Michael’s PICC line (#3) was replaced and he’s at home with TPN/Lipids every day (hoping to move back to 3x a week). He’s gained 4.5 pounds in a little under two weeks, so definitely going in the right direction!
Day five is on target for going home. Michael’s new PICC line is looking good and his blood sugar is reacting fine to pumping all the nutrition at a faster rate. We’re just waiting for all the paper shuffle of going home which is a bit more than usual as home nursing and TPN delivery has to be set up as well.
We have been very much blessed in Michael’s life with people who have gone above and beyond their jobs–his pulmonologists never flagged in their stance of taking each issue and dealing with it as it came and keeping him alive despite all the odds when he was young. His hematologist came in the picture when he was eight and not only saved his life, but gave him a better life for the next 10 years, and today has taken on all the responsibility of coordinating his care and working to make his quality of life as high as possible. My mother has been unflagging in her support of Michael, myself, and my family. When I think of the hours she has spent in doctor’s offices even back when she had young children of her own, I am overwhelmed by her love and support. She gives Michael the space to just be and forget about his issues. These are big gifts, and there are many more friends and family who have stood by for years in good and in bad.
Sometimes, however, its a little thing that can go unnoticed in the middle of the big things. The first PICC that Michael had was a nightmare, not because of the PICC itself, but because all of the dressings we tried irritated his skin so badly that just before they pulled it due to skin breakdown, the whole patch would just fall off as another layer of his skin fell off. As a mother, it was painful and frustrating to watch. Just before they pulled it, our nurse brought a different type of patch that another of her patients gave us to try–it stopped the inflammation but it was too late for the skin on that arm, it needed to heal up. PICC number two we started right off with the Sorbaview patch that our supply company was finally able to get a hold of in a small form that we had to piggy back, but it was worth it to keep his skin in better condition.
PICC #3 came and we had two Sorbaviews left to use for the initial placement but due to strange and irritating “rules” we couldn’t even manage to buy them off the supply company due to the fact that a) he wasn’t a patient b) they didn’t have orders c) even if they had orders they couldn’t dispense to someone in a hospital. It’s a PATCH, not something you can sell on the street corner or black market! After a round about frustrating conversation I hit the web, perfectly willing to buy them but again frustrated at being unable to do so. They are being trialed by hospitals at the moment. I really wanted to get my hands on the newer Sorbaview Shield which would let us ditch one more object with adhesive on it (statlock to hold it in place). We didn’t need the statlock as they had stitched it on his skin, but that meant I wanted it VERY secure–I want this PICC to last as long as possible. We HAD to change his patch in the hospital as it was full of blood from the placement and pooled blood breaks down skin but I so did not want to put anything with tegaderm on it and start the breakdown as well–frustrating! Anyway, no luck, couldn’t even buy them. I finally called the company directly and fell in love with Martha.
At first I was told that they couldn’t sell them to the public because they were on trial with hospitals but that she could see about possibly donating some, she was very sweet and gracious and asked some questions about Michael, his health, his reaction to all the other brands we tried and said she would contact someone. Within fifteen minutes she called back saying they would be sending us some! Yesterday we got a box of 100 Sorbaview Shields that will give us almost two years of patch changes! Thank you Martha and Centurian!!!!!!!!!
It seems like such a little thing to write such a long post about, but Michael needs a PICC line, which needs to be kept sterile and safe as possible from infection, which needs to not destroy his skin and cause him pain and frustration. It made my week!
And the nurse just came in and we can do the paperwork and get out of here!
Monday morning we came to the hematology floor early to have his bloodwork checked before having his 3rd PICC placed in the afternoon. His platelets came back 22,000–as usually with his platelets, I think we’re again at record low. They decided to go ahead and give him a transfusion even though he had reacted to the transfusion in July–they weren’t giving him near the amount, pre-medicated him with Tylenol and Benedryl (for hives) and ran it slowly. He did fine with it, the Benedryl IV knocked him flat and he got a couple hives, but nothing too bad.
The PICC insertion was pretty rough, and by pretty rough, I mean very rough–he felt like he was in a torture chamber. His first two went quite well, so I’m not entirely sure why this one went so badly. They used the Emla cream (numbs the skin) and were quite generous in using local pain killers but it just wasn’t cutting it and the poking for the pain killers bothered him more than the pain in some ways. To top it off, they sewed it in place and he wasn’t prepared for that–very upsetting to him. His other two were held in place with a thing called a stat-lock that was like a clamp that stuck to his skin and was changed with each dressing change. I see the advantages to sewing it in place as putting it in and out of new stat-locks always resulted in the PICC line coming out little by little–not to mention less things sticking to his skin. SIGH, all in all a very exhausting and terrible day.
Today his spirits have been much improved and he has benefited from the Benedryl IV being out of his system and no more Tylenol 3–not to mention great company and supportive notes and bribes (Craig, you know who you are). It’s difficult to see how the PICC site is doing as it’s a pretty big mess as he bled a bit more than usual due to his platelets being low. We’ll be changing it tomorrow and will see how it looks.
We’re hoping to go home by Thursday, things are looking good for that so far!
It’s been awhile since my last update but it is late so I will try to do a quick catch up. We had planned on having a central line put into Michael’s chest as a permanent IV access for his TPN (nutrition) and blood draws on October 30th. A few weeks before that happened he was hospitalized with a blood infection–he was in the hospital for a week while they hit him hard with a lot of strong antibiotics to kill it off, it was not clearing up as hoped so they pulled his PICC line from his arm (a semi-permanent IV) and sent us home on oral antibiotics with the understanding we’d be having the chest one placed in two weeks. A week before the date we got a call from hematology in Cincinnati (they were also going to do a bone marrow tap while he was put under) and were told that there were concerns with putting him under for the procedure and concerns with his platelets. His GI was out of town so we waited until the following Tuesday before the Friday procedure and Dr. P said that both hematology and anesthesia had serious concerns about going forward with anything that involved putting him under anesthesia and the procedure was cancelled.
Along with his local hematologist we decided to watch his weight and see if we could try to manage without the TPN and if nothing else give him a break without the PICC line. It’s been over 6 weeks now and he has lost an average of a pound a week–a pound in a half in the last week. We saw Dr. P last week and he strongly felt that the PICC should be replaced (PICC lines do not require anesthesia so we can keep putting new ones in when needed every 4-6 months) and the TPN restarted. He felt that the poor nutrition was impacting his respiratory, his ability to fight off and recover from illness, energy, and etc. Michael was very upset about having to have to PICC put back on and understanding that this is something that he will need for the rest of his life. He’s had time to get more comfortable with the idea, but it is difficult for him to accept that this is a long term issue.
Tomorrow we’ll be having the PICC line put in again here in town and then will be admitted to the hospital to ease up to the amount he needs of TPN (we can’t just throw him back on what he was on before, the glucose and etc. can shock the system unless slowly increased). He could be in for up to five days from what we understand.
I know this is all pretty informational, I’ll post a more personal update soon!
After doing Katie’s senior pictures, I had to talk Michael into doing a few pictures for me. By talk, I mean bribe, bargain, and cajole him to let me do “a few” pictures. Joanna and I took him out and got more than a few and I’m thrilled with getting more than twenty of them!! I’ll add those to the portrait ones we did earlier this year and have a nice set of senior pictures for him. I’m not embarrassed to insist my children are gorgeous.
Okay, today is Monday, time to regroup and update where we are at. We are still at the hospital and have a “no earlier than Wednesday” leave date. Michael’s culture’s grow very slowly, so just when they think they are in the clear (and normally would be), the next day they start to grow something. His first three days of cultures grew bacteria, Friday’s we should know pretty well by tonight, which, of course, we are hoping are clear. Apparently bacteria can lurk a bit in corners of the heart and etc. and they want to be sure they have nailed any lurkers, so Wednesday is the earliest.
Of course, if any of the cultures grow anything new, then plan B would have to be enacted. It sounds to me like plan B would be pulling his PICC line and replacing it (although at that point I would discuss whether we could just pull it, be without it for a couple weeks until his central line goes in on the 30th–although they are talking about IV antibiotics at home so then we might as well have the PICC, we’ll see).
Michael is feeling better, no fever spikes, no hives, still a nasty cough but it doesn’t have the really nasty sound of last week. Those cultures (respiratory) are all in the clear so at this point its just a cold we are dealing with–one benefit of all these antibiotics for the blood infection is I doubt pneumonia even had a chance to grow in that climate! He is, understandably, tired of being here, tired of being in this room, and ready to go home. Still, as far as hospital rooms go, he has a pretty sweet set up with most of the comforts of home.
One interesting twist, we cringe at colds with him, for good reason. When we hear the sound of a cough shift we get a cold knot in the pit of our stomachs, especially now with his respiratory function so low. In this instance, though, having that (unrelated) cough was hugely important. Sepsis becomes incredibly dangerous if not caught in the first 24-48 hours, exponentially so for kid with compromised immune system. It takes a lot for Michael to have ANY kind of a fever so I doubt that he ever would have gotten a blood culture done that soon if it hadn’t been for us going in Monday for an x-ray of his lungs. Conversely, without the blood infection he probably would not be nailed with high powered antibiotics which have certainly kept pneumonia away. Just saying. Things happen for a reason and him having two unrelated issues whose treatments interplayed to benefit the other ends up being a good thing—-though obviously having neither would be best!
We had our hopes raised and then dashed today 
and for an hour or two we thought we would be heading home today. Michael had a rough night last night with coughing, a splurt of hives and a dash of mild fever, and being up all night going to the bathroom from the TPN. This morning his hem (blood doctor) came in and said that nothing had grown and they believed the first culture must have been contaminated and that she was comfortable with him going home. I talked to the nurse as I felt uneasy as *something* was obviously up for him to be doing any kind of fever and his coughing was really awful although everyone agreed it was keeping stuff up and out of his lungs. We hadn’t met with his pulmonologist yesterday so she called and he stopped over to see Michael. He said his lungs sounded good for him and, like the hem, was comfortable sending him home after they did a different culture way down his nose into his throat and that he would call something in tomorrow if any infection was running around there. I still felt uneasy about it but I understand it is important for him to *not* be in the hospital if at all possible so as not to catch something there, so I told Michael that it looked like he would be going home. He was half asleep and said he didn’t want to go home (a first time in 19 years), shocked, I asked him why, and he said he was too sick to go home and went back to sleep. Ten minutes later, his hem Dr. zipped in and said, “Um, sorry, but you aren’t going anywhere.” Apparently Michael knows his own body, the culture from yesterday (which was *after* he was nailed with some heavy hitting antibiotics) had grown more than one type of germ/bacteria/infection whatever you will.
So they did a culture down his nose and into his throat for his lung doctor, we’ll see what that shows tomorrow on that end. The plan is to hit him again with the “big gun” antibiotic that they hit him with Monday and changed up the secondary antibiotic to one they hope is more effective killing the bacteria. As I understand it, the goal is to hit hard and knock it out of his system before it can overwhelm his system and put his body in shock (sepsis). His nurse said not to expect to go home until sometime next week, so we’ll just take it a day at a time.
Hopefully he gets more sleep tonight since it isn’t a TPN night (which makes him go to the bathroom all night), we could use a good night sleep!
Michael has had a cold that got nasty over the weekend, and then on Monday he ran a slight temperature, which with his immune issues is something to pay attention to. We went in to see his hematologist because his lung doctor was very busy to get an x-ray. She went ahead and cultured his throat, nose, and blood and we went home to wait as his x-ray didn’t look too bad. Yesterday morning (Tuesday) his doctor called and said his blood culture was positive for an infection in the blood and that we needed to come in. The cultures take a range of 24 hours to 4 days for full growth so they didn’t have a particular strain or known sensitivity (which antibiotic would best kill it) but they didn’t want to wait that long. He came in and they hit him with two antibiotics, one of which is a “big gun”–which also started some hives, but they caught them quickly. Right now he is on a second antibiotic every 8 hours while they find out more from the first culture and start to get results from the second culture they did yesterday and they took another early this morning. His fever was up last night and he had a bit of a rough patch but he was feeling better around 10 or 11pm and he slept pretty good (other than the normal getting woke up every couple hours being checked) last night with some medication.
His fever is down this morning, though his heart rate was up a bit and he is tired and has some pain around his left rib cage. So we’re just in a “wait and see” mode at the moment as we wait for test results.
We went to Cincinnati just for a quick visit for Dr. P (his GI) to see him as he will be out of town 3 weeks next month so it would be too long between checkups–they usually try to coordinate visits with other doctors. His weight is holding well on the same TPN volume doing it every other day, so that is great news as we really like having “off nights” with no hookup. His potassium was low so he is going to adjust that and retest after a couple weeks. We also discussed the central line as Michael’s local hematologist who will be coordinating all his care now was 100% for the central line. He recommended Dr. Ryckman there because he is the doctor who closed up an old, scarred up feeding tube site this year–we really liked him and found him very gracious and compassionate. Dr. P said he has had recent conversations with Dr. R about the central line and even about his rib cage (Dr. R is a general and thoracic surgeon), so I contacted their office today to get the ball rolling on that. We will be having a BMB done at the same time to make use of the sedation as obviously we want to sedate him as little as possible.
His bloodwork from Monday continued its ever creeping down:
Platelets: 24,000 (all time low I believe, again, just watching for now)
White Cells: 1.8 (this is low for him on GCSF)
ANC: 1200 (above 1000, so just going to watch the next one)
HGB: 10.5 (will transfuse under 10)
I will post when we have a date on the central line/BMB, other than that, I believe our next trip will be in November to see Dr. H and Dr. P and meet up with Sue, Dee, and Clarke from New Orleans 
We’re back home now from our trip to Cincinnati after seeing Dr. P (GI) and Dr. H (SDS, hem).
GI: Michael weighed 83.5 pounds, 13.5 pounds up from the start of TPN, fantastic! They don’t want him to put on anymore weight though, and want to find a dosage that holds him steady between 81.5-84 pounds (he is 4′8″ tall). We discussed the fact that Michael obviously cannot go back to no nutritional support or he will simply go back to the way he was pre-TPN. We have to make a long term decision in whether to keep him on TPN and place a central line (same as his PICC, but in his chest–more permanent) OR place a J G tube in his stomach that will pass through the stomach into the small intestine for formula feedings. There are pros and cons for both and we are currently leaning towards the central line due to access for blood draws/IVs and etc. but we are still weighing the options. Either one will have to be placed under sedation, but we are hoping to do his bone marrow tap at the same time and maximize the sedation–we’re waiting to hear back about that. For now, they are going to give him the same mix of TPN every other day to see if that will maintain his weight–it may go to 5x a week if he starts loosing again.
HEM: There is not much to do at this point except watch and deal with each situation as it comes. His bone marrow is obviously declining but without the possibility of doing a bone marrow transfusion we will have to support with regular transfusions when the time comes. His white cells are staying steady with his every other day GCSF shots, he is currently at a dose of 5mcg per kilo, which is acceptable and gives us some wiggle room to increase if his white cells start dropping more or he gets more infections. When you get close to 15mcg per kilo you are running a risk of triggering leukemia. His red blood cells can be transfused when they need to, not getting close on that yet. His platelets are the ones that are steadily dropping, Mondays results being 26,000 and 27,000 (Toledo labs) the week before. They were at 34,000 on Tuesday in Cinci which, while better than 26,000, are still the lowest since Sept. taken in Cinci, so the downward trend is quite obvious. Dr. Harris wants to hold off giving a platelet transfusion as long as possible as a) the more transfusions you get the higher risk of reaction and b) the more transfusions you get the higher the likelihood the body stops reacting to them. Because Michael isn’t running around playing sports, they would like to try and let it go down to 10,000 before transfusing but will start watching for a trigger point under 20,000.
So the goal both nutritionally and hematologically is to keep him maximized as best as possible and watch and wait–and I really don’t do waiting well. It was discouraging to be in Cincinnati without a positive goal to shoot for and to be shifting from proactive to supportive. I’ll be setting up consultations with all his local doctors next week to get everyone on the same page and get input from them as well as to where to go from here.
We’re in Cincinnati tonight for a couple appointments and a get-together. We’ll be seeing Dr. H (hematologist) and Dr. P (GI) to figure out a plan for maximizing Michael’s health. Michael has his blood drawn every week to keep his TPN (IV nutrition) regulated and while we were on the way here the nursing company called me. His platelets had come back at 26,000 which is an all time low for him, she was concerned and wanted to make sure a doctor was made aware. I let her know we’d be seeing doctors tomorrow and she emailed me the counts flagged:
WBC 3.1 LOW (this isn’t too bad for him actually)RBC 3.41 LOWHGB 11.5 LOWHEMATOCRIT 32.9 LOWPLATELET COUNT 26 LOWRDW 15.2 HIGHABSOLUTE LYMPHOCYTES 0.6 LOW
We’ll go over it all with Dr. H tomorrow and see what needs to be done. Obviously we have to address his reaction to the platelets on the surgery day as he is going to need a transfusion at some point–this is one of the more immediate things that need to be figured out. I’ll post more tomorrow night.
Quite a few people in our SDS group are in town for appointments so there is a get-together tomorrow night of around 7 families, so we’re looking forward to that!
