A very dry report of our last trip to Boston

Peter had his check-up in Boston about two weeks ago.  We crammed in his intestinal rehab clinic visit, a follow-up with Dr. Jennings (his surgeon), a sedated cardiac MRI, a lab draw, a bone age scan (an x-ray), and a DEXA scan all into two days. 

Peter is doing well enough overall to stay off TPN for now, but he is continuing to lose weight over the last several months.  His arm circumference and skin fold measurements have both gone down.  He has grown some in height, so he has been able to digest enough calories for lateral growth, but if he cannot stop losing weight, then he may need to go back on TPN to supplement his growth.  It looks like his caloric intake is about 2300 calories a day, which should be enough, but there is some degree of malabsorption.  We are currently trying to squeeze in a little more calories here and there over the next couple of months to see if we can prevent a need to return to TPN. 

Peter's vitamin A and D levels are remaining low even with high supplementation.  His platelets are under 30,000 now, which is a little lower than is has been.  To put that is reference, a normal count for most people is 150,000 to 400,000.  He still clots quite well though, which Dr. Kamin says is because his body has slowly gotten to that point and the platelets have functionally adapted.  His white count is still considerably low as well (under 2).  Looking at better lab news, the iron supplementation has proven good for resolving his anemia.  The rest of the labs seemed OK enough, with the exception of calcium in his urine seeming to go up higher.  Our plan is that he will have no calcium-containing foods for 4 hours before the next urine sample, and hopefully we will see less calcium in the urine.

Bone-wise, it looks like Peter's osteopenia is no worse.  It is still present, but his bone-age is about right for his age, and that is good news.  

Besides the let-down that Peter may have to return to TPN, the other disappointment was in the results of his cardiac MRI.  The MRI showed that he now has dilated ventricles, which he did not have in the past.  Dr. Kamin discussed possible causes with several of the doctors who are very familiar with Peter's abdominal issues and they do not feel this is related to intestine, liver, or spleen.  That is a bit of a relief, as I don't know how much more surgery Peter's abdomen could take.  There are two nutritional labs that are going to be drawn this week to see if they could be causing any of this, but that being the culprit is a long shot.  The most likely area of concern is the heart or lung and I expect that will be the focus of additional testing. 

Our priorities over the cardiac and nutritional follow up are that Peter gets to go trick-or-treating with his siblings and that he gets to celebrate his birthday at home, also with his siblings.  Dr. Kamin thought we would be able to work that out just fine.  Peter has envisioned such plans for trick-or-treating and for his birthday, and none of us want to disrupt those plans! 

Peter's formula and diet

This is Peter's diet now that he is off TPN.  This post is in response to Kelly's question, but if it might be helpful to anyone else, please feel free to read as well.

Peter is on unflavored Pediasure Peptide 1.0.  He takes 300 mL by G-tube at night, and to that we add 67 grams of Duocal and 15 grams of Beneprotein.  Throughout the day, Peter eats the following by mouth:
400 grams plain Cabot Greek Yogurt (it has 22 grams of fat per cup)
180 to 200 grams of pureed fruit
180 grams of pureed meat
90 to 100 grams of pureed vegetable with about 5 grams unsalted butter
630 ml of Lactaid milk
60 to 240 ml of water
We add 10 grams of Duocal to his daytime food twice a day
About 1 Tbsp of Barleans Omega Swirl fish oil (He just started that a month ago, now that he is off IV Omegaven.  So far, it is the only enteral fish oil that doesn’t make him smell like fish.  We use the Key Lime flavored one.)
He will take additional foods on some days, like an ounce of fresh fruit, or an ounce of cookies, but that food is more like a “bonus” to his regular intake.

We have found that finding foods low enough in sodium is a huge challenge.  Commercial jarred baby food is generally pretty low in sodium.  We have been buying jars of baby food for Peter for years, even though he is seven.   The Duocal and Beneprotein have no sodium, so they add more calories and protein to Peter’s formula without added sodium or fluid, both of which aggravate his ascites.

Although Peter can chew many foods well, he is slow with the process, and by the time he has taken just a few bites, he says he is full.  We have basically been tricking his mind/body by feeding him his pureed food quick enough that it all gets in him before he feels full.  For example, for dinner, he will have two jars of pureed meat (120 ounces) within 3 to 5 minutes.  We set the timer for 10 minutes and he is free to go play then.  After the timer rings, he comes back to finish the rest of his dinner, which is a third jar of pureed meat and 90 grams of pureed vegetable.  Then he is done his meal.  His lunch and breakfast are done in the same manner, but with the Cabot yogurt and pureed fruit. 

One of us usually feeds Peter, as when he feeds himself, he goes so slow that his body tells him he is full before he has gotten very far.  That may sound very drastic, but in our case the need to get Peter off TPN to preserve the liver far outweighed the need for him to be able to feed himself independently.  (He already has possible cirrhosis, definite fibrosis.)  He holds and drinks his own milk, although he needs constant encouragement to drink it three times a day.  Any snacks he feeds himself.  As we move further along, I expect he will eventually take over his own feedings and even move away from purees.  We are attempting some of that now that we have more free time without the TPN and ostomy issues.

Of note, it took us several years to work up to this amount of feeds with Peter.  He literally started with 5 ml of milk once a day at about age 1.  There were many times over several years that we would push Peter too hard with enteral feeds and he would vomit.  Then we would have to back down from the amount of feeds we had gotten up to and try a more gradual approach to the new feeding goal.  At this point, we seem to be at a good point with Peter’s caloric enteral intake, where he is slowly gaining some weight, but not feeling so full he is nauseated.

Lastly, Peter has most of his intestine.  It has scarring and a unique pathway, but it is mostly all there.  From what I understand about short-gut, having enough bowel length is a very big factor in getting off TPN/IV fluids.

Riding on the scooter now

This summer, Peter has made many strides physically.  He says having the ostomy bag gone has made it more comfortable to run.  I also wonder if being off TPN has made him generally feel better.  Just a few months ago, Peter had very little interest in riding a scooter even one time down the driveway.  He would go ever so slowly and complain the whole way.  Now he is able to ride the scooter halfway around the block and back, actually enjoying himself and going at enough speed to glide part of the way.  Go Peter!

Updates on health and enoying summer

Peter is still doing well without his PICC line.  He has been TPN-free for four months and without a central line (his PICC line) for about five weeks.  He hasn't really gained much weight, but he has grown a little bit in height.  His arm circumference measurement was only slightly down, but still in good range, so we can now wait to go up to Boston for two more months instead of going up once a month. 

Peter's check up included a cardiac exam, complete with echo and EKG.  His jugular veins seem pronounced, as mentioned in the previous post, and after the echo, there is concern about his heart being in a "high cardiac output state."  He will get a cardiac MRI when we are up next.  The high cardiac output could be due to the portal hypertension or possibly a new arteriovenous malformation (AVM).  If this is a new AVM, it should not lead to a situation like we encountered with the liver, but rather should be capable of getting closed off if it can be found.  OR, the MRI may show that he really doesn't have such a high output after all.  Apparently, the MRI can take more accurate measurements of the cardiac output than an echo.  His heart rhythm is good and he is not having outwardly visible cardiac symptoms.  There is not an urgency for the test, so we are going to get the test done when we are back in Boston for his usual CAIR visist again. 

The endoscopy went well.  Peter's esophageal veins were essentially unchanged and there was nothing that needed to be banded.  That is excellent news.  Although we see HUGE collateral veins on his abdomen, which extend all the way up to his armpits, they are not showing up in the esophagus where a bleed could be life-threatening.  I have heard it put that the collateral veins are helping to decompress his portal hypertension, so despite the aesthetic issue of them being visible all over his belly, they are better for his health.  

Peter's stool tests indicate better food absorption than we thought, so that was more good news.  Also, he absorbed his iron well enough last month that his iron stores are in a normal range and he was able to go down considerably in the dose.   We are now trying to cut back on his Protonix (pantoprazole), which is a proton-pump inhibitor, used for controlling acid reflux.  He is down to 8 mg from 20 mg, and seems to be tolerating the wean fine at this point.  He has been on daily acid reflux meds since his early hospitalization as an infant.  To me, it would seem that the more we work his gut with feeds, the greater the chance of gastric distress in a person who has had the GI issues he has had.  It is nice to see that may not be the case.

Back to the PICC-less Peter.... he has been enjoying his first summer of getting really wet.  He still won't admit to liking baths, but he has usually rushed into the tub within a minute of the water going on.  Below are some photos from the summer.  Peter has finally been able to, and willing to, get into the wading pool on some of the hot days.  He splashed around in the water and even ventured kayaking on our last trip to Boston, where we visited with an aunt and uncle on the lake. This summer has been his first time in the water, and he has had so much fun! 

Peter is still into landmarks and geography, and on this trip, Peter got to see the CT State House building up close, instead of our usual whizzing by at 65 MPH on the highway.  He was so pleased to get a chance to see it up close!  Yes, this child is a bit of a geography fanatic.  How many children do you know who at age seven pour over atlases and can tell you the names and locations of places like United Arab Emirates, the Platte River, and the capitals of nearly every country in the world?  The doctors and nurses all get a kick out of quizzing him to see if they can stump him.  Now we need to find a friend for him who likes geography and wants to share this interest with him!

The PICC line has been removed!!

We are happy to announce that Peter had his PICC line removed by the team in Boston this past Thursday!!  Alleluia!! 

Right after line was pulled out, pressure being held over the site. 

Happiness to have no more PICC, to understand that the hard work of eating has paid off, and to realize that pulling the PICC really did not hurt after all.

Peter has not been without a central line (in his case, a PICC line) since he was 5 months old.  He is now 7 years and 7 months old. 

It is strange, but delightful, to not have a PICC line to worry about right now.  Coram and I are going to arrange the pick-up of his four infusion pumps this week: one for TPN, one for Omegaven, one for IV fluids, and one for IV iron.  Bedtime is now just meds through the G-tube and hooking up the formula.  No ostomies, no IV flushes or IV meds.  There has been so many significant achievements over the years, and especially over the past several months.  We are so grateful!! 

After such great news, I am a little reluctant to mention the downside to Thursday's visit in Boston, yet there are some things still concerning the team.  One is that Peter's abdominal and chest veins are more prominent than ever.  Dr. Jennings thinks the veins in the neck are also distended.  We will follow up next month with an endoscopy to look at the esophageal veins, and possibly an echocardiogram of the heart to check on the vessel size there.  The concern, as I understand, is that his portal hypertension may have gone up.  The ultrasound of the abdomen on this past visit looked good though, so it is possible that these more prominent veins are only looking more distended, but are not actually more problematic.  Peter also may be having some absorption issues in his gut.  We are sorting that out with some stool testing from home and increasing some of his calories and vitamins to see if that helps. 

One could ask why the PICC line was pulled out if everything is not perfect.  Peter has gone two months without using his PICC line for IV fluid or TPN.  He has gone one month without needing IV iron, and in fact has brought up his iron stores better with enteral iron over the last month.  Continuing with the main goal as preservation of the liver, it is better for him to have no central line than to later on have to put one in again if he demonstrates need for one.  A central line is made of plastic and bacteria love plastic, so there is always a fairly high risk of infection that is fairly when someone has a central line.  Infections from central lines can quickly lead to sepsis and organ damage, and the liver can be particularly affected.  We also go back to that quality of life issue, where it makes sense to get rid of something burdensome if it is not really needed.  Peter can now get wet (summertime!) from head to toe and there is no line to worry about.  He isn't so keen on the idea of going in a pool, but is thrilled to be able to wash his own hands and not have me worry that he'll get his PICC line wet.  Won't it be grand if he can keep the PICC line out for good!