SickKids

Life without Peter is very difficult. I don't know what to even say about it outloud or online, so I won't even attempt anything here at this point. Lots of tears and heartache continue, good days and bad, but a lot of emptiness without Peter, for all of us. Meanwhile, these commercials recently came to my attention, and if there is anyone who visits this blog anymore, they are worth seeing, although they will also make you cry.

This commercial is one I saw posted on one of the facebook medical sites I used to visit for support with Peter's care. It does a pretty good job capturing the "mom" experience of a chronically ill child. SickKids VS: MomStrong

https://www.youtube.com/watch?v=HvNF0yFUcx0#action=share

This next one also captures what it is like to watch your chronically ill child go through countless procedures as he is growing up. This is the commercial that reminded me so much of the endless procedures, doctor visit, medical tests, fears, and hopes Peter went through. SickKids VS: This Is Why 

https://www.youtube.com/watch?v=vh-WS1FGKTM

Peter went through a lot in his short life. He was a brave kid, like so many who face chronic and/or serious illness. He met a lot of amazing, loving medical heroes along the way, too, patients and staff. https://www.youtube.com/watch?v=78mNZeDaMtk&feature=youtu.be

Farewell Dearest Peter, Rest in Peace

So much has happened since I posted a year-and-a-half ago. Most of that delay has simply been because Peter has been busy -  medically, being a kid, and as part of a semi-large family. 

In the following months, I may eventually update more to his blog, or let things just remain as they are. For now, I share with you portions of Peter's obituary. The photo on the rooftop of the hospital captures his smile and enthusiasm throughout that whole last day. We miss him terribly. 

On August 9, at about 3 AM, Peter Joseph Michalski passed away at the age of 13. 

Peter was preparing to enter seventh grade this year with his younger brother, D. This would have been their first school experience having been home-schooled in part due to Peter’s frequent medical travel and a compromised immune system. His constant companion and best friend, D, was looking forward to accompanying his big brother on this new adventure. 

Peter's life-long medical and hospital journey began at four months old with three emergency admittances and nearly a year-long hospital stay. He had multiple major surgeries in that time related to his liver and intestines and resulting complications. There were many more subsequent operations and procedures in the years following, along with several years of in-home nursing care and therapies.

Peter’s medical care was transferred from Philadelphia to Boston Children’s Hospital after more than half a year inpatient in order to be part of a trial therapy with Omegaven, an innovative lipid therapy for those requiring IV nutrition. His ongoing care and hospitalizations required travel to Boston every six to eight weeks with the doctors, nurses, and support staff becoming his friends and a caring extended family.  

Peter’s great interest in life was the globe he wished to eventually travel. Early on, places and cultures captured his imagination and through a continual study of maps and atlases, he achieved a starling mastery. He visited places vicariously through the trips of others and their photos, inquiring of details with probing questions about the number of floors in a particular building, whether they had seen a prominent landmark, and of course, what they ate along the way. He had committed to memory the nations of the world, the capitals, notable architecture, and an array of facts to such an extent, that, though an unlikely contender with his disarming playful and cheerful manner, he handily won a geography bee locally. 

But, it was people he really loved, and it was hard not to love Peter back. He had an authentic interest in others and would never tire of another person’s presence. He made everyone feel like they were interesting and worth listening to. He wanted people to know him, who he was, and what excited him about life and the world that many take for granted. As someone from Boston Children's Hospital noted, he was like the Mayor of the hospital, everyone knew him and faces lit up as he would recall the details from the last time they talked, beaming with a wide genuine smile. 

He touched the lives of all who encountered him including people around the world who were aware of his illness and who sent prayers and support. 

As Peter was being wheeled into what would have been a multi-organ transplant surgery that never had a chance to proceed in full, he was chatting with the doctors and nurses about where they might be traveling next. He was happy and excited going into surgery, he had no fear, he was not in pain, and most importantly, he was at peace with God surrounded by his family and many others who loved him. 

Peter was the fourth of five children.

He will be deeply missed. Peter Joseph, pray for us. 

In lieu of flowers, donations to the Oley Foundation in memory of Peter would be appreciated. The Oley Foundation is a large support network for people who depend upon IV nutrition and/or enteral tube feeding, of which Peter depended for most of his life. Peter found great pleasure in  attending one of their annual conferences last year, and meeting dozens of other people on IV/tube feeding, just like himself. https://oley.org/donations/donate.asp?id=8905

Back on TPN... as of last April!

I'm glad that these blog accounts don't just disappear after long silences. Maybe it is best to just update in summary form from time to time, versus delivering updates blow by blow. It does make for some very long pauses, but it also means less time on the computer, too. Here is the latest in summary fashion.

Last April, after 4 years off TPN, Peter went back on TPN. He had been having a slow nutritional decline, which was only obvious in retrospect. We were noticing small, but increasing issues, such as hand tremors; episodic "pins and needles" in his hands, feet, and legs; a strange month-long eruption of red-brown petechiae-like spots over both legs; heavy dandruff. Peter started losing weight, and then he went into a full month of GI distress/increased gut motility, ending up with a loss of about 10 pounds over a two month period, and episodes of rectal bleeding. The team was concerned not only about the nutritional losses, but the possibility and likelihood that Peter was entering liver failure. Easter weekend, Peter was admitted to CHOP and put on IV hydration and then went up to Boston Children's Hospital to get a central line (a Broviac instead of a PICC this time) and start TPN. The team agreed he could also go back on Omegaven as his lipid. Within a month or two, Peter had regained every bit of the weight he had lost, and the unusual symptoms he had prior to restarting TPN had gone away. Peter's labs started looking a little bit better as well.

Peter is happy about going to Boston every two months again, as he loves to travel and cities are of great interest to him. He is quite frustrated having a central line and TPN, though. His liver is being carefully watched and monitored, as most of the images and tests suggest it is in some stage of cirrhosis. We spent many months this past year thinking that he will imminently need to get re-evaluated for a multivisceral transplant. As of our last visit to Boston, though, the decision is that we can hold off on listing a little longer for now, given his overall gains and improving labs. He will get another MRI of his liver in four months, and we'll see how that compares to the one he had towards the end of 2017.

On a less medical note, Peter is 12 years old now! He still enjoys geography and fact books and trying new foods (even though his appetite is down now that he is on TPN again). In addition to wanting to be a world traveler, chef, cartographer, and artist, this year he learned about Thomas Edison and has become inspired to add "inventor" to his list of career aspirations.

Physicians as part of the cure

Peter still has ups and downs with liver health and nutrition absorption, but overall he is doing fairly well. One of these days I'll update the health issues, but he is well enough that things are busy in a good way, leaving the writing of updates on the computer as low priority. It did seem worthy of logging on today to post the following though.

St. Hildegard is one of the saints of the day today. Per the site, http://www.catholicireland.net/saint-day/  :
 
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"... St. Hildegard of Bingen [was] a visionary and mystic in medieval Germany....  On the question of illness she has these wise words to say: ‘The best of all remedies remains the doctor’s compassion and his participation in the suffering’."

Upon reading the quote, it struck me again how special are Drs. Kamin, Puder, Jennings, Kim, Fawaz, and several other dedicated physicians that Peter has had along the way. They possess a compassion and a willingness to participate in the lives of their patients, which transcend patient care beyond medicine. They don't shelf patient care once we have left the office, but continue to think and work on solutions to improve Peter's quality of life. Their dedication to patients and families is remarkable, and we have been blessed to have them in our lives!

an update on Peter's teeth

Look at this smile! Isn't it great?!
5 teeth have been color-corrected so far. Peter will have the last bottom front tooth covered in February, and then the dentist will assess where we stand in correcting the teeth that flank those front bottom four. I am not sure exactly the dentist has used to do this, other than to etch the teeth first, paint the with some type of tooth paint like white-out, and then use a composite to cover the teeth. If I find out more specifically what was done, I'll post it for reference.

getting teeth color corrected... finally

two newly bonded teeth

This top photo is the progress that has been made on correcting the color of Peter's teeth.  So far, just the top two have been done, and they may still need some further shaping, but they are definitely no longer brown.  The dentist painted them with some type of dental "white-out" to block out the dark color of his own teeth and then did composite bonding over that.  The lower teeth will be corrected next, in December.  Peter will still need orthodontia at about age 12, but the braces are expected to go right over the bonded teeth without much of an issue.  We are all excited to finally have a solution to correcting the color of Peter's teeth!

prior to top two teeth bonding

a perfect 10!

We celebrated Peter’s 10^th birthday a week ago!  His birthday is on All Saints’ Day, the day after Halloween.  He had a great weekend, trick-or-treating as a knight and then a birthday party with lots of family the next day. 

the brave knight

a happy 10th birthday

Peter has been feeling good and growing tall.  He has become much more interested in playing with traditional toys for boys this last year – cars, trucks, Legos. His favorite go-to items are still books, particularly geography books, atlases, and travel books, but he is definitely incorporating more play into his daily activities than he used to.  

Feeding therapy last fall/winter/spring definitely paid off.  Peter took a break for the summer, but the learning continued on its own and he is now eating two meals a day that involve real chewing.  He is still slower to eat during many of the meals, especially when there are veggies or meat he doesn't like as much, but progress continues. 

Peter went to Boston Children’s Hospital for his check up in August.  The hepatologist started Peter on lasix (furosemide) in August.  The hope is to reduce Peter's ascites and let him eat more sodium than he has been eating. One of Peter's chief complaints with food has been the desire for flavor and even with huge amounts of spice, the absence of salt still keeps a lot of foods "tasteless" in his words.  I don't see that the ascites has really left, but adding more sodium to his low-salt diet hasn't increased his ascites visibly either, so the lasix must be doing what we had hoped.

His upper endoscopy was good with no need to band the small varices that he has.  When labs were drawn, his INR was up to 1.6 from the 1.3 value he had a few months ago, and that caused some concern. However, the hepatologist felt we should try a dose of high-dose vitamin K to see if that would reduce the INR, since we know that Peter has trouble absorbing other fat-soluble vitamins. (On 8000 IU of enteral vitamin D per day, his vitamin D level is still not as high as desired, for example.)  We had tried this almost a full year ago with no real response, but it seemed worth another try.  If the INR stayed up, then it was likely that the liver is declining in function and is getting increasingly cirrhotic. If the INR responded, then the liver, while not exactly healthy, is still working well enough and the elevated INR was due more to intestinal malabsorption than declining liver function.  Peter received the vitamin K intramuscularly and when the labs were drawn several days later, his INR had decreased back down to 1.3!  Success!!!  The plan now is for Peter to receive vitamin K intramuscularly every month. 

Peter is in the process of getting the color of his teeth addressed.  He had a couple bleaching sessions that ended up not changing tooth color at all. Then, when at U of PA for an orthodontic consult, we were asked if we would like to address the color of his teeth. I nearly fell over at the question, as I have been asking and asking for help with the teeth color over the years, and here I was approached instead. So far, we are just working on the top two front teeth. I’ll post photos once they are done, but the process is taking more than one day to complete due to Peter’s inability to stay still long enough in the dental chair.  

In case you might be wondering where Peter's glasses are in the above photos, they are sitting broken in his glasses case as we await a replacement pair.  He had a mishap with a blanket in the living room and that was the end of that pair of glasses.   :/

A few more photos from the year to add to the update...

a long-anticipated view from the Top of the Hub in the Prudential Center, Boston

 

first visit to the beach

loving the rides at Hershey Park 

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...
				View on www.change.org	Preview by Yahoo

 

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. 

I never did post about Peter's catheterization that he had in November.  He did well with the procedure, but the results have been difficult to interpret.  The doctors have discussed and rediscussed the results, but the cause of the enlarged heart and increased O2 saturation in the right ventricle is unclear.  One of the causes could be another AVM (arteriovenous malformation) which has yet to be found, but would likely be in the abdomen.  None of the many tests he has had over the years has found one though.  Another cause could be that the liver is declining in health, but Peter's overall health and labs do not seem to be really pointing to that.  The portal hypertension Peter has could be causing it, but during the catheterization, the portal pressures were "reasonable."  So for now, the cause remains uncertain.  The plan is to get another ECHO in March and compare it to this past July's ECHO.  We are all hoping that the cardiac changes that seem to have occurred over the last three years are not progressive, and the ECHO should be a good way to evaluate that.  If they are getting worse, then we will need to explore the possible causes a little further to see if we can stop further changes.

I do want to share the very good news that Peter continues to be doing well off TPN.  His overall growth was stable enough at November's CAIR that we can wait 4 months for our return check-up, with a lab check in between.   This is the longest that we have ever been able to go between visits!

Cardiac and weight updates

The nutritional tests that were done a couple weeks ago have come back.  They are normal, so the team does not feel that the cardiac issues are related to a nutritional issue.

The plan now is for Peter to have a cardiac catheterization to see what the cause is for the increased cardiac pressures and enlarged ventricles.  The cardiologist kindly called me at home to discuss the procedure and why he feels we should proceed with it.  Basically, he feels Peter's heart itself is not the issue, but since this is a fairly big change since his last cardiac imaging from two years ago, the cause needs to be found before the heart itself does become compromised and begin to work less effectively.

Although it only takes a little bit of room for me to type this up, the amount of discussion and consultation that went into this decision has been quite detailed and involved several top doctors at Boston Children's Hospital.  I am a little worried about the catheterization, but only because I know it is invasive, not because of doubt on the parts of the physicians.     

On the weight issue, I can't see that Peter has lost any more weight, but it is unclear if he has gained any.  I can tell he has a little more fluid in his belly, so that adds some more weight to the scale, but his arms do not seem to be measuring any less around, so I will venture that he is at least holding with his real weight.  We've definitely bumped up his calories since the last visit, with another 80 ml of formula per night, an additional 20 grams of Duocal per day, and about two to three tablespoons of peanut butter per week.  (Skippy now makes chocolate PB and honey PB, which he thinks are terrific.  He had given up on plain PB a long time ago, but thinks these flavors are great.)

Peter will be eight years old in just thirteen more days!!  He wants Klondike bars as his "cake," and a lengthy trip to the library so he can look at lots of books on architecture and geography.  He is such a character! 

Overnight feeds with no leaks!

I have to share a great item with those of you who use G-tubes.  I came across it from another parent's site, but it took me 7 years of G-tube feeds to find out about it, so I am trying to spread the word from this blog as well. 

It is a little device used to secure the feeding connector and keep it from disconnecting from the port of the feeding tube.  It does not put holes in clothing like safety pins, nor is it sharp and capable of piercing tubes or skin like a safety pin.  It is reusable and we are still on our first one even after 4+ months of use. 

It is called an AMT Clamp Feeding Tube Connector.  I have been able to obtain it through both of the enteral suppliers that Peter has had.  (We just switched to a new provider last month, and I am so happy, but that is anther post for another time.)   Here are photos of the AMT Clamp, along with reference numbers and what it looks like in use:

Before clamp is attached to port. 

(I always tape medport closed to keep it from opening overnight, 

in case you are wondering why it is taped.  You could use the

 clamp without tape on the medport if you wanted to.)

       With clamp attached to port.

                                                  

 Clamp is tiny, flexible, and reusable

 As you can see, we run Peter's tube through the neckline of his shirt, rather than under.  For him, that works better at keeping the feeding tube from kinking overnight. 

I hope this information helps other tube-feeders out there. We've had no feeding tube leaks and no torn bedclothes from safety pins since starting with this product!

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!