Teeth updates, fat-soluble vitamin deficiencies, and eating

Peter's teeth color has been discussed many times and research suggests that there seems to be some evidence that bilirubin-stained teeth sometimes respond to bleaching.  As I wrote in an earlier post, we were planning to give bleaching a try.  Around February, we tried bleaching Peter's two top front teeth several times through the local dentist as a trial to lighten the discoloration.  Unfortunately, and though not completely surprising, somewhat disappointingly, there was no color change at all.

We are trying Peter on pancreatic enzymes as of a couple weeks ago.  Peter's fat-soluble vitamin lab values remain low and seem to be falling over the last several months, despite the fact that he is taking in high doses of vitamins A, D, E, and K.  At CAIR in Februrary, we discussed his stool and tests were run to see if his pancreas is working well.  The lab values for the pancreas (pancreatic amylase, I believe) came back normal as they have in the past.  Dr. Kamin said there is research that with short bowel, some people improve their fat digestion with extra pancreatic enzymes, so it seems worth a shot to see if they help him.  His stool is looking more normal and we should be checking labs sometime soon to see if his fat-soluble vitamin levels are responding as well.  He doesn't seem to be putting on any weight as of yet, but we are excited about the potential of correcting the vitamin deficiencies anyway. 

Peter is continuing with feeding and occupational therapy to try to improve his eating.  He had been chewing in such a way that was fatiguing him early in his meal and would often pocket his food and even gag at times.  The feeding therapist feels like he has improved the chewing skills well, and we are in OT now trying to help to desensitize some of the eating reactions/resistance he has.  Peter repeatedly has stated he wants to get rid of his G-tube, as it leaks and hurts with some frequency, so we started these therapies to help him work towards that goal.  He takes in 1000 calories of formula through the G-tube overnight, so it is still a long road ahead before he can fit in that many additional calories in during the day and get rid of the G-tube, but we are at least making some progress.  As we go along, his short bowel symptoms may put a halt to how much he can eat and make it so the overnight feeds need to continue, but at least the ability to chew will not and perhaps he will enjoy eating more along the way now that his eating skills are improving.

We got the approval from the CAIR team that Peter can wait another 6 months before going to Boston again.  Twice a year visits seem like a luxury compared to 6 to 12 visits a year!

Help save The Prouty Garden at Boston Children's Hospital

This is an email sent out earlier today.  I am posting it in hopes that others will read it and want to sign the petition as well.  

 

 

 

Hello to all of you.

The Prouty Garden at Boston Children's Hospital is at great risk of being bulldozed down for more hospital space.  The Prouty Garden is truly an oasis in the middle of the hospital's grounds.  Peter and I went there countless times during his many hospitalizations and medical visits.  It never failed to cheer us up, rain, snow, sun, heat, or cold.  It was a break from being confined indoors, from being a patient.  Peter found snails, rabbits, squirrels, smelled the flowers, tasted the rain, felt the warm sun on his face.  Having experienced a lengthy hospital stay at another hospital without any place for patients to go outdoors, I can emphatically state how much that garden is helpful to the patients and families at BCH.

Two years ago, a petition was started in effort to save the garden. There have also been several news articles about the probable loss of the garden  At this point it looks like there aren't that many more signatures needed to hit the petition goal of 10,000 signatures, and I imagine the goal would be surpassed if more people who have visited BCH knew about the petition.  Would you consider reading the petition and signing?  Would you also consider asking others you know to sign?  The site is change.org, and to keep them from sending you more petitions, just hit the Unsubscribe link in the email that they send you.  This is the link for the Prouty Garden petition.   Boston Children's Hospital: Preserve Prouty Garden

				Boston Children's Hospital: Preserve Prouty Garden The Prouty Garden is a priceless treasure. Designed by the Olmsted Brothers, it opened in 1956 as an endowed gift from Mrs. Prouty, who was a devoted friend and ben...
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Thank you,

Kathryn

Photos and drawings from summer and fall 2014

Here are some photos from the past several months.  Peter's wish is to be a world traveler.  We took several trips closer to home than around the world.  Peter draws a lot, and you might be able to tell by his subject matter that he spends a good amount of time looking over travel books. 

Independence Hall

At the Liberty Bell

Boston  Commons August 2014

Massachussetts State Capitol building

On hotel roof in Atlanta, GA

Greenville Zoo, SC

9 years old now....

Almost eight months between entries seems a bit long for a blog!  The good news is that things have just been busy with some of the more normal things in life, so there has been less time or need to chronicle the happenings with Peter.  He just had his 9th birthday though, so it seems like a good time to catch up on where things are with him.

This summer, Peter had several trips to the swimming pool and found that he really enjoys going in the water.  We also took him to Georgia for a wedding, and discovered how much he enjoys dancing if he has a partner, which he was very good at obtaining.  We had known that he is very social, but had no idea that he would actually work the room the way he did.  Other special events were included spending a day at Hershey Park, a day at Dutch Wonderland, and an evening at a carnival.  He was quite adventurous on the rides this year.  We also took him to Luray Caverns, several playgrounds and a clean McDonalds indoor climbing maze, and of course there were several times that we enjoyed fun times with friends and family. 

Peter and I made our first solo trip to Boston Children's Hospital this summer and we did just fine... no vomiting, only minor issues of getting lost, traffic not too bad.  Peter and I were up there several days and had several little excursions in the city in between doctors' visits, at Peter's request.  We had the privilege of being the recipients to a real act of random kindness while we were there.  On the Wednesday morning of our visit, Peter had an endoscopy and two molars pulled.  (The permanent tooth was actually growing right through the baby tooth and needed to be pulled; the dentist thought we should go ahead and take out the same tooth on the other side while we were at it, since it was loose.)  It was absolutely pouring sheets of rain outside, and I realized that the umbrellas I had brought were inadequate, as one was too tiny and the other would not stay open.  I told Peter that we would need to catch a shuttle back to where we were staying and hang out there for the afternoon instead of going to the big mall that he had wanted to go to, as we would get too wet trying to get to the car and catching the shuttle under the awning of the hospital was the only way to keep dry.  A woman overheard us and offered to trade her working jumbo-sized umbrella for our tiny, battered one.  I was a little reluctant to accept her umbrella, as it was an unfair trade to her, but she insisted that she would not have offered if she had not been sincere.  A few minutes later, she then decided that we should keep our tiny one and just take hers as well, so Peter and I both had an umbrella to hold.  I asked her how she would keep dry, and she said her raincoat would suffice and that she would be very happy if we took her umbrella so that Peter could go off to the mall and enjoy the rest of the day the way he had been hoping.  It might seem like such a little act of kindness, but it absolutely made such a difference in our day.  Peter was able to walk around instead of sitting back at our room; at the mall he had a meal that he still talks fondly about; and we were both made to feel very special by the kindness of a stranger... an angel in our eyes.

Peter's bilirubin and INR have bumped up a little bit over the past year.  That, along with the cardiology findings in February, prompted Dr. Kamin to have us, meet with the hepatologist (liver doctor), Dr. Fawaz.  Dr. Kamin has talked with her extensively over the years regarding Peter, but we had not met her in person before.  She was very attentive and took two full pages of handwritten notes as we discussed Peter's history.  Like everyone else, she is not really sure whether Peter's issues are primarily due to the liver or to the clot he had in the portal vein.  She said his biopsy from four years ago did show some cirrhotic changes to the liver, but in early stages of cirrhosis, it may be possible to reverse the damage.  There is no real way to know whether the cirrhosis is better or worse without a biopsy, but w did run two indirect tests on his liver.  The first was to give two shots of vitamin K to see if the liver would respond and decrease Peter's INR at all; it only made the tiniest drop in the INR number.  We also did a trial run off his diruetic, but after a week he gained about three pounds of fluid and he needed to restart it.  Basically, he failed both indirect liver tests, but at this point, no biopsy of the liver is planned.  Peter had a huge growth spurt between his spring and summer CAIR visits and it seems imprudent to do an invasive biopsy unless there are more obvious signs it is needed.  Meanwhile, we added oral fish oil to his diet to see if that helps his liver.  The Omegaven team has many patients who switch to oral fish oil once they go off TPN/Omegaven, and those patients seem to benefit from continuing with oral fish oil once the IV fish oil (Omegaven) is stopped.  We had started oral fish oil for a few months after Peter stopped TPN, but then stopped giving it since I thought it was making his stools loose.  We started it again in mid-September, but have not had any labs done yet to see if it has had any positive effect on his liver labs.  It does not seem to be hurting, at any rate.  He is taking Complete Omega Nordic Naturals at the suggestion of the Omegaven team, and it has a lemon flavor to it which Peter does not mind.

We did need to go up on Peter's enteral feeds overnight.  If I recall correctly, he is getting 1000 calories by G-tube overnight now.  He had been getting 860 calories overnight prior to the August CAIR visit.  As I said earlier, Peter had a good growth spurt between CAIR visits, but his weight did not keep up with his height and the team decided it best to increase his caloric intake. 

Peter started feeding therapy earlier this month.  We are working to get his chewing better coordinated so he can start eating more throughout the day and maybe go back to less formula in time.  Peter has asked almost daily when he can get rid of his G-tube, so it seems like a good goal to work on ways to help him eat more effectively, and it makes him an eager participant in his therapy.

Lastly, we have some dental plans for him at this point.  Although the staining in Peter's teeth is throughout the teeth, some of the dentists think it may be possible, though others say unlikely, that bleaching may help.  Between the dentist in Boston and the local dentist, we are devising a plan to try to bleach the front top two teeth to see what happens.  Boston suggested then trying composite veneers if there is no luck with bleach.  However, the local dentist, and the several other dentists that we have consulted, say to avoid veneers until he is older, primarily due to the increased risk of decay underneath the veneers in the younger years.  Peter's molars are all coming in white, and at this point he only has 8 permanent teeth that are brown (2 on the top and 6 on the bottom).  We are glad to see that not all of the teeth will need to be corrected in color, especially if bleaching does not actually work.  More to follow once the bleaching has been done.....

Happy Anniversary!

Peter’s one year anniversary off TPN was March 21st!  Though I never updated on this blog, we did celebrate this great achievement with him.  It such a blessing that he was able to get off TPN and has been able to stay off it. 

Peter had his medical visit to Boston Children’s Hospital the day before his anniversary, coincidentally.  Like the last visit, he is able to wait another four months before his next check-up.   We feel almost spoiled at the four-month intervals compared to the six-to-eight-week intervals that had been necessary for so many years.  At his CAIR appointment, his arm circumference and skin fold indicated he has had some real weight gain versus just fluid gain.  His Echocardiogram and EKG indicated that the heart is stable with no changes since the last tests.  The cardiologist said that “stable” is our goal for Peter.  The cause of the heart issues we have been assessing the last couple visits is believed to be liver disease/portal hypertension.  Since Peter is off TPN and the liver seems stable, the heart should remain stable as well, since something  about the liver/portal hypertension is causing the heart to work harder.   We will repeat the Echo and EKG in another six to twelve months, again hoping there are no changes. 

We still continue to see calcium in Peter’s urine.  That never stopped once he came off TPN.  A 24-hour urine test confirmed this, and we have seen elevated calcium in all of his urine tests the last couple years.  He has been put on potassium citrate twice a day to try to alkalinize the urine so kidney stones do not form.  Fortunately, he likes the taste of this medication.

Peter has been working hard at learning to chew better.  A goal that we set for him is to get off purees and eating solid foods.  He has had very little trouble over the years when it comes to meltable types of carbohydrates, such as crackers.  However, when he would chew items such as meat, nuts, fruits, or vegetables, they would end up pouched in his cheek, instead of getting swallowed.  A few months ago, we told Peter that when he learns to eat enough solids that we can use them for his dinner instead of using jarred baby foods, then we will take him to see Philadelphia.  Well, as the spring warms up and dries out, we will be going there, as he has done a great job with this. 

Sadly, Peter is becoming more aware of his physical differences.  We hope to find a way to correct the color of his teeth this summer, and I think after summer we will need to look into lasering the tiny broken veins on his face as we did a couple years ago.  They have become a more prominent again, as the dermatologist suggested they might.  Peter doesn’t seem focused on these issues, but it is apparent to us that other people do notice and sometimes even react to them.  We can’t do anything to get his belly lesser in size, although it has been a long standing hope that as he grows the portal hypertension will lessen and his ascites will decrease.  His belly sticks out quite far from the rest of his thin body, but he fortunately does not seem to worry about that, and his clothing conceals the size of his belly fairly well. 

His concern about being different is mainly about  having a G-tube and how that makes him different.  It seems best to let the G-tube be his focus then, as that is something he knows he can work on getting rid of one day if he wants to really try.   It is his decision on how hard to try, and we think it does him good to have that sense of control.   He isn’t really trying to get rid of it now, but it seems to satisfy him to know that he can likely get rid of it one day when he feels like he wants to put forth that amount of effort in eating.  We’ve showed him many photos of bellies with G-tubes and discussed some other kids we’ve met who also have G-tubes, but that is not convincing him that he likes his.  He only takes in 400 ml of formula overnight and everything else is by mouth during the day, so it is possible he could switch over to taking everything by mouth when he feels he wants to begin that process. 

Peter will be finishing up second grade in another couple of months.  He is homeschooled  and we’ve been working hard to get him caught up in his studies so that he is on par with other second graders.  It is amazing how much developmental time was lost between surgeries, recoveries, being in the hospital for a year as a baby, and just not feeling well.  It is even more amazing to see how much he has caught up over this last year, with studies and physical activity.   He is still riding his Big Wheel and has a surprisingly good throwing arm with the Nerf football.  I’ll try to get some of his artwork up on the computer soon; the occupational therapist he used to have would be pleased with the control he has over his pens and pencils these days.