picc line infusion

Our new routine for infusing Zander (via his picc line) is quick and easy. We usually try to do it while he is sleeping (before Travis leaves for work). Even if he wakes up he is a pretty happy camper (as long as he is involved). Here are some pictures from this mornings infusion.












check the line, flush it with saline, check for blood return.












just waking up













help mix the factor


















helping push the factor












get every drop!













saline flush













and done!!

Ramblings of The Mom

My daughters have done a fabulous job of creating this blog for those who have the courage to follow it. While I have kept up with the blog, I have been somewhat reluctant to post, but the past week has reminded me of my own journey that began in 1973 when I married the Grand Pooba Hemophiliac (also known as Grandpa Rod). At that time in Montana there was no doctor or medical facility personnel that really knew anything about this crazy bleeding disorder, so it was on the family and more specifically Grandpa Rod to learn about and manage this condition. The treatment product and approach to treatment was okay for the time. We carried our own factor to the ER on many occasions before we could infuse at home. Every bleed was a trip to the ER where we mixed the factor, administered the factor, helped ourselves to a few rolls of paper tape, got a shot of morphine and headed home. Lots of memories of trying to get home before the full effect of the morphine set in. Imagine me in Montana in the middle of winter trying to get a drug induced, wheelchair ridin' husband through 30 inches of snow, into a van with no power lift to a safe place before he started singing "Dead Skunk". The nurses put us in a room and left us alone, because they knew nothing about the process. I knew we were in trouble when I saw two or three nurses gathered with the factor "directions" trying to figure out what to do with the two little bottles of "stuff". This is not an indictment of the nursing profession, because we had our share of terrific nurses, but rather an acknowledgment that hemophilia was not often seen or treated, so Grandpa Rod became the teacher of many.

The HTC at Doernbecher provides a different perspective on the treatment for hemophilia. The personnel is trained and the doctors are knowledgeable, fabulous and caring. The treatment for my 3 grandsons is superior to the treatment of my husband's era. He used to say, "someday, kids with hemophilia will probably just take a pill or something instead of all the infusions". Clearly, research hasn't progressed quite that far, but with the regular infusions (prophy), these kids are light years ahead of their grandpa.

Bright future aside, it was a little eery to be in the hospital room with one of my sweet grandbabies and his parents last week. Mom and dad were just trying to soak up the required learning to assist their son--asking every question and filling their parent library with information that will be recalled for future events. There is a little twinge of sadness, as I would prefer my family not have this experience. I suppose it would have been much easier not to have had children -- that would have been the solution for some -- but we would have missed out on the fantastic journey that has come with four daughters and subsequently 13 grandchildren. I would have been a total loner in that scenario. I mean, I'm real real fun, but even I would have grown weary of being around me!

I love this family and let's face it, for some the challenges are not health related, but all have their own challenges to bear. At least, we were somewhat prepared for this potential and thanks to research and medical advances the path is much more sophisticated than it was in 1950 (that sounds like a real long time ago and I don't want anyone to infer my age based on that number) and I look forward to the role of grandparent with this generation.

I love freedom

I infused Zander while he slept today....FABULOUS!

... and nothing happened

We went to the fountains.  Maybe you are thinking "A nubie walking hemophiliac trying to keep his balance on wet pavement.  Don't do it lady.  Don't do it."

Harris did a downward dog right onto his forehead (actually, that might be a totally different yoga position).  Contrary to my previous assertions about "fountains are better than pools because no one can drown", he nearly drowned because downward dog and standing water apparently aren't good friends.  Also, he scratched up his head.  Nothing happened.

Same fountain, different day, he did what my family refers to as a "belly whomp" on the concrete and then sort of rolled up onto one shoulder and the side of his face.  He looked like one of those slow-mo shots that they use on that show "Wipe Out".  (Just for the record - I hate that show, and only watch it when my husband has Hulu held hostage.  I mean how many times can you watch people get hurled into the mud by a Wylie Coyote inspired punching arm and still think it's funny? Seriously people.)  Nothing happened.

We have been through a fall down the stairs (it was a slow roll), immunizations, falling, tripping, banging, pulling chairs onto our chests, being lifted by our 4 year old brother in a rather unconventional fashion, an ingrown toenail and a chest x-ray because Harris may or may not have swallowed a Lego guy's head.  (Waste of an x-ray, as it turned out) and we've only had one trauma related infusion so far.  Piano leg - one, Harris' head- zero.

Mostly, so far, "nothing" happens a lot more than "something"... which would make for a bloody boring episode of Wipeout, but a pretty bloody good life.

Done and Done

We are home from the hospital!! We went in yesterday morning to get Zander's picc line placed. When we got there the doctor and nurses were a little concerned at the swelling in his right arm. They told us that he might need to be admitted, so he could receive proper care. I could only think about one thing at a time and first I wanted to get the picc line in him and then we could worry about his right arm bleed.

The actual procedure was so quick, maybe 30 minutes. When we went back to see him he was still out (probably getting the best sleep of his life). They did a chest x-ray to check the picc and it was in a perfect spot.

Then the excitement started. The picc nurse went to change the dressing and it was soaked with blood. The hematologist came in and said just make sure it is getting lots of pressure. I started to get worried as I watched them try and get the bleeding to stop, I mean it was like watching a movie, this didn't work, let's try this....this went on for a while. Many doctors and nurses were coming and going and there was a lot of quiet talking. By the time Zander's bleeding was under control he had lost a lot of blood, when the doctor said, "he needs a blood transfusion" I got sick.


Here is what happened, even with the massive doses of factor before and after the procedure, the inhibitor was doing what an inhibitor does, it eats up the factor. Further more, he is having a pretty good bleed in his arm. There is only so much the factor can do, there were just too many issues going on. Zander was admitted to the hospital and was getting his special factor (Novo 7) every two hours. At one point they also gave him a HUGE does of his regular factor(Advate), trying to trick that stinking inhibitor.


This morning his numbers all were looking better (the blood transfusion did its job) and we drew blood (ironic right) for more tests. The tests came back that the inhibitor was down, and his clotting factor was up. WE DID IT! We fooled the inhibitor.


Now, we are doing that massive doses of Advate daily. With the picc line we are able to do that at home. We still will have to go to the center to have the dressing changed and to have a lot of labs done to monitor factor level and the inhibitor.


Zander was back to himself this morning, getting into everything in his hospital room and was thrilled to leave. His arm that started all this is covered in a pretty impressive bruise, reminiscent of my dad's bruises.

Here are our hospital chronicles.

my, what large biceps you have!

This morning I noticed Zander's right arm feeling a little swollen. When I went to spend some time with Awny, I asked her to take a look and she said it definitely looks bigger than his left arm and that is was very warm to the touch. We measured it was about an inch and a half bigger than his other arm. After monitoring it throughout the morning, I called the HTC and they scheduled him to come in. We saw our nurses (I can tell how concerned they are for Zander) and a hematologist, everyone agreed he was having a muscle bleed. The best we can gather is maybe on Monday during his blood draw his tourniquet was too tight or maybe we held him down wrong, whatever it was, he has a bleed.
The tricky part is, he is needing to use a different medication and we had not received it so after jumping through some hoops and thanks to a lot of great nurses we got it. Our plan was to get an IV in today and give him a dose today and one tomorrow. We need to see how he responds to this need medication and how his inhibitor will affect his treatments.
Sadly, we poked and poked and poked (Travis and I joked it is a lot of pokes when you loose track of how many you ended with) we think it was 7 pokes it total. We did not end up with an IV, we decided it was time to just get the factor in. We accessed a vein in his head and that was that.
The plan for tomorrow is to get a picc line put in and that way we will be able to give him his fancy new factor and he wont have to get all those stinkin' sticks.
There is a chance that there wont be a picc team available for us but we have our sedation time all set up. I am learning quickly that we have to roll with the punches, sometimes we put a plan in place and something like this happens, it's all about being flexible and making sure we do what is best for our sweet little man.
I will post tomorrow and of course have pictures of Z's new medical accessory.

Another adventure

The last couple of weeks prophy has been rough, more than one poke and a grouchy kid when it is all said and done. Last week, after two pokes we were going to stop and one nurse said maybe we should go to the lab and they can do his infusion (they are pretty amazing when it comes to hitting little veins) they said in order for the lab to administer factor they have to do a lab (is that why they call it that?). We decided to test for an inhibitor. It has been a few months since we did his first test (came back negative) and Zander has had more than 30 doses of factor so it is a slim chance, but that was the plan.
Last week came and went, no word on the inhibitor test. I asked Travis if he thought we were going by the rule, no news is good news. Then when we went in for prophy today things seemed off. Harris went back first (as always) and not long after they came out and said they were going to take an inhibitor test on Harris. Some warning lights went off in my head, I didn't think it seemed right that Harris was being tested. Then we went back for our turn, they wanted to do Zander's vitals, I said, "no, we did that last week." The nurse explained we were seeing a Dr. and we needed to do vitals. When we walked into the room I said to Travis, he has an inhibitor. Why else would we be meeting with a doctor.
Our regular nurses came in (looking like someone just hit their dog) and Travis said, "spill it." They hemmed and hawed and said the doctor would be in to talk to us but that yes, Zander's inhibitor test came back positive.
My first instinct was to cry, it was overwhelming, but what good was crying going to do. I took a breath and asked if I could get a pen and some paper so I could take notes. My mind was racing a thousand miles a minute. Questions were swirling around.
When the doctor came in we talked a lot, we learned that his inhibitor is low, 1.9 Bethesda units, (high is more than 5 BU's) That is a good thing. An inhibitor happens in 1/3 of boys with hemophilia. Zander's body is attacking the protein that we are putting into his body with prophy, the body sees those proteins as foreign. The remedy is doing super doses of factor, they have seen kids overcome inhibitors in as little as three months. It would probably mean factor daily, which also brings up the question of accessing his veins. It isn't likely we would be able to hit a vein everyday, they are just too small. We may have to get a port or a pic line. Lots of questions and lots of answers to come.
Today he was retested (to see where his levels are) and we have another appointment to discuss our game plan. I am sure I will be posting often to update you (whoever you are) on this new adventure.

victory

I just wanted to write an update on Zander's immunization debacle. When I got billed for our co-pay to the ER, I felt like I shouldn't have to pay it because it was an immunization administered the wrong way that landed us in the emergency room. So, I called Kaiser and asked how I go about appealing the charge. The man I spoke with was nice and told me I would get some paperwork in the mail and then I would be assigned a case manager and it is a fairly lengthy process. I filled out the paper work (this was at the beginning of June) and today the 13th of July I heard back from Kaiser. Here is the best part of the letter.





I know I won't win all my fights with insurance companies but right now I am 1 for 1.