Still off TPN and making many enteral changes

Peter is still off IV nutrition.  He had his CAIR appointment in Boston last week.  He grew another centimeter taller, but the weight gain is not true weight gain, but rather is fluid retention.  We are trying to concentrate his overnight formula and cut back on the volume.  He had gotten up to 440 ml of formula overnight, and we are cutting back to 400 ml formula, with extra Duocal in it to add more calories.

Meanwhile, we are trying to get rid of his IV iron.  This week he started enteral iron drops instead.  The recommended dose was surprisingly high to me, but the team has researched carefully and assured me this is the correct amount.  Hearing the dose for Peter and knowing that he only had his ostomy closed two and a half months ago and that this medication may cause constipation even at normal doses, I have been very cautious as we start our way up to what has been prescribed. He is supposed to get 69 mg, twice a day.  So far, I have him up to 23 mg twice a day and he is doing OK.  The reason for the really high dose is that his stores are below normal.  He has been taking IV iron for years, but after his last two surgeries, has not gotten his iron levels back to a normal range.  The plan is to keep working him up to a normal range enterally, to prove that his bowel can absorb and handle the iron that he needs. 

If his bowel can handle his iron needs, that gets us closer to getting the IV PICC line out on our next visit to Boston.  To get the PICC line out, he will need to demonstrate that he has no need for it for IV nutrition or IV meds (iron is the only med he is currently still taking IV).  Labs can always be drawn without the PICC line, although that has always been a nice perk to having one.

Other news is that Peter's zinc and vitamin D stores are low.  We are going to begin supplementing with extra zinc, beyond what is in his multivitamin.  The vitamin D stores have been an ongoing issue for a good while, with supplementing going as high as 8000 IU to as low as 2000 IU.  We are going to try 4000 IU this month and see how he does on that. 

Meanwhile, Peter's multivitamin will be changing, as the company is no longer making the vitamin, Source CF.  Peter does not have CF (cystic fibrosis), but requires a multivitamin with high amount of fat soluble vitamins, and this one is made for patients with CF.  Peter will switch to AquADEKs brand now.  It is not exactly the same as Source CF in vitamin amounts or ingredients, but there is little choice when trying to find other brands of fat-soluble vitamins that are actually made to be water-soluble for those who may have fat malabsorption.

As you can see, there are many changes taking place with Peter's enteral intake.  The "funny" thing about all of these changes though, is that patients are not supposed to make more than one change at a time, so that if there is a side-effect, it is more obvious what has caused it.  I am trying to space out the changes as best I can, and hope that there are no unwanted side-effects, but if there are, that I can figure out what new change caused it.  There will be lots of detailed record-keeping this month!

Almost 7 years to the date....

Peter had his CAIR (Center for Advanced Intestinal Rehabilitation) check-up on Thursday, a month post surgery, as planned.  We went with the expectation that the team would reduce his TPN from 4 nights/week to 3 nights/week and then we'd continue working on reducing that further over the next month or two, eventually getting down to none within two months or so.  To our complete, wonderful surprise, the team said they'd like to see Peter stop TPN completely now! His TPN calories are few enough that he should be able to compensate for them by diet.  He already receives his multivitamin by mouth, so TPN is not necessary for delivery of vitamins.  His volume of stool is low enough that he does not need the fluid in TPN for fluid replacement.  In fact, Peter needs less fluid since he is so prone to ascites.  So, we have all agreed to stop Peter's TPN and see how he does over the next month.  He will go back for another check-up then, to see what his labs are like and whether he has been able to maintain his weight.

Back in January 2012, Peter went off TPN, but within a month, he had lost significant weight.  His face had so little fat on it that his forehead would wrinkle like an old man's and his bottom was starting to look wasted.  He no longer has the ostomy though, so as long as his intestine is able to handle and absorb the increasing amounts of food he will need to eat, he stands a much better chance at succeeding in staying off TPN this time.

Peter's good news occurs almost seven years to the date when we found out that he was sick and needed hospitalization.  Lent 2006 began with Peter and I going to the hospital for a week to find out why he was so anemic.  Now, near the end of Lent 2013, we feel an extra reason for gratitude this Easter.  While Peter will always have some health issues of real concern, it is important to rejoice in the small and big blessings that we receive along the way. 

Another surgical success

Look who's missing an ostomy bag!

Our little warrior's battle-scarred belly

Fishing in the tub

Look who's wearing real pants!

Feeling good

I am hoping these photos illustrate what has been new with Peter lately.  He has has last stoma taken down in early February.  That was his ileostomy, which we had unsuccessfully attempted to close last Fall.  It is now closed, and you can see the scar in the top photo, on his left lower abdomen.  The darker circular area of skin around the suture line shows is where he has worn an ostomy bag for the last 6 3/4 years.  I think that coloration will eventually go away, and the suture line will lighten in color over time.  The white dressing that is in the photos is just gauze and tape to secure his MICkey G-tube button, not anything related to the surgery. 

Peter has had to wear overalls or one-piece outfits up until this time to keep his ostomy bag from getting tugged on by the waist of pants and from getting snagged on something and popped if we kept in on the outside of a pair of pants.  (Remember, he is a little boy, and rolling on top of toys on the floor is part of his nature.)  The problem with overalls has been that he cannot get the straps over his shoulders and fastened by himself.  As of two days ago, he has discovered that pants are now the way to go (elastic waist for now, snap and zipper later on). 

We also convinced him to try the tub with a little bribery of computer time.  He has had sponge baths for almost 7 years, ever since he had his first PICC line back in April of 2006.  We have tried tub baths about three other times, but a wet ostomy bag was very upsetting to him.  Today there was no bag to get wet, so he felt more comfortable trying again.  The big smile was there a good bit, so I think he actually liked the bath too.  Though he has a PICC line protector on, it isn't a great fit on him, so we just kept his arm out of the water when it was on.  If he really begins to enjoy tub baths, maybe we'll get a better PICC protector, or if things go according to plan (that is always a big IF), then maybe he will get off TPN in the next several months and not even need one.

Back to the surgery....Peter spent 11 days in the hospital post-op, which was 3 days less than we anticipated.  There were a few shaky days, where we were waiting to see if Peter was going to need a stent put into his liver before we went home to help with bile flow due to dilated bile ducts (an unexpected issue uncovered with his MRI), and watching to see if the closed ostomy surgical site would open back up and the surgery fail like last time, especially as people voiced concerns that the fascia underneath is just "paper-thin."  The liver stent may or may not ever be needed, but at this juncture it was determined we can just watch and see.  In the end, this surgery gave us just a little difficulty with the surgical healing afterwards, but that was nothing compared to the infection and poor healing of the prior two surgeries, or the ineffectiveness of the first fistula surgery prior to that. 

We have had difficulty embracing this new reality of care for Peter as we should.  It is an easier amount of care not having an ostomy, and no longer having that underlying nagging worry about the ostomy bag popping, leaking, or needing to be emptied.   So often there have been health issues that crop up, and Peter's prior three surgeries were been laden with problems, so we've been tenuous in our enthusiasm and in posting about this surgery, almost waiting for an issue to occur yet again.  I don't know if we are being wisely cautious or just foolishly pessimistic.  I don't want to keep waiting for something to go wrong though, so today I am posting the very good news about Peter's progress.  We are grateful for the success of his surgery, for the freedoms we are finding with no ostomy, and for the satisfaction he has found in his new independence.  We have so much to be thankful for!


(This 3/3 entry updated 3/4, with a few details I'd left out.  Hopefully it all makes sense!)

Strep, potty, eyes, and independence

So much time has gone by since the last post.... again. 

Peter had strep throat right before Christmas.  Aside from the common cold, this was Peter's first "normal childhood illness" that I can recall.  It turned out that 5 out of the 7 of us had it, but no one knew until I ended up making the hour and 20 minute drive to the ER at CHOP just before dawn with an axillary temperature of 102.6 degrees.  That isn't too terrible of a temp for most people, but if you have a central line (which is a PICC line in Peter's case) it is a big concern for infection of the line and possibly ensuing sepsis.  The "good" news about it all though, was that Dr. Kamin started Peter on antibiotics at home earlier that day when his temp was lower, after the home nurse had drawn labs and cultures.  That one dose of antibiotic was enough to get Peter back on track within a few hours of the fever spiking higher.  The positive rapid strep test performed at the ER and negative cultures at the 15 hour mark, gave us the OK to go home from the ER after 7 hours, instead of going inpatient.  What a relief that was!  After Peter was diagnosed, a quick trip to the pediatrician confirmed strep for two of his siblings as well.  At that point, Chris and I knew we had it even with no diagnosis. The frustrating part of the whole scenario though, was that NO ONE locally would see him.  His local pediatrician could have diagnosed strep in the office earlier that day, but their rule is "temp and central line = ER."  The local ER will not draw labs off a PICC line though, nor do they really handle pediatric patients like Peter, as I found out via phone call that day.  Had Dr. Kamin not been willing to help us manage things at home with Peter, the event would have been an inpatient stay, with the "big guns" antibiotics that are used for line infections, quite costly for the insurance company, and frustrating for all of us, most of all Peter who just wanted to be home again.  I will never be able to thank him enough for all the dedicated care he has given Peter over the years.

Potty training has gotten much better since the last post.  I owe the improvement to Peter's oldest brother who allowed Peter to borrow the used ipod he bought if he would go potty quietly and successfully.  It worked like a charm that first time and has worked every time since.  Peter still can't figure out how to go successfully without it, but at this point I am fine with his dependence on it.  If we can keep him stooling rectally, especially without straining, then his next surgery to close the stoma should go well.  The bottle nipple in the stoma still leaks a lot, but enough goes down rectally to give him and his bowel proper practice. 

Peter went to a Wills Eye opthalmologist last week to re-evaulate his lazy eyes on the recommendation of the local opthalmologist.  We were trying to determine if patching would help.  To our surprise and relief, the Wills Eye doctor feels that Peter does not have lazy eyes, and gets his vision at 20/30 with glasses on.  We will return in 4 months to re-evaluate and make sure he is continuing on this good path.

Independence is finally beginning to kick in, at least a little bit.  We went bowling two weeks ago, and as he is getting ready to bowl his turn, Peter told me, "I've got it, Mom.  I can do it myself."  This was huge, especially on the heels of a week's worth of insistence that he was unable to put his own shirt on when getting dressed in the morning.  Aside from one shotput launch of the ball down the alley, he did quite well bowling on his own.  This week, he is coming over to show me that he has put his own shirt on, his own shoes on, even volunteering to brush his teeth.... big steps for this seven year old!

Potty training is not fun

About three weeks ago, after some emails back and forth with Peter's surgeon and the wound care nurse, we started using a baby bottle nipple in the stoma of the ileostomy to help divert poop back down the colon instead of out the stoma.  The stoma still allows a good deal of stool to exit into the ostomy bag, but about 200 ml of stool does get diverted down through the colon with the bottle nipple in place.  The bottle nipple is place in by inserting the tip into the stoma, with the part that would touch the bottle sticking out.  This is placed inside the ostomy bag, and then an abdominal binder is placed over that to keep the nipple from popping back out of the stoma.  It has worked fairly well in that he can still use the stoma as an emergency exit for stool, but it does have its limitations.  For one, the stool from the bag smells horrible, like the plastic material that the nipple is reacting some to the stool.  I can't see any visible problem, and I do put in a new nipple often.  If I leave the nipple out, the smell goes away, so I don't think it is an issue with the stool itself.  The other issue is that the binder then limits how much stool and gas can be contained in the ostomy bag.  The binder basically compresses the entire bag so there is room for only about 1/3 of the bag's usual capacity.  This means there are more trips to empty the bag of gas/stool, as well as more leaks of the bag if I don't get it emptied soon enough.  We tried a leg bag attached to the ostomy bag to give some more room, but the stool is also very thick and doesn't go down freely, so it didn't help.

Aside from those complaints, what we have determined is that the colon works very well at concentrating stool and making formed poop.  This is great news, and what we expected, but had not been able to actually determine up until now.

Peter is having a terrible time learning to use the potty for stool.  He has been potty trained with urine for a couple of years now, but he does not like using the potty for stool.  In fact, he does not like making stool rectally at all, and we are battling that now.  After the surgeries last month, he would yell and carry on, jumping up and down trying to not go to the bathroom and just having little bits come out in his diaper.  I thought that would clear up once we got home, but it basically has not.  We have major bribes going on to get him to go on the potty, but he gets on there and gets frustrated.  He says he doesn't like the feeling of going poop rectally, but that it doesn't hurt.  He is getting a tiny bit better at going in his diaper without quite as much carrying on, but that is only sometimes.  Chris is going to give potty training a shot this weekend to see if he can get Peter to go on the potty.   I think the more he holds in rectally, the more backs out the ileostomy, so it would help so much if he would just let it out.. potty or diaper.

Peter has always been very dramatic with learning things that have been physically challenging or scary to him.  When he learned to sit up sometime around 17 months old, he screamed as we would sit him up or prop him up.  Once he mastered sitting, no more yelling about it.  Then he did the same screaming learning how to stand.  Once he could stand well, no more yelling about that either.  This went on with walking, crawling (which he learned after walking), playing at the playground, riding a bike, swinging, etc.  I often wondered what the neighbors thought about his screaming, wondering if they thought we were hurting him, as what child is that afraid of learning so many new things?!  Anyway, I do believe he is screaming more out of fear of this unknown/foreign feeling than out of pain, as he doesn't act like he is in pain and doesn't complain of pain.  I so much look forward to his mastery of going potty and more peace in the house!  This yelling goes on one to several times a day and can get quite time consuming and frustrating for all of us.

On to some more pleasant news, Peter is getting a night off TPN tonight.  He has been on TPN 7 nights a week since his surgeries, to help with wound healing and to give him a little extra beefing up for the next surgery.  I sure do miss the freedom we had of being on TPN only 4 nights a week before the surgery.  Getting the TPN set up takes about 20 minutes, which isn't so bad, although it it so nice NOT having to set it up when there have been nights off.  The biggest problem of it is getting up twice during the night to change over the Omegaven bottle.  Last night I was up 5 times between the Omegaven pump, Peter having to go pee-pee, his little brother having to go pee-pee, and the feeding pump going off because the enteral feeding tube had kinked.  Chris has offered to get up to change over the Omegaven, but he does not fall back asleep easily whereas I do, so I don't think that is a good option.  Getting Peter off TPN is the better option.  To do that, he needs to get the ostomy closed so he can use the rest of his bowel for food absorption.  To get the ostomy closed, he needs to make poop rectally and keep that colon in use so it gets bigger in diameter and we no longer need the emergency exit through the ostomy. 

I never counted on Peter refusing to poop when we talked about getting the colon reconnected.  It is funny in a way, but I am sure we will find more humor in it at some point later in life once he is past this point.