An explanation of the PICC line

And so begins two long weeks of IV antibiotics. I just keep telling myself that at least we're doing it from home. This morning was particularly tough, given that we don't usually roll out of bed until about 8, but this morning had to get up at 6 to get Drew started on his antibiotics. We also had to do his breathing treatments and airway clearance done at the same time because we had to leave the house by 8:30 with everyone dressed and fed to get Ella to school on time. We had it all together, or so I thought. Everyone was cranky from being woken up and no one wanted to cooperate. Regardless, we made it out the door at 8:30 on the nose and had Ella to school at 8:45, the same time when Drew lost his breakfast. I don't know if it was an upset stomach because of the antibiotics, or to much to eat this morning, or car sickness, or a combination of all of the above, but it wasn't pretty. Ella was crying that she didn't want to go into school and the teacher basically grabbed her and rushed her in the door so I could take care of Drew. I had nothing in the car to clean him up with but some wipes, and prego mama can't handle the smell of puke (non-prego mama doesn't do so well with it either). So poor puke covered Drew, Lily and I headed home for some marathon carseat cleaning. Cleaning puke out of a carseat is high on my list of the worst things in the world to have to do. And when it has to happen quickly so its all washed and dried and put back together in time to go pick Ella up, it just adds to the fun. And the poor thing can't go in the bathtub with the PICC line in, so I just had to do a wipe down as best I could. I was able to put the twins down for a little nap (which they don't usually take in the morning anymore, but given they got up so early, they were beat) and get the car all cleaned up too. Then I had a popsicle and felt bad for myself for 5 minutes. That's how my morning has been so far.

I wanted to post some information on PICCs because I don't think a lot of people understand what they are. PICC stands for Peripherally Inserted Central Catheter. Here's what I found online about them:

A PICC is a long, slender, small, flexible tube that is inserted into a peripheral vein, typically in the upper arm, and advanced until the catheter tip terminates in a large vein in the chest near the heart to obtain intravenous access. It is similar to other central lines as it terminates into a large vessel near the heart. However, unlike other central lines, its point of entry is from the periphery of the body the extremities. And typically the upper arm is the area of choice.

A PICC line provides the best of both worlds concerning venous access. Similar to a standard IV, it is inserted in the arm, and usually in the upper arm under the benefits of ultrasound visualization. Also, PICCs differ from peripheral IV access but similar to central lines in that a PICCs termination point is centrally located in the body allowing for treatment that could not be obtained from standard periphery IV access. In addition, PICC insertions are less invasive, have decreased complication risk associated with them, and remain for a much longer duration than other central or periphery access devices.

Using ultrasound technology to visualize a deep, large vessel in the upper arm, the PICC catheter is inserted by a specially trained and certified PICC nurse specialist. Post insertion at the bedside, a chest x-ray is obtained to confirm ideal placement.

http://picclinenursing.com/picc_why.html

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And here's a picture of where the PICC goes

And this is what it looks like on Drew's arm (this isn't his arm, but he has the same set up). When we administer the antibiotics, we just hook a tube up to this little blue port. There are no needles, it just screws in. We have to scrub it with antibacterial stuff and wear gloves and make sure everything is super clean/sterile to change the antibiotics the several times a day that we do it. It doesn't hurt him at all. He doesn't like getting the PICC line because that does hurt a little and he is restrained for it, but once its in, it doesn't hurt him at all to get his treatments.

So there you have it. Let me know if you have any questions!

This is your life.

Our big news is public. We're expecting baby #4 on or around March 21, 2012 and I'm excited. There are lots of questions I've gotten since I started telling people - are you nervous? will you get the baby tested for CF before they are born? will you find out the sex? The answers are no and yes, no, and no. I'm not terribly nervous. Of course I hope and pray that this baby will be born without Cystic Fibrosis and there is a 75% chance of that. The baby could be tested via DNA test done by amnio while in my belly, but it wouldn't change anything for us so we're not having it tested until its born. Honestly, there is nothing that they can do while I'm pregnant if they found out that the baby has CF, so there is no reason for the risks associated with an amnio. We also will not find out the sex. We didn't find out with Ella and I loved the surprise. We only found out with the twins by accident and having had kids both knowing and not knowing, I strongly prefer the not knowing. To each his own, but for us, the surprise in the delivery room is worth the wait. We're excited, and I hope everyone who reads this is equally as excited for us.

I was on a conference call last week with the the Public Policy team of the CFF and the other State Advocacy Chairs to discuss how to expand our reach and have a greater impact on government. I told Martin after the call that I just don't understand how all parents and friends and relatives aren't as involved in this as they can be. Congress isn't just going to make these big decisions willy nilly without hearing from you and I what is important and why its so important. I encourage and urge all of you to join the CF advocacy program, sending letters to your local decision makers that will affect and improve the quality of life for kids like Drew.

Tomorrow we have to go into the hospital for the day to get a PICC line to start IV antibiotics again for 2 weeks. Drew had Pseudomonas on his last culture, but the last time that he grew that before this was almost 6 months ago. Therefore they're not considering him colonized and cycling him on Tobi. Either the Pseudomonas went away and came back, or it had been suppressed so well by the last round of IV antibiotics that it wasn't showing up on his cultures or causing any problems. Either way, because it was gone for more than 8 weeks, they're making another attempt at eradication with these IV antibiotics. When talking with our doctor, she mentioned that in talking with several other doctors in our practice that there is some new evidence to show that when trying to eradicate Pseudomonas, a two week course of IV antibiotics followed by 2 cycles of Tobi (28 days of inhaled antibiotics) seemed to show more promising results. We're not certain that we're going that route yet, but should know by the end of the week. As for tomorrow, Martin will be taking Drew because the IV is done under floroscopy (live x-ray) and prego mama can't be in there with him. Also, tomorrow is Ella's first day of preschool and I simply can't miss it :)

If I sparked your interest in writing your members of congress, here's what I recently sent to mine. Feel free to copy, paste and add your own two cents. It will make a difference, I promise it will! This is my passion.

While I understand the challenges you face in reducing the deficit and balancing the budget, cystic fibrosis patients need your help to ensure that NIH can fund research that can produce new treatments, and the FDA can swiftly and thoroughly review them. These critical agencies must be able to move new therapies quickly from bench to bedside.


Drew looks healthy on the outside, but he is fighting a progressive and fatal disease. Everyday, Drew spends between 2 and 3 hours doing airway clearance and breathing treatments to keep his lungs healthy and clear of mucus. We have cabinets full of medication that Drew must take on a daily basis. The best part about Drew is that he does it all with a smile on his face. We are learning more every day, and, through Drew, we've discovered a new world of support, education, and opportunity for those living with Cystic Fibrosis. Unfortunately, it does not change the fact that there is currently no cure. We can change that!

This year has been an amazing one for the entire CF community. The money that WE have raised over the past many years helped to develop the first drug to target the basic defect of Cystic Fibrosis. That drug, VX-770, will be going to the FDA for approval late this year. I can only imagine how different our lives will be with this drug and the others in the pipeline. I can only imagine my baby healthy, without Cystic Fibrosis. We are so close to a cure. It's almost here, but we still need your help.

Strong, sustained NIH funding is essential to national priorities of better health and economic vitalization.

These important agencies contribute to the nation's economic strength by creating skilled, high-paying jobs. In 2007, NIH grants and contracts created and supported more than 350,000 jobs across the United States. A well-funded FDA can help ensure promising research is translated into new cures and reduce the cost of health care on the American economy and for American families.

Once again I urge you to actively support robust funding for NIH and FDA in Fiscal Years 2011 and 2012 and help us create better tomorrows for those with cystic fibrosis and all rare diseases.

Thank you again for your time!

Quatro

I'm reusing a blog post that I wrote several months ago, with a little to add onto the end. Happy reading!

Right after Drew was diagnosed with CF, I immediately felt 100,000 different feelings. You're scared because you don't know what it is. You're sad because your baby is sick. You're guilty, knowing that this genetic disease was passed onto your baby through you, and you wish more than anything that you could take that away. But then you look at your beautiful little being and can't help but be overwhelmed by love. As much as I hate everything about CF and Drew having it, it has changed me in ways I couldn't have ever imagined. It has made me more patient. It has opened my mind. It has taught me not to judge others unless I have walked in their shoes because you never really know the situation that you are passing judgement on.

ANYWAY, I always wanted a big family. I just love everything about being a mom, from the hugs and kisses right down to the snotty noses and potty training messes. Not that there was every a solid plan put in place, but we had discussed it from time to time and thought that the twins probably weren't going to be the last of the babies to pass through this house. Its scary to think that if we do have another baby at some point that they too could be affected by CF. I've thought a lot about it. I get upset from time to time thinking about it because on one hand I think I couldn't possibly do this with another kid. Its hard with one. Not the hours of treatments and doctors appointments, I could find time for more of that if I needed to. I'm talking about emotionally and mentally hard. No one likes seeing their kid sick, and I believe that its 1,000,000 times harder when its completely out of your control, as in no cure. But then I get even more upset thinking about the possibility of not having any more kids. I'm torn and I think about it all the time.

Martin and I have talked about it, and he said that he won't let fear be the reason that we wouldn't have more kids. And as more and more time goes on and I'm more angry at the disease than afraid of it, I agree. If I let fear win, then CF wins, and CF isn't going to win in this house.

But here's what got me. In the post I read, someone commented this: " I don't have CF and I don't think it's fair to knowingly put that on my child." I did not put this on Drew. It sucks that he has CF, and it sucks that its genetic and it is passed from parent to child, knowingly or unknowingly, but I did not put this on him. Another woman said "What burns my biscuits is when people call us names for deciding to go ahead and have children. I've read posts from those who call us uninformed, selfish, negligent and hateful. One nurse even commented that it should be illegal to procreate! What?! " Exactly!! WHAT?! I will tell you that the people who think that we're "putting this on the kids" or who think we are "uninformed, selfish, negligent and hateful" are actually the ones who are uninformed, selfish, negligent and hateful. These are probably the same people who would choose to have an abortion if they would learn that something wasn't right during their pregnancy, and I'm the one being called selfish. That baby, any baby, needs the love of two people more than anything in the world, from the first moment it becomes a being. Parents who are blessed with a child with any sort of disability, no matter how mild or severe, are the strong ones. We are the knowledgeable ones. We are not selfish, in any capacity. We give our everything, everyday, so that our kids can be whatever it is they are here to be. Andhateful? How dare you!! How DARE you call me hateful for wanting to have more children. I am the opposite of hateful. I have more love to give that you in the simple fact that I want more of these wonderful beings. I have the most wonderful gift that I can give the world and its called children. I know that there is a 25% chance that any of my children will be affected by CF but that doesn't make them any more or less worthy of existance. I know a LOT about the disease. And I participate in the search for the cure, so that God forbid one of your children ever be affected by something so horrible, there may be more help, more answers, a cure. Maybe my son will go on to find that cure.

I wanted to finally make the announcement that our hearts will be even more full next spring when we welcome baby #4, or Quatro and he/she is currently being called. There is a 25% chance that this baby will have CF. There is a 75% chance that they will not. We're praying for the latter, but will take whatever we get!

Ugh

The culture came back positive with pseudomonas. We're getting a PICC line on Tuesday to start IV antibiotics.

Latest Clinic Visit

We had a routine clinic visit on Monday morning and it went pretty well. We're still waiting on the results of his culture and are hoping it comes back clean. Two other things that came from the appointment was scheduling a hi-res CT scan and a discussion about whether to start on Azithromycin three days a week.

First, the CT. We had talked about it with our doctors before and decided that now was a good a time as any to get it done. Our clinic has started to talk more about this procedure recently, as some studies have shown that a hi-res CT scan can show bronchiectasis early on better than any other test and medicines and therapies can be adjusted to try to better clear the airways preventing infection and further bronchiectasis. Bronchoscopy is good at looking at mucus plugging and other airway abnormalities, but can't see into the smaller, lower airways where lung damage starts. The hi-res CT is useful over time to see how lung disease progresses. I hate talking about lung disease in my 1 year old, but with Cystic Fibrosis, its just the reality. Anyway, his doctor said that she suspects that they will see some lung disease just because of his inability to appropriately clear secretions. She said that some kids have it at this age and some don't, but if she had to guess, she would suspect that he has some damage. The procedure is non-invasive, but he does have to get put under for it. We have it scheduled for the end of the month. I won't worry about it until a little later on :)

The other thing that we discussed at this clinic visit was putting Drew on regular Azithromycin. Azithromycin is an antibiotic (you may know it as a z-pak) used to treat common bacterial infections. But it wouldn't be used as an antibiotic in Drew's case. Here's some information about this from the CFF website:

Azithromycin is a commonly used antibiotic made by Pfizer, Inc. and sold as Zithromax^®. Many people have taken this drug to treat pneumonia, sore throats or ear infections.

In 1999, the Cystic Fibrosis Foundation recognized that azithromycin might help people with cystic fibrosis (CF) who have a positive sputum culture for Pseudomonas aeruginosa(Pseudomonas) and designed a clinical trial to test that possibility.

During the first clinical trial, lung function (FEV₁), weight, use of antibiotics, and days spent in the hospital to treat lung infections were watched for changes. The people with CF who were involved in the study were split into two groups. One group took a placebo—an inactive pill that has no treatment value (sometimes called a sugar pill). The other group took azithromycin.

During this trial, neither the patients nor the CF researchers knew which patients took azithromycin or placebo pills. Over six months, the group that took the azithromycin had about a 6 percent improvement in their lung function, fewer pulmonary exacerbations, took fewer antibiotics (other than azithromycin) and had an increase in their weight. They also spent 47 percent fewer days in the hospital for the treatment of a lung infection.

In the second study, the kids with CF did not have Pseudomonas for at least one year, were 6 to 18 years of age, and had mild to moderate lung disease, although most had mild lung disease.

Over six months, the group that took the azithromycin had fewer exacerbations, took fewer oral antibiotics and had an increase in their weight.

Lung function was about the same in those who took placebo and those who took azithromycin. However, the people in the azithromycin group reported less coughing than the group taking the placebo.

That sounds like reasonably good evidence for me to get him on Azithromycin. My only hesitation is that it then takes Azithromycin out of the running as an antibiotic when he does get a lung infection, and Azithromycin is something that we've had reasonable success with when we needed to use it in the past. If Drew would be on Azithromycin 3 days a week ongoing, his body will build up a resistance to its antibiotic effects, thus eliminating it as an effective treatment option for us. There are plenty of other antibiotics that he can get put on if he gets sick, but hes on so many antibiotics all the time that there is always a fear of his body building up resistance and us not having the options we need to treat his infections.

We decided to see what we see in the CT and then make a decision on the Azithromycin. If he does have reasonable lung disease and bronchiectasis then we will more likely than not go ahead and get him on it. Maybe if he looks good too, I'm just not sure yet.

Cross your fingers and say a prayer for a good culture report!

My Meeting

Today was the big day and it went awesome! At the last minute, I got a call from the Congresswoman's district scheduler and something had come up with the Congresswoman and she wasn't going to be able to make it to our meeting, but I was given the option of meeting with her staff. Definitely not the option I wanted, but it was a foot in the door, and it turned out to be spectacular!

I met Lisa Burns (Drew's doc) about 15 minutes beforehand to briefly go over what I wanted to discuss. I had told her that I was nervous. I like to think of myself as well educated and knowledgeable in many areas, however government is not one of those areas. When I had been discussing this with Martin, he told me to just keep in mind that she works for us, that we are her constituents and she represents us in Congress. Good point Martin Moore, good point. So I went in and we met with the Deputy District Director named Sara. I told her a little bit about Drew and our CF journey before going into the nuts and bolts of why I was there. She was a really nice lady who seemed very interested in what we were talking about, asking lots of questions and seeming to genuinely care about our discussion. She mentioned that she hadn't know much about CF until recently when a close friend had a baby who was born with it. It was as if the meeting with the Congresswoman was supposed to fall through so we could meet with this lady. I mean couldn't have worked out more perfectly.

I told her that Cystic Fibrosis isn't federally funded, and that I wasn't there asking for their support to get funding (though should they choose to, I'd be okay with that). What I wanted was support in getting the treatments through the FDA for approval, and the way she could help was to support budget cuts that DO NOT affect the FDA. Additionally, on matters concerning the NIH (National Institute of Health), support funding and oppose all cuts, as budget cuts to the NIH could cause the life saving research that's going on in places like Cincinnati to come to a halt. There is a group in Congress know as the Congressional CF Caucus and I said that if I had 1 action item for the Congresswoman it would be to ask for her to join this group. This group of representatives fights for the things listed above, and to have Congresswoman Schmidt as a part of that group would be huge. A large portion of her Congressional District utilized the services offered by Cincinnati Children's Hospital, one of the leading CF research hospitals in the country. And I'm sure that a fair number of the employees of the hospital live within her district as well. To have her fighting for a cure with us would be huge, and I'll write and educate and inform until we get there.

It really was a great meeting and I'm glad that I was able to set it up and go through with it. I was definitely nervous going in, but after the meeting, I realized that if I'm not fighting for Drew's life, no one will. I need to be his voice and I need to fight for what he needs, for what we need. I very briefly touched on Medicaid, simply noting that my husband has a great job and we have great insurance, but without our secondary insurance for Drew, Medicaid, we would be paying something like $74,000 a year, just for medications!!! Actually, we probably wouldn't be because we don't have an extra $74,000 laying around (I know, surprising) and what would happen is he would have to go without treatments and his health would deteriorate. And my heart would break at the same time. I asked for her support in preserving programs like Ohio's Bureau for Children with Medical Handicaps (Medicaid) for children and adults with CF so that they can receive the treatments they need to live the life they deserve.

I left the meeting and let Sara know that I would still love the opportunity to meet with Congresswoman Schmidt, and hopefully that request will come to fruition at some point. But for now I'm happy with what I accomplished.