Pregnancy Outcomes In Women With CF

Someone posted this in a CF mothers group I am in and thought it was great info to pass on especially to women with CF who may be thinking about becoming pregnant. It discusses the outcomes of pregnancy in women with CF. As women (and men) with CF are living longer and healthier lives we are seeing more and more CFers becoming parents and I am glad there is some data being collected on this subject.

http://www.cysticfibrosisnews.com/effect-of-pregnancy-on-cf/?utm_source=All&utm_campaign=CF-January+28%2C+2014&utm_medium=email

Port Problems

My hospital stay was only supposed to be four days maximum, but due to many issues including a long holiday weekend my departure kept getting delayed. Tuesday morning, when my insurance company office was open again, I was given the approval to get home IVs and was preparing to go home. All of the sudden a doctor walks in (during my treatments) to talk to me. He started explaining how a port is inserted and that ideally a port tubing ends in the superior vena cava, just upstream of the right atrium (at this point I am wondering why the heck he is telling me this since I got my port 2 months ago). He then went on to tell me that mine was significantly lower in a place that could cause life threatening problems. 

Needless to say, I was speechless. I have had the port for two months with no mention of any issues. I also did not understand how radiology, who used imaging to place my port, could have placed it too deep. The doctor told me that if I choose to have the port removed and then put back in they would try to get me in as soon as possible, but that it would delay my discharge. My head was spinning! Here I was getting all ready to go home and suddenly I am facing an unexpected surgery. 

I started processing out loud to the doctor (resident?). I don't understand how two months passed without any mention of this issue. Why did radiology not see this when they did imaging after the placement? The doctor (not my CF doc) explained that the pharmacy looks at all x-rays of port and PICC placements before issuing home IVs and they are the ones that noticed the port placement issue and refused to send IVs since they deemed using the port as unsafe.

I was now even more confused at this point! What x-ray did they get this info from? The doc left to investigate more. He came back and said they were send X-rays from an outside facility. Now I was baffled! I have not had any x-rays since 2008 (all in the hospital) and my port was placed 2 months ago so what x-ray could they possibly be looking at. The RT in the room pulled up my x-ray history. I asked him to check my last x-ray and to see if I had a PICC placement in that x-ray. Sure enough, my last x-ray was 2008 and I did have a PICC. 

This new informatin brought a huge sense of relief. There is no way they were looking at the correct imaging of my chest 2 months ago because it simply does not exist. I am assuming my PICC placement in 2008 was too deep and they were not looking at my port, but rather at a PICC placed 6 years ago! Someone at the pharmacy needs to check dates before giving such information. And the doctor (who I believe was a resident) should have done his homework before coming in and scaring the crap out of me.

We did an x-ray in the hospital just to confirm the placement of the port and sure enough my port was just fine. So after all that stress and confusion I am happy to say that my port problem is not actually a problem at all!

Skin Problems With The Port

I have had my share of skin issues and allergies with my PICC line so it was no surprise that the skin around my port would have issues too. The second night of my hospital stay I was snuggling in bed with my daughter when she got tangled in my port tubing and accidentally pulled the port needle right out! I called the nurse expecting her to re-access it with no problems. What I didn't notice was that my skin was already reacting to the new hypo-allergenic tape we were using.

The nurse took one look at my skin and was convinced I was allergic to Betadine (I AM allergic to Chloroprep so we use Betadine to clean my skin) since we were using hypoallergenic tape. I tried explaining over and over that it was the tape since I am allergic to essentially all tape, but for some reason she was certain it was the Betadine. She went to talk to some other nurses and doctors (it was evening so my doc wasn't available) and they all agreed that they had to use either chloroprep (which I am allergic to) or Betadine to clean the access site and until they knew that Betadine wasn't causing the allergy they refused to use it on my port.

This left me annoyed because I know that I am not allergic to Betadine, but nobody would believe me. They agreed to cover a few patches of skin with Betadine to see if I reacted. They also placed an IV using the hypoallergenic tape (that I knew was the culprit) with the intention of checking it the next day for a reaction.

Of course, the Betadine did not react and my IV site (using the hypoallergenic tape) went crazy with blisters. When my doctor came in he brought two new types of tape that we decided to try. I think we have found two solutions for now!! We cover the port with several layers of sterile gauze and then tape the gauze down with Hy-Tape or Kind Tape (pediatric). I am so relieved to finally have a tape that does not make my skin blister.

When 3 turns into 6

After admitting to myself that I really needed to be admitted tot he hospital in order to hopefully end this year of bad health I knew I could not be away from my daughter for a full two weeks. I called my CF nurse and she agreed that I really only needed a few days of intense CPT and then I would be fine to go home and finish home IVs. I was told 3-4 days would be as long as I would have to be in the hospital. Of course, knowing the way hospitals work I told myself that I would be out in 4-5 days tops. 

Yeah, so apparently when you are a stay at home mom you don't pay attention to holidays and when you don't pay attention to holidays and you get admitted to the hospital you set yourself up for disaster. On Friday (day 3) I was told I would most likely go home the next day, but I also know weekends can be a little tricky. I figured I would be out by Sunday (day 5) as a worst case scenario. And then someone reminded me that it was a long weekend which suddenly makes setting up my home IVs even more challenging. As of now I am going on day 6(!!!!!!) of my 4 day max stay and am not sure I can get home until Tuesday due to my insurance office not being open. I feel like I might go crazy being away from my heart and soul, my daughter this long. I have officially decided I am never ever going to agree to an admission before thoroughly checking the calendar and making sure there are absolutely no holidays anywhere near my potential release date. 

The only thing that is keeping me going is knowing that my daughter is handling the time away from me amazingly well. She visits everyday, often twice and we snuggle, eat snacks, sing songs, and play. She then goes home and has done so well playing with her daddy and grandpa (my dad) who have been caring for her. She loved coming to the hospital and playing with a bear my sister gave me (that K named Roar). Yesterday when she didn't want to leave she decided she would willingly leave if she could take Roar with her. She now sleeps cuddled up to her teddy Roar instead of her mama. I seriously cannot wait until Roar gets the boot and mama can snuggle her baby again.

Learning Something New

It has been a very long time since I have had a RT do my treatments with me since I usually do home IVs. During this stay I have learned from my RTs a little bit about my unique case of CF. First, I realized I am very productive. I have had several comments from a few RTs along the lines of,

"Wow, you sure coughed up a lot with that treatment"

"That must have been really effective, look how much mucus you got out of your lungs."

"I can't believe how much you coughed out"

Which is cool and all, but if you ask me it seems I am coughing up the same (if not a little less) than usual. Apparently, I am a very productive cyster. Lovely.

Second, I apparently have very thin mucus. RTs like to look in my spit cup and make comments on my mucus and one that comes up is how thin it is. I guess if you are going to have copious amount of mucus it helps for it to be super thin. 

Pro and Cons of Hospital Life

We all know being in the hospital is lame. Besides the obvious like being cooped up in a teeny tiny room (especially since it has been sunny and warm in my corner of the world), not having any privacy, control, or loved ones there are other cons that I forgot about since last time I was in the hospital. However, ever the optimist, there are a few perks to the hospital life (and it is not the "free" food). Here is a quick list of my random personal pros and cons to my current hospital stay.

Cons:

1. Having an audience for treatments: Don't get me wrong I like the RTs, but I always find it awkward to have someone watch me while I cough my brains out, turn red in the face, and spit in a cup.
2. Pooping: It happens to all of us, but why oh why does someone always walk in 22 seconds after I go number two? I mean, really?!
3. Sleep: Between patients screaming down the hall (dementia patients?) and nurses coming in the room in the middle of the night to the carts being rolled around outside the door I just can't sleep. In fact, I am pretty sure I slept more the few weeks after my daughter was born and I nursed her every 2 hours than I have slept in the past four night.

Pros:

1. No Responsibility: I find home IVs somewhat exhausting. Waking through the night to do infusions, setting timers for meds, making time for therapy all while cooking, cleaning, and being a mom. It is nice to have someone else keep track of times and meds and therapy.
2. Selfishness: When you become a mother you lose the right to be selfish. You have another human who depends on you 100% so you often come second or third or fourth. The nice part about being in the hospital is that everyone, including yourself, is focused 100% on getting YOUR health back to where it needs to be. 
3. Improvement: I am finding that I am feeling better much faster than when I do home IVs, but that is probably because I do my Vest 4x/day for 30 minutes plus percussion 4x/day for 20 minutes, and I bike 2-3x/day for 30 minutes. That is about 4 1/2 hours of airway clearance (not including nebs and meds) a day. There is no way I would get that type of therapy at home, but shhhh, don't tell my doc.

Locked Up

Saturday night I was suddenly jolted awake by a sharp stabbing in my left lung. I frantically was gasping for breath, but it seemed that each breath I took brought on excruciating pain making it difficult to breath at all. I climbed out of bed, crawled to the living room and rolled into a ball and tried to decide what to do next. K was sound asleep in the other room and my husband was at work until 4 in the morning so I felt stuck in the house, but was worried I may have collapsed a lung and needed to go to the ER. I called my parents to come to the house thinking I would be going directly to the ER and needed someone to stay with Kaylee. While I waited I called the on call doctor to get his advice.

As I waited for my parents to arrive the pain started to subside slightly, the panic and fear started to ease and I realized that I recognized this pain. The stabbing, sharp pain that comes with every inhalation, the feeling of being unable to catch your breath, the crazy PAIN all point to my old friend, pleurisy. In order to avoid exposure to the flu I decided to wait until the on call pulmonologist called before going to the ER because Pleurisy is not necessarily an emergency situation.

The pulmonologist never actually called back and I eventually fell asleep so the next morning I decided to try calling again. Thankfully, this doctor called back right away and agreed that my symptoms pointed to pleurisy and told me to go to clinic on Monday to have the CF doc check it out. I have had pleurisy twice before and my doctor always prescribed oral meds, motrin, and increased physio and hydration. I figured this time would be the same.

At clinic, the doctor was very concerned. He told me that he was sure I had plugging which is what was causing the pain. He felt that with how hard my last year has been in addition to this current plugging he really wanted me hospitalized. He explained that in the hospital they could give me more CPT, more hydration, and more rest than I could at home being a mommy. As hard as it was to agree to hospitalization I knew that he was right. My husband can't take 2 weeks off of work to take care of K and there is no way I could possibly do as much at home caring for a toddler as I could if I was trapped in the hospital.

I keep reminding myself 3 or 4 days away from K now will hopefully make me a much better healthier mommy that will be around longer to watch her grow up. I have been in here since Tuesday night and my heart is breaking being away from my daughter, but I understand that sometimes CF pushes its way to the front of our lives, demanding that it get all our attention and care. At this moment in time I need the extra care, I need this time for CPT, I need to get better for her, for us.

19 months

Dear Princess,
This month has been all about colors for you! You are absolutely obsessed with your colors! You want to know what color everything is! This tree. That book. The dog. The wall. You know all your colors in Spanish except yellow which you will only say in English. If we try to tell you in Spanish you look at us like we grew two heads and correct us in English. You can identify yellow from miles away and you have almost perfected "nana" (naranja) "verve" (verde) and "rojo". The other colors get a little confusing and you often need a little help identifying them, but you are getting much better. Shopping is fun with you because you love to identify the color of the clothes as you walk by. You get pretty frustrated with me when you ask me what color something is (in your own made up babble language) and I don't know what you are asking. I usual figure it our once you start raising your voice. I now default to telling you colors if I am ever in doubt of what you want.

Thing You Like: You are obsessed with books. I remember babysitting kids as a teenager when they were in this stage and I thought it was cute, reading 8 million books in a row. As a mom, not so much. I mean, I LOVE reading to you and I LOVE that you love books. But reading the same books over and over and over every single day gets a little...dull. But the cool thing about reading is you are getting interested in letters and although you think you know all your letters you really only know the letter "o". You love to look for letter O when I read to you and you point them out on each page. Pretty cool!

You are also obsessed with counting to four in Spanish. Although your cuatro comes out as "caca" which always makes me laugh and is a little embarrassing in public! Your favorite thing to count are your toes (apparently you only have 4 toes, but we are working on number 5!)

Things You Dislike: You get so very frustrated when we don't know what you are saying. I understand because when I was learning Spanish I would often say things perfectly, in my mind, and nobody would understand. I found it one of the most frustrating things in the world so I totally get your tantrums on that one!

What I Like About This Age: You are so so very independent. You play by yourself so much that sometimes I feel left out! You have the best imagination and you are such a jokester, you are constantly making us laugh! 

New Words: Since you talk nonstop I thought I would put some of the funny words you say wrong because that is half the fun of listening to you talk!

• gub gub: shoes
• Aloh- Hola (backwards)
• pio pio - chicken. Pio pio is what baby chicks say (Spanish) so I think it is funny/gross you call your food what the animal says)
• baby chee chee - raisins (baby dried cherries)
• Brrrrrrm- car (she specifies if it is mama's,  dada's, or Kaylees (stroller)

Flu Phobia

At the risk of sounding like a paranoid psycho I am terrified of getting the flu this year. Last February, 11 months ago, I caught the flu for the first time. I was so sick I couldn't function (getting from the bed to bathroom was a huge feat) for over 2 weeks. I was sick, but somewhat functioning with help for several weeks after that. I was extremely fatigued and unable to preform normal duties because of this fatigue for months. It took me about 9 months to recover and at 11 months out I still feel like, although recovered, I am forever weakened health wise by the experience. I don't bounce back from colds like I did before, I get run down much easier and I just don't feel the same.

I recently bought clorox wipes for grocery carts, baskets, or other places that sick people may have touched. I always feel slightly embarrassed taking out my wipes and sanitizing everything because I am not normally a germaphobe, but avoiding the flu is worth the moments of embarrassment. I wear my gloves when I go to the ATM or other public places so I can pull my gloves off after touching everything (germs!) and throw them aside. Hand sanitizer is my new best friend. I no longer worry about hurting feelings if a kid at the park or library is coughing near my daughter I instantly pick her up and move.

On the radio last night I heard there have already been 5 deaths from the flu in the city I live in. Obviously, that did not ease my fears of getting the dreaded flu. I know I need to live life and can't just barricade myself in the house, but I am not going to lie- there are times I wish I could (and not go completely crazy from boredom).

Any tips on avoiding the flu through the winter months?