It's a CF Thing...

Over this past year and a half I have "come out" of the CF closet. My family and closest friends knew about my illness, but many of my acquaintances or co-workers had no clue. It was quite easy to hide. Or, if they knew, they assumed that it must have not been so bad since I "looked" fine from the outside. This past 6 months of hospitalizations and almost constant IV treatments probably took some by surprise.

I have found that some people are still confused about my illness, since I still look the same on the outside. I think it's so easy for people to understand that someone is sick when they see outward proof of an illness or aggressive treatment. Much of the impact of CF is internal. So, I wanted to share some of the outward physical manifestations of Cystic Fibrosis, just to raise awareness.

Cough: A CF cough for most is very congested sounding. A coughing fit can last several minutes. Most people with CF cough every single day...I do. Some people with CF cough so hard that they open up a wound or blood vessel in their lungs and can cough up large amounts of blood. This is called Hemoptysis.

Salty Skin: In the middle ages in Europe they were informally diagnosing children with an unknown disease, simply based on salty skin. They used to say, "a child who tastes salty from a kiss on the brow...is hexed and soon must die". The disease that they were talking about was CF. My sweat is salty. I look like I have frosted skin when sweat dries on my face. Ick...sometimes it even burns my skin and eyes. On a positive note, I'm sure it exfoliates my skin at the same time!

Clubbed Fingernails: Due to a lack of Oxygen, most people with CF experience clubbed fingers. The nail beds and tips of the fingers are larger than what's considered normal. My pinkies and thumbs are OK so far, but my index, middle and ring fingers are clubbed...though not severely.

Barrel Chest: When people have illnesses that cause a chronic cough, they can actually change the shape of their rib cage. The rib cage takes on a more circular shape, then it's normal oval shape. This sometimes occurs in people with CF. I am pretty certain that this symptom doesn't impact me yet.

"CF Belly": I kinda made this one up... I haven't seen anything written on this topic, but I have spoken about it with several of my girlfriends with CF. That means it's definitely true! People with CF typically have difficulty keeping weight on. The CF pancreas does not digest fats. When the pancreas is deficient, it's called pancreatic insufficiency. CFer's who are PI take enzymes so that they can digest more of the foods and nutrients that they ingest. I am pancreatic sufficient. My pancreas still digests some of the fat and nutrients I take in, however not all of them. I am just not at the point yet where I need to take enzymes.

(Nope, this is not MY belly)

CF patients are encouraged to eat a lot of very high fat food. I have no problem with this part of the disease! My CF doctors have also encouraged me to keep a little layer of extra fat stored on me, since when I get sick it will melt away. Mission accomplished on that one! However, when us CF ladies get together we often feel the need to compare our CF belly's. Many of us have a similar body type, including skinnier arms and legs, along with the 4 month pregnant belly look. I'm sure the belly has to do with digestion...I don't know. I just know that no amount of crunches are going to flatten this girls CF belly :-/ I want to hear from my Cysters out there... Any of you have the "CF belly"?

A little health update. Not too much has changed in the last few weeks. I am still on the IV and nebulized Amikacin, along with the oral Zyvox and Azithromycin. My white blood cells and platelets have been behaving. I have a clinic visit next Wednesday, at which I will have my PICC pulled. No more IV's after Wednesday! Whew!

The Theory of Natural Selection

'Tis the season to give thanks. This year, more than any of my previous years on this plant, I am thankful that Darwin's Theory of Natural Selection is no longer relevant in the human species. I am just sitting here, at age 35, laughing at the whole idea of "Survival of the Fittest". Nope, no longer the truth here in 2010. Today, sometimes the broken do survive.

What does this old fogey from the 19th century know anyways? People thought the Earth was flat back then...

I am thankful that I was born in 1975, at a time when medical science started advancing at a tremendous pace. Having no siblings or cousins with CF we were asked to think about where CF may have derived from in my family. My mother had an aunt die in childhood in the 1920's or 1930's of "whooping cough" ~ likely CF before the diagnosis existed. My father had 3 siblings, Earl, Eugene and Loraine who all died before the age of 8 of either "Whooping Cough" or "Consumption" ~ also likely CF.

If I were born a century, or even 20-30 years earlier I would have no doubt died at age 2 when I had horrible pneumonia for almost the entire year. If I were spared at the age of 2, I would certainly have died at age 16 when I had a majorly debilitating case of mono. If I miraculously survived the mono, I would have likely passed when I had a horrible case of C Diff that earned me a week in the hospital and a month long recovery. If that didn't get me, I am pretty sure that the lung infections that my medical team I have been working to control over the past 2 years would take me. Yet, I am still here! I am alive and thriving and mothering and working and wiving. I am so thankful to have been given what I see as 33 "bonus years". Years that would have never been mine to have if I had been alive in the days when Darwin's Theory was the word. Much of my life has been like icing on a cake...none guaranteed or even expected.

I am really, truly a lucky girl! When I am physically knocked down by my disease, which I have been much of the time over the past 6 months, I tend to forget how lucky I am. Yet, I know that I am almost as lucky a they come. I have been allowed to live in overtime. My wish is for the next generation of CFer's, the little one's being diagnosed now, to be even more lucky than I have been. I want them to be given many more "bonus years" than those I have been given. I want CF to be something that is managed and not deadly. Even better, I want a cure!

Today I am a thankful girl! I wish you all a Happy Thanksgiving! Count your many blessings...even if only for today.

Clinic Visit ~ The Good & The Bad

I just got back from clinic. Do you want the good news or the bad news first? I'll go with the bad, so we can end on a positive note...

I got the H-Flu...AKA Haemophilus Influenzae. Yep, I cultured a new bug. This one is often seen in conjunction with meningitis. This is baffling to me since I have been on IV's for nearly 8 of the last 10 weeks. I know that having a depleted immune system makes me more susceptible to picking up new bugs, but I just imagined that being on 4 antibiotics would protect me. I guess that's not how it works :-/ Dr. Dazzle explained that H-Flu is often resistant to Cifoxitin, so that's why it still grew when I was on the IV's in Sept/Oct. This IV Amikacin that I started 2 weeks ago should suppress the H-flu. Dr. Dazzle is not at all worried, so I'm not going to worry either. This is another one of those bugs that will never go away. Instead we will work to keep it under control to avoid lung damage. So now, here's what the insides of my lungs look like...

H-Flu

Mycobacterium Abssessus

Staph

These are my enemies! It's a little frustrating to have new bugs to manage. It likely will make it a little more difficult in the future to figure out which bug is the culprit when I have an exacerbation.

In my last post I shamelessly bragged about how much energy I had, even though my blood counts were low. I forgot to knock on wood while writing that post. That energy evaporated on Sunday. On Monday I took a 6 hour nap, yesterday I took a 4 hour nap, and today I had a tremendously difficult time getting up and out the door to my clinic appointment. I have been tired when I wake up in the morning and no amount of napping replenishes my energy. I was relieved to hear that my counts have jumped a bit and are back to being low/normal. I was also relieved to hear Dr. Dazzle say that extreme fatigue is one of the main side effects of IV Amikacin. He compared it to the fatigue that cancer patients experience while undergoing chemotherapy. I know it's not a great symptom, but it was great to hear that this is normal. I no longer feel like a lazy slob. I feel like I can now give myself permission to rest when I need, knowing that no amount of fighting against it is going to take it away. So sleep I will do...

I brought up my hearing issues and Dr. Dazzle is scheduling another complete work-up from audiology. We'll see if my hearing has been compromised and if so, in what ways. I wouldn't be surprised if I've sacrificed a bit of the hearing in my right ear for the sake of this treatment.

That brings us to the great news! I blew AMAZING PFT's! I haven't had PFT's this high since 2005, prior to having my son. They were 3% higher than last time, which was 4% higher than my baseline. Basically, I'm 7% better than my baseline on my FEV1. Good stuff! I never share my PFT #'s on my blog...just a choice I made when I started blogging. Yet, believe me when I say that they were good :-)

I gotta go take a nap now. G'night all!

Can You Hear Me Now?

I have had an amazing amount of energy this week and I'm not complaining. I got a call from my nurse on Friday saying that my blood counts are all out of whack again. Not surprisingly, my Hemoglobin was normal, hence the energy. However, my white count dropped back to 2. If they get to 1, which they probably are by now, I must give myself another Neupogen shot. My platelets are also giving me a run for my money right now. A normal platelet count is between 150,o00 and 300,000. Mine were 63,000 on Thursday when I had my last blood draw. My bone marrow better stop being lazy.

The other thing that I've noticed is that the hearing in my right ear feels different. It's a strange sensation. Twice this past week I have been stopped in my tracks from REALLY loud ringing in my right ear. It was so unpleasant that my immediate reaction was to grab my ear and shake my head until it stopped. It only lasted about 20 seconds, but it is making me a bit paranoid. Hearing loss is the biggest concern while I'm on this IV Amikacin. I feel like I am still able to hear well, though.

So I have a clinic visit on Thursday and I have some questions for Dr. Dazzle...surprise, surprise! I have 4 weeks left that I need to get in on these IV's. Will we continue this dance if my counts continue bouncing around? Could this turn into some type of permanent bone marrow condition? Is the ringing in my ears OK if it is only occasional? Could this become permanent?

I have been thankful for the burst of energy I had this past week! I had tons of fun. I kissed a camel named Charlie, I went out with my girlfriends, we refinanced the house and I finished my Christmas shopping. Life is Grand!

Minor Annoyances

This week has been full of what I would call minor annoyances. I am very thankful that I was able to be released from the hospital last Wednesday. I had meals prepared and sent over from caring friends much of the week. I am tolerating the IV Amikacin wonderfully. I am just to the point where I think I'm done with the inconveniences associated with treating this stupid disease.

Annoyance #1 - Time Management
The progression of my CF has not changed the fact that I lead a busy life and stuff has got to get done. I really have no excuse when I am tolerating my medications and feeling relatively OK. Every day I have to meticulously plan my day to ensure that I get all 3 1/2 - 4 hours of my treatments in each day. I had a busy weekend with the family and fitting everything in was very tough. It was not impossible, just frustrating. There have been many times where I just can't fit everything in and I don't get done all that I wanted. I always end up having to give up something I love, like working out :-( I have been able to get to my workout class 3 times in the past 5 days, though. I just have to work extra hard and plan my time well so I can get there.

Annoyance #2 - Surveys/Letters from Insurance Companies
With 4 hospital stays and 2 ER visits in the past 4 months I have gotten MANY quality assurance surveys from these hospitals. To be honest, I have no idea which survey goes with which stay. I feel obligated to fill these out, so they sit in my pile of things I need to do...but they never make it to the top of the priority list for reasons such as "Annoyance #1". I also keep getting daily letters from my insurance company saying I have been approved for this or I have to use a different pharmacy for that. I am a Social Worker. It's my JOB to help people navigate their insurance plans, but when I think about the hoops I have to jump through it boggles my mind. The amount of information is overwhelming!

Today was the kicker... I got a form letter that said that I was "approved for home care or hospice services". I wish my insurance company would send me a survey like the one's I get from the hospitals. I would tell them where to shove their form letters. Couldn't they have one letter that goes to those who have been approved for home care and a second for people approved for hospice? It would have been nice if they could have even just circled, underlined or bolded the home care selection. Do they know something I don't? So either I am able to have a nurse come to the house twice a week to monitor my care or I have six months or less left to live! Crazy form letters...

Annoyance #3 - PICC lines
Yes, they are super, duper convenient. However, I am a nervous wreck with mine now, since my last one ended up infected. This one has been very sore and itchy so far. I think the adjustment that had to be done, the bleeding that caused and the 20 minutes of pressure to the PICC site as a result created one sore arm. I think it's itchy, because even though I've had a PICC most of the past 4 months, I have not yet mastered the art of keeping the site dry in the shower. Saran wrap does not work, nor does saran wrap taped into place with medical tape. I spend 15 minutes prior to every shower trying to wrap my arm myself, cursing at the saran wrap as it sticks to everything possible, except my arm. Yet, I still end up with a wet dressing. Grr. One day I will master this art.

Here is a HUGE don't for all my CF friends with PICC lines out there. If you have an extension, say like a 14 inch extension, and you finish your antibiotics leaving the extension dangling when you run to the bathroom...DO NOT allow your PICC extension to fall into the toilet. I'm not saying I did or anything, but I would suggest that you DON'T. If you do I would suggest running, not walking, to your supplies and immediately changing your cap and extension line! I mean, this sounds like it would be a good plan if it were to ever happen to anyone... Not me, of course.

Basically, I'm doing very well on this drug. My counts came back up with a jolt of Neupogen last week. I have some energy that I didn't seem to have on the other IV antibiotics I was on prior to this one. My hearing and vision seem to be OK so far, although I know that I may not notice those changes immediately. My cough it non-existent at this point. All good stuff! Even with these "annoyances" I really can't complain :-)

Events of Day #2

This is my 50th blog post! Holy moly, I must have an awful lot to say...

So today wasn't horrible, but there were a couple of little surprises thrown in. First, I found out that my white blood count is back down to 1. I have 280 neutrophils. Basically, this is no where near enough. My body is not equipped to fight off ANY infection right now. I am neutropenic. The only puzzling thing this time around is that we don't really know why. Two weeks ago when my white count was depleted we knew it was the Cifoxitin. I went off of it at the time, took Neupogen shots, my counts recovered and all was right with the world. This time the only thing we are thinking is that the Zyvox must have caused this drop. This is not a common side effect of Zyvox, but it appears that I always get all of the fun side effects...so why not? So it's back on Neupogen for me. I will do 3 shots this week, get my blood draw twice a week, and continue with Neupogen as needed. The cruddy part is that I need to try to stay on the Zyvox for a full year. I've only been on it for 2 months. We'll see how long we can go along like this, but eventually, we may have to discontinue :-(

The second fun occurrence of the day was that the PICC team came in a few hours ago to tell me that they took another look at my Xray following my PICC placement and they realized that the line was too short. It wasn't far enough into my Superior Vena Cava. Therefore, I had to have an adjustment. Since they just needed to replace the line inside, not create a whole new hole, I turned down the drugs they offered me to take the edge off. Here's my advice! If you take anything away from this post, please let it be this. ALWAYS TAKE THE DRUGS THEY OFFER YOU TO "TAKE THE EDGE OFF". Oh my, the "adjustment" was not such a comfortable process. Nope, not at all. I sucked it up and didn't let it show, but I was in some pain :-/ I love the PICC nurses, though, so they made it more tolerable.

I am tolerating the IV Amikacin well and I think I'm still set to head home tomorrow so I can do all of this stuff for myself for the next 6 weeks. Yeah for home!!!

A "High" Hello from the Hospital

A little "high" hello from the hospital...

Not nearly as bad as "David After Dentist"!