Cheeseburger!

About two months ago I meant to post this after it happened, but as things have been busy I am posting it now. 

We were discussing dinner plans in the morning and Peter overheard us say that we would have hamburgers for dinner.  He piped up, "Can I have a cheeseburger for dinner?"  To put this in context as to why this would stand out for us, Peter's dinners consist of Stage 1 Gerber baby meat, a pureed vegetable, and a token amount of crackers/Cheerios/chips/other table food.  This request was an unusual one for him.  It is also one of the wishes that I have had for him for the past couple of years... to see his ability to eat expand so much that I will one day see him eat a burger. 

Of course we said that we would make him a cheeseburger, and went on with the morning.  Several times throughout the day he remarked that he wanted his very own cheeseburger, so I started thinking about how to make that happen.  Usually we will give him a piece of what he asks for and he will lick it, taste it, nibble it, and maybe eat it.  I didn't know what he would do with a whole burger, but did wonder what he would do with a whole burger too.  As dinnertime approached, it hit me that I could make a tiny one and use part of a hotdog bun to make a slider-sized cheeseburger.  Peter was so excited about this that he volunteered to set the table too. 

Dinner came and he wanted ketchup and mustard on the burger.  He examined the burger, then held it up and took a bite out of it.  We were all on the edges of our seats, camera out.  He loved the burger!  Being short-gut, on TPN 7 nights a week, with limited food intake which is mostly pureed or liquid, he only ate about 1/4 of the burger, but he absolutely enjoyed himself.  We told him we would save the leftover part for him and we did.  The next morning he asked for that burger again and ate another 1/4.  Lunchtime came and he wanted it again.  In the end, he ate almost all of that burger. 

 Dinner

 Breakfast

  Lunch

He hasn't asked for another burger yet, but has gone on to try a little bit of ham, kielbasa with seeded mustard, hotdog with ketchup and mustard, and the Indian spinach dish, palak paneer.  Boy do we get a kick out of him eating.... medical grade formula, baby food, and then break out the seeded mustard and kielbasa.  His feeding therapists who worked with him from last Spring through late Fall, have enjoyed hearing of his new conquests as well.

MRI, vascular clinic, and surgical decisions

Peter has his MRI last week.  The MRI shows that Peter has 3 lesions in the liver, but the likelihood is that they are most likely related to the cirrhosis/fibrosis, as opposed to cancer. It is possible they have been there for years, as we haven't had an MRI of the liver since 2006 (I think), and it was apparently difficult to see even one on ultrasound. Peter moved during the MRI at a crucial point, so at some point he will need another imaging of the lesions, but for now the doctors are very comfortable that we can proceed with surgery and that Peter is not entering some kind of rapid decline. We think that it is unlikely that his liver disease has gotten worse, as the function does not suggest it is at all as bad as the biopsy or radiology tests indicate. Peter is probably borderline cirrhosis/fibrosis.

That all said, we discussed the risks of surgery and how it could affect the liver.  Since our last visit, Dr. Kamin consulted with the liver docs and everyone is in agreement that Peter's liver has at least a 90% chance of pulling him through surgery and recovery without much insult to the liver. Great odds in Chris's and my opinions!  There are of course real risks with that 10%, as Dr. Jennings reminds us, but there are also dangers to doing nothing or transplant as well.

The plan for surgery will be as follows.  Both Dr. Jennings (surgeon) and Dr. Kamin (GI) will be in surgery.   The surgery will connect the good portion of bowel above the fistula with the good portion of bowel below the fistula, so Peter can pass food all the way through without losing some along the way. That should in turn slow down the fistula enormously, as there won't be a direct passage to it. It will then truly be a "mucus fistula" and drain only what it produces itself.  Dr. Jennings will have the whole OR day reserved for Peter in the event it is necessary, but this procedure should be fairly straightforward. It is the bleeding from portal hypertension and past surgical adhesions that are the big concerns during surgery, and I suppose post-op as well.  The liver health comes into play during post-op recovery. 

The Vascular Anomalies Clinic (VAC) also went well. The doctors feel the spider veins and red spots are most likely due to liver health versus a separate vascular condition. They are not 100% sure of that, and were very upfront about that, but for now we are going with the thought that they are related to the liver. The larger arm is likely a compromised blood flow to the arm, but as it causes no problem other than size there is nothing necessary to do for it. The hand getting bigger post PICC seems to point to a small clot or pinching of the vessel. If they can get clearance to use the laser in the OR, they may laser the spider veins on his face when he has the abdominal surgery.

Good news, good visit, ...... and good weather this time too!

Liver concerns and another cancelled surgery

We recently returned from another visit to Boston which entailed more than our usual CAIR visit.  This visit also involved an abdominal ultrasound to look at the liver and kidneys, a CT to look at the chest, and endoscopies of the esophagus, fistula, and ileostomy. 

The ultrasound suggests that we need to follow up with a liver MRI to help further establish whether Peter's liver is declining.  There is a lesion on ultrasound, which hopefully is nothing, but could be showing that the liver is entering the stages of cirrhosis, versus staying in his current stage of fibrosis.  The ultrasound of the kidneys was to see if kidney stones are developing, as Peter has high levels of calcium in the urine now.  His nephrologist at CHOP feels that the IGA nephropathy is still in remission, and that the calcium is likely due to the high levels of IV sodium needed to compensate for the fistula losses, or due to too much vitamin D.   We hope to get the cause identified and removed shortly, and fortuately we do not see calcium build up in the kidneys.

The CT of the chest was ordered because the Vascular Anomalies Clinic physicians saw enlarged chest vessels on a portion of an old abdominal CT and want clarity if he has enlarged pulmonary vessels.  Per the CT, he does.  A "quick" trip over to cardiology established that the cardiologist is not alarmed, and the cardiac echo and EKG now are the same as a year ago.  However, the chest may need a follow up MRI as well.... to be determined soon.

The endoscopy was the big test that we were waiting for on pins and needles, as it was to reveal whether Dr. Jennings would be able to close the now high-output fistula next week.  The disappointing news is that the bowel is more complicated than hoped.  He has two "chambers" in his bowel below the stomach, which both have connections to the fistula, and the fistula is physically necessary for draining a loop of bowel that goes nowhere.  That leads us back to the idea of needing a bigger surgery on the bowel, trying to determine if the liver is healthy enough for recovery from a larger surgery, and whether a shunt to decompress the portal hypertension is needed or not.  We essentially have 3 options it seems: 
1. do nothing (but is Peter headed for liver failure now, and we are unable to advance feeds on Peter without the fistula going up in output),
2. take a chance on the surgery (but Dr. Jennings does not want to do that without establishing a reasonable chance that the liver can handle the surgical recovery, hence the liver imaging we did and are still planning), or
3. do transplant (but multivisceral transplants come with their own big set of problems and serious risks, and we are not clear yet if we could do an isolated liver transplant on Peter).  

We will be returning to Boston soon for the MRI, the actual meeting with the Vascular Anomalies Clinic, and hopefully leaving with answers as to whether this child is going to have surgery to get his bowel fixed enough so that we can resume advancing feeds and begin weaning him off TPN like we did a little over a year ago.