More updates

The last post was actually from Feb 19, even though dated the same as today, so these are new updates.  We restarted formula last night, just 200 ml at 20 ml per hour.  Peter will just do plain formula, no flavoring, as that will lower the osmolality of the formula and may help decrease the likelihood of diarrhea if it was related to the flavored formula he had been on.  I had read that the formula (Pediasure Peptide 1.0) was on the lower side in osmolality compared to other formulas, but didn't realize that there is a considerable difference in it when choosing flavored formula versus plain.  Just like in the "real world," there are added sugars and colorings in medical-grade formulas.  Now the obvious question is, "Why?!"  With the substantial number of people on medical-grade, prescription-only formulas, couldn't someone do a better job with the composition of them?  I've tasted his strawberry formula and it is too sweet.  It hides the flavor of the plain formula well, but I don't know that the sweetness is as necessary as the strawberry flavor.  Then there is the bright pink color that is created with red food dye FD&C #3.  For some reason plain and vanilla don't need a fancy color, but strawberry does?  There are other formulas on the market, but "plain" is a difficult flavor with all of them, and some of the other formulas we've tried are too thick, come only in vanilla (a flavor that makes him vomit), or are just don't suit his needs.  Maybe some entrepreneur out there will come up with better formula options, like color-free, or low-sugar not artificial.  Fortunately, Peter has a G-tube, so we can give the plain formula to him now overnight and he won't have to taste it.  Hopefully we'll have better luck with this lesser osmolality.

We have found that sodium is becoming a problem now that Peter wants to eat.  Peter has always had problems in retaining sodium and then developing ascites in his belly from accompanying fluid retention.  The best measure to prevent this is just to keep his sodium intake low.  I mistakenly thought that we had some wiggle room while off formula and let him have Cheez-Its, goldfish, pretzels, etc, all loaded with salt.  It only took a week of that too see his belly begin to get big.  Fortunately he is responding well with extra water and taking away the salty foods, and he is losing the ascites.  Unfortunately, he is only six and can't understand why I would buy him these snacks only to take them away.  He actually said that to me in almost those exact words, crying profusely as he said it.  It is quite a juggle with him to work on good foods when all these years we have tried to get him to eat anything, anything, just for the practice of chewing and swallowing.  If he ate 12 goldfish in a day that was impressive.  Now he wants 25 goldfish, and 15 animal cookies, and 20 Cheez-Its, all at one sitting, repeated three times a day.  He is eating three good meals a day, with things like plain yogurt and meat, veggie, and fruit purees, but it is these snack foods which give him the practice of feeding himself and which he absolutely craves.  We are starting to leave out little pieces of kiwi and apple for him to practice with new snack foods, and I'm sure he'll make the transition over to better foods, but it is hard to see him so frustrated trying to understand the concept of moderation now after trying so hard to get him to eat at all.  We went on an outing to the grocery store this past weekend to pick up new foods to try.  That is when we picked up the snack foods listed above, but he also picked up grapefruit and a turnip that he said he MUST try.  "I don't know what a red grapefruit or a turnip tastes like, Mom.  I HAVE to know what they taste like.  I've never tried them before."  I guess we'll go back to the store again this weekend and find some low-sodium things to eat.  He picked out four kinds of cereal to eat when we were there this weekend, but they are not holding his interest like the animal cookies and the salty foods.  He loves bold flavors, but bold flavors seems to have high sodium.  Other short-bowel patients have shared that they crave salty, bold flavors, so Peter's cravings may be more physiologic than psychologic.  I think we are going to have to get pretty creative in finding ways to give strong flavor with low sodium. 

Back on TPN every night

Peter had his check-up in Boston earlier this month.  Peter is now back on 7 nights of TPN and all formula was taken away, since his ostomy outputs were so high and he was so skinny when they saw him. As much as we have enjoyed the freedom of lesser nights of TPN, we agreed that it was best to just put him back on it and get a little more weight on him. His upper arms are actually skinner than his forearms now, and his bottom just looks wasted. Ironically, he seems to feel good with lesser weight. With the adding of TPN, the other decision made was to remove all formula from his diet for a while and just let him eat what he wants. It felt like a big set-back, but we were reassured by the docs that in doing all of this, watching what happens to his ostomy output now will help in the diagnosis of why he seems to be dumping out all that we put into him. Infection has been ruled out, and we are trying to determine if this was a long-lasting transient problem post antibiotic use, an issue with his formula, or if he is actually demonstrating a need for the ostomy to be taken down so he has more bowel length to work with. (That will be another high-risk surgery, so no one wants to just go ahead and do it at this point unless he shows us it is necessary.)

The results of this have been that his ostomy output has gone way, way down, and his weight is going back up. In fact, we were able to shave off some of the volume of TPN this weekend, and even though he still has it for 7 nights a week, the next step in this process will be to start shaving off one night, then two, etc. 

But the silver lining of all of this is that he is hungry and wanting to eat real food now! So, we have been going up on his real food instead of using formula, a little each day, and so far his ostomy output is still down. He is really having a good time with food, pretty much grazing all day. It is such a blessing to see him want to eat instead of trying to convince him of the merits of drinking formula. I am still trying to work on the concept that cookies are more of a treat than part of a meal, but we'll get there. We'll likely have to add some formula into his diet again, but most likely we will be able to just do that at night if he is willing to keep advancing in real food during the day. 

I think to many people the excitement of this real foods achievement is very abstract.  I know that I would have a hard time understanding why it is exciting to see a six year old grazing on a piece of grapefruit or goldfish or tasting ranch dressing, if I had not have the experience with Peter that I have had.  He is not a "picky eater," but rather a kid who has always had such a keen interest in what other people are eating, in reading and asking about different types of food, but never with an appetite to taste them.  All of a sudden he is hungry and wanting to know what these things taste like.  It is like a whole new world has opened up for him.  As we quietly lamented the return of the TPN regime, a beautiful new dimension opened up for him!

20 days later, TPN is coming back

January 6 was Peter's last night of TPN... we hoped. Tomorrow night he'll start back on it again. He has been losing weight steadily since his surgery, which was good since he was overweight on TPN and fluids, but only good up to a point. His ideal weight is likely 47 to 48 pounds, but he is now 44 1/2 pounds and decidely skinny and bony... not just thin, but frankly bony.  It is normal to lose some weight when transitioning off TPN, but he is dropping too much weight too fast.  

The immediately apparent issues that have complicated Peter's success off TPN are unstable daily ostomy outputs that can vary from just under 600mL/day to over 1200mL/day and then the responsive action to take away his enteral feeds and place him on Pedialyte, which has considerably fewer calories than his formula.  He is now on six Imodium capsules a day, and some days it seems to help, other days it doesn't seem to do a thing.  At this point it is unclear whether the formula he is on needs to be substituted out for another one, or whether this is an issue with his bowel. As his ostomy does slow down considerably with Pedialyte, the output amount is likely due to the formula. We will be looking further into all of this over the next couple of weeks, perhaps going onto a different formula or substituting out some of the formula for something else. 

People have asked why he is on formula instead of real food.  The typical protocol in coming off TPN is to be on formula, as formula is more nutrient and calorie dense than real food, so a person can take in less formula than food. This gives the bowel time to gradually adapt to a more normal intestinal functioning.  Peter does eat some real food, so it is possible we might have success feeding him more real food in place of some of his formula, but he frankly doesn't eat enough to sustain without formula or TPN.  Unfortunately, with the reinstitution of TPN, we also know his appetite will go down.  It is a difficult catch to be on TPN - it can prevent or treat malnutrition, but it can also reduce the desire to eat.   Everyone has held out as long as possible in putting him back on TPN, but at this point we all agree that he needs to regain some of the lost weight and definitely not lose any more.  The goal is to still get back off TPN as soon as possible though.   

Peter is disappointed that he needs to go back on TPN, as are we, but he is only going to be on three nights a week and will likely go down to two nights a week once he gains a little of this lost weight back.  We are, however, happy that he will get back on Omegaven again, as he had to stop Omegaven once he stopped TPN.  We had tried enteral fish oil after he ended Omegaven and that did not fare well with his ostomy output, so Peter has not had the benefits of those great omega 3 fatty acids in the 20 days he's been off TPN.  We had a great break from the tedium of TPN set-up, but honestly, the last two weeks have been rather harried in keeping up with the dealings of his fluctuating high ostomy output: calculations, phone calls, emails, and boluses of Pedialyte, sometimes every half hour, in an effort to keep him off TPN.  Resuming TPN may be the less complicated option at this point.     

We decided to give Peter a "bonus" to restarting TPN by telling him we will have another celebration when he comes back off TPN.  He is already looking forward to it.  "Will I get to open more presents?" he asked tonight.  I just love the thought process of a six year old!

A little video of Peter's party

We tried to take some video of Peter's little party for coming off TPN.  Most of what was captured is mayhem, but this one at least captures the excitement that he and his siblings have about this special milestone.

The significance of the watusi gift is only this - Peter has a great appreciation for animals and he has a huge collection of plastic animals at this point.  After he got home from his long hospital stay in Sept-Oct, we took him to the zoo, where he saw a watusi.  It was an impressive animal!  He has been wanting a plastic watusi since that time, but there is just no plastic watusi to be found.  The day of his party, his sister came across this animal, which actually is a Texas Longhorn, but awfully close in appearance to a watusi, and felt that if we cut the tag off, Peter might just be convinced it is indeed his long sought-after watusi.  So far, he is convinced that it is! 

Peter is OFF TPN - hooray!

The big news is that Peter is now officially off TPN!  In less than four months after his surgery in September, he has gone from 7 nights per week of TPN dependency to zero dependency on TPN!  He is taking in almost 1300 ml of formula daily, along with 120 to 200 ml of yogurt and pureed baby foods, and about 120 to 250 of water a day.  A great thing with Peter being six years old is that he has been able to understand why I am feeding him formula several times a day even though he doesn't care to drink it. He has been able to decide that he wants to be free from TPN and to understand that for him to accomplish that goal, he needs to drink the formula. We had a little party for him to celebrate his end of TPN and he was so excited. He decided that he wanted sugar cookies (reduced in sugar for him) and that they were to be shaped cookies, not drop cookies, and he wanted to help make them. He also wanted strawberries for his party. We then added some surprises for him with crepe paper streamers, a banner, a candle to blow out, and some unexpected gifts and cards. He had a wonderful time and the celebration gave him some well-deserved recognition for the effort he has put into coming off TPN. 

Peter has been on TPN since April 2006.  The most he was ever able to come off was three nights a week for a few months, and then the fistula started putting out huge volumes of output and he had to go back to 7 nights per week of TPN.  It almost doesn't feel real that he is actually off TPN since we have been using it for so many years.  I am posting some photos of what his nightly set-up on TPN was.  We will miss the great health benefits that we know Omegaven gave to Peter, but will certainly not miss the twice-per-night changing out of the old Omegaven bottle for the new one and resetting of the pump.   His nightly set-up now is just a little IV push medication, setting up his tube feedings, and hooking up his ostomy to a drainage bag.  He, and we, are enjoying the freedom that comes with being off TPN.

Supplies needed to set up TPN and Omegaven.  I forgot to include the 50mL bag of saline which we hang at 6am to "chase" the remainder of Omegaven down the IV tubing. Below are photos of what the set-up looks like after injecting the multivitamins and connecting the tubes.

Omegaven

This yellow bag is what the TPN looks like after the multivitamins have been added.  This yellow bag and little pump are what is inside the maroon bag below.



This was Peter's nightly set-up with TPN, Omegaven, and enteral feeds.  The tiny little white and teal pump and pink bag behind it are all that will be remaining on his IV pole, as they delivery his near-700 milliliters of enteral feedings overnight.  The ostomy drainage bag is still there too, but hanging off the bottom of his bed.  That will go away whenever the ileostomy gets surgically reversed. 

Hopefully this is goodbye to TPN for good, but if not, we'll enjoy the freedom while it lasts!