For once I allowed CF to take over, NEVER AGAIN!

I came home from the hospital Wednesday afternoon. It was a nice and quick 3 night/2 day stay! Enough time to get the picc line in, get my meds straight, and to get me pumped to get my health back on track! I am on home IV and oral antibiotics for 3 weeks and then I will follow up with Dr. K to see if there was any improvement in my PFTs. They are also going to see how the whole Diabetes thing is going.

So let me tell you about my scare this past summer. Classes ended and it was time for my Intro to Hospital Pharmacy rotation. I was pretty excited b/c ever since I was 16 years old I have only worked retail pharmacy and I wanted to see what else was out there. I completed my required rotation and enjoyed it so much that they hired me on as a pharmacy intern. So at this point I was working both at the hospital full time and part time at CVS. I wasn't allowing myself time to rest, exercise, or even eat like I should. I was putting my health "in the back seat" instead of making it my number one priority. BOY that was a huge mistake! I began to loose the weight that I had managed to put on during the previous 4 months. I went from 121 to 110 within 2 weeks. I then developed this horrible productive cough that just wouldn't stop. Much worse than my regular "cf cough." Have you ever heard those 80 year old smokers that sound like they are going to hock up something the size of a softball while they are coughing? Yea well I sounded even worse than that! I knew then that it was only going to get worse until I got some IV antibiotics in my system or as us CFers call it, a "tune up." I figured I better get on into the hospital if I could get my insurance to go through because I was supposed to be in one of my friend's wedding in Charleston, SC in a few weeks.

About that time I received some wonderful news that my husband would be coming home for his 2 week leave from Iraq and that he would arrive on our 1 year wedding anniversary! Needless to say I was almost as happy as the day I married him! So I continued to push myself b/c I would be damned if I was going to spend any of Brian's time at home in the hospital. So I called Dr. K and asked him to call in a couple of what I like to call my "miracle drugs" to see if they would get me through Brian's visit and the wedding that I was going to be in. Against his better judgement he phoned in Cipro and Prednisone but warning me that if I felt the least bit worse, to call him immediately.

Well the day came and my husband was finally here. I was overwhelmed with excitement to see him but he knew something was wrong. As the days went on I continued to get worse and worse. The shortness of breath became almost unbearable and the coughing continued to increase to the point that I was coughing up blood and pieces of lung tissue. But me being the stubborn idiot that I tend to be, insisted that we still head to Myrtle Beach to spend a few days. If you know me at all, you know that I absolutely love the beach and I could lay out on the beach until the tide comes in and washes the sand away. Or swim in the ocean until my fingers and toes are as wrinkled as they can be. But this time all my body would allow me to do was sit on the balcony and watch the waves crash from the second floor. I could barely walk from the bathroom to the bedroom without stopping to catch my breath. So I knew there was no use in trying to walk downstairs to the beach. I remember looking at Brian with tears in my eyes apologizing over and over for being so sick. I couldn't stand the fact that I had let "this STUPID disease" take over my body! I mean my husband and I had been away from each other pretty much 7 months and of those 7 months he had been fighting for our country 4 of those months. He deserved a wonderful time at home doing whatever it was he wanted to do. He didn't deserve this! He shouldn't have to spend his whole 2 weeks taking care of his sick wife. I became overly frustrated and cursed my disease in every imaginable way. I pretty much threw myself a pity party but I didn't invite anyone. And being the most wonderful husband in the world he told me time and time again that he was doing exactly what he wanted to be doing, and that was spending time with me. He always had a smile on his face and told me every chance he could that he loved me more than anything in the world and he would do anything and everything for me until the day he died. And I knew he meant every word of it, but in the back of my mind it was still eating away at me that I was keeping us from doing so much. We couldn't go for walks on the beach, we couldn't go ride the go-carts in south myrtle, we couldn't walk around the mall, we couldn't even go to the movies b/c the walk from the parking lot was just too far.

Then the day came that I had to take him back to the airport. With tears streaming down my face I watched him walk into the terminal, praying that he wouldn't turn around and see the pain in my eyes. I was scared to death of losing him and I was scared to death of this disease that may shorten our time together on this earth. What if this was the last time I would ever see him? Yes I know that was a little dramatic, but when your husband goes off to war you tend to think the extreme could happen and in my opinion you are entitled to be a little "debbie downer." But I kept telling myself that God was going to look after him because if there ever was a man that deserved a guardian angel, it was indeed my husband.

I left the airport and headed home. My best friend Sara and her husband were coming to pick me up on their way to Charleston so I needed to make sure I had everything packed up for the wedding. Mom begged me over and over to call my friend and tell her that I just couldn't make it to the wedding because I needed to go to the hospital. But I just couldn't do that to her. It was her wedding day, and even if I had to ride in a wheelchair to the alter, I wanted to be there for her.

So I finally made it into the shower and over the past few days showers had become the hardest thing for my lungs to endure. I don't know what it was but it would take everything I had to wash my hair. I learned later that anyone with low oxygen saturation finds it almost impossible to lift their hands above their head which is the reason showering and drying my hair had become so difficult. Well anyways, I went to rinse my hair out and I ended up passing out and hitting my head against the tile wall. When I came to, I called for mom to help. At this point she was beyond furious that I still insisted to go to this wedding so she grabbed my phone and said, "I'm calling her!" At this point I am crying my eyes out and I begged and pleaded with her not to call. She finally said, well if I take your temperature right now and you have a fever, we are going to the hospital! So she took my temperature and it was 102.5. And without allowing me to say anything she grabbed my phone and called the soon to be bride to tell her the news. Not only had I messed up Brian's time at home but now I had messed up one of my best friends weddings. GREAT, WAY TO GO CF! PERFECT TIMING!

Anyways mom called the CF clinic and within the next 24 hours I was admitted into the hospital. My oxygen saturation was in the upper 70s (normal is 96-100). My PFTs were in the low 20s and since I can remember they had always been in upper 70s and 80s. They immediately put me on 4 liters of oxygen and began running all kinds of tests. Later they told me that I had pneumonia in both lungs and that it had become so severe that some of my lung tissue was permanently damaged and for the first time in my life I heard the words, "Lung Transplant." I guess I always thought I was invincible and I would beat the odds of CF without ever getting a transplant. They went on to tell me that I needed to start considering it for the future because my lungs could not take many more of these episodes, and something needed to be done before there was any other organ involvement. My mom and I were both in complete shock. I mean in the back of our minds we knew it was inevitable that I would need one but to hear the words actually come out of a physicians mouth sounded like nails on a chalkboard. We just kind of looked at each other knowing that the other one was just as scared. Not knowing what to say, I just looked at the physician and knodded my head. I mean really, what do you say at a moment like this? Do you say, "Ok doc, thank you for scaring the $&@* out of me," or "thank you doc for ruining my day." I mean really? I know he was only doing his job but I still wish my regular physician had of been the one to bring it up or that the physician had of eased us into the conversation instead of "you need a transplant before your other organs go to crap." haha No he didn't say it in those exact words, but he might as well have.

Anyways after a few days on IV antibiotics (Cefepime and Tobraymcin) they evaluated me for home oxygen use. I had always been fortunate enough not to need oxygen at home or as CFers call it, "being put on a leash." After a quick 6 lap walk with the Physical Therapist my stats dropped well into the lower 80s, upper 70s and my heart rate maxed at 170. So needless to say I was sent home on oxygen.

I ended up spending over a week in the hospital because it was taking so long for the antibiotics to work their magic since I had let this infection get out of control. When I was discharged I was sent home on both IV antibiotics for an additional 2 weeks. I also had a determination that I had never had before to beat the odds of CF and show it that I WAS THE BOSS! Screw a lung transplant, I was going to hang on to these lungs for as long as I possibly could. So everyday when I would get home from class I would put on my oxygen and exercise until tears rolled down my cheeks. And for the first time since I had entered pharmacy school, I made it the entire semester without getting sick. I barely even coughed which was amazing.
(Not only does my coughing disturb everyone in the class, but do you know how aggravating it is to hold in a cough when you are trying to listen to a professor. And the longer you hold it in the worse it gets, to the point that you explode and have the leave the classroom while people stare at you? Even heard a guy whisper one day as I was walking out, "go smoke another one." I mean that honestly hurt when I heard that! Was that really necessary?)

It wasn't until after exams that another lung infection decided to hang its weary head. But this time I decided not to be stubborn and go ahead and head on in for my 40,000 mile tune up. So here I am, home on IVs. My lungs are already feeling much better and if I could just get the weight up and this diabetes under control I hope to be good for another semester! I'm really looking forward to this semester for many reasons. For one, my husband will be home in a month and also because this is my last semester of class!!

I have faith that God is keeping an eye on me and I am going to make it through another semester with his help! And I can't say it enough, THANK YOU FOR ALL OF YOUR PRAYERS AND SWEET MESSAGES! I know I may seem down at times but I know that God has a plan for me and I intend on sticking around for a long time! I couldn't do it without him, my husband, my family, and my friends! All of you are amazing and I love ya'll so very much!

Baby Talk...

Well, I went off of my birth control in April of 2009 because I knew getting pregnant was going to be a difficult task between my CF and Brian's previous chemotherapy treatments when he had testicular cancer in 2006. So giving my body enough time off of birth control would be one less thing hindering success of conception. Dr. K and I also had the "baby talk" today. Dr. K told me that if I wanted to become pregnant, I needed to gain weight, get my diabetes under control, and get my lung function at it's peak and to do it fast. And when he says fast, he means within the next 2 years. Only problem with that is, I still have a year and a half left of pharmacy school and I cannot imagine being pregnant with CF on my rotations. I am guessing that I wouldn't be the most pleasant person to be around if that becomes the case! haha BUT you gotta do what you gotta do right?

Brian and I have been together for 5 years come Jan 2010 and have been married for a year and a half. Of that year and a half he has been in Iraq for a year. Brian and I both have always wanted a child. To me having a baby would mean everything to me and I wouldn't even hesitate to risk my life to bring a healthy baby into this world. But to Brian, he doesn't even wanna consider pregnancy if it will cost me my life. I try to tell him that if something happens to me rather it be now or in the future at least there will be a beautiful child to carry on my memory. To me it's reality, to him it's painful to think about. And I completely understand, bc if I were in his shoes I would be the exact same way! So this is something we really have to talk about and we have to make a decision pretty fast. Brian will be home from Iraq in February and we will be meeting with Dr. K so he can discuss with both of us the complications involved in CF pregnancy. I have an open mind about this and if all else fails there is always the possibility of adoption! So not only do I have motivation to gain weight for my own health but also for our future child. So my goal is to get that 10 pound weight gain by the time he gets home! :)

Diabetes and Feeding Tube

Well today is Tuesday and since I was admitted on Sunday night I feel like a new person already! It is amazing what a little tune-up can do for a CFer... But there was some bad news this time. I now have CFRD (Cystic Fibrosis Related Diabetes), which is very common in CF patients. My HA1c was 7.7 and you want it to be less than 5. My fasting blood sugar has been between 135 and 150, my non-fasting blood glucose since I have been in here has ranged from 198 to 366. Fasting blood sugar should be less than 100 and non-fasting should be less than 200. So they have started me on insulin, I have to give myself 3 shots a day before each meal. I know some people are scared to death of needles but when you have CF needles are a part of life ad you get use to it! You have no other choice between all the vaccines, blood tests, picc lines, IVs, and diabetic needles. So in my opinion a small little prick on my finger 3 x's a day and 3 shots into my abdomen, thigh, or arm 3 x's a day will be a piece of cake. Anything is better than being sick! This whole diabetes thing explains alot, it explains my huge weight loss over the past year and my frequent lung infections. So hopefully once I get my blood sugar under control, I can get my weight back up.

Speaking of weight, Dr. K came in to see me this afternoon. Dr. K is my main CF doctor who I love. He is one of the smartest doctors I've ever met and my favorite thing about him is he doesn't try to beat around the bush or tell you what you want to hear. He is honest even if it hurts. And him knowing I'm a pharmacy student he respects my opinions even more so and likes to hear my input. He isn't afraid to step on my toes or put a scare in me. I also LOVE his Nurse Practioner, she is wonderful and does everything she can for me. I have never felt so comfortable about a team of doctors as I do here at Duke. They are great and know what they are doing. I may be a Carolina fan at heart, but when it comes to my health, DUKE is #1!! Duke is the reason I am alive today, along with the support of my friends and family! No matter what you do for yourself, if you don't have a great team of doctors and a support system you can only go so far.

Anyways, back to my weight and the honesty of Dr. K. He came into my hospital room and sat on my bed and looked down at me from the top of his glasses and said, "you are too damn thin!" He then went on to tell me that a feeding tube would be in my best interest. He tends to joke alot, but I knew this wasn't a Dr. K joke. So I just kind of looked at him with a blank stare. I didn't know what to say because a feeding tube just sounds horrible to me on so many different levels. The shallow part of me just thinks about the cosmetic aspect. I am obsessed with shopping and buying clothes, and a cute top with a tube hanging out of your belly "just ain't cute!" Other things I worry about are infection, pain, and inconvenience of it all. Dr. K knows me all too well, and says "I can already guess what you are going to say." And he proceeds to tell me that "you wouldn't find it cosmetically appealing as a female." Yup he hit the nail on the head, and I looked at him and told him that was 80% of it. He then said, "well I like your honesty and I am going to make a deal with you. If you can gain 10 pounds in 10 weeks there will be no feeding tube." I quickly took him up on his offer!

So for the next 10 weeks I not only need to stay away from germs, and increase my exercise but I have to get this diabetes under control and gain some weight!! I know people think it sounds so easy to gain weight, but when you have CF it can be quite a chore! So I'm gonna try as hard as I can to incorperate >2500 calories/day, exercise, medication compliance (which isn't a problem for me), my vest, and breathing exercises into my everyday pharmacy school routine. Pharmacy school is tough enough without the stress of CF, but I'm not complaining! Because becoming a pharmacist has been my dream since I was 16 years old. And working in the health care field has been my dream since I was a child.

I remember my first hospital admission like it was yesterday..

This is my 6th admission into the hospital in the last 4 years and my 6th admission ever in my life. So I like to consider myself blessed. Most CFers are in and out of the hospital at least twice a year from time of diagnosis.

I'll never forget my first admission, I was scared to death. I was 21 years old and my husband (boyfriend at the time) was at his annual national guard training in Mississippi I was gettng our things packed up to move to Angier. We had moved home to RR for the summer because I had just graduated from UNC-W and was going to start my Masters in Clinical Research then Pharmacy School at Campbell. All of a sudden I developed this sharp pain right under my sternum that radiated across my left lung. With each breath I took the pain would get worse and worse. It honestly felt like someone was stabbing a hundred ice pics into my chest. On a scale of 1-10, I would have rated it a 20... And if you know me at all, you know I'm a pretty tough cookie and I tend not to complain about aches and pains so I decided not to tell anyone. I kept telling myself I had pulled a muscle from cutting grass with the push mower the day before. I came up with every excuse in the book just because I didn't want to tell anyone and have them worry about me.

Well 2 days passed and the pain had only gotten worse. I would wake up in the middle of the night crying because it would hurt so bad. I was even taking cough suppressants to keep myself from coughing. Couging and yawning would shoot my pain score to a 50. If you know anything about CF, you know that cough suppressants are the worse thing for us. We have to cough to get the "gunk" out, if we don't it will settle in our lungs and attract bacteria. This bacteria then begins to multiply and the symptoms of lung infection start.

Anyways, I finally gave in and got in my car with tears streaming down my face and drove to my parents house. I am sure I scared the living day lights out of them by showing up at 3am but i didn't know what else to do. Mom got up and started to get dressed because she was determined to take me to the ER. I begged and pleaded for us to wait until in the morning and see if the pain would get better. And like I always did, I made excuses telling her I pulled a muscle and just needed time to heal. But that I wanted to stay with them because I don't know about any of you, but when I get sick or hurt, I want my mommy! haha Anyways that morning when we woke up the pain was still there so my mom called the CF clinic. Of course when something goes wrong, your physician that knows everything about your history isn't there. So mom had them to page the doctor on call.

When the physician called us back my mom went on to tell her my symptoms. When mom got off the phone she told me that we were going to the clinic and to pack a bag just in case they admitted me into the hospital. As soon as I heard the word "hospital" I wanted to run away screaming. Why in the world would I need to go into the hospital for a pulled muscle, that is stupid. What kind of doctor does this woman think she is. Anyways after realizing that there was no need in fighting my parents on this, I got my stuff together and we headed to Durham.

When I got there they took an X-ray because they thought I may have pneumothorax (collapsed lung). Thank goodness that wasn't the case. They then sent me to do PFTs. I was thinking, you have to be kidding me, I've already told you it hurts like hell when I breath and cough and now you want me to breath as hard as I can into a machine? But anyways, I didn't protest, I just went ahead to do as they said. Well needless to say that didn't go too well. When I went to blow out for them to test my FEV1, I fell onto the floor grabbing my chest and in tears. I thought I was in pain before, but now the pain was unbelievable. So they took me back to my examination room and we waited for the doctor. I think a thousand different nurses came in to check on me, asking me if I needed anything. But of course they couldn't give me anything for pain besides ibuprofen and acetaminophen because most pain medications contain codeine, and codeine is a cough suppressant.

Finally the physician came in and told me that she had looked at my xrays and there appeared to be alot of mucus plugs. Which means there were areas in my lungs that oxygen wasn't reaching because they were blocked by mucus. And that this could contribute to the pain I was experiencing. She then proceeded to tell me that she wanted to admit me into the hospital for IV antibiotics and around the clock monitoring. Scared to death not only of going into the hospital but also because my boyfriend was in Mississippi and I wanted him there with me. I was admitted that night and started on Piperacillin/Tazobactam (Zosyn) and Tobramycin because MSSA (Methicillin Susceptible Staph Aureus) and Psudomonas Aeruginosa were found in my sputum culture. After about 5 days in the hospital they released me on home IV antibiotics for an additional 9 days. Zosyn was scheduled every 6 hours and tobramycin every 12 so I had to set my alarms to make sure I never missed a dose. But I wasn't complaining bc anything was better than being in the hospital and getting woken up at 4 in the morning to get random blood drawn or stats taken or to weigh me. I mean is it really necessary to wake me up from dead sleep to weigh me, why can't you just wait until the morning? Well I spent those 9 days taking extra care of myself by exercising and eating high calorie high protein diet. And eventually that horrible pain went away once the oxygen reached those areas in my lungs.

I know you are thinking, why in the world are you eating high cal/protein? Well in CF, thick mucus blocks the passage of digestive enzymes from pancreas to the duodenum. These enzymes are responsible for breaking down and utilizing fats and proteins therefore, we can become malnourished. Therefore we have to take enzymes everytime we eat something that contains fats or proteins. We also tend to lose alot of weight when we get a lung infection because all of our nutrients are pretty much going to feed the nasty bacteria. They like for us to be "overweight" or in CF terms "healthy-weight." Up until this past year and a half I have been at my "healthy-weight" which was 140 pounds. But after getting extremely sick in 2008 I lost 38 pounds in 3 months. I am currently up to 110 pounds and trying my best to gain at least 10 more pounds so that the doctor doesn't give me a feeding tube. Cuz I mean, who wants a pharmacist with a feeding tube hanging out of them? Grossss!

Well anyways, I will go into detail later about how sick I have gotten in the past and how a couple of times I went to sleep praying that I wouldn't wake up in the morning. Yea, CF can play a toll on your mind. I know all you hear about is the respiratory, digestive, and reproductive involvement, but they never think to tell people that depression is often a factor. But like I said, I'll go into that on another day.

I am in the hospital today for my scheduled "tune-up" so that I will be healthy when school starts back next week and when my husband returns from Iraq in February. I was admitted at 6pm tonight and in the past 5 hours I have had a CXR (chest xray), 10 tubes of blood drawn, a nasal saline wash, albuterol treatment, 1 successful IV started after 3 sticks that consisted of digging into my veins (ughhh, man that hurts), a heart monitor placed on me with 5 leads that are irritating my skin, and they have the pulse-ox meter taped to my finger to monitor me tonight to see if I have sleep apnea. I have the usual bugs (staph and pseudomonas) which are both sensitive to most antibiotics, except for 2 of the 3 aminoglycosides -gent and amikacin. Thank goodness I am currently not having problems with resistance, lets hope it stays this way.

But anyways, they are still waiting on some blood cultures and to see if my aspergillus shows up again. Currently I don't have MRSA or any acid fast bacilli (Tuberculosis)THANK GOODNESS!! I owe my current success to the fact that I am a germ-a-phobe :) You can ask anyone that knows me, I am obsessed with hand sanitizer! I put it on everytime I touch something that I know someone else has touched and ALWAYS before I eat! People at school think I'm crazy, but they just don't understand that a simple 2 week cold to them can turn into a total nightmare for me that lasts for months!

Well I am going to try to get some sleep and I'll try to update you tomorrow after the round of doctors come through.

My introduction...

Where do I begin? My name is Claudia and I am a 25 year old married pharmacy student with Cystic Fibrosis. Since I am studying in the medical field and our everyday language consists of medical terms and medication names that are a mile long and strange medical abbreviations, I may have a tendency to use them in my blog. But I am going to try my best to put it in the simplest of terms, and if there is something you don't understand, please don't hesitate to ask me! The key to being a great pharmacist is to be able to translate all the medical jargon we learn into something that someone with a 5th grade reading level could understand.

In case some of you do not know what Cystic Fibrosis (CF) is, it is a life threatening genetic disease that involves multiple organs (most commonly lungs and pancreas). It is due to a mutation in the gene CFTR (Cystic Fibrosis Transmembrane Conductance Regulator). Without at least 1 fully functioning CFTR gene, sodium chloride and water are unable to move into and out of the cells. This leads to production of thick mucus which can build up in various organs of the body and can cause severe complications. There are many types of mutations that can occur with the CFTR, therefore CF severity can range from asymptomatic to life threatening. I have met CF patients that only had pulmonary complications and others who only had digestive or reproductive organ issues. In my case, I have both digestive and pulmonary complications.

I was diagnosed with CF at the age of 3 and 1/2. Mom knew something was wrong with me within a few days of bringing me home from the hospital after I was born. No matter how much she fed me, I just never seemed to get full. I had severe diarrhea after each feeding and as I grew older and started eating solid food, everything would pass through me undigested. I began looking like those poor starving kids in 3rd world countries with my swollen tummy and tiny arms and legs. She took me to doctor after doctor trying to figure out what was wrong with me. Then one day I caught a cold that just didn't seem to go away. So she took me to the ER and one of the doctors there mentioned the possibility of Cystic Fibrosis. So mom immediately made an appointment with a CF specialist in Greenville, NC. At my appointment they performed a sweat test on me. This is a special test that measures the amount of chloride that is released in sweat. I was immediately diagnosed with CF at the conclusion of the test.

Like any parent that finds out there is something wrong with their child, my parents were petrified. All they had ever heard about CF was "fatal lung disease" and that most children didn't live past 12 years old. After they got over their initial shell shock, they made the decision to treat me like any other kid (except for adding in medicine, chest pt, and breathing treatments) and not to put limitations on my life. They didn't want to put me in a bubble, they wanted me to feel like a "normal kid." So I was involved in girl scouts, acrobatics, gymnastics, jazz, tap, ballet, karate, softball, swimming, and playing the clarinet in the band. And I went right on through high school without ever being admitted into the hospital! Most people didn't even know I had CF because I was very discrete about it. It was almost as if I was ashamed of my disease. I didn't want others feeling sorry for me or treating me any different. I remember I would tell kids in my class that the reason I coughed so much was because I had allergies. When I had to take my enzymes before lunch I would turn my head or excuse myself to the restroom so that no one would see me. It wasn't until my second year in college that I became more open about my CF. I had finally realized that CF was a part of who I was and if someone couldn't accept it, then they weren't even worth my time.

It amazes me still today that alot of people don't even know what Cystic Fibrosis is. Some people just refer to it as "a lung disease." But it is so much more! It involves so many organs and affects so many peoples lives! Over the years there have been so many advances in the treatment of symptoms of CF, which has allowed people to bypass the initial 12 y/o then 18 y/o and now 30 y/o life expectancy. I hope one day there will be a cure so all of us CFers aka Cysters can "breathe easy."