Sunday, November 17, 2013

Clinical Trials: A Vital Part of Cystic Fibrosis Treament Advancements


Clinical trials are vital part of advancing treatments for CF patients. Fundraising is the genesis for new treatments because it allows researchers to create new treatments, but clinical trials are the bridge from a breakthrough in the lab to a new medicine actually being available on the open market. It is the often overlooked last step in getting a new medication approved by the FDA for the open market. As a consequence, new medications languish for extended periods because the researchers cannot recruit enough participants in the clinical trials.
As a 30 year old with CF I recognize the importance of clinical trials as I have been alive to see many great breakthroughs for CF in my lifetime. I have participated in various clinical / research trials in CF for over two decades. Some of these studies have involved mucociliary clearance, drug studies, observational studies, genetic marker studies and early intervention studies. Currently, I am participating in a study drug clinical trial and thrilled with the ease of taking the study medication.

The CFF drug development pipeline has never looked as promising as it does today. There are several medications with great potential being studied in CF patients. There are studies ranging from CFTR modulation which Vertex is currently leading. Vertex’s Kalydeco was successfully approved after extensive clinical trials in CF patients with G551D mutation. Vertex also has VX-809+Ivacaftor (Kalydeco) and VX-661+Ivacaftor (Kalydeco) in trials for CF patients who are homozygous F508del as well as those who are heterozygous f508del.

Additionally, there are first-ever inhaled antibiotic therapies being studied in CF patients. For example, Savara Pharma’s (
www.aerovanc.com) lead product, inhaled dry powder Aero Vanc, is being studied in CF patients who culture MRSA. Aero Vanc could be a huge advancement in the treatment of MRSA CF lung infections. Currently, there are no commercially available inhaled antibiotics for MRSA. Subsequently, those with CF and MRSA currently have to take very strong oral and IV antibiotics for every exacerbation. For those with Pseudomonas, inhaled Levofloxacin and Arikace are also in development in clinical trials. These new inhaled antibiotic therapies have potential in lessening the bacterial burden in CF patients’airways. Thus, leading to reduced airway infections and subsequent irreversible lung damage.

Participating in clinical trials is not as challenging as one may think. Patients are reimbursed for study participation, travel (mileage), parking, food consumed during study visits and sometimes overnight stays at a hotel depending upon distance traveled by patient. The study sponsors do their best to make study participation less inconvenient to CF patients and their families.

Great sources for online searches of CF clinical trials are
www.clinicaltrials.gov, www.cff.org drug pipeline, and company websites. A great example of a patient-friendly company website with easy-to-navigate study site info is Savara Pharma’s www.aerovanc.com. Savara makes it easy for patient’s to select their state and click on the pin to obtain contact info for the study coordinator and doctor conducting their trial. Obtaining study site contact information is easier than ever thanks to the internet and especially social media.

Best of luck to you in your efforts of participation in CF research. Just remember to always consult your CF doctor prior to screening or joining any clinical trials.

Thursday, August 30, 2012

Anemia, Hair Loss, and Iron Infusions

Anemia occurs when the body does not have enough healthy red blood cells, which is vital for carrying oxygen into body tissues. Anemia can be caused by several conditions. However, for the purpose of this blog I am going to share my experience with anemia which began after I experienced massive hemoptysis in February 2011.

                                       My Hair is Falling Out!
One year following my experience with massive hemoptysis, in February 2012, I decided to go to my salon for pampering and candy coffee. The girl washing my hair kept saying how beautiful my hair looked and how soft it was. I was thrilled with my new hairstyle and couldn't wait to get home to show it off. Well, just before leaving the salon my stylist gave me a large handheld mirror to check my hair before I left; it looked lovely. But there was a major problem. I realized the lighting in the salon shined through the front of my fine, thin, blonde hair and my scalp was clearly visible through my hair. What?!? How did I not notice this before now? I knew I had been losing an unsual amount of hair because we needed to use draino weekly due to my hair clogging the shower drain. I was losing hair all over the house, even even stopping my vacuum cleaner's rotating brush in its tracks. Every few times I vacuumed I had to spend an hour cutting my hair out of the bristles on the vacuum cleaner's spinning brush. The most prominent area of hair loss was the top of my scalp and around my face. Something was wrong within my body. I asked myself, "What is wrong with me now?" I was determined to find the answer sooner than later.

                               Dermatology Consult for Hair Loss
I saw a dermatologist who I'd seen years before when he was a resident on the pulmonary team. I told Dr. S. about my hair loss. He listened to me thoroughly and patiently as I described my hair falling out. His attending seemed to think my hair looked fine and was not thinning; I respectfully disagreed with her stating that I was indeed losing an unusual amount of hair for me. Thankfully, Dr. S. decided to test me for anemia and thyroid conditions. A few weeks later I was readmitted to the hospital and I called Dr. S. as soon as my labs returned showing that I was anemic. He then explained to me that I had been anemic for quite some time and that it can contribute to hair loss. I was surprised.

Dr. S. recommended that I speak to my team about iron infusions and I did (for a reference point, my ferritin level was only one point higher than the lowest number the lab uses in the reference range). I was given two sodium ferric gluconate infusions during this admission. Two infusions of SFG were not enough to restore my RBCs. SFG is typically given several times over several weeks to restore WBCs. Needless to say, my symptoms of anemia, hair loss, and poor hair growth continued.

                         Hematology Referral and Iron Infusion
A few months passed, my hair continued to fall out, and I was in and out of the hospital. Finally, I decided to schedule an appointment, based upon Dr. S.'s recommendation, with Dr. M. at the hematology clinic. I wasn't expecting a miracle. Dr. M. and I discussed symptoms of anemia: fatigue, memory fog, hair loss, and pale skin. I was experiencing all of the aforementioned symptoms. I'd become so used to walking around with these symptoms that I accepted them as my new "baseline of health" except for hair loss which obviously concerned me. I was severely fatigued, which I attributed to being "CF sick" as I was in the midst of what seemed to be a never ending exacerbation with frequent hemoptysis, fevers, and hospitalizations. If it wasn't for my complete devotion to my lungs and being consumed by taking care of my lungs, I probably would have spoken up sooner about my hair loss and thus received proper treatment for anemia. 

Within 24 hours of seeing my hematologist I was sitting in the infusion center and receiving a large bag of Iron Dextran. This type of iron typically only needs to be given once to restore all of the iron as opposed to the sodium ferric gluconate I was given during my admission, which requires multiple infusions over several weeks to replenish iron levels.

                                            Side Effects
I experienced the common side effects of body aches, fever, and fatigue for the entire week following the Iron Dextran infusion. But after one week the side effects disappeared and I hoped for the best not knowing if the infusion would make any difference in how I was feeling or the awful hair loss that prompted me to see a dermatologist in the first place.

                                Six Weeks Later: Improvements!
It has nearly been six weeks since my iron infusion. I have certainly experienced an improvement in my overall sense of well being. For example, I used to feel shaky and cold (glucose was normal) throughout the day even when the temperature outside was 100+ degrees. I complained of everyone "freezing me with the a/c" in car rides, restaurants, and when I visited family members. Now I crank up the a/c full blast because I actually get hot and sweaty! I had not seen my golden ivory complexion in over a year. I was pale with a gray under cast to my skin that I hid with Makeup Forever HD foundation. Now? My natural complexion is back in addition to rosy cheeks! Other than side effects to some medications, I do not feel like I have "memory fog" like I once did. I have also noticed a mild improvement in my energy. Rather than passing out from exhaustion before 9pm I am now able to stay awake until 10pm and I do not need naps as often as I did before my iron infusion. Lastly, I am happy to report my hair has finally stopped falling out and I see new hair growth coming in around my hairline! I cherish these little baby hairs growing in, even the gray and white ones!

The best part of all? My red blood cells are, for the first time in a year and half, within normal limits! I am no longer anemic! Furthermore, the size of my red blood cells have increased! My hematologist believes that having my RBCs within normal limits will buy me some wiggle room due to having frequent hemoptysis. She also said, "The more anemic you are, the smaller your blood cells are, and the more likely you will be to experience issues with bleeding." Finally, my hematologist took me off the GI irritating iron supplements indefinitely. She said when I need to "fill up my iron tank" I will have an iron dextran infusion since it helped me significantly faster than the supplements I was taking without the harsh GI side effects.

                              

                                            Symptoms of Anemia
1. Feeling Weak

2. Headaches

3. Problems concentrating

4. Palness of the skin

5. Blue color to the whites of eyes

6. Light-headedness upon standing

7. Shortness of breath (not indicative of anemia in CF patients due to underlying lung disease).

8. Sore tongue



 

Thursday, August 23, 2012

Support Systems, Asking for Help, & Cheerleaders!

While having CF is stressful and challenging, the support from our loved ones can make a significant impact on our quality of life. Those we surround ourself with can encourage or discourage us, and thus may play a pivotal role during our healing process.

I feel very fortunate to have an incredible support system in my life. My loved ones have encouraged me to reach for my dreams, have lifted my spirits when I felt defeated, have held my hand when I was scared, have wiped away my tears when sad, and have brightened my day when I needed it most. Additionally, these very same family members have always ensured to let me know they will help me any time I need it and have never made me feel like a burden. As someone with CF, who needs much more help at home than your average person, I hate asking for help. This is when my support system steps in and says, "No. You need to rest. Let me do this for you." Sometimes admitting I need help feels like defeat. But when my loved ones ride in on their white horses to help me, my body sighs in relief (mental happy dance! Woo!).

                                         I Love Cheerleaders!

My loved ones have been my cheerleaders throughout my journey with CF. They are here to celebrate with me when life is going well, and here to help get me back on my feet after CF knocks me down. I find that when I am sick and asking for help I tend to say, "I am sorry for asking you this, but will you _____ for me?" I know I do not need to apologize for asking for help, but I can't help it. Everyone I know is carrying around their own stress and when I add my needs to their load, I worry that it will become too much for them. My only hope is that they all know just how much I love and appreciate them in addition to being grateful for their compassion and generosity. I love my cheerleaders, especially when their pom-poms come in the form of a home cooked meal.

                                     CF Support Groups

Another great support system is support within the CF community. With the exception of a few people, my experience with other CFers has been positive, uplifting, encouraging, and informative. I truly enjoy being on Skype, texting, and FB chatting with my Cysters. These women are the most amazing group of women I have ever met! In addition to beautiful friendships blossoming, great recipes (maybe I shouldn't write this blog at dinner time, food is on my brain), vast information about personal experience with CF, and funny bathroom jokes, some of you have become my personal heroes.

                                      To the Caregiver(s):

If you find yourself wondering what you can do to help someone you love who is dealing with a chronic illness look no farther than your average daily tasks: taking the dogs outside, making a simple meal, offering to rent a movie, offering to go sit with your loved one just for company, offering to make a donation to the foundation which funds research for their illness, picking up groceries, or offering to ride with your loved one to a doctor's appointment. Though these all seem like simple things, they are not easily accomplished by someone who is not feeling well.
 
                                          To the Patient:

Ask for help when you need it and ensure your family and friends know how grateful you are for their kindness. Surround yourself with positive people and those who have an open heart and mind when you need to talk about your feelings, fears, and concerns. If someone tries to make you feel bad for talking about your physical suffering or isn't interested in your problems then it's time to distance yourself from that person. Never feel like you have to hide a huge part of your life from others, especially your CF. Those who love you will always want to be "in the know" when it comes to your mental and physical health and will be in line to help you when you need it. There will be peaks and valleys, but having those you love most at your side will make the valleys less difficult.

Wednesday, May 23, 2012

Cysters and Fibros: Loving and Losing Friends to Cystic Fibrosis

With the internet and increasing survival of CF patients many of us have found fulfilling friendships within the CF community, and some have even found romance within the CF community. In fact, it was a CF patient who coined the terms "Cysters and Fibros" just a few years ago. Since the birth of "Cysters and Fibros" many of us have enjoyed using this term to describe our more-than-friendship-but-family-surviving-CF-together perspective in the CF community. For a decade I have enjoyed researching CF online, seeking out support groups, and befriending others with CF. I have met some of the most incredible people who have CF, and unfortunately I have lost some of the most amazing friends I have ever had. I admire many of our Cysters and FiBros; we battle the same disease, offer advice to one another, and become a pillar of support for one another in dealing with this devastating disease. When I'm at home spending hours on breathing treatments, vest treatments, or just feeling bad I can turn to FaceBook or Skype and chat with another Cyster or Fibro who is also doing treatments or not feeling up to getting out of the house.

My first "group" of CF friends were found over a decade ago. I discovered a CF message board and e-mail support group full of older, wiser, more experienced CFers. I was only 17 and had not experienced many serious complications from CF (yet). What I discovered was a great group of people, but also the fact that being an adult with CF is (1) a blessing to survive childhood (2) full of complications, hospital stays, and often times great suffering. My "group" consisted of CFers across the United States. I thought it was nothing short of amazing (keep in mind this is over a decade ago) that I met people with CF from New York, California, Pennsylvania, and Maryland. This was back before Skype and when the only pictures you could see of one another were uploaded onto AIM or slowly sent through e-mail or snail mail.

My first group of CF friends consisted of Jim, BJ, Don, Kristin and Joey.

Jim, BJ, and Don had already received lung transplant when I met them via CF message boards.

Within six months of getting to know Jim, he died from rejection. His suffering was long and miserable and extremely sad for me to hear him talk about. The night before he died we talked about religion and he asked me to pray that he would die in his sleep. I refused. But he died later that night, in his sleep. This was my first experience with CF and death; I was heartbroken for weeks.

Then there was BJ. His transplant was successful and his health remained stable for two years. He did not like to go out much, but enjoyed talking on the phone. He and I would exchange gifts in the mail. But one day I called and he sounded terribly weak. He was not up to talking and was not his usual self. His body was rejecting his transplanted lungs. His doctors were not hopeful. We e-mailed daily and then one day I did not receive a reply from him. I worried. I called his house. There was no answer. Two weeks passed, and finally I received the bad news that BJ died. A few weeks later I received a Beanie Baby stuffed animal dog named "Courage" that BJ bought me for Christmas shortly before he died. His mother mailed it to me along with a note BJ wrote thanking me for my friendship and telling me to "stay strong" and "fight CF harder than anything you have ever fought for." Shortly before BJ passed I found out I had CFRD and was frustrated that I would have to take insulin shots daily. BJ talked about all he had gone through with transplant and said, "You are stronger than you think Meranda. You will see. Give yourself more credit. It's just a shot. You will go through a lot more with CF." He was right. I turned out to be much stronger than I ever imagined and I have faced great challenges with CF. These days, insulin shots are the easiest part of my daily regimen.

Don, was, well Don was full of life. His transplant was a success and he lived five years post-transplant. Don was in love with life and his personality was one that you could not help but find infectious. Don was all about having fun, laughing, and had the funniest voice I have ever heard. I liken his speech pattern to that of a high pitched, fast paced, better enunciated version of Donald Duck. And, yes, Don's first name was indeed Donald. Don was the eldest of our group, but his perspective on life was probably more similar to an 18 year old. He did not worry about life or health problems. He simply wanted to live and love. Eventually Don met and married a beautiful woman whom he cherished. She worked in the medical field and would stay by his side when his health began to fail. Even in the midst of death Don remained hopeful. He said he always knew he would be transplanted and remained positive. Unfortunately, after celebrating 5 years of post-transplant life Don experienced a bowel obstruction that would lead to surgery and subsequent complications. Don passed away due to complications relating to bowel surgery; his lungs and heart were still in excellent shape when he died

Kristin was very sick from CF and a multitude of secondary CF complications including CF liver disease. She had so many complications from CF I could not fathom, at that time, how she could have so many secondary complications and still be alive. She was not compliant with her therapies, which always prompted me to practically beg her to start taking. I was concerned about her and wanted her to get better. I knew she needed her breathing treatments to get better, but she assured me that they did not help her and they were a waste of her time. Unfortunately, as time passed Kristin became less receptive to my calls and e-mails. She was dying. She knew she was dying and she knew that I knew she was dying. I couldn't cheer her up as much as I wanted to. She was suffering. She was living in the hospital. Her lungs were failing, she was not a candidate for lung and liver transplant due to history of noncompliance--which was fine because she did not want one anyway. Despite having a feeding tube she became very underweight. One of our last conversations she talked about Heaven, and how she knew she'd never be sick again when she died. We said we would dance together in Heaven someday. I knew that would be one of the last times I would speak to her. She was terribly sad, not only because she was dying, but because she felt as though she was dying alone. Despite her giving her all into her failed romantic relationships, she never met a guy who was willing to stand by her side and love her as she endured the sufferings of CF. It was heart breaking to watch her date loser after loser. Eventually I received a call from Joey saying Kristin died--and we cried together over the phone for over an hour. Now our CF group was down to two.

Sweet Memories: Kristin and Joey were friends even before I came along in the group. We used to prank call each other on three-way when Joey or Kristin was in the hospital. We would all laugh so hard we would spend what seemed like hours coughing and choking from laughing so hard. Although, looking back on it, I'm sure as soon as they heard my strong southern accent they knew it was me trying to pull a prank. I now realize they were likely laughing at my inability to change my southern accent to sound like someone from up North rather than a little country girl. I'm sure I sounded much like Forrest Gump before puberty if he were trying to imitate a Northern accent.

We are now in the year of 2012. Joey and I are still alive, though we went a few years without talking to one another. Both of our lives have changed a lot since we lost contact with one another. Joey received a double lung transplant and is now happily in a relationship with a mutual friend I introduced him to years ago. Joey and I text each other and have talked on the phone a few times over the last several months. I'm grateful that he's still alive and has found love. I hope to see him continue to do well and remain happy for years to come. Joey is the last of my original group of CF friends. We have talked about being the last two left from our original group of friends and how difficult it is for us to watch friends pass away. After talking about the sadness of losing our friends, we sat in silence for a few moments as though we were both realizing the same thing: CF life is not for the weak hearted. Simply put, "It's not easy being wheezy, baby."

I have made new friends with CF. Some of them are still living and others have since passed away. It has been difficult. It has been heart breaking. I have learned a lot about CF and especially life through my Cysters and Fibros. I am grateful for social networking and the ability to connect with my CF friends across the world. I am always eager to meet new friends and lend a helping hand where I can. And I cherish the friendships I have with my Cysters and Fibros.

If you have time please see: http://65redroses.livejournal.com/ the documentary about Eva and her life with CF is very inspiring. Eva's story featured her friendship with two other CF women and how they all helped one another. You can also purchase the 65RedRoses DVD through this website. Eva's documentary was recently featured on the Oprah Winfrey Network OWN.

Monday, May 21, 2012

Cayston (Aztreonan for inhalation): My Experience

Cayston

 Cayston was approved (Gilead News Release) in the United States for treatment of chronic Pseudomonas Areuginosa infections in patients with Cystic Fibrosis in February 2010. My first dose of Cayston was July 3, 2010. This is a short blog entry about my experience with Cayston.

Brief Background

I have cultured Pseudomonas Areuginosa since 1996. When I first began to culture Pseudomonas there was not (yet) an FDA approved inhaled antibiotic available to CF patients. In December 1997 Tobi (inhaled Tobramycin) was FDA approved to treat CF lung infections caused by Pseudomonas. It would take 13 long years before a second inhaled antibiotic would be approved for CF related Pseudomonas infections. In February 2010 CF patients were finally able to breathe a sigh of relief after Cayston was finally approved by the FDA.


Personal Experience with Cayston

 I have ran a chronic low grade fever since August 2009, but I can remember waking up with a fever as far back as March 2009. My lung function (PFTs) were great. According to my doctors I looked great. But I felt awful. After complaining about increasing symptoms of a CF exacerbation my CF doctor prescribed me Cayston in July 2010. Within a few days of starting Cayston my fever subsided and I began to feel better. My airways began to open back up (a sign that the inflammation was improving in my airways), my energy increased, my appetite increased, and after months of feeling terrible I felt good. I was gaining strength by the day and felt like I was finally making progress. After my 28 days of Cayston were over my symptoms of fever, shortness of breath, fatigue, and decreased appetite returned. It would be several months before I would use Cayston again. But when I used Cayston (this time during a hospitalization for a pulmonary exacerbation) I once again felt relief from the symptoms of chronic infection that was plaguing my tiny body. I was prescribed and used Cayston three times in the year 2011 each time with improvement of my pulmonary status.  

2012

In mid-April of 2012 I was once again prescribed Cayston and instructed to cycle it ever other month. Yesterday was my 28th day of treatment with Cayston and I managed to stay out of the hospital the full 28 days which is an accomplishment for me these days. I only experience one mild episode of hemoptysis (link to my blog on my experience with hemoptysis) during this cycle of Cayston. During my April-May 2012 cycle of Cayston my chronic fever was alleviated, my appetite improved which lead to a 6 lb weight gain, my energy increased, my heart rate decreased (my heart rate increases due to infection and difficulty breathing, my oxygen sats remained stable, and my quality of life improved.


In Closing

I used my iPhone calendar to remind me 28 days from my last dose of my Cayston to restart Cayston to ensure that I do not miss a single day that I could be on the medication. I believe Cayston is a wonderful addition to the standard prescribed therapies for those of us living with CF and colonized with Pseudomonas Areuginosa infections. Click here to learn about the Pseudomonas Genome Database.

Thursday, February 23, 2012

Attention CF Centers: Reputation Matters


I am fortunate enough to keep in contact with 1,000+ CF patients across the world in addition to CF patients at my center. We (CF patients/families) share our unique experiences about living with CF and our health care provider experiences with one another. We confide in one another and ask one another for advice; we also share our deepest fears with one another. One topic we all discuss in the CF community is our CF centers. While the CFF provides CF center stats about various issues from average lung function to average BMI for each clinic, it does not provide patient satisfaction stats. A CF center's reputation is not built solely on stats. Reputation is also built on patient satisfaction. We talk, so listen up.


We, CF patients, all want to believe we receiving care from the best CF center and CF specialist. We want to believe we are receiving state of the art care by leading CF researchers. We want to believe our doctors are going to save our lives. We need to have confidence in our providers. We compare our doctors to other CF patient's doctors and our personal experiences with CF center physicians. Our experiences with healthcare providers play a role in who we recommend to our friends and who we advise to avoid. Every doctor has his or her strengths and weaknesses. Some doctors have better bedside manner than others. Some doctors do not want to be bothered after hours or on weekends. Some doctors will respond to our e-mails, others won't. I have CF friends whose CF doctors have given out their personal cell phone number with instructions to call should an emergency arise. We share our CF center experience with each other in hopes of getting our friends to better CF care or even for transplant purposes. Hello, my personal CF clinic, you can thank me for encouraging some of my Cysters and FiBros to switch to our center. But you can take the credit for them staying.

Let's say a patient has a bad experience with a CF doctor, nurse, or team member. What are they likely to do? They're going to write about it on FaceBook, Twitter, and/or personal blogs (welcome to social networking), they're going to call their family and friends, they're going to Skype their CF friends and tell them about it, they're going to e-mail others, etc. In today's internet age not much is kept secret. In fact, I have known CFers who have recorded their conversation with their CF doctor via iPhone and later play it to others to prove their doctor has a rotten bedside manner. I know because I heard the conversation myself; it was mind blowing. Needless to say I would not recommend any CF patient to this particular physician.

When I'm hospitalized I live on FaceBook, Skype, FaceTime, and e-mail. During Skype calls with my Cysters and FiBros who are also admitted, we give laptop tours of our hospital rooms and compare: who has the bigger TV, who has the bigger hospital room, best bathroom, view from hospital window, etc. When I see CF patients who have beautiful views from their hospital rooms and I'm staring at a brick wall from my window I think, "I wonder what else is better at their CF center?" But what matters more than all those things is this: who has the better CF center, team of CF doctors, who is receiving top care, and especially who has better inpatient experiences. You could have the best CF team in the country, but if the inpatient side of care is miserable, patients are going to hate receiving care at your center.

Patients are talking. Your giving us something to talk about. While hospitals are working hard to keep our medical records private, many of us in the CF community are posting details about our health and health care providers on FaceBook. We're sharing our personal xrays, CT scan images, even pictures of procedures online. Why? Because we want to compare notes with others going through the same complications. We're learning from one another. We're realizing that every CF doctor has a different opinion about what test results mean. We're realizing medicine is practiced and is not an exact science, yet we realize when we're not receiving the best available care. We're getting smarter about CF, we're learning along with you, and we're comparing you to others in your field. We're talking about you and it's affecting the reputation of your CF center and you as a professional.

Your CF center's reputation rides on the back of the decisions you do or do not make for your patients. I believe this is great for the CF patient community, because it's breeding competition for new patients. We're jumping ship when it's sinking and finding solace in the CF center that's full steam ahead. If your losing CF patients to other centers, ask yourself why. With CF patients having an average life expectancy of just 37 years old, we don't have time to waste away at a CF center we're unhappy with. CF may not be as profitable as cancer in terms of insurance or research dollars, but it's still a competitive field. Just remember that next time you think your bedside manner doesn't matter, you just may end up being the topic of discussion on FaceBook, Twitter, Skype, or other social media outlets.

In closing, in the first paragraph of this  blog I posted my personal chest xray (CXR) taken a few months ago at my CF center. I posted it on FaceBook, which then prompted my Cysters and FiBros to post their CXRs as well. Within a week of me posting my CXR at least 70 of my CF friends posted their CXRs, some including PFTs next to the CXR. It was interesting to see so many CXRs from CF patients in all walks of life!

Sunday, February 12, 2012

Hemoptysis Changed My Life

One year ago in February 2011 I had My First Experience with Massive Hemoptysis (bleeding from the lungs).  I had to be embolized in an emergency surgery to stop the bleeding, but unfortunately it took over 12 hours after I entered the Emergency Room for me to be taken to ICU and then the VIR operating room. The lengthy delay between presenting with hemoptysis in the ER, and having the embolization was due to a misdiagnosis and miscommunication via my ER resident. I was first told by this young resident that I simply had a case of CF-pneumonia. I immediately voiced my doubts--while blood continued to fill my mouth--, but the young resident failed to properly communicate my symptoms and concerns to her attending physician. Additionally, it was not until the attending radiologist read my CT Scan several hours after I entered the ER and after I was diagnosed with "normal CF-pneumonia" that I was diagnosed with a pulmonary hemorrhage and was then taken to ICU where I waited a few more hours until embolization. Perhaps if communication had not broken down in the ER I could have received my embolization sooner than 12+ hours after it began and the experience would not have been as stressful.

Here I am almost exactly one year from the day I experienced massive hemoptysis. I have been admitted so many times due to hemoptysis and CF exacerbations that I decided to stop keeping track. There are days when I wake up and feel good, or at least my perception of good; I am rested and eager to start my day. The first thing I take when I wake up is my Aciphex for reflux and my Advair 500/50. I brush my teeth and gargle any residual Advair out of my mouth as I begin looking forward to doing my CPT and breathing treatments. Why would I look forward to breathing treatments? They allow me to breathe better; my lungs love breathing treatments and thus I enjoy my breathing treatments. Nothing feels better than the ease of breathing immediately after Hypertonic Saline and Pulmozyme--nothing.

Thanks to regular episodes of hemoptysis I have to constantly check for blood streaks in my sputum. Sometimes I taste or smell blood and I don't see it for a few minutes or hours. Sometimes I feel great and SPLAT! Blood starts showing up when I cough. It frustrates me, it scares me, it stresses me out, and it forces me to change my daily plans on numerous occasions. For example, I enjoy breathing treatments and airway clearance because they make me feel so much better. However, one must stop all inhaled treatments and airway clearance for 24 hours after even a small bleed. This means not only am I coughing up blood, but I have the added discomfort of extra congestion. The only thing I can do is get a glass of ice and sit down in front of the TV. I feel like my day is wasted and that I am a a couch potato contributing nothing to society. It is very disheartening.

As I sit there on the couch resting and munching on ice in hopes of preventing a small bleed from worsening into a massive bleed I try various things to keep my mind busy and relax. I read books, magazines, watch TV, and then I always find myself pondering life and especially how different my life has turned out now that I experience hemoptysis regularly. I can't sleep, because I am on high alert for fear that my bleeding will become significant and I will have to rush to the hospital. Quite frankly the stress that comes along with hemoptysis is second to none in my life.

Moreover, being forced to stop my treatments due to hemoptysis seems cruel. I know CFers who skip treatments just because they are too lazy to use them. I know CFers who skip treatments because they feel like they feel fine and don't need them. Not me. In the last decade I have not missed treatments for any reason other than ones out of my control. I know my lungs need these treatments in an effort to reduce damage and future complications associated with CF. But hemoptysis has changed all of that.

I never know how my day will end or how it will begin. Sometimes I make plans with family or friends only to cancel on them at the last moment because of hemoptysis. There have been multiple occasions when I have looked forward to something for days, weeks, or months only to find myself frustrated and sad that I was unable to participate because of hemoptysis. When someone tells me I look better or that they are happy I am feeling better when I get out of the hospital I wince. All I can think is that my life could change in an instant all because of hemoptysis. What I unfortunately learned from experience is that you can be the most compliant patient in the world, have normal spirometry, and yet hemoptysis can fundamentally change your life in a flash.

The most frustrating part is that there does not seem to be a very effective way to treat hemoptysis in CF. Embolization helps some patients, but not all. If an embolization does not stop massive hemoptysis a lobectomy may be performed, but there is the risk of death from the massive hemoptysis that necessitated the procedure, and the procedure itself. The thought of dying from massive hemoptysis is unpleasant. It's a valid fear because I have watched other CFers die from hemoptysis and complications that were directly related to hemoptysis. I just wish my doctors could find the exact answer to my hemoptysis and a treatment that would alleviate the problem.

Monday, January 2, 2012

Monitoring CF Symptoms and Symptoms of a CF Exacerbation

Symptoms of a CF exacerbation may vary from one person to the next; however, many of the symptoms are similar in pediatric and adult patients with CF. In this blog entry I am listing a few devices to help monitor symptoms at home as well as symptoms of an exacerbation. Remember, not all patients will experience all symptoms listed during an exacerbation.


Recommendations for At-home Monitoring of Symptoms


1PiKo-1 Electronic Peak Flow & FEV1 Meter will help you keep track of your lung function at home. My CF center uses this meter in CF patients so that we can monitor our lung function regularly in an effort to detect an exacerbation or drop in lung function before CF clinic and before we become symptomatic. My CF center also gave me a printout containing my FEV1 References, which give me the FEV1 value in liters and the percent predicted FEV1. I also like that the PiKo device gives a digital report of FEV1, FEV6, and FEV1/FEV6 which are all important in monitoring lung function. This is a portable, easy-to-use, and affordable device if your center cannot provide you with one. This data in PiKo-1 can be downloaded by you or your doctor's office so that they can also keep track of your lung function. Please note I do not recommend using this device during episodes of hemoptysis. You can purchase this device on several websites, including Amazon-PiKo-1 Peak Flow Meter. There are other versions of this device, including a PiKo-6 which is the model I have. Lastly, ensure that your at-home PFT device is consistent with the PFT machine at your CF center for best accuracy.

2Fingertip Pulse Oximeter is another great device to have at home. This device will measure your blood oxygen sats as well as pulse rate, which may change during an exacerbation. Typically when I begin to experience an exacerbation I notice my heart rate goes up over a few days and my 02 sats decrease. I have checked my Pulse Ox with the one at my CF clinic and the numbers are consistently the same.

3. Blood Pressure Monitor is also a useful device to monitor symptoms of a CF exacerbation and baseline symptoms at home. Some patients with CF experience a drop in blood pressure during an exacerbation whereas others experience an increase in blood pressure. My blood pressure typically increases during an exacerbation, which is why it is important for me to monitor my blood pressure closely.

4.Thermometer is very useful in determining a change in temperature which is typically indicative of an exacerbation or acute infection. Sometimes a person with CF may have symptoms of a low grade fever, but not know he or she is actually running a temperature. Monitoring your temperature daily is useful to keep an eye on any sudden changes that may occur before a fever spikes. For example, I began running a chronic low grade fever in August 2009 and it was not until I purchased a thermometer that I realized all the symptoms I was experiencing were actually caused by a low grade fever.

The aforementioned devices can be used to detect an exacerbation early. Each morning I wake up I check my temperature, blood pressure, oxygen sats and pulse rate. This allows me to keep a record of how well my body is or is not functioning. so that once Additionally, I use my PiKo-6 device twice weekly (I skip my PiKo-6 during episodes of hemoptysis and for at least 24 hours after hemoptysis), and call my doctor if I experience a significant decrease in lung function. Monitoring lung function, oxygen sats, pulse, temperature, and blood pressure are ways I measure my body's status. Just as important as my stats are acute symptoms that I may be experiencing, which are also key indicators of a CF exacerbation.

Symptoms of a CF exacerbation:

 
1. Fever
2. Dyspnea (Shortness of Breath)
3. Hemoptysis (Coughing Up Blood)

4. Wheezing (Whistling Sounds Occuring During Breathing)
5. Increased Cough and increased sputum
6. Increased Pulse Rate
7. Increased Respiratory Rate (Breaths Per Minute)
8. Decreased Lung Function
9. Sore Throat
10. Sinus Pain, drainage, or tenderness
11. Fatigue, malaise, or lethargy
12. Decreased appetite and/or weight loss
13. Shortness of breath
14. Color change in baseline sputum
15. New or increased Cyanosis (Bluish Color of Skin)
16. Change in complexion (paler than usual, bluish tint to skin)
17. Increased White Blood Cell Count (WBC)
18. Napping or needing to sleep longer hours than usual
19. Nausea
20. Chest Pain (Pressure-like pain, sharp pain when inhaling or exhaling)

21. Back Pain (lung pain can feel as though it is originating in the back)
22. Irritability
23. Difficulty sleeping
24. Worsening reflux
25. Changes in radiographic reports (Chest x-ray or CT scan)
26. Change in smell or viscosity of sputum
27. Increase in blood glucose or difficulty controlling blood glucose

28. Night sweats

Friday, November 11, 2011

My First Portacath and Tips!

Are you considering a Portacath? Do you already have one and want to learn about my experience with the device? If so, read on!

After years of having PICC lines (Peripherally Inserted Central Catheter) placed to receive weeks of intravenous antibiotics for Cystic Fibrosis I decided to consider having a Implantable Port Device placed so that I would no longer have to deal with getting a PICC line for each hospitalization requiring weeks of IV antibiotics. I considered having a port placed for several months prior to talking to my CF doctor about it.

On January 4th 2011 I experienced Hemoptysis for the first time after experiencing chronic low grade fevers for nearly a year. Experiencing hemoptysis was a clear indication that my lungs were declining and this in combination with feeling unwell for over a year, I knew it was time for a longer-term solution. I decided it was time to consider a portacath. Why?

  1. My last PICC experience was unpleasant to say the least. Initially a nurse tried to thread the PICC, but after several minutes of her being unsuccessful (which I blame, in part, due to the fact that she was too busy gossiping about a friend and was clearly in a foul mood) I had to demand an M.D. to be paged to VIR to place my PICC; the M.D. had the line placed in record time and without pain! However, the thought of another distracted nurse causing me unnecessary pain during a PICC procedure made me think twice about having another PICC placed.
  2. Hemoptysis on January 4, 2011: I realized that my lungs were no longer functioning as well as my PFTs (Pulmonary Function Tests) reflected. For a year I suffered with chronic low grade fevers, fatigue, increased shortness of breath, and the inability to tolerate much physical exertion. Although my lungs looked good on paper--I felt terrible.
  3. I wanted a long-term solution that would allow me to preserve my veins in the future as opposed to having countless PICC lines and labs that would only add further damage to my veins in the future.
  4. Many of my friends with CF have or had Ports and most of them have had positive experiences with them. 
  5. I considered the facts: I was quite sick for over a year, my lungs were not functioning as well as they used to, and my last PICC was pulled just 6 months prior to having my Port placed. Why continue having PICC after PICC placed and damaging healthy vessels when a port can eliminate the need of having multiple lines placed over a short time period? 

While I did ask many of my Cysters and Fibros about their Port experiences I wish I knew more about Ports prior to having mine placed. Because Ports are commonly placed in hospitals and in CF patients I believe nurses and doctors see them as routine. They may forget that a Port is NEW to a patient and the patient may be completely clueless about the placement, process, recovery and what to do if ____ or ___ occurs. I heard so many GREAT reviews about Ports that I was clueless in terms of how they actually function, the fact that a needle was used to access a port once a week during IV therapy, and what is abnormal.

My CF doctor was the Attending Pulmonologist during my hospitalization in January. She and I discussed having a port placed and within a couple of days I was in VIR having my port placed. The procedure went well and I did not experience any complications. It took me approximately two weeks to feel fully heal from my port surgery. I am pleased with my decision to have a port placed. It has not been smooth sailing by any means, but it has saved my veins from numerous PICC lines and blood draws. Since having my port placed January 10th I have had two instances where tPA (Tissue Plasminogen Activator) had to be injected into my port due to it not giving a blood return.

Unfortunately shortly after going home to do home IV's I learned very quickly of something no one told me was a potential complication of a Port: the mixing of two medications causing the port needle to crystallize. Of course, the doctors and nurses I saw in VIR (Vascular Interventional Radiology) told me that they had never seen a port needle crystallize before. But I know I am not the only person who has experienced this complication. It was not a problem that originated with my port, but the mixing of two medications: Zosyn and Micafungin. What I now know is that the formation of crystals would have occurred in a PICC, port, or a peripheral IV. Therefore, having a Port did not raise the risk of the medications crystallizing. (Click picture to enlarge)

My crystallized port needle was changed out and the line thankfully continued to work. But it took two emergency trips to CF clinic, VIR, and another hospitalization before anyone realized the combination of Zosyn and Micafungin were causing crystallization. That said, if you are prescribed Zosyn and Micafungin be aware that the combination of these two medications can cause crystallization in your line (port, PICC, midline, etc.).


                              Port Advice:

1. Use AquaGuard port covers to shower. Reinforce the AquaGuardAquaGuard adhesive and additional tape around the edges will be more effective if you take a cotton ball and rubbing alchohol to thorougly clean the area that you are placing the AquaGuard and tape around the edges.

2. Learn how to access and deaccess your own port. You will find this will save you time in the long run. Find a nurse you trust and know uses sterile technique to teach you proper protocol to access your port. You want to learn from the best. A nurse named Kathy L., who used to work in my CF Center clinic, taught me how to access and deaccess my port. She told me what to do and what not to do and to make sure others access my port the way she taught me, because when your accessing a port you have to be perfect in sterility to avoid potential infections and complications. Thank you, Kathy, for teaching me so much!

3. Port needles and dressing must be changed weekly. Always keep extra supply of port needles, tape, saline, heparin, alcohol wipes, biopatches, and dressing in supply. Sometimes your port dressing will peel back and need to be replaced before the week is up, and this is when you will need the extra supplies on hand. This is also when your knowledge of accessing your own port will save you time and an extra visit from your home health nurse.

4. Keep an information card with you at all times that includes the doctor who placed your port, contact information, location of your port (ie: upper right chest), size/type of port, and type/size needle that must be used with your port.

5. Teach your family members how your port should be accessed. You never know when you will be in an emergency situation or unconscious and this is a time in which your family member(s) having knowledge of your port will be in your best interest. Your family member(s) will be able to ensure the nurse accessing your port is (1) knowledgable and (2) uses sterile technique to reduce the risk of infection associated with port access. The more of your family and friends you educate on sterility with port access, the better your chances are of reducing or eliminating infection risk due to improper access of your port.

6. Keep a close eye on your port site. If you see any redness or irritation on the site call your CF doctor immediately. It could be a minor skin irritation or it could be signs of infection. If your CF doctor is as wonderful as mine, he or she will not mind you e-mailing a picture of the site to get an idea of what is going on--and ordering blood cultures if needed.

7. Always use a gentle non-irritating soap on your port site, even when it is not accessed. I use Cetaphil Gentle Cleasning Bar because it does not dry out my skin and does not cause any irritation.

8. IV Course: When your port is accessed for weeks at a time always deaccess your port and shower prior to reacessing your port. You will want to take this time to clean off any residual tape that may be lingering, which could harbor higher levels of bacteria. I use Detachol to remove my port dressing, large clumps of residual glue leftover from the dressing, and then I clean thoroughly with alchohol wipes. Sometimes I will find sticky areas even after all of this, which is when I take a soft wash cloth and soak in it warm water with antibacterial soap to gently scrub the sticky areas on my skin. Caution: do not scrub the actual site of the port where your needle accesses your skin! Only scrub the areas where the dressing glue sticks to your skin as this is the only area where you will find residual glue left behind. After I finish my shower I once again take clean alchohol wipes and wipe over my port site once again, this time including my access area, prior to setting up my sterile area to reaccess my port.

9. Flush your port slowly and draw back blood slowly. I have found that when I am admitted and my nurses are rushing to get blood from my port they draw back the syringe quickly and this is when my blood return starts to get sluggish and eventually tries to clot. What I found works best for me is flushing slowly, pumping saline into my port, and gently drawing back for a blood return. The blood does not have to enter your clave before you know you have a blood return. As soon as you see the beautiful line of blood in the beginning of the port needle line, you have a blood return and it's time to flush it back and begin your next step, whether it is an antibiotic or using heparin to hep-lock your line.

10. Protect your port! I have found there are nurses I trust wholeheartedly with my port and others I have caught being unclean when accessing my port. For example, never let a nurse access your port without first using an alchohol wipe to thoroughly clean your clave. I was told a minimum of 15 scrubs with an alchohol wipe side-to-side around the diameter of the clave is what it takes to kill bacteria that may be lurking on your clave. I have also caught nurses opening up clean alchohol wipes, throwing them on my bed (which I cough all over and put my dirty feet in, by the way) then trying to use them on my clave to access my IV line! At first I was hesitant to say anything, but the wonderful Kathy L. taught me to worry more about preventing infection and less about being nice. All nurses are taught to be sterile and use proper technique when accessing a line and you have to enforce these teachings sometimes. SCRUB THE HUB!

11. Use the skin protectant that comes in your dressing change kits. For several months I did not use the skin protectant swab stick that came in my dressing kits. My skin became red and irritated from being accessed for nearly 9 months and it burned when I cleaned it with alchohol wipes at times. What I found is that the skin protectant that comes in my dressing kits work! These skin protectant swabs are sterile and should be used as the last step during dressing changes. Only apply the swab after the germicidal formulas (alchohol and ChloraPrep) have dried and only apply the skin protectant in the areas where dressing will be applied. Do not apply skin protectant over the port site where your needle will be inserted!

12. Set up space at home for accessing your own port: I use a long mirrow and a folding breakfast-in-bed tray as part of my port access. Obviously the mirror is so that I can see what I am doing, but the tray serves a very helpful purpose of allowing me to set up my supplies without dropping anything. First, I clean the breakfast-in-bed tray (which is only used for my port access) with Chlorox wipes and allow it to dry. Then I gather 2 of everything I need to access my port: needles, biopatches, saline flushes, heparin flushes, dressing kits, SorbaView dressing, an extra pair of sterile gloves, and a box of alchohol wipes. This ensures that if I drop something I can leave it on the floor and open up a new package without having to worry about leaving my port supplies unattended. After I finish accessing my port and my site is covered with dressing, I throw away all opened dressing supplies; all unopened port supplies are returned to my medicine cart for future use.

13. If your port site accidentally gets wet: Remove dressing immediately, deaccess your port, clean the site thoroughly and reaccess using sterile technique. If your port site got wet in the shower see tip #2 again and reinforce with extra tape. Sometimes it helps to hold a dry wash cloth over your port site in addition to the AquaGuard and extra tape around the edges to soak up any water that may get near your port side or run down your neck during showering.

14. You can use your vest after having a port placed: When I first had my port placed I called Hill-Rom the Vest company and they sent me two squares of a durable foam that I used to go around my port site until it fully healed. This helped prevent irritation to my incision and port site. You can use any piece of durable foam you have around the house, just make sure you cut out an opening large enough to allow your port needle to rest inside of without the foam pressing down on it. Basically you will want to cut a dohnut shaped hole into the middle of the foam, because this is where your port will be resting during vest treatments. However, you may want to wait a day or two after port placement to use your vest during which time you will need to use an alternative form of airway clearance.

If you have any questions please feel free to ask. I am an open book and will do my best to answer your questions.

Wednesday, November 9, 2011

Updates Coming Soon!

Hello fellow Cysters, Fibros, and parents of my little Cysters and Fibros! I apologize I have not updated my blog recently. I have received several e-mails asking me how I am doing, asking for blog updates, new pictures, and basically everything that has been going on with my life.

I have several blog entries saved in my "drafts" folder. The reason my blog entries are still in my drafts folder is because I have a lot of information I am excited to share with you, but I am trying to condense and simplify the information as much as possible.

I am constantly editing the information to make it easier to understand without significant knowledge of medical terminology and without being bored-to-tears. That said, I'm trying to avoid blog entries that read more like a medical book than a blog; this is something extremely challenging for me to do!

Lastly, if you would like to request a special CF related blog topic, please feel free to do so. I enjoy receiving feedback from my readers!


Please stay tuned, new blog entries are on the way!




 
Upcoming topics!
In No Particular Order
  • Got Anaerobes?
  • My First Portacath
  • Gastroparesis (Slow Gastric Emptying), Aspiration, Hemoptysis and Cystic Fibrosis--My Experience
  • Gastroparesis and (FDA/Non-FDA Approved Medications
  • "My Favorites" Links to CF Related Web Resources
  • An Educated CF Patient: Survival and Quality of Life
  • Importance of a "Healthcare Emergency Folder" and What You Should Include
  • Tips to Stay Organized at Home with Mountains of Medications, Nebulizers, Bottles, Equiptment, Storage and More!
  • Dear CF Doctors: A Patient's Thoughts
  • Your Fired! Doctors who Blame Your Physical Symptoms on a Psychological Illness Instead of Correctly Diagnosing Your Physiological Illness
  • Medical Record Inaccuracies and Misdiagnoses: Affecting Your Future Healthcare
  • Teaching Hospitals and Cystic Fibrosis: Tired of Teaching Residents and Nursing Students?
  • Importance of a Strong Support System