Monday, November 22, 2010
The removal of the port left a rather large wound on his chest which will need to be taken care of for awhile. Not really looking forward to undressing the wound and repacking it, but we'll manage somehow.
Sam's surgery last night revealed some more concern for site access. It appears that during an earlier surgery Sam's right jugular was severely damaged or tied off. This isn't that uncommon, but would have been nice to know. Going back through the history of Sam's central lines it would have had to have been his very first line that was in that vein and the line was removed in Milwaukee - but none of the history that was sent to Boston or Madison has any notes regarding the line being in that vein and it is listed as being in the Right Subclavian vein.
After surgery last night they started to ramp up Sam's glucose. It always seems that once we get off of his normal TPN cycle we have issues and this time will be no different. His blood sugar spiked and remained high all night even when they reduced his intake down considerably. This meant having to check blood sugar every hour last night.
Not sure yet when we are headed home, but today is looking less likely. They would prefer to have his blood sugar be a little more stable before we leave.
Sunday, November 21, 2010
We have not been able to maintain Sam's blood sugar using his temporary IV that was placed. He just simply requires too much sugar. For the last six hours we have watched his glucose hover in the 30's no matter what we have done to try to raise it. Therefore they are going to take Sam into surgery tonight, remove his old port, and put in a larger line.
The surgeon is on his way up to talk to me and it will be the same surgeon from this morning that I did not agree with. I am not sure that I want Sam to have a PICC line in his arm for the next several weeks or months, and I do not like where he wants to place a new central line for Sam.
I will update again once we are out of recovery later tonight.
Last Sunday we changed out Sam's needle and dressing and the site looked the best that I have seen it since we began this whole ordeal. Yesterday (Saturday) I went to de-access Sam (take out the old needle) and as I was working to get the dressing off could see that the skin under the needle head looked discolored. As I got the needle out I realized that it was not discolored skin that I was seeing but the silicone on the top of his port. The skin in the middle of his port, about 1 cm around, was just gone.
Since Sam is completely dependent on his TPN and IV fluids, he had to have an IV placed yesterday to keep him hydrated. It did not go well. It took several attempts over the course of 5 hours to get an IV placed. In the process I think he was stuck at least 10 times and we blew three veins. his poor arms and legs look terrible. Since his TPN concentration is too strong to put through this type of IV we are just keeping him hydrated.
We were scheduled to go into surgery this morning to have his port removed, but we are now putting it off until tomorrow as there has been some disagreement as to what to replace the port with and when to do it. Our surgeon will be back on tomorrow so hopefully we can all come up with a plan that everyone can agree on.
Sunday, October 17, 2010
Sam has been inpatient since Wednesday at Children's in Neenah. He spiked a fever Wednesday which led us to assume that we were probably looking at another blood stream infection so we had cultures done and started our standard antibiotic treatment for the staph that he seems to be best friends with.
By Wednesday evening we were getting a little concerned as Sam was acting sick, which he never does. He was very sleepy and lethargic and he still had a fever. Normally when we start his antibiotic he perks back up after the first dose. Then we got the dreaded phone call from the pediatricians office. Sam was already growing out profuse numbers of Gram Negative Rods. Definitely not staph, we assumed we were dealing with E-Coli and we needed to switch antibiotics. We made the decision to take him onto the floor rather than dealing with an overnight stay at the ER while we arranged for our home health company to deliver new antibiotics.
Thursday morning Sam still had a fever. To add to that we began having difficulty drawing blood from Sam's central line. This was especially bad since we needed to be able to culture the blood off of his line to monitor the infection. Then we got back the strain identification results from his original blood culture and it wasn't what we expected. I heard the doctor say Klebsiella oxytoca and then my brain thought of a great number of really awful things. Klebsiella can be nasty. It is virulent and mutates easily to develop resistance to antibiotics.
To add to all of that the skin over the top of Sam's port looked terrible. Many calls were made to Madison and Boston. Between everyone we decided it was best to keep Sam in Neenah and keep treating to see if we could clear the infection and whatever was blocking his line from drawing.
Late Friday night, after the second round of TPA to help unblock his line, we were able to get blood from his central line for cultures and labs. His labs were still screwy, but I just learned that the culture from Friday night is still clear and Sam's line is working beautifully. We also just changed the dressing over Sam's port needle and his skin looks almost perfect, better than we have seen it in weeks.
We are just waiting on lab results from this morning. If labs are looking good (which should be the case because he is definitely acting like he feels better) then we just need to wait for arrangements to be made to get our home care company to deliver some antibiotics (on a Sunday) and then we might be able to get out of here. 5 days here is way too long. Sam is bored out of his mind and if I have to watch Lilo and Stitch one more time I am not sure what will happen, but it will not be good.
Of course we never believe the H word (home) until we are actually in the car driving away, but things are looking good.
Monday, September 20, 2010
So, we kinda left everyone hanging with the last post. Sam and I made it back to Wisconsin and we finally got a negative culture and the all clear to head to the wedding instead of the hospital. The wedding was a blast, Sam had such a good time dancing and chasing all of the other kids around at the reception.
I was hoping that the line infection would be a blessing in disguise. We had been battling a recurring site infection at Sam's broviac site on and off for several months. With the line infection we were on a nice long course of antibiotics and his site started to look healthiest I had seen it in awhile. Unfortunately as soon as we stopped antibiotics the site started to look messy again and within a short time the infection was back. We decided that it was time to consider removing the line and having it replaced.
We met with Sam's new surgeon from Madison for the first time to have him assess Sam's site and get his opinion. Dr. Lundwent to school in Boston, and did his residency and fellowship at Children's in Boston. This was the guy that our team in Boston wanted us to use as Sam's local surgeon, so we had high hopes when we walked into this appointment that we were going to end up with a good addition to Sam's medical team. We weren't disappointed.
If you have ever read our entries about, or heard us talk about, Dr. Puder you will know that we hold him in very high regard. Dr. Lund has come in a very close second place.
Dr. Lund agreed that it was time to pull the line, and recommended replacing Sam's Broviac with a port. The Broviac is a tunneled central line that enters Sam's chest and then is tunneled under the skin to a vein in his neck. This leaves a long tube hanging out of Sam's chest that we hook up his TPN (nutrition) , hydration, and IV medications to. The problem we were having is the the site where the tube entered Sam's chest kept getting infected. The port is similar, but instead of of a tube hanging out of Sam's chest there is a small titanium disk implanted under Sam's skin. This disk has a silicone top on it. To use it a small needle is inserted through Sam's skin and into the port. This means no tube and no large wound on his chest.
After weighing the decision for several days, we made the decision to give the port a try. Once of the benefits that we would gain is that we can de-access (take the needle and IV tubing completely out) any time we need to. This allows us to give Sam a full bath without having to worry about contaminating his site. He can also swim in the pool unprotected when he is de-accessed and we can allow him to skim in lakes and rivers which he has never done before.
Sam's surgery on August 30th went very well. Dr. Lund was able to re-use the existing vein opening so we did not loose any access sites for Sam, and the new port worked great - but don't get too excited yet.
The needle in Sam's port can stay in for up to 7 days. To allow Sam to heal from the surgery and for the swelling to go down, they wanted us to leave the original needle in for 5 to 7 days and then change it out weekly or more often at home once we were comfortable with the procedure. Our nurse came out to the house and changed out the needle with no issues the first week. The plan was for me to start learning the next week.
When our nurse came out the following week, Deb decided at the last minute that she wanted to learn how to place the needle. We went through the cleaning process and Deb inserted the needle. The placement looked good, but she could not get any blood to draw into the line. The nurse had her pull the needle out and try again. Still no luck. The nurse took over and tried several more times (keep in mind that each attempt means sticking the needle back through the skin into the port). She was stumped as each time she was certain that the needle was in the port. After several calls to two hospitals, she tried one more time. Placement was good again. This time she attempted to flush the port before trying to get blood return which was suggested by the pediactic nurses at the hospital that we had talked to. Everything worked beautifully this time.
Unfortunately, this meant that the skin on the top of Sam's port was pretty torn up an irritated. If you've been reading our website for awhile I am sure you can guess what I am going to say next. Sam has developed and infection on the skin over his port. We have been treating with antibiotics and changing the needle out every 3 to 4 days, hopefully we will start to see improvement this week.
Otherwise Sam has continued to thrive. He continues to show interest in trying food and has shown steady gains in his speech development and use of sign language. He very quickly developed an interest in books (although he main interst at first was destroying them) and we now have to read several every night before bed.
With all of this going on we neglected to update the website for almost two months. There were several things that happened all one after another that made me question whether or not we were going to continue to share Sam's story so publicly. I think the Sam update was enough for tonight, but I will update again soon and explain what else has been going on in our lives and what the rest of the family has been up to.
Thursday, July 15, 2010
Since our last update we have continued to be busy. Sam has been battling a site infection for the last few weeks, after two separate courses of antibiotics the infection was gone and we were waiting for the site to heal.
Sam and I are in Boston today for a check up and to pick up more Omegaven. We still have not worked out a way to bring Omegaven to Madison for Sam, but I have something in the works that would help tremendously with the trips to Boston. By far the biggest expense for these trips is the airfare. I may have found an oranization today that is willing to fund the airfare portion of out trips, and I will post more details as soon as I find them out.
Our schedule for this week was hectic. Sam and I were set to head to Boston Wednesday morning on the 6 AM flight, meet up with Bradley's family, have a clinic appointment, catch the 6 AM flight back to Wisconsin on Friday, head to Fond du Lac WI for my brother's wedding rehersal and then be at the wedding on Saturday.
Tuesday morning Sam woke up with a fever of 101.5. Normally this wouldn't be a big deal but, becuase Sam has a permanent central line, this can be an indication of infection so we headed into the ER. Of course when we got to the ER we were in for a treat.
There is an ER doctor that I have had issues with in the past. To be blunt he is one of the biggest jerks I have ever encountered in the medical profession and we have met a lot of doctors. If you are not a trauma and you are not bleeding out on the floor then you are wasting his time. The first thing that he said to me was "Explain to me why you are here instead of at the clinic, it is just a fever". Things went down hill form there. The main reason that we were at the ER is that we can get immediate results on labwork, and it is extremely difficult to get a peripheral blood draw off of Sam. The other reason is that depending on what the preliminary lab results say, there is a very good chance that we will be admitted to the hospital. An infection of this type can be VERY serious and can quickly go from being nothing to being deadly in a short amount of time.
I explained to this doctor what we needed, read the list of clutures and lab work that I needed. He said that he would need to consult with someone who knew more about Sam. I explained that our pediatrician was off for the day, but there were others at that office that had enough knowledge of Sam to help, I also gave him the information for Boston and let him know that everyone on the Peds floor at the hospital (which is three floors above the ER) knows Sam well and could also help. With all of this information in hand he left the room, letting me know that it would probably be awile becuase he was very busy. Let me add here that at this time there was nobody in the waiting room at the ER and we were, in fact, also the onlypatients in the ER.
FOUR HOURS pass and we still hadn't even had cultures drawn. The doctor stops in to let me know that he is still waiting to hear back from the pediaticians office.
I called Deb and asked to to call the peds office to find out what was going on. I am sure you will be shocked to find out that he never called them. Nor did he call anyone else that I had asked. Deb ended up calling the Peds floor at the hospital and one of the pediatric specialists came down to help us out. We got cultures drawn and disussed what our plan would be while we waited for the culture results. Since Sam was acting like nothing was wrong and he wasn't showing any symtopms of being septic, we decided that it would be pointless to admit him to the hospital when they wouldn't do anything for him that we couldn't do at home. We went home with two IV antibiotics. Sam ws still looking OK on Wednesday morning so we headed for the airport.
Upon landing in Boston I received the results of Sam's cultures, both the culture off of his line and the peripheral culture were growing profuse amounts of gram positive rods. Looks like staph, I am still waiting on exact identification.
This meant that we needed an additional culture on Wednesday, so I met up with Bradley's family and we headed to the hospital. Boston was a little hesitant to let Sam come back to the hotel with me and they would not normally treat a blood infection outpatient. We worked it out and I agreed that if there was any change we would come back to the hospital right away.
Today we had our clininc appointment. Unfortunately the cultures from yesterday grew out again, which means that we will need to have blood cultures done again tomorrow in Wisconsin. Protocol is that if there are three positive cultures while on antibiotics, the central line will need to be pulled so we can clear the infection from Sam's system and replace the line. This would mean that if Friday's cultures are still positive they would want Sam admitted in Madison on Saturday. Great. I asked if he looks OK and we aren't having any other issues if it would be safe to wait until Sunday. Right now the answer to that question is "We'll cross that bridge when we come to it". Sam's White Blood Cell count was EXTREMELY HIGH on Tuesday (which is an indication of infection), it was much better on Wednesday. This could mean that we are keeping the infection under control but not getting rid of it. Hoepfully we can get a clear culture today or tomorrow.
The only other changes from our clinic vist today are increasing his iron dosage and frequency. We reduced the dose and frequency in May and Sam has become slightly more anemic since then. The other "shocking" news is that we are actually REDUCING Sam's calories in his TPN as he gained too much weight in the last two months (I can hear my mother already as she always thinks he isn't getting enough calories).
With all of this going on we still had time to meet with Bradley's family all day yesterday. I have spoken on the phone to them many times over the last few months and it was nice to meet them in person. The weather wasn't the best in Boston on Wednesday so we spent some time hanging around at the hotel. I had hoped that Sam and Jackson (Bradley's brother) would hit it off, but no luck there. Sam just wasn't in the mood to deal with other kids. We completed the day by having dinner with another "Omegaven Mom" when Genevieve's mom, Tiffany, took a break from the hospital to joined us. It was nice to meet her and give her a little break.
This morning we had breakfast with Bradley's family and Blaise's family before we headed to our clinic appointment. All in all it was a busy two days. Tomorrow motning we will head to the airport at 4 AM to head back to Wisconsin.
To top off the week, we received several inches of rain at home yesterday. Severe storms knocked out power during the night which caused our sump pump to not run and we now have wet carpet in the basement family room that we just finished this spring. Deb is doing her best to get everything dried out and hopefully we can salvage everything.
I will update as we get more information about Sam's infection, but I will leave you with a picture of what Sam is supposed to wear on Saturday. Here's hoping that he isn't wearing an ill fitting hospital gown instead.
Tuesday, June 15, 2010
Since our last post, Sam and I traveled to Boston for a checkup. Traveling was hell this time as we spent a fair amount of time sitting on the runway both on our way to Boston and on the way home. Sam was not pleased. Overall the rest of the trip went OK. Sam's labs are looking good and his growth is following the same curve that it has for the last several months.
We had his central line site looked at while we were there as the stitches were causing a fair amount of skin irritation. Since it wasn't affecting the rest of the site the decision was made to try to leave the stitches in for a few more weeks.
Unfortunately the irritation caused Sam to rub at the site which ripped the stitches out just a week or so after we got back form Boston. Within days he developed a site infection. We treated with antibiotics and the site seemed to improve and begin to heal. Yesterday the site looked red again and was discharging a small amount of puss again. We are back on antibiotics and waiting to see if we can clear the infection completely this time.
Last year we spent almost the entire summer dealing with multiple site infections. We were hoping for a fun filled summer this year, we will have to wait to see what happens. We had a lot of plans last year and most of them got canceled. I just don't want to disappoint Ryan this summer by spending multiple weeks inpatient.
In between dealing with Sam's site infections, we have been very busy already this summer. Sam got to swim for the first time (and for those of you reading that cringed at the mention of Sam, we have some up with a system that keeps his central line completely dry). Sam is not able to swim in rivers, lakes, or public pools. There is just too great of a risk of his site getting wet and contaminated with bacteria. We already have enough of that going on. We were able to purchase an inexpensive pool to set up in the backyard that is chlorinated and filtered. Since we can manage the water and we know the pool is clean, we are comfortable with Sam swimming in it. Sam is definitely a fan of the water. We had beautiful weather at the end of May and were able to get in the pool a few times. June has not been as kind and we have been spending the last two weeks dealing with what feels like non stop rain and cold weather.
Ryan and Sam also received an early Christmas present from Oma and Opa in Georgia. Our old swing set was here when we bought the house, and was not in very good shape. Ryan and Sam both love the new one and it is much safer, and a lot more fun, then our old one. Putting it together was not as bad as I originally though it would be (Thanks for the help Scott!) but it still meant a few long days of work to get it all done.
We have also learned that Sam has a very hard time regulating his temperature when the weather is warm. He overheats very easily, even when he is hooked up to IV hydration during active times. Luckily, he realizes that he doesn't feel good and heads for the house or a shady spot when he starts to overheat. It is going to take careful planning to manage this during warm weather outings this summer, and we are planning on spending as much time as possible this summer outdoors and having fun.
On the Omegaven front, we still have not made any progress. We are still working with Madison to work on a few options to try to get Omegaven here for us, but I am not very optimistic. Sam and I are headed back to Boston on the 14th of July for a quick check up before we hurry back to be in Wisconsin for my brother's wedding. We will also be meeting up with Bradley's family while we are there. We still don't know if we will be able to return to Boston in September, but we are doing everything we can to continue with Boston and Omegaven for as long as possible.
Thursday, May 6, 2010
First off, our friend Carter Cates is not doing well. Sean and Chelle have not had a chance to update their site yet, but I have been following updates on Facebook all day. Carter is in severe respiratory and renal failure. About an hour before I started writing this Carter was taken to surgery. At the time I started writing this Carter had already had a bronchoscopy and was intubated. They are now attempting to place a catheter in his right jugular so that they can start dialysis. This is very severe and very scary for the Cates family. Carter has been through a lot in his short life and he just can't seem to catch a break lately. Carter's website is www.cartermcates.com , please keep this family in your thoughts and leave them a guestbook message to let them know that you are thinking of them.
Now I want to go back in time a few years. Two years ago Sam had been transfered to Children's Hospital of Wisconsin Milwaukee from the NICU here in Appleton. We still had no idea what was wrong with Sam. He was just over 8 weeks old. It would be just about a week before we got a diagnosis and started to attempt to figure out what the future had in store for Sam. The future looked pretty grim. Everything we read on line about Microvillus Inclusion disease was pretty dire. We didn't know about Omegaven. We had not yet found Bo's family. We were very alone and it was a very scary time.
Fast forward two years. A short while ago we were contacted by a family in New York. I almost deleted the email without reading it, the subject line said "We don't know what to do. Can you please help?". I thought it was spam. For some reason I opened it anyway and this is the message that I found:
Dear Mr. and Mrs. O’Conner,
First off I can’t tell you how much of an inspiration Sam has been for us right now. My name is Scott Shutka and my wife is Kristy. We live on Long Island in New York and we have a 17 month old boy named Jackson as well as a 4 week old boy named Bradley. We found out over the past few days that Bradley has Microvillous Inclusion Disorder much like Sam. I am sure you know the pain and unanswered questions we must have. I am not very computer savvy so it would be easier if I were able to talk over the phone. If you have any time in your busy schedule day or night that you are able to speak to us, we would greatly appreciate it. We really need some answers and the doctor’s don’t seem to know very much about this genetic disorder right now as they are awaiting guidance from a gastrointestinal specialist. Most of what I have learned about it is from online resources. I was hoping to get more insight and guidance from anyone who has personal experience as that is the best resource around. We thank you in advance for any help you are able to give us. "
Well, at least I didn't delete it. We have been in contact with Bradley's family through email and phone calls ever since. While our hearts break for them as we know exactly the range of emotions that they were experiencing, we were excited to have the opportunity to share out knowledge and experiences with them. We were also able to put them in touch with Bo's family so we were able to almost double our knowledge and experience to help this family out. Within a few weeks of our first conversation, Bradley was able to start receiving Omegaven while his bilirubin levels we still relatively low. They are just starting to make plans to take Bradley home. It is amazing how different their experience over the last few weeks has been from what we experienced when Sam was diagnosed. They are still working though some uncertainty and red tape, but I truly believe that they will be much better off because of Sam's and Bo's experiences.
Bradley's parent have just started a Caringbridge site recently for him. If you would like to follow his story, please visit:
So what's been going on with Sam? Not much really. Sam has been battling a spring cold along with the rest of our family. He has been very active and loves to be able to spend more time outside now that the weather is nice.
We had our first appointment in Madison a few weeks back, and it went well. We talked at length about where we would like to go with Sam's care and what our expectations are and everyone there seems to be on board. We will be back down in Madison for another visit in June as we are now officially moving all of Sam's specialty care from Milwaukee/Boston to Madison. We will still be seen in Boston and they will always be our secondary care team for Sam.
Unfortunately this has not completely solved the dilemma that we are in. We were hoping to be able to have Madison provide Omegaven for Sam. As of today it sounds like that is probably not going to happen. While we can still hold out hope for things to work out, we need to be realistic at the same time and plan for the fact that the probability is low.
So where does that leave us? Well, to be honest it leaves us in a very very scary situation. I am traveling to Boston in a few weeks with Sam, while there we will receive a 30 day supply of Omegaven. One month later we will do labs locally and have another 30 days shipped to us. If we do not return to Boston in July, it will most likely mean the end of Sam's Omegaven journey. We are looking at the possibility that Sam may need to go back on the dreaded intralipid that caused so much damage to him two short years ago. Because Sam is older now, he may handle intralipid just fine with no adverse reaction, but the idea is still not a pleasant one and this was not a decision that we ever wanted to have to make.
We simply can not afford to continue to travel to Boston every eight weeks. When Sam was born with Microvillus Inclusion Disease we went from being a two income family to being a one income family. Deb works part time, but that barely makes up for the income loss that I take every year by taking FMLA leave when Sam is in hospital or to travel to Boston. Raising Sam has not been cheap. The money that was raised almost two years ago during Sam's benefit is just about depleted, and we would like to keep a small reserve of that money in the event of any urgent situation that might arise for Sam.
We still have two months to get things worked out, but everyday that goes by gets us a little close to that day when the Omegaven may run out.
Well, this turned out to be a bummer of an update. I will try to get a happier update and some new pictures posted soon...
Monday, April 12, 2010
Sam has been doing very well. He has "graduated" from physical therapy as we have blown away all of his goals. He is still having speech therapy weekly and if you listen close you can catch a lot of words inside what used to be a whole lot of blabbering. Sam loves to talk on the phone, "Hello" and "Goodbye" over and over again. His new fascination lately has been the word "WOW!".
We have also been working on potty training, but that is not going quite as well as we hoped. Sam loves to sit on the potty chair, as long as he is fully clothes and wearing a diaper. He just doesn't like the way the seat feels on his bare but. Oh well, he will get there eventually. He is showing awareness of the fact that he is pooping and likes to hide when he does it. Although this can be frustrating (especially when he is leaking all over the floor), it is a good sign and something to work with. The bad side of this is that Sam is not as tolerant of dirty diapers as he used to be overnight. Starting about a week ago we are now up every night and least 2 to 3 times to change his diaper. We have been so spoiled for so long as Sam has slept through the night since we first brought him home when he was 3 months old. Not having to get up to feed a baby was one (small) benefit that we at least gained with Sam's disease.
Sam enjoyed Easter, but was not really into the hidden eggs so much one he realized that they had candy in them. That was fine with Ryan who, of course, got to eat all of the chocolate.
With summer upon us and Sam being more active we have had to increase the amount of hydration that Sam received through his IV. This has become a challenge due to the fact that Sam is not used to being "hooked up" to an IV during the day when he is not in his crib. Since he doesn't remember to take his bag with him and the dog doesn't see the line stretched between the bag and Sam, we have had to start using a backback for Sam during the day when he needs hydration. He screamed bloody murder for the first hour, but we refused to give in and he eventually forgot that it was there and went about his business of climbing and running. This will, hopefully, make it much easier to manage trips outdoors and camping this summer.
Buddy is growing way too fast and has doubled in size in two months. At 25 weeks old he is now 43 pounds. The kids still love having the dog around, but Sam knows that when Buddy gets excited it is time to be picked up. It didn't take him long to figure out how easy it is for Buddy to knock him down.
Sam and Ryan have been getting along very well lately, except for the days when Sam decides to bully Ryan.
In other news, Sam has been accepted into the Littlest Heros Project. The Littlest Heros Project is a network of photographers that provide free photo sessions to any child who suffers from any type of serious illness or life altering disability. We are excited to hopefully get some really good, professional pictures of Sam.
Next Monday we will be traveling to Madison to meet who will hopefully become Sam's new primary GI doctor. We will also be meeting with the nutrition group and the social worker at American Family Children's Hospital in Madison on the same day. If all goes well, we will be transitioning the primary responsibility for Sam's care to Madison over the next two months. Sam and Jason are headed back to Boston in May and then, depending on our Omegaven situation, we may not be back to Boston for 4 to 6 months!!
Sometime since our last post I added a bunch of pictures from Sam's birthday to the photo section, enjoy!
Saturday, March 20, 2010
We started out early Wednesday morning with a venous ultrasound of Sam's jugular and subclavian veins. This was done to determine the amount of vein narrowing that Sam has due to the multiple times that we have had to replace his central line. The last replacement that we did in September was a difficult placement and the surgical report from that placement made us very nervous about running out of access points in Sam's upper body. There are only so many veins in the body that can support a central venous line. Our other options would be to have a CVL placed in his femoral artery, but we are trying to avoid a placement there as it would mean having an exit site below his waist. Having a line below the waist for Sam would mean having a line covered in stool at least once a day. That would lead to even more infection risk. If we do loose access sites, there is the possibility that the situation could change as Sam gets older and grows, some previously narrowed or damaged sites may become accessible again.
The ultrasound itself was a complete nightmare. We started at 8:50 in the morning and the procedure should have lasted 20 minutes. We ended up being a half an hour late for a 10:00 appointment with Dr. Puder. I explained to the ultrasound tech that if I tried to restrain Sam, he would resist and get angry so it was best to try to work with him lying on the table without holding him down. That went fine for 5 minutes until she needed him to turn his head away from her. He got mad and that was pretty much the end of him cooperating. After listening to him scream for 20 minutes I suggested that we have him sit in his car seat/stroller, but she didn't think that would work. At 9:55 she decided that it was time to have a different tech come in to try to finish the procedure which gave me a chance to try to calm Sam down. I strapped him into his car seat and rocked him almost to sleep.
The second tech came in and asked if we could try just leaving him in his seat to finish the ultrasound. I told her that I thought that was a great idea. Too bad nobody thought of that earlier. Within 10 minutes the ultrasound was complete and we made a mad dash for Dr. Puder's office.
When we made it to Dr. Puder's office we went over the results of the ultrasound. The results were not anywhere near as bad as he, or I, expected them to be. There is some slight narrowing in the right subclavian, but the left looks normal. Both jugular veins look good as well. The current line was already in the left subclavian so I was still holding out hope that we wouldn't have to pull the line and risk damaging the vein. I knew before asking though that Dr. Puder was going to want to replace it. It appeared that Sam may have been starting a new infection at the exit site as there was a small amount of drainage. This on top of the fact that the site looked terrible, was not healing, and had a mess of granulation that kept getting irritated, meant that it was time for a replacement.
With that agreed upon we moved onto a discussion regarding the direction that we are heading with Sam's medical care. I am pretty sure he wasn't shocked as he knew we were frustrated with Children's in Milwaukee over not allowing us to use Omegaven while inpatient and the fact that there are certain doctors there who should be more involved in Sam's case that seem to just not care anymore since we made the decision to go to Boston. (More about the planned changes in a little bit). We also discussed Sam's future in the Omegaven study. We simply can not afford to continue to travel to Boston every eight weeks. There is enough money in Sam's benefit account to make a few more trips, but we having been doing this for almost two years already and it is just becoming too much. No matter what happens we hope to continue to travel to Boston at least one or twice a year at a minimum. We would like to keep Dr. Puder and his team involved in Sam's care for as long as we can. Since our insurance will not cover Omegaven we are really in a tough situation as there are not any facilities locally that have the funding to provide the drug for us at no charge. We left things as they are for now as a final decision on what we are going to do with Omegaven will depend on a huge number of factors.
We headed over to surgery to check Sam in and get him ready for surgery. While Sam was in surgery I got to spend a little bit of time with Carter Cates and his parents Sean and Chelle. I was nice to spend time with them as we really enjoy their company and we always seem to miss each other in Boston. The last time Sam and Carter were both in Boston at the same time we weren't able to meet Carter.
Surgery went well. Dr. Puder was able to re-wire Sam's Central line using the existing vein site which meant that we did not cause any further damage to Sam's veins and did not cause anymore access issues for the future. A re-wire is done by making an incision in the existing central line close to Sam's neck. A wire is then inserted through the incision into the existing line to the tip. When the old central line is pulled out the new one is then slipped over the wire and the new line is tunneled under the skin to a new exit site. Sam's new site is lower on his chest which will hopefully allow us to protect it better. It will be harder for him to get his had into the top of his shirt and reach the dressing now. We are also protecting the dressing and line using a Summer's Life Guard under his shirt and an ace bandage wrapped around him on top of his shirt while he sleeps. The new site looks great so far and is already healing well.
Sam was pretty angry in recovery and we have to give him some additional morphine to help calm him down. This put him back to sleep and he was out cold for over an hour. When I finally got him to wake up he was in a much better mood and we headed out of the hospital and back to the hotel.
On Thursday we had our CAIR appointment. I was nervous going into this appointment because I wasn't sure how the team was going to react to the changes that we want to make to Sam's care. Our only experience with this type of change was in Milwaukee when we told them we were going to Boston for Omegaven. It was not really a pleasant experience.
In comparison, the discussions with the CAIR team went very very well. Now that we have spoken to them we can share what we are planning.
We are currently working on building a new local team of doctors for Sam at American Family Children's Hospital in Madison. We will be selecting each member of our new team and we will be very up front with them as to what we are looking for. This team will gradually replace the CAIR team in Boston as Sam's primary care team over the next 4 to 6 months and the Boston team will be our secondary team. Once this transition is complete we will still continue to see the CAIR team in Boston, but much less frequently that we currently see them. At first we will still see them three times a year, but we will probably go down to once or twice a year when Sam turns three. We are asking our team in Madison to be collaborative with Boston. Both teams will be kept in the loop as to what is going on at all times and we are not going to tolerate any of the political garbage that we experienced in Milwaukee. This change is important for several reasons, but the main reason is that in a crisis situation we need a team here is Wisconsin that is comfortable and familiar with Sam. It is also important to make this change so that in the event that we can not continue to travel to Boston, due to the cost, we already have a team here is can take over his care completely.
This has been a difficult decision to make. Boston, for the most part, has done a very good job of managing Sam's care from halfway across the country. They have listened to our input and have helped us to keep Sam healthy and growing for the last 19 months. They have resources that Madison does not have and this is why we will continue to keep our team there active in Sam's care for as long as possible. There may come a day when a situation arises with Sam that can not be handled in Madison and we always will know where to go if that happens.
The main thing that we have to work through is what to do about Omegaven. Omegaven has saved Sam's life and we are not ready to stop providing it for Sam. Working with the team in Boston we have come up with a few solutions on what we can do:
>Nothing. We continue to participate in the Boston clinical trial. This would mean finding a way to fund returning to Boston every eight weeks until the drug receives FDA approval and can be prescribed locally and paid for by our insurance.
>Stop giving Sam Omegaven and go back to using Intralipid. Other children who were suffering from TPN associated liver disease, went on Omegaven, got healthy, and then had to stop Omegaven for whatever reason, have done well going back on the standard Intralipid formula. It is possible that this could be the case for Sam. In the event that we start to see damage to Sam's liver we could go back to using Omegaven.
>Stop giving Sam Omegaven and attempt to supplement him with Enteral fish oil orally. There is a great possibility that this wouldn't work, but it is worth discussing or possibly testing. For this to work we would have to get Sam to take fish oil orally, on a regular basis (which doesn't sound like fun), and his gut has to absorb it. We have no idea if his gut will absorb any or a portion of fish oil given orally so we would need to perform a test by giving him the fish oil orally for awhile and then run labs to see how much he is absorbing. If he absorbs it at all I do not think it will be a high amount so this could mean giving him massive amounts of fish oil orally every single day and dealing with whatever this might do to his already stinky stool.
>Get Omegaven locally. Well, we have already tried this and haven't had much luck. Since our primary insurance will not cover Omegaven and we have not found a hospital locally that has funding to cover the cost of the drug it just wasn't an option. So what if there were a third option? What if Fresenius Kabi, the company that produces Omegaven, was nice enough to provide Omegaven to a local facility at no charge so that they could provide it to Sam? Well, that is exactly what we are waiting to find out. It will take some coordination between Madison and Boston, and there is no guarantee that Fresenius will agree to it, but we have a guardian angel in Boston who is working on it for us.
It was a busy three days in Boston. It is going to be a busy couple of months getting everything set up and coordinated with a new team of doctors in Madison. After all of our discussions with everyone in Boston though, I am convinced that the decisions we are making now and the steps we are taking are what is best for Sam and his health.
I was actually sad to leave Boston after this visit. I got to spend a fair amount of time with Sean Cates while we were there. It was nice to give him a break from being at the hospital and I enjoyed getting to know him and Chelle a little bit better. We talked for hours (over several beers) about Sam and Carter and Hospitals and Doctors. It sucks that I am in a situation to have those kinds of conversations and that they are in a situation to have those types of conversations, but I am glad there are people like the Cates family out there to have those conversations with.
Unfortunately we ended the Boston trip on a bad note. I caught a bug from somewhere in Boston and it hit me while Sam and I were waiting to get on our flight home. By the time the first flight took off I was miserable. Landing was horrendous as I could not get the pressure in my ears to equalize. This caused excruciating paid while landing. I ended up with two perforated ear drums and a double ear infection.
I haven't updated pictures in awhile and I will try to get that done this week. I need to get the pictures from Sam's birthday uploaded and Sean Cates took a bunch of excellent pictures of Sam in the garden at Children's Hospital Boston which I will post as soon as I get a chance,
Tuesday, March 16, 2010
Although Sam's infection cleared up with the last round of antibiotics, the site really hadn't healed at all yet. I planned ahead and had Dr. Puder put Sam on the surgery schedule for Wednesday since we were going to be in Boston anyway. Tomorrow is turning out to be a very busy day.
Tomorrow morning Sam is having an ultrasound done to look at his veins. This is being done because his last line placement was very difficult and took several times longer than a line placement should take. This study will help us narrow down where Sam still has veins that can be used for central access and which ones are not available. We may gain back some of the unavailable sites as he (and his veins) grow, but we need to be able to keeps these lines and sites healthy for more than a few months.
Tonight when I changed Sam's dressing, I noticed that Sam's site has some discharge again and it looks like there may be another infection brewing. Tomorrow at 1:30 (so like 4:30 hospital time) Sam will have a new line placed. Hopefully everything will go well and Sam and I will both be back at the hotel tomorrow night.
I will post again tomorrow one we are out of recovery.
Monday, March 8, 2010
Sam's party went great and it was a very fun day for Sam. We managed to somehow only take about 5 pictures from the entire day, so if you were here and took any pictures, please email them to me. I will post a bunch on the site as soon as I can get the memory card out of my mom's camera.
As soon as I can get a little sleep I may come back to finish the post that I originally intended to post yesterday.
Sunday, March 7, 2010
While we are there, I am going to have central access and surgery take a look at his site and although it doesn't really look infected anymore it is still an ugly mess. I am not going through a line repair tonight just to turn around and head back down there on Monday or Tuesday.
Friday, March 5, 2010
At of 11 PM tonight, Sam's party is a GO. His site doesn't look great, but I have seen it look worse. The infection is better and I have no doubt we will make it through tomorrow with no issues.
To top that, I think our house is cleaner than I have ever seen it in two years.
Wednesday, March 3, 2010
My frustration with the hospital grew continually with each moment that I spent there and it was probably a good thing that we got out of there today as I don't think I could have taken anymore. I had hoped that I could meet with GI, surgery, and the central line tem before I left so that we could try to put our heads together to help come up with a plan for future stays to help get Omegaven for Sam while he is inpatient in Milwaukee. That never happened. I wanted to meet with the central line team to try to brainstorm ideas on ways to keep Sam away from his line. That never happened either.
So we are home with antibiotics and will we continue them for 5 more days. Sam's site is better than it was, but it is not great. I would say that we have about a 25% chance of making this line last past our Boston trip in two weeks and a 50% chance that we will end up back in Milwaukee next week.
To add to our frustration, we were only home an hour and Sam had already destroyed another dressing.
Tonight when I checked on him 45 minutes after he went to bed he had already worked his way out of the wrap that we put around him to protect his line and dressing from his hands.
It does look like we will at least be home for the weekend so we are moving ahead with plans for Sam's birthday party. I may sleep through it though.
Tuesday, March 2, 2010
There are certainties that we will have to deal with during every admission whether it is in Milwaukee or Boston. Some good and some bad.
These are teaching hospitals which means a lot of students. Typically these people have no bedside manner and very little experience. Combine that with their superiority complex and "I know everything" attitude typically leads to confrontation. With that being said, I somehow managed during this stay to meet not one, but two, residents that I didn't want to hurt. It was refreshing to talk to a GI and surgical resident who actually wanted to hear about Sam's history, wanted to hear my input, and actually showed in interest in Sam instead of trying to shove their almighty and all knowing wisdom down my throat.
Unfortunately other things have not gone as well. The hospitals investigational review board has denied our request to continue Sam's "investigational" therpary (a.k.a. Omegaven, the one thing that made Sam healthy and doesn't damage his liver). I won't go into a lot of detail on this, but pretty much the entire day Monday was spent trying to work out a solution. It just isn't going to happen. More about this later.
The plan since yesterday was to continue IV antibiotics and give Sam's site a chance to heal. We want to do everything we can to try to save his line as there are only so many places that a line of this type can be inserted and we don't want to use them all up. This plan means leaving the hospital on Wednesday and going home to continue antibiotics. The plan is a good one and I was hopeful that we could at least extend the life of this line. It does appear that the infection got to the cuff on Sam's line. It is extrememly hard to completely get rid of the bacteria from the cuff so there is a good possibility that even if we can get the infection under control and if we can get the site to heal, the infection could come back at any time. We have seen this before, sometimes as soon as we stop the antibiotics the infection comes back.
I was hopeful that things would go as planned and that we would be heading home on Wednesday. Tonight we changed Sam's dressing and I am not quite as hopeful after getting an up close look at the site. In my opinion his site looks worse today than it did yesterday. I will still give the antibiotics a chance to work, but I am nervous about leaving the hospital with his site in this shape. Part of the problem lies in the fact that once we are discharged, we are two hours away. The other part of the problem is that is is really not healthy for Sam to be in the ER for another 7 to 8 hours if we need to be readmitted. Our other option would be to try to wait this out and have the line changed in Boston as we are scheduled to head there in two weeks for clinic. At least then we can avoid the ER. Neither option is very appealing.
Sam is bored and ready to get out of here. We are ready to celebrate the fact that we have beaten the odds and we want Sam home this weekend to celebrate with us. I do not like to have to make these decisions that can have such a huge impact on Sam's health and I am not looking forward to rounds tomorrow when I have to voice my concerns.
There is also the fact that as it stands right now we will not be able to supply Omegaven to Sam while we are inpatient here. Whether we extend this stay or some back next week this problem is not going to go away.
We have been considering making some major changes to Sam's care team for some time now. At this point I am not really sure what that means. Something has to change. We are stuck in a situation where our belief is that in order to maintain Sam's health in the best way possible we need to continue to participate in the Omegaven study. Unfortunately that means traveling to Boston every eight weeks. I am not entirely certain how much longer we can do that.
Each trip out there just got more expensive as Sam tunred two and is now required to have his own seat on the plane. Each trip, with travel, lodging, meals, cab fares, and whatever else comes up costs between $1500 and$2000. On top of that, we have to add the expense of lost wages for both Deb and I as we take a varying amount of unpaid time off of work to manage all of this. It also means that the team that we rely on for the majority of Sam's care is over 1000 miles away from us. Due to ego's and hospital politics this also creates additional problems when we need to seek care closer to home.
We know that something has to change, but we are just not certain what that is yet, our current hospital stay has really driven home the fact that our current situation is not ideal and is not best for Sam's health. Our optimal situation would allow us to pick out the best parts of each team and move them all into our basement. Since that isn't going to happen we need to look at what is best for Sam and try to make it work. That will most likely mean eliminating some parts of our team and including new parts. It may mean completely moving all of Sam's care to a new hospital.
Hopefully during rounds in the moring we can finalize a plan for our current infection treatment and decide how to proceed for the next few days/weeks. Deb and I are also going to decide tomorrow whether we think we are going to be home on Saturday and move ahead with party plans or cancel everything and try to plan for later.
Monday, March 1, 2010
Sam and I spent almost 7 hours in the ER last night and finally got up to our room around 2:30 this morning. We both managed to get almost three full hours of sleep in before shift change and rounds.
Today we are just hanging out waiting to talk with everyone and coming up with a plan for the next few days.
I will update more later today once I get my computer (which I somehow left by the back door at home). It is hard to get out of the room to use the computer in the lounge so I will most likely not update during the day today.
Saturday, February 27, 2010
Since Sam's recent infection he was healing nicely and his central line site looked really good. Unfortunately Sam ripped his dressing and pulled on his line earlier this week. He pulled hard enough to actually tear the site open and move the cuff (which is a piece of plastic that his body "heals" onto after the line is placed and holds it in his body). Since that time, his site has deteriorated.
Tomorrow I will be taking off his dressing to clean the wound and redress it. Tonight I can see that he is still bleeding and there may be some other discharge as well.
Depending on how the site looks on Sunday, we may be headed to Milwaukee for an admission where we will do everything we can to attempt to save the site. If the infection has spread to the cuff we will most likely not get this infection to clear up and could mean surgery to place a new line. This will be more complicated than it has been in the past as we have to either have a Femoral line placed (in his leg) or have them search for a usable vein elsewhere.
Still hoping that the site looks worse now then it really is. It is very possible that I could get the dressing off and clean the site and it could look fine.
Either way, we are moving forward with plans for next Saturday. Hopefully if we do end up in Milwaukee we can get in and get out and be home in time for the party.
Saturday, February 13, 2010
In other news, we have a new addition to our family. Buddy came to live with us on Thursday. He is a 4 month old Black Lab/German Shepard mix. He currently enjoys licking the children and peeing on the carpet.
Buddy is very mellow and a perfect match for Ryan and Sam. He is going to be a big boy so he will be able to tolerate years of "attention" from the kids.
Wednesday, February 10, 2010
Nothing else new, and not really much to update. Sam has not been phased by the infection or the screwy schedule that we have to follow in order to infuse his antibiotics at home. In fact, he slept through a lab draw at 9 tonight. If only I could sleep so peacefully.
Tuesday, February 9, 2010
Having a child who visits the ER regularly and has frequent inpatient stays is a different experience than going to the ER with a broken arm. Almost everyone at our local hospital knows Sam. This normally makes it very easy to get the treatment that he needs. We normally speed through the ER and the ER doctor almost always pages the peds specialist for us as soon as we get there. They are the people that know Sam the best and have cared for him the most locally.
So when a certain doctor walked into our exam room last night I was less than pleased. I have dealt with guy before and knew right then and there that I was in for a long night. He does not feel the need to contact Peds. He doesn't want to hear our ideas on how Sam should be treated. He couldn't even be bothered to look at Sam's site to see what it looked like. He refuses to consult with Boston or Milwaukee. I doesn't feel that a central line site infection is an emergency and we should have just waited to be treated in clinic in the morning. He really just doesn't care. Why would you become a doctor if this is how you feel?
Many parents with medically fragile children deal with this attitude on a regular basis. We never experience this locally and that is the reason that we try to stay here whenever we can and only move to a "bigger" hospital when things get beyond what they are comfortable dealing with. The next time Sam needs to go to the ER, I will be calling first to see who the ER doctor is before heading to the ER. A 2 hour drive to Milwaukee is better than ever having to deal with that doctor again.
We will be headed into the clinic this afternoon for a check on Sam's site. He is still asleep so I haven't been able to take a look at it yet, but hopefully it is looking the same or better than last night.
Monday, February 8, 2010
That ended tonight. I am sitting in the ER with Sam waiting on labs and to talk to the ER doctor (who is the same jerk that was here the last time we were at the ER). Sam's site is infected, again. The infection looks pretty mild, but that can change in a hurry. Hopefully we caught the infection before it made it to the cuff (which it looks to me like we did) so we can start antibiotics and hopefully save his line.
I am not sure yet if the are going to just do a dose of antibiotics and then send us home or admit us yet, but I will update when I know more...
Thursday, February 4, 2010
Sam continues to thrive and loves trying new tastes. Eating for Sam is not really eating, swallowing is not happening for him, but he does enjoy tasting food and sitting with us at the table. His favorite taste so far is lemon juice.
No other new news to report on Sam. We are busy trying to finish up a few last small remodeling chores and getting the house ready for Sam's big day.
If you have a moment, please keep the Cates family in your thoughts and prayers. Carter has been giving the doctors in Texas a run for their money lately. Cater's website can be viewed at www.cartermcates.com.
Monday, January 18, 2010
Since our last update we have been halfway across the country, twice, spent a day with our friends in Michigan, and celebrated Christmas at least 6 times (which led to a much too large addition of toys to the playroom). Somewhere in between all of that we managed to almost finish several remodeling projects at the house. Our new family room in the basement turned out great and Sam LOVES having the extra space to run around. Literally, he can RUN around in this room - it is HUGE!
Christmas started for us on Christmas Eve at Nana's house, then at home for Christmas morning, and then back down to Grandma's housr for Christmas afternoon. Unfortunately Christmas Eve almost marked the return of Sam's vomiting after two months with no issues.
The day after Christmas we packed up the van with all of our gear and medical supplies and started on our way to Georgia to visit Oma and Opa. Although the drive went well and the kids were well behaved (Thanks to the TV in the van and Ryan's Leapster), spending that much time driving did not sit well with Sam. His vomiting escalated to the point of actually starting to worry us, but we have since determined that he may also suffer from motion sickness. Traveling that far away from Sam's doctors and being in a situation where we have to even consider the possibility of having Sam see a doctor was nerve wracking, but we made it OK.
We made a spit stop for an afternoon to visit friends in Atlanta before continuing on our journey.
We celebrated Christmas at Oma's house a few times and Sam got to meet lot's of family for the first time. The weather was not as warm as we had hoped, but we did get outside to take a hayride and see a whole farm full of Christmas lights. For six years I have heard how much warmer it is in Georgia than it is in Wisconsin. Good thing we brought along winter coats because we definitely needed them. Unusually cold weather hit the area so we didn't venture out of the house too much.
We took a detour on our way back to Wisconsin to Michigan which would have been a nice drive except for hitting snow and a closed interstate. A closed interstate meant driving through the mountains on a winding two lane road which did not help out much with Sam's vomiting issues, but it was a beautiful drive. Once in Michigan we spent the day visiting with the Velarde-Chan family in Kalamazoo. Bo (who also has Microvillus Inclusion Disease), Sam, and Ryan had a blast playing (although I am pretty sure that they managed to wear out all 5 adults that were in the house that day). It is always nice to be able to spend the days with Bo's family and compare notes on things going on with the boys. The day went by way to fast and we can't wait to see them again in March.
Once we returned to Wisconsin we had a break for a few days before we headed back to Grandma's house to celebrate Christmas one more time with her side of the family.
A few days later it was time for Sam to head back to Boston for a check up. Ugh, more traveling.
Sam's check up went well. Still no answers on what is causing his vomiting. It comes and goes and is definitely worse when we travel. Both Hunter and Bo have similar issues so we are going to just chalk it up to part of his disease for now and will look more into it if it begins to cause other problems for him. Sam is growing well, and is actually getting to a point on the growth charts that he is starting to get too heavy for his height. Clinic ran smoothly and Sam is doing so well that there really wasn't much to discuss or change in his medication or TPN. Satisfied that we are still keeping Sam as healthy as we can, we headed back to Wisconsin with another months supply of Omegaven in hand.
In other news, Sam has started to try to communicate more verbally and through signing the last few weeks. It is always out of the blue that he adds a new word to his vocabulary. His favorites right now are "Thank You" and "gentle".
That about wraps up the last month. I uploaded tons of pictures from Christmas and our trip. Make sure you check out the photo of Sam and Bo giving each other a big hug.
Don't forget, Sam's Birthday party is March 6th, mark your calendars. Everyone is welcome to stop by anytime between 11 and 5. I will post more details on the party soon.