Cystic Fibrosis Podcast 196: Managing CF as a Track Athlete
Cystic Fibrosis Podcast 197: Moving from Middle School to High School
Cystic Fibrosis Podcast 196: Managing CF as a Track Athlete
Cystic Fibrosis Podcast 197: Moving from Middle School to High School
By Michele Wilson PhD
The buildup of mucus in the lungs is an ongoing challenge faced by people with cystic fibrosis, and knowing whether they should seek medical attention is not always clear.
Recently, Mologic – a developer of personalized diagnostics – have developed a tool which they hope will help guide people with cystic fibrosis so they can avoid unnecessary stays in hospital.
The app-embedded algorithm converts data collected from a urinary test to a traffic light result, which indicates whether a patient is stable or in need of medical intervention.
Recently, Mologic, announced that they are launching a clinical trial to assess the company’s urine-based diagnostic tool, ‘HeadsUp’.
To learn more about how this point-of-care diagnostic tool could help improve healthcare for people with cystic fibrosis, we spoke with Gita Parekh, Head of R&D at Mologic.
How do you define pulmonary exacerbation, and why is it important that it is monitored in people with cystic fibrosis? Continue reading Monitoring Pulmonary Exacerbation in Cystic Fibrosis: The Hunt for Urine-based Biomarkers Begins
A note from CF Roundtable: Please do not stop using your Vest or other HFCWO device because of this impractical study. There are important differences in this study that make it not applicable to CF and therefore, not meaningful for us. First, healthy volunteers without CF were enrolled. Too many adults with CF have experienced significant benefits with these HFCWO devices, not to mention the preventive benefits. To imply these devices worsen lung function when used by a healthy nonCF person vs one with CF – with the usual accompanying inflammation, mucus +/- bronchiectasis, etc – is not practical. Second, these healthy subjects tested all 4 HFCWO devices in one day. Even when I repeat spirometry in one day, my lung function numbers most often decline over time, even with rest periods in between. The researchers tested lung function with a rest period of 15 minutes on these healthy individuals after use of the HFCWO device then moved on to the next device. Very impractical and again – not applicable to use by individuals with CF.
By Iqra Mumal
A clinical study into high-frequency chest wall oscillation vests — assessing their short-term impact on standard measures of lung function before and during use — challenges the view that these devices work through airflow bias in the lungs, the process responsible for mucus movement when breathing.
Findings, using established tests that include forced vital capacity (FVC), forced expiratory volume (FEV1), and forced expiratory flow (FEF25%-75%), suggest “that the concept of HFCWO vest-induced cephalad airflow bias is not supported by standard spirometry measurements,” researchers concluded. “None of the vest groups showed statistically significant increased airflow in the lungs.” Continue reading Airway Clearance Vests Fail to Show Measurable Short-term Lung Benefits in Study
By HH Patel, M.Pharm
Similar to spirometry and pulse oximetry, lung plethysmography is a tool within the broad umbrella of pulmonary function tests. It is a type of diagnostic procedure used to measure the amount of air in the lungs after inhalation or exhalation.
Also referred to as body plethysmography, lung plethysmography provides an idea about how efficiently the lungs are functioning. It is also especially useful in characterizing types of pulmonary abnormality and implementing appropriate treatment approaches depending on the diagnosis.
By Valerie Waters and Keith Grimwood
Cystic fibrosis (CF) is a genetic, multi-system disease due to mutations in the cystic fibrosis conductance regulator (CFTR) gene, leading to ineffective anion channel activity . The resulting impaired mucociliary clearance permits initial acquisition of Pseudomonas aeruginosa and, if untreated, the establishment of persistent infection in the CF airways. It has long been recognized that chronic infection, often characterized by a mucoid P. aeruginosa phenotype, is associated with more rapid lung function decline and earlier death in individuals with CF [, , ]. Defining chronic P. aeruginosa infection is, therefore, an important step in identifying CF patients most at risk of lung disease progression. Traditionally, the Leed’s criteria has been used to define chronicity (as having >50% of sputum cultures being P. aeruginosa positive in the preceding 12 months), as it is the only clinically validated definition . However, the Leed’s criteria are difficult to implement in young children unable to provide sputum and further limited by the required number of sputum samples and follow-up time .
In this issue of the Journal, studies by Heltshe et al. and Boutin et al. aim to re-define what chronic P. aeruginosa infection means in CF. In a retrospective cohort study using data from the US CF Foundation Patient Registry, Heltshe et al. followed close to 6000 early-diagnosed CF children for approximately 6 years . Two-thirds acquired P. aeruginosa infection and of those, 6% had an initial mucoid phenotype. Furthermore, the majority (87%) of children who developed mucoid infection did so before meeting the definition of chronic infection (at least 3 yearly quarters P. aeruginosa positive in the preceding year). Initial P. aeruginosa infection with a mucoid phenotype has been previously described and is a recognized risk factor for failure of antimicrobial eradication therapy [, , ]. Whether this initial acquisition of a mucoid phenotype represents prior adaptation of P. aeruginosa in the CF host (either undetected or transmitted from a patient with chronic infection) or simply infection with an environmental strain particularly well-suited to the CF airways, is as of yet unknown . It is clear, though, that mucoid P. aeruginosa does have an adaptive advantage in early CF infection as mucoidy was associated with an almost three-fold increased risk of transition to chronic infection in this current study. Despite the presence of this risk factor, however, only 13% of P. aeruginosa infected patients went on to develop chronic infection. Although Heltshe et al. did not provide details as to eradication strategies used in this cohort, this low incidence of persistent infection does speak to the overall effectiveness of current antimicrobial treatment for early P. aeruginosa infection.
Boutin et al. took their investigation a step further by using molecular methods, specifically quantitative polymerase chain reaction (qPCR), to define chronic P. aeruginosa infection . In their study, patients with chronic infection had significantly higher levels of P. aeruginosa as measured by qPCR compared to those with intermittent infection. A single P. aeruginosa qPCR measurement in sputum had a sensitivity of 84% (with a specificity of 85%) in detecting chronic infection using a threshold of 103.4 colony forming units (CFU)/ml. A single sputum PCR measure had the advantage of not requiring 12 months of culture results as per the Leed’s criteria . Furthermore, in their small study sample size, PCR was more discriminatory than mucoidy status in predicting chronicity, not surprisingly, given that alginate production (conferring mucoidy) is only one of several virulence factors contributing to the establishment of persistent P. aeruginosa infection in CF . When used in throat swab samples, qPCR had a considerably lower sensitivity (82%) and specificity (56%) in detecting chronic infection, likely due in part to the lower bacterial burden observed in this specimen, compared to sputum. The low specificity of PCR in this setting (positive PCR, negative culture) may reflect the fact that a molecular signal may precede culture positivity. Early detection of P. aeruginosa infection, before culture conversion, in CF patients was originally suggested decades ago using serologic and, more recently, molecular methods [, , ]. Serology, however, has proven disappointing at identifying early P. aeruginosa infection . Nevertheless, early detection may still be possible using highly-sensitive PCR techniques for identifying lower airway P. aeruginosa infection in a young, non-expectorating child. In the study by Boutin et al., P. aeruginosa detection in throat swabs by PCR alone was linked to a positive culture in sputum in three-quarters of cases. Previous studies comparing oropharyngeal cultures to bronchoalveolar lavage (BAL) cultures in children with CF demonstrated that oropharyngeal cultures had a positive predictive value of only 44%, but a negative predictive value of 95% in diagnosing lower airway P. aeruginosa infection . Performing P. aeruginosa qPCR on culture negative throat swabs may further improve the diagnosis of lower airway infection in young children with CF who are unable to produce sputum, but this approach will still need to be validated by comparative studies employing BAL fluid samples. Unfortunately, using confirmatory induced sputum samples as suggested by Boutin et al., may produce unreliable results as these specimens are poor predictors of lower airway pathogens cultured from BAL specimens in young children with CF . Finally, it is yet to be determined whether an earlier diagnosis of P. aeruginosa infection leads to improved eradication success rates and superior clinical outcomes.
In summary, the recent studies by Heltshe et al. and Boutin et al. further our understanding of how chronic P. aeruginosa infection develops in CF and how to better recognize it [7,12]. Ultimately, prevention of chronic P. aeruginosa infection and its deleterious effects on lung function and survival is the goal.
Original article in Journal of Cystic Fibrosis here.
By Ella Balasa
Looking up at the rising wall of stone, sweat droplets beading on my forehead, I think about the hundreds of steps between me and the top of the walls of the city of Dubrovnik, Croatia. I want to see the view from the top, but I feel the discomfort of what-ifs welling inside me … what if I hold up the line going up the stairs because I need breaks? What if I pass out from shortness of breath? What if my lung collapses again from taking such heavy breaths with only 25 percent FEV1?
Those were my thoughts last August during my European adventure.
The first six months of 2017 had been difficult. I had three surgeries — each two months apart — on my lung because of a reoccurring lung collapse. I spent weeks in the hospital and then weeks recovering at home. I went from barely walking around my house to building up the strength to walk on the treadmill for 30 minutes a day, only to restart the process each time after the next two surgeries. There were moments I never thought I would get stronger, that I’d be confined to my house with 24/7 supplemental oxygen, chained to an oxygen concentrator that allowed me to breathe.
Slowly I got stronger and — after the third surgery — the lung held. I had been planning this trip since before my medical issues began, and I wanted to make it a reality. I already had to cancel a trip to Vegas for my 25th birthday and a Fourth of July get-together with my best friends. I would be heartbroken if I had to add this trip to that list.
In the days leading up to my trip, the fear of another lung collapse (pneumothorax) still terrified me. A pneumothorax occurs when air is trapped between your chest wall and your lung. This trapped air pushes on the lung, allowing less room for the lung itself in the chest cavity, thereby collapsing it.
When there is a decrease in air pressure at higher altitudes, air molecules expand, occupying more space. Because of my history of lung collapses, there was a chance that I might have a slight air pocket between my lung and chest wall. If so, the altitude change in an airplane could have expanded this air pocket, making the collapse much larger and dangerous.
Some might think it’s too risky to travel outside the country if you have a chronic illness, where the possibility of needing medical attention is high, and the constant awareness of symptoms and management of medications and treatments are a necessity.
There certainly are times when the risks outweigh the benefits. In my situation, there will always be a risk, but the level of potential pleasure to be gained makes an attempt worthwhile.
Being prepared was important and eliminated some of the anxiety associated with travel. It was also necessary to relax about the parts that were not in my control.
I made sure I had my flight insured and bought travel insurance, and I carried the documents with me. I counted and packed the amount of medications I would need, plus extra.
I did not worry about packing light. I require the amount of luggage of a family of four. In the past, this has embarrassed me. We all stereotype women and their extra bags, but I need: A rolling luggage bag for my vest, a roller for my oxygen concentrator, my suitcase of clothes and personal products, and a carry-on backpack of medications. I do not check any of my nebulizing medications and machine, inhalers, enzymes, and antibiotics in case my suitcase gets lost. These are the items I have to have, and it would be a nightmare tracking them down in a foreign country.
I opted for special services through the airline for assistance with getting from one gate to the next between flights and to help carry heavy bags. Having 25 percent lung function, it’s tiresome to walk distances, and it’s not possible for me to carry anything remotely heavy. This was the first time I had used this service. I’ve never liked being seen as different or needing special accommodations. However, I have realized, as my disease progresses, that doing everything everyone else does is not always possible, and it’s OK.
And, it turned out to be a wise decision. As I got off one of my flights, I was met by an airline employee with a wheelchair and a sign with my name. I had 20 minutes before my next flight was to depart JFK airport in New York, and my gate was at the other end of the terminal. With only 10 minutes to go, this gentleman started running as he wheeled me through the airport. By the time we got to the gate, he was profusely sweating. I was the last one to board! I would have missed my flight without this assistance.
First, I learned to be comfortable with strangers seeing me doing CF-related stuff, like wearing a mask and using an oxygen concentrator on an airplane, and doing a breathing treatment on a park bench, while coughing and spitting into tissues. Here is a picture of me doing exactly that in Split, Croatia.
Guest post by: Kathy Russell
Today was a terrific day! We have just experienced a three-day series of high temperatures in the 80s. In the middle of summer that would be quite normal, but getting that kind of weather in April is extremely rare in my part of Oregon. I made the most of those days.
In our front yard, we have a very old and very large black walnut tree. There is a brick planter built around the base of the tree and there are various plants, including several sword ferns, growing in it. The sword ferns are beautiful if they are properly groomed. Each year, when the weather permits, I get out and cut away all of the old fronds to make room for new growth.
I didn’t get to do that last year because of my health. I just didn’t have the energy to do the bending and twisting that the task entails. Also, since I am on continuous oxygen, it makes getting out to the tree a bit problematic. Dragging my portable oxygen concentrator (POC) while I am pruning the ferns is a bit of a pain. I bend over and cut some old fronds, then I have to stand up and drag the POC to my next position. It makes it more of a chore and a lot less fun than it used to be before I was on oxygen.
Yesterday I worked on a couple of ferns that I could reach with the length of hose that is on my big concentrator. That was fairly workable, but I couldn’t go beyond the range of my hose. Also, I couldn’t reach two of the ferns. After about an hour I was getting too hot and decided to stop working on a large fern that was at the end of my tether.
Today, my husband took my large concentrator outside and plugged it into an outdoor outlet. With the 75 feet of hose that I have on it, I had a lot of freedom to move around. I was able to finish the fern that I left yesterday and finish the final two. I didn’t have to worry about running out of hose length and I felt so unencumbered. It was so great to be able to move around like a normal person. I absolutely loved that feeling of freedom. It was almost like not even being on oxygen.
My oxygen saturation stayed in a very good range and I got a couple of hours of fresh air. I was mostly in shade so I didn’t have to worry about being in the sun too long. Having the ability to move around and not have to drag a POC was a real gift as far as I am concerned.
By: Diogo Pinto
Researchers have linked variations in the mix of microorganisms in cystic fibrosis patients’ airways to their disease outcomes.
The findings in the journal PLOS One were in an article titled “Fluctuations in airway bacterial communities associated with clinical states and disease stages in cystic fibrosis.”
CF patients typically have particular strains of bacterial and fungus in their airways. The usual bacteria suspects include Pseudomonas, Achromobacter, Burkholderia, Haemophilus, Staphylococcus, and Stenotrophomonas.
Other bacteria and fungi also inhabit CF patients’ airways, however. These include anaerobic species that do not need oxygen to grow and spread.
Not only do the microbial communities in CF patients’ airways vary by type of microorganism, but also in the relative abundance of each species.
Researchers decide to see if the prevalence and relative abundance of typical CF pathogens and anaerobic microorganisms play a role in the severity of patients’ disease and their lung function.
They analyzed 631 sputum samples collected over 10 years from 111 patients.
The team classified the stage of patients’ disease on the basis of their lung function scores. The yardstick they used was forced expiratory volume in one second, or FEV1. They considered an early stage of the disease to be an FEV1 score higher than 70, an intermediate stage a score of 40 to 70, and an advanced stage a score lower than 40.
Researchers classified disease aggressiveness — mild, moderate or severe — on the basis of change in FEV1 relative to age.
They discovered a link between variations in the prevalance of the six typical CF pathogens, plus nine anaerobic species, and changes in a patient’s disease stage and lung function.
To continue reading, click here.
By: Sydna Marshall
A few weeks ago I found myself in the ER at midnight in a new city. Let me backup the story a bit. I had planned a road trip to Dallas with my bestie (we’ll call her “A” for now) for a beauty conference. Two days before the trip I ran a 102.6 fever for the better part of the day. The morning of, as I was doing my treatments and finishing my packing, I felt the familiar band of pain around my chest. I mentally ruled it out as merely remnants of pain from my blockage earlier in the week and carried on with my morning. I drove the hour north to pick up A and start our trek up to Dallas. We stopped for tacos and queso along the way. I took some pain meds for that persistent and annoying band of pain. A few hours later, we checked into our hotel, picked up our beauty boxes for the conference, and had a happy hour cocktail in the bar followed by sushi in the rotating tower.
Fast forward to 9p: CF changes on a dime. Suddenly, that pesky band of pain that I’ve absently noticed and ignored for the day is front and center. I can barely take a breath, much less a full breath, post-treatment. By 11p, I’ve laid in bed silently crying as the pain spikes up to an 8 and then back to a 6. It dawns on me that the band of pain is pleuritic pain. After texting multiple Cysters and weighing the pain with the inability to breathe, my recent 20% drop in lung functions and the fever I ran earlier, I finally make the decision to wake up A (who is for once sleeping peacefully, without interruptions, in the absence of her four kiddos) and have her drive me to the ER.
We arrive at the ER with this naïve idea that my CF clinic, albeit on-call at this late hour, will communicate with the CF clinic in Dallas. I’d already given A all of the information, phone numbers, and instructions for getting everyone, including my husband Adam, on the same page. Over the course of the evening and early morning hours, hundreds of texts and calls between A, Adam, and the on-call care team at home transpire in an effort to expedite the process. Since it’s not my first rodeo with pleuritic pain, I’ve already determined before we even got settled in the ER that I desperately need instant-relief pain meds and a chest X-ray. Am I the only one who self-diagnoses? When you’re in and out of the doctor for the litany of health problems in addition to CF, you become the expert on your own body. I digress.
Over the course of the 12 hours in the ER, my port is accessed a total of four times, with one of them being a needle repositioning, before we get anywhere. To administer IV medication and run blood tests, two different nurses start dueling peripheral lines, one in my left hand and the other in my right elbow. Meanwhile, other nurses attempt to get my port working, which won’t flush or draw back blood. My vein blows on one of the lines, and the other is dangerously close. I have a chest X-ray taken, a CT scan with contrast of my lungs, every blood test imaginable, an EKG, several rounds of morphine, two doses of vancomycin and two albuterol treatments. I’m told I have a potential pulmonary embolism, a virus causing pleurisy, a mucus plug, or sepsis. Twelve hours in, and about 10 minutes after Adam arrives at the Dallas ER, my repeated requests to be moved to my home clinic, care team, and hospital are heard and I’m care-flighted from Dallas back home (Adam has to drive back home). Once admitted to my home hospital, they have me repeat nearly every test the Dallas ER did less than 24 hours prior as none of my medical records transferred with me from the ER. Five days later the medical records from the ER finally make their way to my home hospital and care team. In the end, it was determined that I had a virus, which accounted for the difficulty in breathing, pleuritic pain, and fever. It was a very long, traumatic, stressful, and a trying 12 hours away from home. And, I missed my conference entirely, but that’s another story.
I’ve since had some time to reflect on this jaunt to the ER. The biggest takeaway for me – CF clinics do communicate but getting the ER to communicate with the CF care team is nearly impossible. Having a port is a blessing, but it requires orders from your doctor, not just any doctor, to access and use heparin or cath flow in the event that it’s not working properly (or, in my case, repeatedly accessed incorrectly). I learned that complaining of chest pain at a new hospital where none of my medical records are accessible means a round of tests to rule out heart problems, despite knowing that it’s my lungs. I learned that transferring medical records from one hospital to another is a royal pain in the you-know-what.
Hindsight is always 20/20, but I know I could have avoided the entire debacle if only I had heeded my inner voice the morning I left for Dallas when I first felt the band of pain around my lungs. For me, it’s often hard to gauge when it’s important to say no and upend plans, especially when it impacts friends and family around me. If a trip to Walgreens completes a vacation in my house, am I an overachiever for my trip to the ER?