Tuesday, July 27, 2010
Thursday, July 22, 2010
I've spent the past few days on the phone, with I can't tell you how many different people, trying to get all of the drugs we need. Insurance doesn't want to cover some of them, and others are only available to us through mail order at this point. I'll fight the good fight with these people and get what we need. Its just been on my mind so I thought I'd share.
Tuesday, July 20, 2010
Monday, July 19, 2010
"How it is," for someone with cystic fibrosis, is that each breath is a small triumph. The genetic conditio churns out gluey mucus that will clog and inexorably shut down the lungs if not jarred loose and hocked up at least twice a day. That entails half-hour sessions of gentle thumps (parents of CFbabies call them "pitty pats") at more than a dozen prescribed locations on the back and chest, or wearing a vibrating vest powered by an air hose, or using a handheld "positive expiratory pressure" device. The sessions are punctuated with vigorous "huff coughs" to expel the sticky stuff. CF children learn the technique as toddlers.
There's more. The mucus blocks the ducts in the pancreas that release vital digestive enzymes generated by the organ. To prevent malnutrition, downing handfuls of enzyme capsules is mandatory before eating so much as a cracker or drinking a glass of milk. Many children still can't absorb the amount of nutrients they need, and must have a high-calorie supplement dripped into their stomach at night through a port in their belly or a tube down their nose. About half of those with the condition, including Lang, develop CF-related diabetes because of gummed-up ducts in the pancreas and have to take insulin injections. A who's who of deadly microorganisms love to multiply in the built-up mucus and cause lung infections. For patients who get repeat lung infections—like Lang, who spends a couple of weeks in the hospital for every six outside it—30-minute daily sessions at home, inhaling an antibiotic and tolerating the unpleasant aftertaste, are encouraged.
Even after 12 years of caring for CF children, Jamie Wooldridge, a pulmonologist in the Cystic Fibrosis Center at Cincinnati Children's, purses her lips and shakes her head in disbelief when she talks about the grueling regimen. "I can't think of another chronic illness that asks so much of families," she says. "Three to four hours. Every day."
The daily grind can never stop because it does not defeat the disease; it only blunts its force so that life can go on for CF children—or most of them. Every year scores die, falling short of adulthood however diligently their families work to keep them alive. And while several drugs now in clinical trials hold out the promise of a cure, their approval could be several years off.
Yet the larger picture also shows encouraging progress. The grip of the disease, which affects an estimated 30,000 U.S. children and adults, has loosened: In the early 1960s, children typically died before they were 10 years old. New drugs and improved care have pushed the median age above 37. The children are healthier, too. They play soccer. They join tumbling teams. They surf.
The pace of progress, moreover, is picking up. Over the past decade, CF specialists have increasingly embraced evidence-based medicine, "best practices," and other health reform tools. Several years ago, the Cystic Fibrosis Foundation, which for more than 50 years has funded basic research and development of drugs like those currently being tested, began bringing together teams from some of its 115 accredited centers, forming collaboratives to exchange information and encourage the spread of successful approaches. In 2006, the foundation took the almost unheard-of step of going public with outcomes data. Anyone can now look up average lung function and body mass index (BMI), the two key CF indicators, for every center's patients at the foundation's website.
When the numbers were released in 2006, the differences from center to center were immediately apparent. Cincinnati Children's, however, had known five years earlier that it had to improve. In 2001, the hospital persuaded the foundation to hand over performance data from a few top-performing centers, expecting that its own numbers would compare favorably. Instead, the figures described a troubling portrait of care that was mediocre or worse. Cincinnati Children's launched an unflinchingly critical self-examination. Top administrators and clinicians invited the parents of the hospital's CF children to a meeting. They displayed the stark data in PowerPoint slides, pledged to do much better, and asked the families to help. "We laid our dirty laundry on the table," says James Acton, director of the cystic fibrosis center at Cincinnati Children's.
Less than 10 years later—a blink in the life of a large institution—the hospital is now among the small handful of elite CFcenters. "They became some of the deepest students of the study of perfection," says Don Berwick, who as CEO of the Institute for Healthcare Improvement in Cambridge, Mass., which promotes practices that enhance safety and quality, frequently advised Cincinnati Children's as the hospital reinvented itself. (This month Berwick took over as newly appointed head of the federal Centers for Medicare and Medicaid Services.)
Cincinnati Children's is not alone in recognizing inadequate performance and vowing to do better; it was one of many hospitals that sought a "persuing perfection" grant from the Robert Wood Johnson Foundation to support its turnaround effort, for example. But it is telling that years before health reform became law in March, the hospital built its road to improvement using many of the same tools Congress wrote into the final package:
Evidence-based medicine. Detractors of EBM worry that it means studies and data and not a physician's accumulated clinical wisdom should dictate care. It doesn't, or shouldn't. The idea, rather, is that well-done research should help guidetreatment. For example, Pseudomonas aeruginosa is a nasty bacterium that has a particular affinity for the mucus in the lungs of CF patients and is responsible for a disproportionate share of their hospitalizations and deaths. Many children with cystic fibrosis show no symptoms of infection but repeatedly test positive for the bug. Given its lethal nature, it makes sense to damp it down to the lowest possible level, and many studies have shown that routinely inhaling the antibiotic Tobramycin keeps the bug at bay.
When Cincinnati Children's began to dissect its own performance and plot out how it could improve, the CF care team and quality analysts saw that the evidence for using Tobi (no one uses the drug's full name) with chronically infected kids was powerful and that the hospital wasn't even in the top quarter of centers in keeping patients on it. It's not an easy drug to use, eating up an hour or more a day with no obvious benefit because of the absence of symptoms. The hospital's doctors intensified their work to educate families on the benefits of Tobi even for children who did not appear ill. Parents were enlisted as partners, reaching out to other parents. (One of them, Tracey Blackwelder, was the mother of four CF children, all under the hospital's care. She is now on staff to help the hospital find similarly motivated parents to join "improvement teams.")
Progress in Tobi use was charted. Now prescriptions are written for about 80 percent of eligible children, says Acton. That compares with fewer than half of patients at about 1 in 7 centers nationally.
Nor had the hospital previously been especially diligent in making sure children with low BMIs received extra calories by mouth or through a tube or port. In 2004, 61 percent of its low-BMI children, almost exactly the national average, got supplemental feeding. The national average now stands at 71 percent. The Cincinnati Children's rate is 91 percent.
Collecting and sharing data. Berwick likes to say that if you want to do better, you first have to know how you're doing. You start tracking the percentage of CF children year by year who get a flu shot, for example, and their average lung function. Then, he says, you make the data available to the public and to other care providers so the top and bottom of the range of performance are identified. That allows you to set realistic benchmarks and goals in a way for all to see.
Gerald O'Connor, a professor of medicine at Dartmouth Medical School and chair of the Cystic Fibrosis Foundation committee that drew up the 2006 plan to display centers' clinical performance, says that opening up the data was "a game-changer," because the centers could observe for themselves that the ranges were too large to explain other than by differences in each center's approach to care. "What people were doing was wildly variable," he says. Lung function figures, for example, showed that children at a few aggressive centers were breathing, on average, as well as children without the disease. These centers hounded families to schedule outpatient visits and reminded them when one was approaching.Lung infections were reduced by broad use of proven medications and successful fluimmunization campaigns.
When the staff at Cincinnati Children's realized in 2001 that average lung function for its CF kids from 6 to 17 was less than 80 percent of normal, trailing the national average of about 84 percent and the top center's 93 percent, they increased the frequency of lung infection checks to every three months instead of once a year. Parents were taught new and better ways to clear their child's airway. Reminders to get flu shots went out. Today, national average lung function has improved to about 93 percent. At Cincinnati Children's it has rocketed to a better-than-normal 102.5 percent.
Learning from other centers. CF center director James Acton examines 8-year-old outpatient Raegan Holley and asks her mother, Dawn, 15 questions displayed on the nearby monitor. Raegan will receive a "pulmonary exacerbation score," or PES, of 0 to 38 according to the number and severity of her currentrespiratory symptoms (such as increased cough for a week or more) and objective measurements (such as blood oxygen level). A perfect PES is 0. Five or higher suggests that she needs a change in her lung-clearing technique or a more effective antibiotic.
The PES score sheet is Acton's favorite example of how the hospital worked with another center to benefit patients. The sheet, developed at Akron Children's Hospital, caught Acton's eye when the director of its CF center presented it at the annual meeting of the North American Cystic Fibrosis Conference conference. When Acton returned, he shared it with the hospital's CF team. The Akron center then helped its Cincinnati counterpart document the extent to which evaluations of outpatients varied from physician to physician, resulting in more- or less-aggressive care depending on each doctor's clinical preferences. "They didn't believe it at first when I showed them the results," says Acton. By standardizing assessments, he argued, care would be improved. And that, he says, is what happened.
Defining ambitious goals. Although Cincinnati Children's now outpaces most other CF centers in the nation, its clinicians and quality specialists are not satisfied. "There's incremental improvement every year," says Bruce Marshall, the Cystic Fibrosis Foundation's vice president for clinical affairs. The hospital's 2011 goals include pushing lung function up a point—to 106.3 percent for kids from ages 6 to 12 and to a lesser but still ambitious 100 percent for those 13 to 17. (As children reach adolescence, many become less faithful to the critical daily routines.)
Nutritional status, CF's other key indicator, now stands at 60 percent for the hospital's CF patients, meaning that 60 percent of the children are at or above the 50th percentile, or average, for BMI. The current national average is slightly above 48 percent; Cincinnati Children's is aiming for 62 percent in 2011.
Improved performance for children with an elevated PES aims even higher. At the moment, a tally of 5 or higher on a clinic visit produces a documented change in the child's care plan about 40 percent of the time. The 2011 target is to double that to 80 percent.
Collaborating with families. The healthcare reform law calls for providers of medical care to involve patients, parents, and other caregivers in "shared decision making," with each a full partner in charting a course of action. That families should actively take part, with their views on appropriate care considered as important as those of the doctors, remains a foreign notion at most hospitals. Not at this one.
Annelise Page was labeled an urgent nutritional risk when she was 7 years old; her BMI was in the lowest 10th percentile. The family and the center's physicians and dietitians had "many difficult discussions" about the best way to help her, says Acton. They "were very clear about balancing quality of life with the need for sometimes intrusive interventions" such as a feeding-tube port into her stomach, which Annelise rejected. ("That won't look good with a bikini.")
Meanwhile she remained at risk. "She tried to consume three to four thousand calories a day but she just couldn't do it," says her mother, Honor Page. "She got so sick of butter and ice cream. She would plead with me for a salad. People really have no idea how hard it is."
About two years ago her doctor, Jamie Wooldridge, brought up the possibility of a nasogastric feeding tube, which would have to be snaked through the girl's nose every night to drip predigested nutrients directly into her stomach. Annelise agreed to give it a try. She mastered the technique, which soon became just another part of her nightly routine. And she gained 30 pounds, topping the 50th percentile. Now 16 and on her school tumbling team, Annelise tracks her BMI on a chart on her bedroom wall. "They listened to her," says her mother. "They went into their bag of tricks and thought, 'What can we offer this family?' "
Adopting electronic records. Health information technology has benefited Cincinnati Children's patients most directly in the form of My Care Connection, a secure Web page that lets families track a child's progress and stay on top of the latest medical plan. There are word descriptions and graphs of the child's most recent lung function data, results of lab and imaging tests, and an updated list of medications and dosages. Parents can put questions to a child's care team and expect answers within a day. "It is a critical philosophical step forward," says Acton, "in transferring control of their healthcare from us to them."
Friday, July 16, 2010
First guess (and my favorite) is that it isn't bacterial, and that's why antibiotics aren't helping. They are treating for the "worst case scenario". In the CF world, bacteria in the lungs is "normal" to some extent. They had believed that there was maybe more bacteria in his lungs than they had originally thought and that a stronger IV antibiotic would help to eradicate anything living in there. Because its not getting better with stronger antibiotics, they think that maybe its not bacterial but viral and will just have to work itself out. That would be great news if it weren't a CF problem but just a bad cold that hes taking a little extra long to get over!
The virus they are actually leaning toward thinking he has is bronchialitis now. Basically, its bronchitis of the lower airways. It happens in everyone, not just CF people, and it sounds bad but its just a virus and will work its way out. Anything that is not bacteria festering in his lungs is okay with me.
You might be wondering why they can't just figure this out and treat it. The thing is that they can try, but it wouldn't be the least minimally invasive process. In older kids, they are able to cough up some of what they have and send that off to a lab to be analyzed to see what bacteria exactly is living in the lungs. In babies like Drew, he regularly has the back of his throat swabbed to determine what, if any, bacteria is present. This is a fairly good way to see what is in his lower airways. If they truly want to see whats happening down they, they need to take him to the OR, knock him out, intubate (as in ventilator), then send something all the way down to his lower airways to collect a mucus sample to send to the lab to see what hes got. Again, the throat swab provides a pretty good view of whats happening down below, but its not 100%.
So based on other kids and the way he sounded when we went in on Monday, they treated for "worst case scenario" which would be bacteria they didn't see from the swab that was growing in his airways and would be rid by stronger antibiotics. Since its not working, they are just trying to figure out what path to take next. They kind of want to rule out anything and everything bad before they say its just a virus, lest it get worse, but they also don't want to do any unnecessary procedures.
I love them at Childrens. I really really do. They care about him and about us. His CF doctors come in to see us several times a day and sit with us to brainstorm and ask questions and see what we think about his care and whether hes getting better or worse and they listen to us and I just love them. Now I just want them to pinpoint whats going on so we can get the H outta there and come home.
This leads me to another topic on my mind which I think I wrote about once before - germs. This hospital stay changes the story for me. I am paranoid and I'm allowed to be. Judge me for it if you want, I don't care. My baby has CF and germs can make him sick, very very sick. I'm going to be cautious. You can't come into my house unless you are willing to use hand sanitizer upon entry. You can't hold Drew. Sorry. If you have, had, or are getting a cold, please don't come over. He will get colds, he will get sick, but if we can avoid it we will. I ask for your consideration. I'm not just a germaphobic paranoid mama. I'm the mama of a very sick little boy who I cannot imagine living without.
I think when we first found out about the CF diagnosis we were both uneducated and a little naive. It is a scary scary horrible disease. The reason the average life expectancy is only 37 is because there are people who die with it when they are young. We are hopeful that the new drugs on the horizon will change thing. Another blogger friend said it perfectly the other day - "Tears flow to think about never having to hear the words "Life Span" again!". But right now, today, I realize the severity of this awful disease. Drew is 4 mo. old and has spent 6 weeks of those 4 months in the hospital...because of CF. It is robbing him of his childhood and us of our baby. I don't want to lose him, ever. I want a cure. Our hope, of course, is that he does lead a healthy, happy, normal life. And right now the only way that I know to ensure that is to keep him as healthy as I can.
So that's where I'm at. Now I'm going to bed. Spending 10 hours in a 10x10 room with two 4mo. olds takes a lot out of a person :)
Wednesday, July 14, 2010
Monday, July 12, 2010
He hasn't got some life threatening cold (disease, yes; cold, no) that is going to bring him down. Hes simply got a ton of mucus in his throat and lungs and belly and they need to make sure that whatever bacteria is causing it gets taken care of before it sets up shop and starts to cause lung damage. There were several options and the IV antibiotics is where we are starting. Hopefully by this weekend he will sound much better and "cough-free" will be his baseline by which we can measure future colds and infections.
Friday, July 9, 2010
Wednesday, July 7, 2010
- The 65 Roses Canoe and Kayak Trip - July 18 - Green Acres Canoe Rentals
- Caving for Cystic Fibrosis - July 31
- Masquerade Ball & Art Auction - August 27 - Brylan's Coffee House Cafe' in Newtown
Saturday, July 3, 2010
Thursday, July 1, 2010
We had discussed with Drew's doctors the course of action that would be taken when he did get sick. They told us that they treat based on symptoms and that not ever cold would turn into a problem. They said that we would should just keep an eye on things for about 5 days, and if they weren't getting better or started to get worse that they would want to see him.