"Bob 'd Builder" for Halloween
Friday, November 27, 2009
Halloween and birthday photos
Our miracle child celebrating his fourth year! There is much to celebrate!!
"Bob 'd Builder" for Halloween
"Bob 'd Builder" for Halloween
Saturday, November 21, 2009
Back from Boston
NORMAL bilirubin - direct and indirect!
NORMAL albumin!
NORMAL hematocrit! (hemaglobin still a little low, but pretty close to normal!)
Wait, there's more....
Another night off TPN now, for a total of three nights off TPN per week!
We had a very good checkup in Boston... good labs too! Our biggest focus this time was that we still need to work on Peter's vitamin D status. Even though we have had him at twice the usual dose for the last 3 months or so, his stores still do not seem to be high enough. He will now go to 600 IU of vitamin D per day. During this trip we found out that Dr. Lo, one of the doctors that we see in CAIR clinic, has his PhD in vitamin D metabolism. That sure puts my mind at ease as we assess Peter's vitamin D, calcium, and overall bone status to assure he gets as far away from his past osteopenia as possible.
NORMAL albumin!
NORMAL hematocrit! (hemaglobin still a little low, but pretty close to normal!)
Wait, there's more....
Another night off TPN now, for a total of three nights off TPN per week!
We had a very good checkup in Boston... good labs too! Our biggest focus this time was that we still need to work on Peter's vitamin D status. Even though we have had him at twice the usual dose for the last 3 months or so, his stores still do not seem to be high enough. He will now go to 600 IU of vitamin D per day. During this trip we found out that Dr. Lo, one of the doctors that we see in CAIR clinic, has his PhD in vitamin D metabolism. That sure puts my mind at ease as we assess Peter's vitamin D, calcium, and overall bone status to assure he gets as far away from his past osteopenia as possible.
Friday, November 13, 2009
A new age, feeds, and the new tooth
Peter has turned four years old! Peter's birthday is All Saints' Day. It was an exciting birthday for all of us, as this is the first year that he really seemed to understand and care that it was his birthday. He even had gift requests - books, puzzles, and chapstick (he is a mouth breather and his lips get very dry if we don't keep up with ointment and chapstick). He decided upon pumpkin pie as his "cake," since he has to have pureed foods still. Better than the pumpkin pie was the whipped cream, though. He has asked for whipped cream again.
Photos of birthday and Halloween to follow soon.
On the medical front, Peter has made good strides (great strides for Peter) with his feeding increase sice we were in Boston nearly two months ago. He has gone from two ounces of baby food daily to five ounces of baby food daily. Vital Jr. has increased from 650 mL per day to 850 mL per day. He is taking a little over half of the Vital Jr. formula overnight via pump, and the rest is by mouth. All of the baby food is by mouth. He is even eating Gerber Puffs and Gerber Li'l Crunchies now. He ate a whole can of Gerber Li'l Crunchies this week in only about 5 days. They look like cheese puffs, and he ate about 85 of them over 5 days!
The new tooth is beginning to emerge. The top is........ BROWN! We are disappointed, needless to say. We are praying that the rest of his teeth will look better. The dentist told us two years ago that the only way to "fix" the color of teeth stained by bilirubin is to veneer them. Bleach will not fix the color. Hopefully they will be strong... more important than color in the long run.
Photos of birthday and Halloween to follow soon.
On the medical front, Peter has made good strides (great strides for Peter) with his feeding increase sice we were in Boston nearly two months ago. He has gone from two ounces of baby food daily to five ounces of baby food daily. Vital Jr. has increased from 650 mL per day to 850 mL per day. He is taking a little over half of the Vital Jr. formula overnight via pump, and the rest is by mouth. All of the baby food is by mouth. He is even eating Gerber Puffs and Gerber Li'l Crunchies now. He ate a whole can of Gerber Li'l Crunchies this week in only about 5 days. They look like cheese puffs, and he ate about 85 of them over 5 days!
The new tooth is beginning to emerge. The top is........ BROWN! We are disappointed, needless to say. We are praying that the rest of his teeth will look better. The dentist told us two years ago that the only way to "fix" the color of teeth stained by bilirubin is to veneer them. Bleach will not fix the color. Hopefully they will be strong... more important than color in the long run.
Saturday, October 17, 2009
Lost a tooth!
Peter just lost his first tooth... and he is two weeks shy of 4 years of age! It is his front lower left tooth, which makes its loss "normal" although it seems an early age to lose a tooth at all. We noticed it was loose only a handful of days ago, and when I brushed his teeth tonight, it just popped right out. We are praying that his permanent teeth will not have the gray, green, and brown colors that his baby teeth have from the longs months where his bilirubin levels were so elevated. No tooth is crowning yet, but I guess we'll find out soon.
Wednesday, September 30, 2009
Two nights off TPN per week!
Peter had his Boston check-up last week. It was a very speedy up and back trip spanning about 40 hours total... would have been 38 but traffic got pretty bad around 10:30 pm on the return trip home. During the trip we stopped to see Great-Nana in Connecticut, which was quite nice. Once we got up to Boston, then we had one comlete ostomy change (entailed all 3 ostomy bags needing to be changed), another duodenotomy change, two enteral feeding administrations along with venting and pausing multiple times, and two TPN administrations, one of which was in the van - I do wonder what people think when we set up his TPN while sitting in our parked van in the middle of the parking lot at dusk! And in between there was the unloading and loading of a van full of ostomy and TPN supplies as well as clothing and food and siblings. A family vacation like no other!!
Peter's weight and height have both gone up enough that we are now able to have only 5 nights of TPN per week! Although we still have 2 nights per week of IV fluid, it feels so liberating to not have to mix the TPN bag or to worry about changing over the Omegaven bottle at midnight. And even better than losing another night of TPN is the fact that the weight gain is because of his gut working rather than TPN alone.
We did some other tweaking of his care, including removal of extra vitamin K and extra vitamin C from the TPN (two less meds in the TPN bags is another liberating move). The reduction in vitamin C was done to hopefully reduce the oxalate we see in his urine so that he will not develop stones in his urine. The vitamin K was removed since we have seen his bleeding times stable over the last year and a half. If we see bleeding times increase, then we can always add the vitamin K back in. We are also going to reduce the amount of calcium he receives in his TPN to decrease the calcium losses we see in his urine. We possibly are just giving him too much calcium and this would be an obvious fix if that is in fact the cause for the calcium in the urine.
The bad news from the trip is that his portal hypertension does seem like it is worsening. While the esophageal scoping done last trip showed that the varices are no worse, CT and ultrasound images of the abdomen show continued pockets of ascites and an enlarging spleen. The problem seems to be from the continued clot in the portal vein versus a worsening of the liver, which is better news than if it involved the liver. The thought seems to be that we will currently do nothing, but we can try a decrease in IV sodium to keep him at 2mEq/kg/day to see if that helps at all. If Peter's platelets continue to drop (they have lowered some over the last few months, but are not scary low) then we may see that we need to act surgically to lower the portal pressure with a shunt. This would help divert the pressure away from the spleen and ultimately then help keep the platelet numbers up. If we see intestinal bleeding return or are unable to advance feeds on Peter, that also may indicate that we need to address a surgical shunt for the portal hypertension. Otherwise we will just keep watch, as he has been doing very well with feeds and no bleeds. Since he has intestinal scarring (probably severe), portal hypertension, is missing part of his liver, and he is still only about as tall as a yardstick, surgery is very tricky, especially a shunt procedure deep inside the body.
We will have to address the portal hypertension before any reconnective surgeries. The plan for any surgery (including the shunt) becomes more contingent upon how truly necessary it is. With the ostomy bags now leaking about every 2-3days now, that news hits a little harder than it normally would. We had been hoping to start talks to close up ostomies in the spring, but that is more for convenience than out of necessity for digestion, so likely closing them up will have to wait. Is this terrible news? No. Disappointing, yes, but not terrible.
Peter certainly seems to be doing well despite the portal hypertension. He had at leaast 10 people in his little exam room for his clinic visit. Several came just to see how well this little miracle boy is doing because he does look that good. And Mr. Peter hammed it up well for his audience. We are blessed that this child does not realize that he has an illness and that he is very happy with himself and in general.
Peter's weight and height have both gone up enough that we are now able to have only 5 nights of TPN per week! Although we still have 2 nights per week of IV fluid, it feels so liberating to not have to mix the TPN bag or to worry about changing over the Omegaven bottle at midnight. And even better than losing another night of TPN is the fact that the weight gain is because of his gut working rather than TPN alone.
We did some other tweaking of his care, including removal of extra vitamin K and extra vitamin C from the TPN (two less meds in the TPN bags is another liberating move). The reduction in vitamin C was done to hopefully reduce the oxalate we see in his urine so that he will not develop stones in his urine. The vitamin K was removed since we have seen his bleeding times stable over the last year and a half. If we see bleeding times increase, then we can always add the vitamin K back in. We are also going to reduce the amount of calcium he receives in his TPN to decrease the calcium losses we see in his urine. We possibly are just giving him too much calcium and this would be an obvious fix if that is in fact the cause for the calcium in the urine.
The bad news from the trip is that his portal hypertension does seem like it is worsening. While the esophageal scoping done last trip showed that the varices are no worse, CT and ultrasound images of the abdomen show continued pockets of ascites and an enlarging spleen. The problem seems to be from the continued clot in the portal vein versus a worsening of the liver, which is better news than if it involved the liver. The thought seems to be that we will currently do nothing, but we can try a decrease in IV sodium to keep him at 2mEq/kg/day to see if that helps at all. If Peter's platelets continue to drop (they have lowered some over the last few months, but are not scary low) then we may see that we need to act surgically to lower the portal pressure with a shunt. This would help divert the pressure away from the spleen and ultimately then help keep the platelet numbers up. If we see intestinal bleeding return or are unable to advance feeds on Peter, that also may indicate that we need to address a surgical shunt for the portal hypertension. Otherwise we will just keep watch, as he has been doing very well with feeds and no bleeds. Since he has intestinal scarring (probably severe), portal hypertension, is missing part of his liver, and he is still only about as tall as a yardstick, surgery is very tricky, especially a shunt procedure deep inside the body.
We will have to address the portal hypertension before any reconnective surgeries. The plan for any surgery (including the shunt) becomes more contingent upon how truly necessary it is. With the ostomy bags now leaking about every 2-3days now, that news hits a little harder than it normally would. We had been hoping to start talks to close up ostomies in the spring, but that is more for convenience than out of necessity for digestion, so likely closing them up will have to wait. Is this terrible news? No. Disappointing, yes, but not terrible.
Peter certainly seems to be doing well despite the portal hypertension. He had at leaast 10 people in his little exam room for his clinic visit. Several came just to see how well this little miracle boy is doing because he does look that good. And Mr. Peter hammed it up well for his audience. We are blessed that this child does not realize that he has an illness and that he is very happy with himself and in general.
Friday, September 4, 2009
Pics from July Boston trip
This is Peter in pre-op, looking at the smiley face Dr. Jennings drew on his index finger and his big toe. Those smiley faces produced three days of amusement for Peter!
This is the finished dressing with nursing pad and tegaderm covering the dressing pictured below.
This is the old dressing with bottle nipple over allevyn, over aquacel, over zinc paste.
When you consider that this dressing got changed one to three times a day b/c the leaking was that bad, you can see why we wanted to get that gastrostomy site fixed! Peter and we are all happy with the new, no-dressing-needed site!
Boston updates... from July!
Peter went up to Boston at the end of July for SURGERY! These were not the intestinal closing surgeries that we hope for... one day. But they were surgeries nonetheless. The big deal about "surgery" for Peter is that we had been told in the past the he is a terrible surgical candidate due to his portal hypertension (great bleeding risk), elevated INR (another bleeding risk), and history of poor healing from earlier surgeries on the abdomen. Dr. Jennings has discussed Peter's surgical risks and possibilities with us before (see old posts). As such, we all felt it was a good idea to do these surgeries to help make Peter more comfortable as well as to see if he heals better then he has in the past and to see if the bleeding turned out to be terrible or not.
The surgeries that Peter had done were to move the gastrostomy to a new site, close the old gastrostomy, and close the bilateral hydroceles. This all came about because we were going to be putting Peter to sleep for an upper endoscopy (EGD)to evaluate his esophageal varices (which are due to portal hypertension). I will try to post pictures of his last gastrostomy site and how we had to dress it to keep it from pouring out. The old dressing consisted of aquacel, allevyn, zinc paste, part of a nipple from a baby bottle to stabilize the G-tube, a nursing pad over all of that, surrounded by tegaderm all along the borders. This would be changed one to three times a day, depending on leakage amounts. Our new beautiful G-tube only needs tape to keep it secure, no dressing, just a little skin prep or zinc to protect from scant G-tube ooze. The old site healed remarkably well with some stitches to close the stomach opening and wet-to-dry dressings to close the rest of the hole. It is just a tiny scar now. The hydroceles healed well too. There are two perfectly-healed thin scars from that procedure. Dr. Jennings and Dr. Kamin said that the amount of bleeding was not much, especially considering that he has portal hypertension. Also, the EGD revealed that there has been no increase in the size of the esophageal varices since the evaluation two years ago.
We have started nighttime pump feeds with Peter...very slow rate to begin with. (Elevating the head of a toddler bed-- to keep him from lying flat and getting formula in his lungs while he is tube-fed-- is not as easy as one would think, as the waterproof mattress is slippery and the sheet then moves quite a bit.) We are hoping then to not have to chase him around the house every hour to drink his formula, and to hopefully leave him more physiologically hungry during the day so he will want to work more on eating food. We are still only giving about two ounces of baby food daily, but aspire to increase that over the next few weeks.
Along with pump feeds overnight we are changing from Peptamen Jr to Vital Jr. They are similar products but the flavor of Vital Jr is better than Peptamen Jr and there is a prebiotic in Vital Jr as well.
We have increased his IV iron to see if he can pull out of his anemia. We went from 15mg iron dextran to 25mg. He had pulled out of the anemia before once we put him on IV iron, but he has gained weight and possibly just needs a bigger dose. I am greatly reluctant to put him on enteral iron b/c of the GI side effects that can come from enteral iron.
Back to Boston later this month. That will be just to check in with the Intestinal Rehab Clinic. We are thinking we will get another reduction in TPN as Peter is gaining weight well. He sure seems to feel good!
We have also started vitamin D drops. Endocrinology wants his vitamin D levels up at 30 or above given his istory of osteopenia. Despite going outdoors daily for one to two hours all Spring and Summer, his vitamin D level is only 22.
The surgeries that Peter had done were to move the gastrostomy to a new site, close the old gastrostomy, and close the bilateral hydroceles. This all came about because we were going to be putting Peter to sleep for an upper endoscopy (EGD)to evaluate his esophageal varices (which are due to portal hypertension). I will try to post pictures of his last gastrostomy site and how we had to dress it to keep it from pouring out. The old dressing consisted of aquacel, allevyn, zinc paste, part of a nipple from a baby bottle to stabilize the G-tube, a nursing pad over all of that, surrounded by tegaderm all along the borders. This would be changed one to three times a day, depending on leakage amounts. Our new beautiful G-tube only needs tape to keep it secure, no dressing, just a little skin prep or zinc to protect from scant G-tube ooze. The old site healed remarkably well with some stitches to close the stomach opening and wet-to-dry dressings to close the rest of the hole. It is just a tiny scar now. The hydroceles healed well too. There are two perfectly-healed thin scars from that procedure. Dr. Jennings and Dr. Kamin said that the amount of bleeding was not much, especially considering that he has portal hypertension. Also, the EGD revealed that there has been no increase in the size of the esophageal varices since the evaluation two years ago.
We have started nighttime pump feeds with Peter...very slow rate to begin with. (Elevating the head of a toddler bed-- to keep him from lying flat and getting formula in his lungs while he is tube-fed-- is not as easy as one would think, as the waterproof mattress is slippery and the sheet then moves quite a bit.) We are hoping then to not have to chase him around the house every hour to drink his formula, and to hopefully leave him more physiologically hungry during the day so he will want to work more on eating food. We are still only giving about two ounces of baby food daily, but aspire to increase that over the next few weeks.
Along with pump feeds overnight we are changing from Peptamen Jr to Vital Jr. They are similar products but the flavor of Vital Jr is better than Peptamen Jr and there is a prebiotic in Vital Jr as well.
We have increased his IV iron to see if he can pull out of his anemia. We went from 15mg iron dextran to 25mg. He had pulled out of the anemia before once we put him on IV iron, but he has gained weight and possibly just needs a bigger dose. I am greatly reluctant to put him on enteral iron b/c of the GI side effects that can come from enteral iron.
Back to Boston later this month. That will be just to check in with the Intestinal Rehab Clinic. We are thinking we will get another reduction in TPN as Peter is gaining weight well. He sure seems to feel good!
We have also started vitamin D drops. Endocrinology wants his vitamin D levels up at 30 or above given his istory of osteopenia. Despite going outdoors daily for one to two hours all Spring and Summer, his vitamin D level is only 22.
Sunday, August 23, 2009
July photos
Peter is moving on to toddler activities such as coloring. We are at a point now (August, despite the pics being from July) where we are able to get him out more and HE is now enjoying it more. It is heartening to see Peter feeling so good and wanting to do more and more things.
Do you see what is peeking out of Peter's shorts? Peter wears two ostomy bags - one for a mid-abdominal fistula and one for his ileostomy. They unfortunately are exposed during shorts season when Peter sits down. See below for a great solution!
I purchased two fabric ostomy bag covers online made by another mom whose child has an ostomy. Peter is wearing a navy blue one here and it really isn't too obviousm covering up what otherwise it quite obvious and rather a bit of a put-off to see poking out of his shorts. This mother's son uses the cover bag so that he can wear his ostomy bag over a pair of shorts. Peter's torso still isn't long enough where we can go to shorts and a shirt, but the cover bags also work well under a onepiece outfit like Peter wears. If you are interested in these bags, we found them at Gus Gear (www.gusgear.net). So far they have held up very well. Peter likes having the ostomy bags covered when we are out too... especially the one with zoo animals on it.
Tuesday, June 16, 2009
Kidney update
We went to the nephrologist today. The protein in the urine is very slight at this point! We are reducing Peter's enalapril from 2.5 mg a day to 1.5 mg a day. We'll return to the nephrologist in 3 months for another check up. He feels Peter still has IGA nephropathy as the cause of glomerulonephritis, but that Peter has a very mild case of it. We will continue to monitor to make sure that it does not increase in severity, but otherwise the report is very, very good!
Saturday, May 23, 2009
First night without TPN
Last night was our first night with no TPN, no Omegaven. He is still on TPN 6 nights a week, but this was our first "night off" from TPN since Boston told us we could go to 6 nights per week instead of 7.
Peter was hooked up to a "simple" bag of 10% dextrose with some added sodium so that he would receive enough fluid to compensate for the volume of TPN he is accustomed to at night. We were not sure how he would do... BUT HE DID GREAT!!! He is very chipper this morning and not a drop of fluid retention is evident. In fact, his urine went up an extra 100mL overnight. Low blood sugar was another potential concern, but we see no signs of that either. Toddler energy should be able to be bottled up and given to parents!
I am curious to see if his desire to eat/drink goes up on the day following no TPN. He is liking the choice in formulas to drink since Boston gave us some samples to try. It looks like we will be going now with Vital Jr. Vanilla, Vital Jr. Strawberry, and plain Elecare. We still get some protesting as we try to fit in 10 feeds of 60mL of formula a day, but the protests seems to be lessening with a choice of what he should drink. He is really enjoying his dinnertime feeds of baby food now too... again with an improvement when given a choice versus just being presented with what he is to eat. And of course the obvious thing is, why wouldn't he want a choice??!! Don't we all want some say in what we are given?!
Peter was hooked up to a "simple" bag of 10% dextrose with some added sodium so that he would receive enough fluid to compensate for the volume of TPN he is accustomed to at night. We were not sure how he would do... BUT HE DID GREAT!!! He is very chipper this morning and not a drop of fluid retention is evident. In fact, his urine went up an extra 100mL overnight. Low blood sugar was another potential concern, but we see no signs of that either. Toddler energy should be able to be bottled up and given to parents!
I am curious to see if his desire to eat/drink goes up on the day following no TPN. He is liking the choice in formulas to drink since Boston gave us some samples to try. It looks like we will be going now with Vital Jr. Vanilla, Vital Jr. Strawberry, and plain Elecare. We still get some protesting as we try to fit in 10 feeds of 60mL of formula a day, but the protests seems to be lessening with a choice of what he should drink. He is really enjoying his dinnertime feeds of baby food now too... again with an improvement when given a choice versus just being presented with what he is to eat. And of course the obvious thing is, why wouldn't he want a choice??!! Don't we all want some say in what we are given?!
Monday, May 18, 2009
Boston visit May 2009
We went up this time for the usual Omegaven/CAIR check-in as well as for an endoscopy to evaluate the improvement or worsening of Peter's esophageal varices which were not very bad in August 2007. We were also going to get Peter's gastrostomy site fixed that it may stop needing a fortress of dressing to keep the leaks under control.
7 hours in the car on Monday, 8 hours in pre-op testing/meetings on Tuesday, and then he got sick on surgery-day which was Wednesday. Although disappointing, surgery was ultimately cancelled b/c he should be in tip-top shape for these elective procedures. Instead, we had blood cultures drawn with one positive culture in the red lumen. However, repeat cultures before antibiotics were started are revealing no infection at all. The decision is to stop the antibiotics after only 5 days. He has had nasal congestion for over two weeks now and possibly he was clearing a mild infection on his own between Wed an Thurs, or somewhere along the line there was contamination of one of the culture tubes. It is hard to be sure the exact reason there was nothing growing with the second set of cultures, but it is relieving that we can stop the antibiotics now and avoid another set of cultures.
Thursday we had CAIR and of course Peter was back to himself, and then we drove back home after that, pulling in to the driveway at 1:45am. Peter could not stop smiling once he got into his bed at home.
The things we found out from our visit to Boston are many, and as follows:
1. Echo of Peter's heart indicates that the enlarged coronary arteries have not changed since the last echo over a year ago. Good news!
2. PICC line is in correct placement still per chest x-ray. Confirmation of correct placement in a growing child is a good thing.
3. Dr. Jennings feels that Peter's gastrostomy may be so prone to leaking partially b/c there is very little muscle supporting the G-tube MICkey button. He is going to close up the original site and create a new gastrostomy altogether. I'll have to post a photo of the crazy dressing we have to do now to keep the current site contained. It is an infection waiting to happen b/c it creates a moist, dark environment, but the only other alternative has been to let the site leak all over the place, letting a good portion of his feeds leave shortly after he is fed. A new site should allow for minimal dressing, minimal leaks, and best of all should allow Dr. Jennings to see how well Peter is able to heal from a "simple" surgical procedure. Peter's portal hypertension, INR of 1.2, and unique bowel structure make surgery much more complicated than a child without all of that. How Peter heals from this will likely give us all some insight into future bowel surgeries for him.
4. Apparently Peter's hydroceles may be repairable at this point too. When we go back up for the surgery and endoscope, Dr. Jennings possibly will correct this problem too.
5. We are able to shave off one night of TPN a week now!! This will leave Peter with only 6 nights of TPN a week, and one night of replacement fluid since he can't currently drink enough to compensate for the fluid he will not receive overnight. The nutritionist said Peter's weight gain is nearly double what it should be at this point, so that allows for the loss of a night of TPN at this point. How exciting!!
6. We will add an extra 5 mg of iron to his TPN once a week since his iron levels are on the lower side again. He is biting and chewing on things now, not food, but things. While I thought that might be a good thing so he becomes more oral and interested in food, it also be a symptom called "pica" indicating that he is low on iron.
7. We were given some samples of other formulas to try in an effort to get Peter to stop resisting his elecare so often. He goes through periods of taking it just fine, and other times it is taking extreme bribing to get him to drink it. We are sampling EO28 Splash and Vital Jr. So far he seems to like them both about as much as he likes the Elecare. The variety allows us to at least give him a choice, but so far nothing is winning him over wholeheartedly.
8. Discussing why Peter tends to do so poorly with his feeds when he eats too closely to bed-time or when he has to sit for long periods of time (in the car or waiting in the hospital), Dr. Kamin feels that it is likely that Peter's digestion is somewhat positional. The duodenum is very scarred and likely has a tendency to balloon with feeds. We notice that Peter eats much better on days he is more active. He is more prone to vomiting when he eats and does not have the opportunity to to move around much. We also notice that he vomits more when he is holding onto fluids and getting more ascites in the abdomen. I didn't ask about the why's on this, but wonder if it could be that as he has more fluid in the abdomen there is less room for movement of the bowel as he is digesting. We are having some issues with feeds now as he recovers from so many days of sitting and possibly from side effects of the antibiotic. As much as Peter enjoys the trip to Boston, we usually have to deal with feed and/or fluid issues for a bit until he settles back out.
I think I have recalled all of the main points from the trip. We feel it was a really encouraging visit!!
Oh, by the way, Peter is in a "big boy bed" now. We replace his crib with a toddler bed a month ago. He has not missed his crib one little bit!
Thursday, March 19, 2009
Spelling Genius??
I don't know that we can declare genius status yet, but we decided to see just how many words Peter is spelling these days, and we get at least 50! He is only 3 years and 4 months old and can spell several 2 letter, 3 letter, 4 letter, 5 letter, 6 letter, and even an 8 letter word accurately. OK, he still isn't jumping or running, and he still isn't eating table food, but what a curiosity is his spelling capability.
The other neat thing we see is how much he has progressed in what he can do physically. We finally captured the backwards bear walk on video before he moves on to some other trick and leaves this one behind. He is now independent on stairs if there is a railing or something else to hold onto. We still hover nearby to catch him if he should stumble, but his confidence and skill are gaining. We are in the process of attempting to learn how to pedal a tiny "Big Wheel" so he can ride in the driveway with his siblings. He is also walking around the block twice a day on many days, versus just once a day, for a total of a mile and a half a day!
One last hurrah is that we are up to 100mL of elecare every two hours now. He can drink the 100mL all in one sitting. He takes 5 feeds like that per day, and then nothing enterally overnight. He is still on TPN and Omegaven 7 nights a week for 12 hours per night, but we are having dreams of reducing some of that now that he is up so far in feeds and still not needing to be vented at night via G tube.
Spelling:
Backwards bear walk: (forgive the protesting halfway through, and the overly-exuberant-mom-talk, I goofed on the first three attempts at videotaping and he was ready to be done the bear walk by the time I finally got the video recording going properly.)
The other neat thing we see is how much he has progressed in what he can do physically. We finally captured the backwards bear walk on video before he moves on to some other trick and leaves this one behind. He is now independent on stairs if there is a railing or something else to hold onto. We still hover nearby to catch him if he should stumble, but his confidence and skill are gaining. We are in the process of attempting to learn how to pedal a tiny "Big Wheel" so he can ride in the driveway with his siblings. He is also walking around the block twice a day on many days, versus just once a day, for a total of a mile and a half a day!
One last hurrah is that we are up to 100mL of elecare every two hours now. He can drink the 100mL all in one sitting. He takes 5 feeds like that per day, and then nothing enterally overnight. He is still on TPN and Omegaven 7 nights a week for 12 hours per night, but we are having dreams of reducing some of that now that he is up so far in feeds and still not needing to be vented at night via G tube.
Spelling:
Backwards bear walk: (forgive the protesting halfway through, and the overly-exuberant-mom-talk, I goofed on the first three attempts at videotaping and he was ready to be done the bear walk by the time I finally got the video recording going properly.)
Monday, March 16, 2009
February's Boston Visit
Peter had the best check-up to date when we went up in late February. He went in spelling words - "a-p-p-l-e is apple," "J is for Jill," "v-i-o-l-i-n is violin," and so on. I have never seen a three year old spell like he does, but Dr. Puder and Kathy Gura say that the Omegaven kids all seem to be really smart, so perhaps it is that good fish oil at work yet again.
After the spelling impressed everyone we went on to focus on his growth and behold, Peter has grown in height and weight, and not just following the curve this time. He grew almost 4cm in two months, and the weight gain is not fluid weight, it is real weight. Dr. Kamin felt his abdomen and we all agree that his abdomen doesn't have the feel it used to. Instead of feeling a little spongy with fluid along the sides, it feels more solid.
Peter had essentially been able to keep the G tube closed for the last month and a half also, which was huge progress compared to our December visit where Peter was needing the G tube put to drainage overnight every night in November and December.
We dropped the dextrose in the TPN a little bit and made plans to advance feeds further. He was and still is at 26 calorie elecare and it was felt he can stay there. We have been working on changing his feeds from 45 mL every hour to 90 mL every two hours and we just reached that goal in the last week. So far, so good. When we get to 115mL every two hours we get to drop a little TPN and begin to try our hand with baby food again.
Currently he takes 10mL of stage 1 baby fruit with his probiotics, but we have not pushed foods further since the focus has been on advancing elecare. Peter is the type of child that does better with one change at a time versus multiple changes, so we can figure out what causes problems more easily (usually more easily said than done though). We are stocked with stage 2 foods in anticipation, but also know that the last two times we fiddled with baby food he blocked up. Hopefully he'll do better this time... we think we'll be able to try the baby foods in another week or so. This time he is on probiotics, so we'll see if that is helpful with the baby foods advancing. Maybe a happier intestine will be less likely to block up. Blockage could also be from stricture, so we'll see as we go along.
More photos to come along soon. :)
After the spelling impressed everyone we went on to focus on his growth and behold, Peter has grown in height and weight, and not just following the curve this time. He grew almost 4cm in two months, and the weight gain is not fluid weight, it is real weight. Dr. Kamin felt his abdomen and we all agree that his abdomen doesn't have the feel it used to. Instead of feeling a little spongy with fluid along the sides, it feels more solid.
Peter had essentially been able to keep the G tube closed for the last month and a half also, which was huge progress compared to our December visit where Peter was needing the G tube put to drainage overnight every night in November and December.
We dropped the dextrose in the TPN a little bit and made plans to advance feeds further. He was and still is at 26 calorie elecare and it was felt he can stay there. We have been working on changing his feeds from 45 mL every hour to 90 mL every two hours and we just reached that goal in the last week. So far, so good. When we get to 115mL every two hours we get to drop a little TPN and begin to try our hand with baby food again.
Currently he takes 10mL of stage 1 baby fruit with his probiotics, but we have not pushed foods further since the focus has been on advancing elecare. Peter is the type of child that does better with one change at a time versus multiple changes, so we can figure out what causes problems more easily (usually more easily said than done though). We are stocked with stage 2 foods in anticipation, but also know that the last two times we fiddled with baby food he blocked up. Hopefully he'll do better this time... we think we'll be able to try the baby foods in another week or so. This time he is on probiotics, so we'll see if that is helpful with the baby foods advancing. Maybe a happier intestine will be less likely to block up. Blockage could also be from stricture, so we'll see as we go along.
More photos to come along soon. :)
Sunday, February 22, 2009
Playing in the snow
The photos are a month old, but here they finally are. Peter had a great time sledding and being out in the snow... until one of his brothers threw snow at him and it went down into his coat. Ah, siblings! One of these days Peter will have his revenge snowball throw.
OK, Peter didn't really clear any of the driveway, but he at least held the shovel for a while and smeared a bit of the snow around.
Thanks for taking me sledding and out in the snow Kristy! (Kristy is one of Peter's wonderful homecare nurses.) I'm sure it is obvious, but the girl in the photo is not Kristy. This is Peter's sister in the photo.
After an hour plus in the snow, it was time for a nap... anywhere!
OK, Peter didn't really clear any of the driveway, but he at least held the shovel for a while and smeared a bit of the snow around.
Thanks for taking me sledding and out in the snow Kristy! (Kristy is one of Peter's wonderful homecare nurses.) I'm sure it is obvious, but the girl in the photo is not Kristy. This is Peter's sister in the photo.
After an hour plus in the snow, it was time for a nap... anywhere!
Thursday, February 5, 2009
Glomerulonephritis
The verdict on Peter's kidneys is that he has glomerulonephritis. from Medline, "Glomerulonephritis is a type of kidney disease caused by inflammation of the internal kidney structures (glomeruli), which help filter waste and fluids from the blood." There can be different reasons for the condition, and it can be mild, moderate, or severe in nature. We think that Peter has had this for many months and that he is presenting as a mild case. The nephrologist feels Peter's cause is likely IgA nephropathy versus some of the other causes that could be the culprit, but the only way to make a definite diagnosis of the cause would be to do a kidney biopsy. Peter is not the ideal candidate for biopsy of any organs due to portal hypertension, so unless he gets acutely worse or does not respond to medication, we will just ride on the fact that he has glomerulonephritis and remain watchful.
He has started on vasotec (enalapril), which is a blood pressure lowering medication. He does not have high blood pressure, which is a blessing since it can go hand-in-hand with kidney disease, but the medication also has the property of protecting the kidney from scarring. Prolonged inflammation of the kidneys from glomerulonephritis can eventually cause scarring of the kidneys and long-term damage. He has been on this medication for several days now and seems to be tolerating it well. We will hope for his labs to show that he handles the medication well and will return to the nephrologist in two months. Peter will likely need to stay on the medication for a good long time, possibly for life. The nephrologist said that sometimes people clear the condition, and sometimes they do not. We are on a wait-and-see approach to see where things end up, but it all sounded encouraging and manageable.
He has started on vasotec (enalapril), which is a blood pressure lowering medication. He does not have high blood pressure, which is a blessing since it can go hand-in-hand with kidney disease, but the medication also has the property of protecting the kidney from scarring. Prolonged inflammation of the kidneys from glomerulonephritis can eventually cause scarring of the kidneys and long-term damage. He has been on this medication for several days now and seems to be tolerating it well. We will hope for his labs to show that he handles the medication well and will return to the nephrologist in two months. Peter will likely need to stay on the medication for a good long time, possibly for life. The nephrologist said that sometimes people clear the condition, and sometimes they do not. We are on a wait-and-see approach to see where things end up, but it all sounded encouraging and manageable.
Monday, February 2, 2009
Peter's Probiotics Regime -Part Two
After posting about Peter's probiotics, I got to thinking about the central line issue that makes many doctors, nurses, and other SBS'ers worry about infection. I thought I would revisit this subject with additional details for other parents/caretakers who are dealing with bacterial overgrowth (BO) and the question of whether to add probiotics or antibiotics.
When we first began treating BO sometime in mid 2007, we went straight for antibiotics given Peter's proneness to intestinal bleeding from portal hypertension (possibly the BO as well) and his recent hospitalizations for a gut translocated organism in June if memory serves correctly, and the fact that he had only come out of the hospital in Feb 2007 after almost a year inpatient. The antibiotics worked very well... for a couple of rounds. We did flagyl for a week, augmentin for a week, and cipro for a week, and then either repeated or took one week off before repeating (can't recall). They were all administered enterally. As we grew accustomed to this routine over a couple of months, Peter began doing funny things while on the enteral antibiotics. The first issue was hives. After the third round of hives we came to recognize that they were occuring when he was on augmentin. We stopped augmentin and the hives left and haven't returned. We continued with just flagyl and cipro until we began to have mystery illnesses while on flagyl. I have written about that in this blog before - see posts from March and April 2008 where Peter was hospitalized and cultured and there was nothing discovered as far as illness. The third time the mystery illness occurred we realized that each time he had been on flagyl. We stopped the flagyl and the illness left the next day. Then all we had left was cipro. We were looking for patterns by this point and found one - enteral bleeding within a few days of starting cipro. (By this point Peter's enteral bleeds had pretty much resolved and were rare occurences compared to the year before, but when cipro came onboard it would predictably return.)
This is when we started the probiotic drink of kefir. It was good stuff, but eventually stopped working, likely due to the small amounts that we are limited to since Peter takes so little in enterally to begin with. Speaking with Peter's GI in Boston about the BO and where to go with it, he suggested that we could try probiotics, although it is considered risky in a child with a central line. Given that we have already been hospitalized because of antibiotics, it seemed worth the risk to try probiotics over antibiotics. The cautions I have been given are that probiotics are LIVE organisms. What has been emphasized to me, is that one of the culprits for infection in a line of a person using probiotics is likely a cross-contamination versus necessarily a gut translocation. In other words, a person may have probiotic on their hands, not realize it before handling the central line and thereby contaminate the line directly. That may not be the route of contamination in all probiotic line infection cases, but just like one would thoroughly wash hands after changing a dirty diaper or ileostomy bag before accessing the line, one should be extremely thorough in cleaning after probiotics before handling the line or accessories. Below are the tips that were given to me along with how we have adapted them to Peter's routine.
The germs, though "good" germs, are still "bad" germs if they get into the bloodstream, so wash hands thoroughly and make sure that the probiotics are handled with diligent care so that they do not get anywhere near the central line. Administer probiotics long before accessing the line or making TPN of handling IV meds. Do not administer or mix or store probiotics anywhere near where you make TPN or handle IV meds. Our routine is that Peter receives the probiotics only after his TPN is down and his lines have been flushed and wrapped in coban for the day. The probiotics are mixed only where food is prepared, not where TPN would ever be prepared, and is mixed over a large dinner plate to catch any excess powder that might fall onto the plate when it is being measured. The powder is mixed with a teaspoon of baby food in a small bowl that is on the plate. The plate is used then as a tray to keep the possibly dirty bottom of the bowl from contaminating another surface. Peter is fed the food containing the probiotic, not allowed to try to feed it to himself. Immediately after feeding it his mouth is wiped with a damp paper towel, the bowl and spoon and plate go into the dishwasher, and the person who administered the food and anyone else who handled the probiotic in anyway washes hands with soap and water.
Peter has been on this regime since late December and though the attention to maintaining strict cleanliness when using the probiotics seems a little strict and uptight, it seems to be working well for us. We'll keep posting updates about the success or lack of it as we go on.
When we first began treating BO sometime in mid 2007, we went straight for antibiotics given Peter's proneness to intestinal bleeding from portal hypertension (possibly the BO as well) and his recent hospitalizations for a gut translocated organism in June if memory serves correctly, and the fact that he had only come out of the hospital in Feb 2007 after almost a year inpatient. The antibiotics worked very well... for a couple of rounds. We did flagyl for a week, augmentin for a week, and cipro for a week, and then either repeated or took one week off before repeating (can't recall). They were all administered enterally. As we grew accustomed to this routine over a couple of months, Peter began doing funny things while on the enteral antibiotics. The first issue was hives. After the third round of hives we came to recognize that they were occuring when he was on augmentin. We stopped augmentin and the hives left and haven't returned. We continued with just flagyl and cipro until we began to have mystery illnesses while on flagyl. I have written about that in this blog before - see posts from March and April 2008 where Peter was hospitalized and cultured and there was nothing discovered as far as illness. The third time the mystery illness occurred we realized that each time he had been on flagyl. We stopped the flagyl and the illness left the next day. Then all we had left was cipro. We were looking for patterns by this point and found one - enteral bleeding within a few days of starting cipro. (By this point Peter's enteral bleeds had pretty much resolved and were rare occurences compared to the year before, but when cipro came onboard it would predictably return.)
This is when we started the probiotic drink of kefir. It was good stuff, but eventually stopped working, likely due to the small amounts that we are limited to since Peter takes so little in enterally to begin with. Speaking with Peter's GI in Boston about the BO and where to go with it, he suggested that we could try probiotics, although it is considered risky in a child with a central line. Given that we have already been hospitalized because of antibiotics, it seemed worth the risk to try probiotics over antibiotics. The cautions I have been given are that probiotics are LIVE organisms. What has been emphasized to me, is that one of the culprits for infection in a line of a person using probiotics is likely a cross-contamination versus necessarily a gut translocation. In other words, a person may have probiotic on their hands, not realize it before handling the central line and thereby contaminate the line directly. That may not be the route of contamination in all probiotic line infection cases, but just like one would thoroughly wash hands after changing a dirty diaper or ileostomy bag before accessing the line, one should be extremely thorough in cleaning after probiotics before handling the line or accessories. Below are the tips that were given to me along with how we have adapted them to Peter's routine.
The germs, though "good" germs, are still "bad" germs if they get into the bloodstream, so wash hands thoroughly and make sure that the probiotics are handled with diligent care so that they do not get anywhere near the central line. Administer probiotics long before accessing the line or making TPN of handling IV meds. Do not administer or mix or store probiotics anywhere near where you make TPN or handle IV meds. Our routine is that Peter receives the probiotics only after his TPN is down and his lines have been flushed and wrapped in coban for the day. The probiotics are mixed only where food is prepared, not where TPN would ever be prepared, and is mixed over a large dinner plate to catch any excess powder that might fall onto the plate when it is being measured. The powder is mixed with a teaspoon of baby food in a small bowl that is on the plate. The plate is used then as a tray to keep the possibly dirty bottom of the bowl from contaminating another surface. Peter is fed the food containing the probiotic, not allowed to try to feed it to himself. Immediately after feeding it his mouth is wiped with a damp paper towel, the bowl and spoon and plate go into the dishwasher, and the person who administered the food and anyone else who handled the probiotic in anyway washes hands with soap and water.
Peter has been on this regime since late December and though the attention to maintaining strict cleanliness when using the probiotics seems a little strict and uptight, it seems to be working well for us. We'll keep posting updates about the success or lack of it as we go on.
Wednesday, January 28, 2009
Peter's probiotics regime - Part One
Kids with short bowel syndrome (SBS) tend to have recurrent bacterial overgrowth (BO) in their intestines. We haven't had to deal with this in ages with Peter, but in December Children's Boston suggested that Peter's difficulty with feeds may be due to BO. ***UPDATE: I think the more frequent abbreviation is SBBO - Short Bowel Bacterial Overgrowth, versus just BO.***
We have had terrible luck with enteral antibiotics in the past, resulting in hives, mystery ailments ending Peter up for hospitalization, and intestinal bleeding. We had tried 3 different enteral antibiotics (flagyl, augmentin, and cipro), each presenting one of those problems. We had put Peter on kefir for several months after the antibiotics had failed and the BO odor was returning. That worked well for many months, but apparently did not work well enough to be the sole prevention for BO, possibly given the small amounts we had to give Peter.
Peter has now been on powdered probiotics since late December, and we think we are seeing a huge improvement! We started on 15-35 Probiotic which has 35 billion organisms per capsule, but it also has enough sodium in it that Peter ultimately began to refuse it due to taste. We moved on to Florastor, which is a yeast organism. He has been on this for 2 weeks and we will keep him on it one more week. The initial response on Florastor was that he smelled quite poopy most of the time, but at this point he does not and he is acting like he feels just wonderful. Perhaps the poopy smell was him clearing out the BO?? He takes the Florastor in a little baby food and is no longer refusing elecare. He is sleeping better at night too!
We will then go to VSL#3, which is quite similar to the 15-35 Probiotic, but no sodium. We'll give that for two weeks, go back to Florastor for two weeks, and then go for a two week break off all probiotics. Meanwhile we are hoping to begin increasing elecare again to see if he can tolerate it better now that the BO is out.
Still waiting to hear back from the nephrologist about what is going on with Peter's kidneys if anything.
We have had terrible luck with enteral antibiotics in the past, resulting in hives, mystery ailments ending Peter up for hospitalization, and intestinal bleeding. We had tried 3 different enteral antibiotics (flagyl, augmentin, and cipro), each presenting one of those problems. We had put Peter on kefir for several months after the antibiotics had failed and the BO odor was returning. That worked well for many months, but apparently did not work well enough to be the sole prevention for BO, possibly given the small amounts we had to give Peter.
Peter has now been on powdered probiotics since late December, and we think we are seeing a huge improvement! We started on 15-35 Probiotic which has 35 billion organisms per capsule, but it also has enough sodium in it that Peter ultimately began to refuse it due to taste. We moved on to Florastor, which is a yeast organism. He has been on this for 2 weeks and we will keep him on it one more week. The initial response on Florastor was that he smelled quite poopy most of the time, but at this point he does not and he is acting like he feels just wonderful. Perhaps the poopy smell was him clearing out the BO?? He takes the Florastor in a little baby food and is no longer refusing elecare. He is sleeping better at night too!
We will then go to VSL#3, which is quite similar to the 15-35 Probiotic, but no sodium. We'll give that for two weeks, go back to Florastor for two weeks, and then go for a two week break off all probiotics. Meanwhile we are hoping to begin increasing elecare again to see if he can tolerate it better now that the BO is out.
Still waiting to hear back from the nephrologist about what is going on with Peter's kidneys if anything.
Wednesday, January 14, 2009
Christmas photos 2008
This year Peter understood that Christmas is baby Jesus' birthday. He understood that presents have gifts inside that you keep. He understood how to open the gifts. And he enjoyed himself! What a lovely Christmas gift and blessing to see the advances from last year to this year!
The endless cold and the fearless wrestler
I have been meaning to post for the last 10 days but have been stuck with an endless cold rendering me essentially useless by the end of the day which is the best time for me to try to update things... after the kids are down and it is quiet. Interestingly, Peter had the bug for only a day and a half, though he has a persistently low white cell count in his labs and has for the last two years at least. It is a whopper of a cold though, striking every one of us plus one of the nurses that works in our house here. It seems to be rebounding on the kids including Peter today.
Peter's albumin yesterday was down from 2.9 to 2.2 and he acts like he is holding onto fluid in the abdomen in his breathing and gait. We hope it is just the cold! When he has been hit with a good illness in the past we would see his albumin get down below two and he would swell up, needing IV albumin and lasix to remove the fluid. I don't see the swelling taking over like in the past, so hopefully he is pulling out of this on his own already.
Prior to the cold we actually have seen some great progress with Peter physically. He still can't jump, but he is doing a whole lot more self moving when playing which imitates many of the positions PT wants him to work on. Also, what is really a big leap forward is that he has started to enjoy crashing into his brothers and rolling around on the floor with them in mock-wrestling moves. His speech is coming along beautifully and he is using full sentences with occasional pronouns and making conclusions about what someone may be feeling based on what they are doing - ie, if I am holding a cup, "Mommy is thirsty."
We are still perplexed with the urinalyses that we are getting over the last few weeks. There is still large amounts of microscopic blood and there is protein in the urine. We are to find a kidney consult locally to see what is happening. We also still are trying to understand why he holds onto fluid as he does. Children's Boston lowered the sodium in his TPN even lower, but so far we don't see much change. But again, perhaps that is because of the nasty cold virus and the proneness to holding fluid when ill. Maybe the kidney consult will give us some clues.
We are still at 30ml per feed with the elecare but have added a tiny bit of stage 1 fruit again. Peter can detect the probiotics in his elecare and we have been seeing an aversion to the elecare, seemingly out of fear that it will not be the pure elecare but will have the probiotic in it. Putting the probiotic in the baby food is so far working out well. We are now trying Florastor probiotic and have great hope that will work as well for him as it has in other "intestinal failure" patients. (I don't particularly like the term intestinal failure and almost panicked when I first saw it on Peter's chart, but it does not mean that his intestine is failing and has no chance, in case you were wondering.)
Peter's albumin yesterday was down from 2.9 to 2.2 and he acts like he is holding onto fluid in the abdomen in his breathing and gait. We hope it is just the cold! When he has been hit with a good illness in the past we would see his albumin get down below two and he would swell up, needing IV albumin and lasix to remove the fluid. I don't see the swelling taking over like in the past, so hopefully he is pulling out of this on his own already.
Prior to the cold we actually have seen some great progress with Peter physically. He still can't jump, but he is doing a whole lot more self moving when playing which imitates many of the positions PT wants him to work on. Also, what is really a big leap forward is that he has started to enjoy crashing into his brothers and rolling around on the floor with them in mock-wrestling moves. His speech is coming along beautifully and he is using full sentences with occasional pronouns and making conclusions about what someone may be feeling based on what they are doing - ie, if I am holding a cup, "Mommy is thirsty."
We are still perplexed with the urinalyses that we are getting over the last few weeks. There is still large amounts of microscopic blood and there is protein in the urine. We are to find a kidney consult locally to see what is happening. We also still are trying to understand why he holds onto fluid as he does. Children's Boston lowered the sodium in his TPN even lower, but so far we don't see much change. But again, perhaps that is because of the nasty cold virus and the proneness to holding fluid when ill. Maybe the kidney consult will give us some clues.
We are still at 30ml per feed with the elecare but have added a tiny bit of stage 1 fruit again. Peter can detect the probiotics in his elecare and we have been seeing an aversion to the elecare, seemingly out of fear that it will not be the pure elecare but will have the probiotic in it. Putting the probiotic in the baby food is so far working out well. We are now trying Florastor probiotic and have great hope that will work as well for him as it has in other "intestinal failure" patients. (I don't particularly like the term intestinal failure and almost panicked when I first saw it on Peter's chart, but it does not mean that his intestine is failing and has no chance, in case you were wondering.)
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