigh..." It just melts our hearts. During the process of clean up Jimmy and I both rack our brains to try to figure out why it happened. Was he coughing or sneezing today (mucous from a cold often results in vomiting), did we speed up the rate of his feed, when making the formula was the ratio of powder to milk right? I think we both feel a little better about it when we identify why. The why for last night? A combination of speeding the rate up and maybe some congestion... He coughed a few times throughout the day and I heard a little congestion in his chest. Then I give myself a silent lecture for not knowing better- a mom thing I guess... As we laid back in bed, Jimmy said "this is what bothers me the most- not the medicine, the feeding tube or anything else.." He was do right. Cystinosis isn't much of a big deal anymore, but hearing him scream from his belly hurting and watching him throw up like that.. You just don't get used to. Fortunately, he probably does this once a month and it's not a daily or weekly. So for today, we will cut back on the amount of formula he gets through his tube, slow down the rate and keep an eye on any respiratory symptoms. Tonight we will slow down the rate and hope for the best... So far, so good. We have a resilient little guy on our hands!

Rituals

Landon, like Jimmy and I, is very much a creature of habit. He does well with his routine and knows what comes next. Well, he is also beginning to create his own routines... For the past week or so, we put Landon to bed for his nap and at night and leave the room. After several minutes he begins screaming and crying. At first I ran in afraid that something was really wrong. Now, I still go on but no longer in a panic... Landon has a Dream Lites Giraffe, a small monkey and his sippy cup in his crib. He throws each item our and cries to have them returned. He says, "oh noo!" as i walk in the room. So, I place giraffe back in his place, monkey in his place and his cup where it belongs. Landon says "nigh nigh" and that's the end of it. Am I encouraging it by doing this twice a day? Yeah, sure. But, he goes right to bed after the ritual and it makes me giggle.

School Days...

The feel of summer coming to an end is in the air. The neighborhood kids start school tomorrow. Landon and I are going to "Meet the Teacher" on Sept 4th. He will be going to school by himself, without me, two days a week for two hours. I would be lying if I said that I wasn't very anxious. I have been cramming in classes at Gymboree over the past few weeks to prepare him for being in a group setting and following class instruction again. I have been pleasantly surprised at how well he functions. He doesn't cling to me as he used to when he first started school last year. I find myself placing myself so that I am in his sight when he wanders off because he used to panic if he didn't see me. The poor kid needs to be away from me, right?! As hard as I try to encourage him to be independent and convince him that he is a big bit, I know I still pull him back a bit. I know that it may be a rough transition for our boy but I also know that he will love it and it will be a wonderful thing for him. It's hard to watch our baby become a big boy but I am such a proud mama!

It's a...

Yesterday was the big day! We; Jimmy, Landon and I, got to see the baby for the first time. As I lay on the table with my belly exposed, I look to see Landon with his shirt up looking at his belly. He wanted a turn next! Our peanut's heart beat was 143. The brain, head, spine, limbs etc looked beautiful. And, we learned that we are being blessed with another little boy!! Jimmy is beyond thrilled, as am I. I was convinced that this baby was a girl. We would have been happy either way but the visions of two, energetic little boys running around the house with their cars and dinosaurs just warms my heart! I always thought I would want a girl but now that I have Landon, who is the best piece in our world, of course I want another like him!!! The Hartz boys- it has a nice ring to it! Let me confess though, I am a little afraid of what these two little boys along with their Daddy are going to do to me... Landon is becoming as silly as Jimmy and they are certainly entertained by each other. A boys of boys, and me- oh Lord. But, I guess I do laugh right along, usually! I guess this mama will just have to be one of the boys!

Choose Your Battles

This morning I attempted to convince Landon to wear his cute, white tennis shoes to the park rather than the usual brown sandals that make his feet smelly! I was successful, kind of... He instead chose brown shoes that are not only a bit too big but clearly meant for the fall/winter months. As I reluctantly put them on, watching him beam with pride, I wondered how it happened that I continue to lose this battle with a 2 year old. Am I just tired and "giving in" to avoid a tantrum? Minutes later I told Landon that we were leaving in one minute and he needed to clean his toys up... I watched my little bottom pick his cars up and put them in one basket, the car track and put it in another (all the "right" basket) and take his ball and mitt in the play room to put them on the basket. I smiled... You must choose your battles as a parent.. Our boy behaves well in public, sits in time out and takes his consequence when misbehaving, he cleans up after himself and follows instruction pretty well... If choosing the shoes that don't go best with the outfit that I choose is where he draws the line then I can handle that... We are doing something right...

Imagination...

I cannot tell you how many times a week I stop and think, "I need to blog about this..." and it just doesn't happen. Life happens and blogging often doesn't. I'm not going to promise to do better, I will promise to try to do better! Last weekend Jimmy and I took Landon to Hershey, PA. We left Friday afternoon and returned home Sunday afternoon. It was a quick trip but honestly, it was nice not to be gone very long. It's been a wonderful, exciting summer but I am beginning to feel the effects of being on the run a lot. Baby #2 raises pokes at me from time to time and says, "Mommy slow down. Remember, you have to take care of me too!" I will mention, by the way, that my sonogram is on Monday and we hope to learn that we have a healthy baby who is developing just perfectly and whether we have a he or she growing baking in there! When I say healthy, I mean that all of the organs are the right size and look just right. We won't know until the baby is about 2 months old whether he/she has Cystinosis or not.. I was asked recently but someone in the Cystinosis community why we are not doing prenatal testing and why I would take that chance.. It's a fair question and one that I imagine other people, who know us personally and who just know us in the cyber world wonder. My answer is, because it's right for our family. Landon is an incredible blessing... He is not damaged. Many of us throughout our lives will have to take medicine for lots of different reasons. We can't predict what our health will be like or what life changing events will occur... Landon and this child are no different. Is life a little more complicated sometimes? Yep. Do I feel sad about what he goes through? At times. Do I know what the future will look like for Landon? No, I have hopes and dreams though. Do other parents know what the future holds for their children? No, but I bet they have hopes and dreams too. I know that my boy is the most special, the sweetest, the cutest, funniest, most entertaining little boy on this planet. If you're a parent, I bet you know that too (about your girl or boy). So why not bring another beautiful, wonderful child in this world? Today I took Landon to a Gymboree class... they are, by the way, wonderful classes! The instructors encourage learning and fun and using your imagination. At the end of the class, as usual, the instructor gave me a piece of paper that included the lessons that we were learned and what skills they touched on along with suggestions for activities to do at home. The instructor pointed one out specifically and told me that it's lots of fun and kids learn a lot from it. When we got home I looked at the lesson. It was very cute and encouraged using the imagination, counting, critical thinking, listening etc.. Suddenly I felt like I am missing the boat a bit. I looked at our calendar for next week... therapy 3 days a week, visit relatives, business meeting for my MOMS group, try to fit in another activity for MOMS group since we've been invisible lately, one day of Gymboree class since school starts soon and our membership will expire.. Add in grocery shopping and the other errands that pop up as the week goes on.. Whew! What about Mommy and Landon activities that involve more than shooting cars across the floor, throwing and catching ball and playing in bed under the covers? Are those things important? Absolutely. But what happened to my imagination? I have wonderful activities at my finger tips thanks to the classes we've attended, child development websites and Pinterest. They are in a virtual folder in Pinterest labeled "Kid Stuff." I have yet to try one of them. Now I know some of you reading this will respond by saying, "you have so much going on. You're doing a great job!" It just makes me re-evaluate things. Would Landon learn a lot of the skills that we are working on in therapy through these kinds of activities? Would making the time to do activities be just as, or more, advantageous then weekly therapy? I never want to look back on the time that we spent when he is this age and have regret. I want to set him up for success.. Decisions, decisions...

Mr. Independent

We have looked forward to our boy getting older, bigger, learning new things... People say not to rush the times because their childhood goes so fast. I have never wanted to rush time but Landon accomplishing new skills and getting bigger means, to us, that he is thriving and his body is healthier... Our little guy running around full of energy means not just that he is being a toddler, but that his electrolytes are within a normal range and that the Cystagon is doing its job. We feel so blessed to watch Landon grow, change and learn right before our eyes. He is becoming more independent... Oh yes, independent. I didn't expect, at the age of two, that he would want to do his own meds and feed. It started by insisting on shaking the formula in the bottle before I poured it in the bag.. Then he liked to help me press down on the syringe to release medicine in the tube.. Now he wants to disconnect and connect the tube from his Mic Key button. He releases the opening on the side where medicine gets distributed. He sits with a syringe and carefully places it in the opening. You can see why we avoid giving him the plastic syringe in his bed whe. We can't supervise, even when he insists, because his attempts have been successful leading to wet sheets and jammies at night. Is it sad that he is so used to all of this? I used to think so but not so much now... I want Landon to learn to manage his disease and what comes along with it as he gets older. And this is a great start. It makes life a bit more interesting for Jimmy and I!

The results are in...

I could just shout from the roof top, I am so excited! Today, we took Landon to have an X-ray done. You may or may not know that he was diagnosed with Rickets when the diagnosis of Cystinosis came. His phosphorus levels were extremely low and phosphorus is what promotes bone health so his bones weren't in great shape... At his last nephrology appointment, they said that we should have the X-ray done to check to see how his bones look now that he has been treated for over a year. Dr. Nyguyen called this afternoon (yes, only hours after we got the X-ray)and said that the difference between the two X-rays were like night and day!! His bones look wonderful!! We spent time talking about some other med changes, additions etc.. At this point, he is doing well, so a lot of what we spoke about today was to be proactive. For example, we discussed increasing the dose of Cystagon that Landon is taking to see how much of a dose he can tolerate. This came from some case studies that Dr. Nguyen read about patients in Europe who take as much Cystagon as they can tolerate (rather than basing it on body weight alone)and there have been reports of WBC levels at .4-.5. Our goal is to keep the level below 1 so to get it to half of that would be incredible! Since Landon really seems to tolerate his meds well, I feel comfortable to experiment a bit to see how much his little belly can tolerate. I also asked her about this little peanut that is growing inside of me now. I may or may not have mentioned that we chose not to move forward with prenatal testing. We will find out after this little one is born if he/she has Cystinosis (by the way, we hope to find out at the end of this month what the sex is, yippee!!). I inquired about the protocol for testing this baby (when will it occur, how)... The baby will be tested the same way Landon was between 2 and 4 weeks of age. They will take blood and send it to the only lab in the US that reads these tests which is located in California. The results generally take a couple of weeks to come back (although we got Landon's back in 10 days as a result of very persistent doctors). They will also perform a genetic test, as they did with Landon, to determine the mutation. The genetic test isn't absolutely necessary and many with Cystinosis have never had this done, but I would like to do it if we have the opportunity. If this peanut has Cystinosis, he/she will not need a feeding tube right away and will not have to be put on Cystagon and supplements right away. It really just depends on electrolyte and cystine levels. The plus for this kiddo is that we will know at a VERY early age so we can begin treatment right away and hopefully not have to worry about Rickets and bone health in the same way that we did with Landon. Only time will tell..

Summer Fun!

I have been meaning to post but it keeps slipping my mind. We spent the week, last week, at the beach in Ocean City, MD. Landon did incredibly well being away from home and a bit off of his routine. There is no doubt that he thrives on it but it's nice to know that he is still his sweet, silly self when we don't totally stick with it. He woke up each morning to a condo full of people he loved who were ready to give him their full attention. What else could a little boy ask for? He slept well at night and napped well so my anxiety about that was put to rest... He did better at the beach and with the water and sand than I expected and even went into the swimming pool. I have to say that he was most comfortable with grandma in the water. Jimmy and I were so pleased and so proud of our big boy. We have one more vacation to look forward to. Landon is going to attend his first concert this month, the Wiggles, and we are going to go to Hershey Park. It has been a great summe and our boy is getting bigger and doing and saying more all the time! He now weighs 22lbs! And the best thing is that he is beginning to eat and swallow so much better. I mentioned before that he put lots of food in his mouth a d would chew it but then spit it out. Jimmy and I have decided to no longer accept him spitting food out. It's just not an option. We remind him to take small bites, to chew and to swallow.. We offer him a drink in between bites and he had had much more success in swallowing! Now, we only started doing this when we noticed him swallowing more food on his own but we have set expectations now and it seems to have helped. The feeding tube is still necessary and we don't plan on weaning him from it anytime soon but this is huge progress! He takes in about 1200 calories from formula each day which promotes growth and the extra calories from food is a big bonus. At this rate he should average about a pound a month in weight gain. He was sick a few times this summer which knocked him back a bit but he has done really rally well overall. At the end of this month, he has an appointment at the Childrens Institute with a feeding team. It is a local rehabilitation facility for children. He has made lots of progress but I think their input and feedback will be helpful. So you see, lots of good and fun things going on. My sister sent me a video of Landon when he was just beginning to walk which was about 9 months ago and it melted my heart and made me so proud. He . He had grown and developed so much in that period of time. I could not be more proud. I just love being his mom and watching him grow. I wi try to post some pictures later! Thats all for now!