Terrible Twos, or Threes?

Good news is that Landon is a typical 3 year old in many ways. The bad news is that Landon is a typical 3 year old, kidding! Kind of... You know how they say that the terrible twos have nothing on the 3s. I didn't believe it. Landon was always so laid back so I thought we may be in the clear. Not so much! I think he is giving up his nap. He did not nap 3 days this week. It used to not be a big problem if he missed his nap. But now, when he doesn't nap he is incredibly wound up and does not listen or follow direction unless he being occupied. And if not given the attention he desires, he does things that he knows will get a reaction from us. It's exhausting! He spends a good bit of time in time out and losing privileges/toys etc.. It's only a phase, I hope! I have been making more of an effort to spend quality time with him and doing more structured activities rather than playing cars for 15 minutes here and there throughout the day. Pinterest has given me ideas for some new activities! We are still working out some issues with Landon starting Procysbi (the 12 hour medication).. He has had blood work twice recently- last week and the week before. The first week he had it was when he was regularly vomiting and last week he had a bad cold.. The first blood draw indicated that he creatinine level went from .03 to .045. This number had consistently been .03 for a while now and to jump up is not a good thing. His nephrologist thought it may be a result of being a bit dehydrated from vomiting. People with Cystinosis can easily get dehydrated. I should mention that creatinine is one measure of kidney functioning so my stomach dropped a bit when she said that. So we had his blood drawn again and added a test that more accurately measures kidney functioning. I took him back for his second blood draw and added the cystine test to see how high his cystine level is and another test to measure kidney functioning. I learned several days later that his creatinine went from .045 the week before to .040 so down a bit but not as low, and as good, as it had been. His bicarbonate level (measures some of his electrolytes) was a bit low but he hasn't had his dose of medicine for this so not something to be concerned about.. The other kidney functioning test (I think it was called the NER) showed a drop from 101 to 64 (the numbers may be off a little because its coming from memory). At 101, his nephrologist reported that his kidneys function just as anyone of his age. She reassured me that although a drop, not for the better, it was not a huge drop and something we should just keep am eye on. Because he was sick, she reported feeling ok about the results. I am praying that this is the case and that when he has a blood draw again in September that the numbers will look like Landon's again. We still do not have the results of the cystine test but if that number doesn't look okay then there is a good chance that I'm going to ask to stop Procysbi and put him back on Cystagon. I have nothing negative to say about the drug because it has provided a much better quality of life for so many in the Cystinosis community but I cannot keep him on a drug if I know that another was working better for him. I'm really jumping ahead in even discussing this but it's my nature. I need to get things fixed if there is even such a thing. The last thing I want to mention is that our 3rd annual Halloween event is scheduled for Nov 2nd. E-mail me for more details!!! LaurenLHartz@gmail.com

Surprises and Hiccups!

I haven't updated since July 15th. So, what's new since then? Potty training. Well, it went much better than I ever expected. He is wearing underpants with very few accidents while he is awake and still wears his diaper when he naps and at night. His diaper is so full when he wakes up. Night training is probably a long time away and I am not at all concerned about it. When his body is ready for it, he will do it. He's not so good at telling us when he has to pee yet although if his pants are off and his potty chair is in the living room, he is great about sitting and going when he has to. It's when his pants are on or the potty chair isn't convenient he has had a few accidents. I have to say that he has not had a single accident while we are out. We put him on the potty every 1 1/2 hours when we are out which I know helps but we went for a walk yesterday and it was over 2 hours before he went potty. Anyway, I am a proud, proud mama! In other news, Landon has started the 12 hour drug, Procysbi. He took his first dose on July 20th. Along with the new medicine, his nephrologist changed a few other doses so that we don't have to give any meds more than 3 times per day. He had 3 medications, including Cystagon, that he took 4 times per day. All in all it has been going well.. We've had a few hiccups and I am so thankful to Landon's nephrologist and some of my friends in the Cystinosis community for helping me to help Landon transition. The hiccup has been increase vomiting. Strange enough but I did not anticipate this. I've always been prepared that when we change doses and add more medication that there is a possibility that he will throw up and we need to work through it. But I didn't prepare myself this time around. I've said it before and I know it to be very true but the better Landon is doing, the harder it is for me to accept the hiccups. The few times this past winter that he got a cold, I really struggled to not get to worked up about it. Is a cold a big deal? Not for the most part, but it means I have to cut back on the amount of formula he gets and his weight gain slows down. Anyway, I have done a bit of experimentation and have written a new schedule and posted it several times. I do not feel that the hiccups are because of the new medication, but I think that there have been a number of changes and I have to experiment and figure out what it is that his body is reacting to. I'm not sure if I explained anything about Procysbi and how it works so I will do that now, to the best of my ability. The difference in Procysbi, as compared to Cystagon, is that Procysbi is released into the intestine rather than the stomach. For those how have a lot of GI issues, this is wonderful because they don't get sick as a result of taking it. When Landon was first diagnosed, he threw up everything that went into his stomach because he had delayed gastric emptying. At the hospital, doctors put a NJ tube in before a G tube which means that all of the fluids, med etc went to his jejunum and intestine and bypassed his stomach. Administering the drug is a little interesting.. Landon gets the drug at 5am and 5pm. I chose these times because he cannot eat 2 hours before and 30 minutes after taking Procysbi. I pull 50mL of applesauce and empty it into a bowl, leaving 10mL in the syringe. I then empty the contents (which remind me of sprinkles) into the bowl with the applesauce and mix it up. I collect that applesauce in the syringe with the leftover 10mL of applesauce into the syringe. Then I collect 30mL of orange juice into another syringe and put some of it in the bowl that has some leftover applesauce and beads from the Procysbi. The orange juice helps to collect the remaining beads. I then push the syringe with the applesauce into the extender of his G tube and orange juice and flush it with water. So that's what's new with us. It's busy but we are getting adjusted to a new routine and I'm enjoying the longer period of sleep. My plan was to stay awake at 5am and work out and get things done before the boys wake up. That happened one day. Oh well.. I'm still working out and still getting what I need to done. =) Oh, and last but not least. We are starting to move on plans for the 3rd Annual Lots of Love for Landon fundraiser. It will be held on November 2nd at the Home Economics Building in South Park (same as the last 2 years). If you want included on the guest list, want to donate a Chinese Auction item or door prize item send me an e-mail at LaurenLHartz@gmail.com.