Tuesday, July 27, 2010

Random Things

We had another check-up at the CF Clinic yesterday. It seems that all doctors are in agreement that Malacia is what's causing this sound Drew is making and not CF. That's good news, I guess. They are still going to do a bronchoscopy sometime soon to look into his airways with a scope and identify for certain that this is the problem, but all signs point that way. Its just something that he will need to grow out of. As he gets a little older, the cartilage that makes up his trachea will get harder and he will no longer make the sound hes making. If any other kid without CF would have malacia, they would sound the same way Drew does. So basically, if you hear him and think "Dear Lord that kid sounds atrocious", know that he is not sick and will grow out of this.

We also got some other great news at the clinic. Drew is in the 80th percentile for height to weight! The goal of the CF Foundation is for kids to reach the 50th percentile by age 2 so hes doing fantastic. You might be thinking "So what?" but as a refresher, weight gain with CF can be very difficult as the pancreas cannot produce the enzymes to break down the fat in food and therefore the body cannot hold onto it, making weight gain difficult. We work very closely with our dietician to monitor Drew's caloric intake - calories per ounce, ounces per bottle, and bottles per day - so that adjustments in the amount of enzymes he takes can be made to ensure proper fat and nutrient absorption. Our dietician is a rock star. I talk to her several times a week about how Drew is doing, what his poop looks like (shiny = fatty = malabsorption) and how many pills he needs per bottle. Over the past 10 years, research has shown that children who have better weight gain and BMI have better lung function tests when they are older. Their growth really strongly correlates with their health overall. So the fact that hes already in the 80th percentile makes me SO so happy. Hopefully this will be good news for the future.

I asked if he could get to fat and the answer was basically no. If he was at like the 99th percentile when he was 2 and was so huge that he couldn't move and play, then it would be come a problem, but right now hes just a pudgy little baby boy that very few people thought he'd ever be, happy as can be. He is just about 3 times bigger than he was when we left the hospital 3 months ago (he was 1mo. old the day we got to go home).

One more thing. I was reading a blog the other day of another mom with a child with CF, and she had a story posted that I wanted to share. Her daughter was in the hospital for 5 months after she was born and when she finally left the hospital she had a g-tube. She and her family participated in the Great Strides Walk, as we did this past spring, and at the event she had the opportunity to talk with someone from the foundation. They spoke with this woman about what hope there was for finding a cure. The woman from the foundation told her that the only hold-up was money. MONEY!! I had mentioned early on when I had started this blog that CF is considered an orphan disease and therefore receives NO FEDERAL FUNDING, relying only on donations from individuals and corporations to fund their research. Lack of money has shut down numerous clinical trials, one of which could have changed our life. There are several very promising, very life changing, life extending drugs in the pipeline right now, but without support they may never come to fruition. We thank everyone so so very much for all of their support this past year during the Great Strides walk, and ask for your continued support of the CF Foundation and of Drew as we continue to fight this battle until we win.

Thursday, July 22, 2010

Pill head

Drew takes over 300 pills a month. Thats of 1 medication - Creon. We will go through 150ml of Prevacid, 90 vials of Albuterol, 30 vials of Pulmozyme, 30ml of Vitamax, 6ml of Iron, and 150ml of Sodium in the next 30 days. We have also been on 2 different antibiotics, steroids, and inhaled saline in the past 3 months. And it doesn't look like its going to get any better. I know he needs all of these, but its still a little scary to think of someone so little being on so much medicine.

I wish he didn't need any of this. There is some hope with 2 new drugs and a gene therapy currently in the pipeline with the CFF. I think I wrote about this before, but I'll talk about it again. Here's a little bit of info from the CFF website on these two promising treatments.

GENE THERAPY (This would be a dream come true!!!)

Because a faulty gene causes cystic fibrosis (CF), adding normal copies of the gene to cells could correct these cells and ultimately cure the disease. This approach is exploring ways to introduce normal copies of the gene into CF airways.

  • Compacted DNA (PLASmin™): Copernicus Therapeutics, supported by a CFFT TDA and the TDN. Using compacted DNA (non-viral) to introduce normal copies of the gene into CF airways. A Phase 1a trial demonstrated chloride current changes in the noses of CF patients, but no evidence of gene expression. The gene therapy product is being reformulated prior to additional clinical trials in an attempt to improve the amount and duration of gene expression.

CFTR MODULATION (CFTR - Cystic Fibrosis Transmembrane Conductance Regulator)

(This is a little further down the pipeline and has been showing promising results in the clinical trials. This would essentially turn CF from a life-shortening disease to a chronic disease, like asthma)

These therapies are designed to correct the function of the defective CFTR protein made by the CF gene, allowing chloride and sodium (salt) to move properly in and out of cells lining the lungs and other organs. (no more thick mucus = no more nasty bacteria!)

  • VX-770: Vertex Pharmaceuticals – VX-770 is supported by CFFT and is a new compound called a "potentiator" that may act upon the CFTR protein and help to open the chloride channel in CF cells. Phase 1 dosing has been completed in healthy volunteers and CF patients. A Phase 2 trial in CF patients with at least one copy of the G551D mutation in their CF gene demonstrated improvements in biological measures of CFTR function (nasal potential difference and sweat chloride) and clinical measures of pulmonary health (FEV1). Two Phase 3 studies (one for pediatric and one for adolescent/adult patients) began in Summer 2009.

  • Ataluren (formerly known as PTC124): PTC Therapeutics – A novel, small molecule compound, that promotes the read-through of premature truncation codons in the CFTR mRNA. It aims to treat CF patients who have what is known as a "nonsense mutation." It has been demonstrated to be safe, orally available and well tolerated in a Phase 1 single-dose trial in healthy volunteers. A Phase 2 trial in CF patients conducted in the United States and Israel demonstrated safety and encouraging biological results. The Phase 3 trial began in Summer 2009, which is a 48-week study of ataluren in people with CF age 6 and older. The main goal of the trial is to find out if ataluren can improve lung function in people with the disease.

  • VX-809: Vertex Pharmaceuticals – VX-809 is supported by CFFT and is a new compound called a “corrector” that helps move the defective CFTR protein to the proper place in the airway cell membrane and improve its function as a chloride channel. A Phase 2a trial began in Spring 2009.
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.

Throw me a bone

I'm ready to catch a break. We went to the ENT today and she really feels that Drew has Tracheomalacia or something else in his airway making the sound that we're hearing so we're going to set up a bronchoscopy. She said it will only take about 30 minutes for her part, but since they're going in, the pulmonologists want to get a sample of the mucus from way down low in his lungs and their part will take an additional 30. Add the time for prep and recovery, he will be away from us for probably about 2 hours. I hate that damn surgery waiting room.

We have a clinic appointment on Monday and I'm anxious to ask our doctor what tracheomalacia means in terms of the CF. I'm a little worried that it won't be good news. I'd even hope for no news, meaning that it has exactly zero affect. However, I think what I'm going to hear is that its a problem because he can't clear that thick mucus affectively. That awful, thick, sticky mucus that bacteria just loves to grow in. The ENT said that if it is tracheomalacia that he should grow out of it by the time hes 2 at the latest. But in my CF thinking head, thats 2 years of bad bacteria not being affectively cleared from his airways which can't be good news. I hope thats not the case. I really really hope its not.

This is what I fear. If they go in and they do determine that its tracheomalacia, I fear we will have hospital stays with every cold. I fear we will be doing airway clearance 3-4 times a day, with extra albuterol and pulmozyme and maybe even courses of inhaled antibiotics. I fear that its just more time spent on treatments and in the hospital where he can't grow and play and exists the way any other kid can. It means less time for our girls. It means more financial stress. It means more heartache.

I hope I'm over thinking this all. I just want to know what the worst case scenario is. I don't want to pretend everything is fine. I'm worried about this. This event. This whole thing. I'm just ready to catch a break.

Tuesday, July 20, 2010

We're Home!

Well, we're home! This is how we got here...

About 3 weeks ago, the whole family came down with a cold. Within a few days, everyone was over it except for Drew. We had been told from the get-go that he wasn't any more susceptible than any of the other kids to getting a cold but would almost certainly have more trouble getting over it, and thats exactly what happened. So he was put on oral antibiotics at home to try to kill any bacteria that may have gotten in there and was causing this phlegm to linger. After 10 days [give or take] with no audible improvement we took him back to the doctor who decided that IV antibiotics would be necessary to kick whatever was causing this horrific sound.

So into the hospital we went. Drew's doctor wasn't on service in the hospital so we had a different doctor from the clinic. He said that it sounded like bronchiolitis. Based on their assessment from listening to him breath and looking at his vitals and general demeanor, the general consensus from the team (doctors, fellows, residents and nurses) was that a few days on the IV antibiotic would make him sound a million times better. That was on Tuesday. By Friday he sounded no different. The kid was happy, but sounded like he had a giant frog in his throat that he just couldn't clear.

So the weekend came and it brought with it a new doctor. Because the antibiotic didn't seem to be making any difference, he started to explore other options. He thought maybe that Drew just had asthma, but that was ruled out when the Albuterol breathing treatments didn't seem to make much of a difference. They thought that maybe there was some bacteria in his lower airways that they were missing that might be resistant to the antibiotic that he was taking, however this seemed like an unlikely explanation as when they swabbed his throat it showed nothing but a little staph and when tested found that it was not resistant to any antibiotics. The other thinking was that maybe it was anatomical - some abnormality with his throat or airways.

On Monday morning there was another "changing of the guards". This doctor listened to him and to all that I had to say about the sounds he was making. He listened to the nurse read through the reports of everyone else's assessments, and at the end he said that he too thought it would something anatomical, but whatever it was we were going to get to the bottom of this so if we could just hang in there and bear with them for a little while longer we would get this all sorted out. He ordered a scope of Drew's upper airways from an ENT doctor (ear, nose, throat). They were looking for something called Laryngomalacia. Basically, its floppiness of the valves over the voice box which creates a noise as the child breathes in which is usually high-pitched and is especially heard during feeding. Its apparently common in infants and causes no harm, and is something that they outgrow as the cartilage around the vocal chords matures. The best part - it has nothing to do with CF!

This all seemed to make sense and so we were anxious for the ENT doctor to arrive to do his scope and solve this mystery sound that was being pegged as an illness. Around 11am the ENT doc arrived and stuck his little camera up Drew's nose and down his throat (that was a lot of fun) and the doc says............everything looks fine. ARGH! I mean I guess thats good that there's no issue with his voice box, but now what? What the hell is going on?

I called our nurse and asked to have the doctor paged so we could discuss what would happen next. Around 2ish the doctor came back and the first thing she said was "We're sending you home." (What? Why? Did you figure this out?) She said that they had ruled out anything bad, that they didn't think this was a CF bacterial infection and had collectively agreed that this was just a virus that he was going to need to work through. They didn't want to do a bronchoscopy because 1)there are risks and 2)he can't be on antibiotics which would mean keeping him in there for a few more days sans antibiotics so that they could get a clean sample of the mucus from way down in the lungs to figure out what crazy bacteria was causing this cough. Rather, they would like to see if with a little more time this could work itself out. They wanted to put him on a 3 day treatment of steroids to see if reducing some of the inflammation would allow for this phlegm to clear, send him him, and see him in a week. If by the end of the month nothing was better then they would go ahead with the bronchoscopy.

Alright! I guess if theres nothing more they can really do for him in the hospital we would just go home and play this out! So while the doctor is writing up the discharge instructions, another ENT doctor came in because the first guy was apparently talking about the mystery with the rest of his gang, and she had some other thoughts. She thought maybe it was Tracheomalacia - basically the same thing as Laryngomalacia but affecting the trachea rather than the voice box. Again, its nothing of huge concern and it fixes itself 99% of the time as the child grows (please dear God let us fall into the 99% if it is infact what is going on). Unfortunately the nose scope doesn't extend into the lower airways so the only way to determine if this is what is truly going on is the bronchoscopy. The doctor said that if it weren't for his cold we may not even hear anything, but that the thick CF mucus was getting sort of hung up on the airways, and because of the looseness or floppiness of the trachea, a cough wasn't getting everything out, hence the never ending frog-in-the-throat sound. She wants to wait until hes better before they do the bronch. I asked why, if shes so certain that this is the problem, we needed to even do the bronch and she said so that we know this is what the problem is rather than struggling through this mystery again next time he gets a cold.

I agree. I would hate to go through this again, another hospital stay, another IV antibiotic treatment, if really the problem weren't a CF related bacteria, but at the same time I hate having to do a bronchoscopy. It will be an outpatient procedure, but he will have to get put out for it and be put on a ventilator while they look down into his lungs. Since they will be down there, they will also collect a mucus sample to test just to make sure that there is no harmful bacteria.

So with that, we were sent home! Monday morning we thought we might be there for a while, and by Monday night we were all home. I spoke with a few of the CF docs and nurses and they all agree that the diagnosis of tracheomalacia seems very logical and appropriate. It makes sense to me that they had trouble diagnosing it as that because they are Pulmonologists, not ENTs, and therefore listening to and treating for a problem with the lungs when really it seems to be a problem with the throat. We have an appointment with the ENT doctor on Thursday so she can see him after hes finished with his steroids, when perhaps a little less inflammation and cough exist, so she can reevaluate and see if she still feels this is whats causing the problem.

We were told that if Drew didn't have CF (someday i hope!) he wouldn't have even been in the hospital. They really just need to treat aggressively when there are signs that bacteria is present to prevent lung disease from rearing its ugly head. Many kids have tracheomalacia and are diagnosed for a number of reasons. Drew just happens to have this extra special thick and sticky mucus thats clinging around his trachea that is producing this sound leading them to believe that this is what the problem is.

But we're home. Everyone is asleep and I had a few minutes to write about it. Thanks for all the thoughts and prayers over the past week. Hopefully the ENT figured this out. Even more hopefully, it will be no big deal and in no time he will sound just like you and me!

Monday, July 19, 2010

I know we're in the right place

A 'Best' Hospital for Cystic Fibrosis Kids - US News and World Report

"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?' "

[Read: Medical Treatment: Patients and 'Shared Decision Making'.]

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

Here's where we're at

So Drew's been in the hospital now for 4 whole days and doesn't sound much different than when we went in. They were expecting some improvement which leads them to believe there may be a few different things going on.

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

Its not what you say, its how you say it

Doctors have a way of really causing unnecessary feat and panic. I'm sure they mean no harm, but I'd like to offer them some advice on how to approach something with a nervous parent.

When Ella was just a few weeks old, she started spitting up, quite a bit at times, and so we took her to the doctor. Before I knew it, we were off to the ER. Martin met us there, me in tears, doctors talking about something called Pyloric Stenosis and how they could feel the "olive" that signified this and she had all the symptoms and that it was just a minor surgery to fix it and on and on they went. One of the residents even asked if he could bring in some of his colleagues to feel the "olive" because it is so rare that you can actually feel this knot in the belly and he wanted others to get the chance. Hours later, they did an ultrasound, and turns out nothing was wrong. They diagnosed her with acid reflux. HELLO PEOPLE!! Could we not START there?! Why do we have to go straight to the worst case scenario sending new parents into a tailspin worrying and calling all family and friends preparing them for an impending surgery. Acid reflux. They wrote a prescription, sent us home, and within 2 days she was fine.

Today, Lily had her 4 mo. checkup. Drew was supposed to be there too but this new hospital stay prevented that from happening. The nightmare that will be getting them back on the same "vaccination schedule" is better served for another post. ANYWAY, Lily had her 4 mo. checkup. They measure her head and her length, weigh her, and then stick us in a room to wait for a doctor. I was going from the doctor with Lily to the hospital to be with Drew so I was anxious to get out of there. Then the doctor comes in and we start talking about how shes doing. She says her head circumference is a little strange. A little strange? What does that mean? Is her brain not growing? Is something wrong? Shes apparently gone from the 40th percentile to the 15th percentile which isn't "normal. Normal? Who defines normal? JUST TELL ME WHAT THIS MEANS!!!! So I ask what she expected it to be and she says well I was just expecting it to maybe be a half centimeter bigger. Seriously? SERIOUSLY? She says oh I'm not concerned or anything, I was just noticing that she made a jump in percentages. Well then WHY bring it up lady? You KNOW i'm already stretched pretty thin on the stress scale! Just let it go! If it shouldn't be of concern for me, then don't bring it up, I don't wanna know! I really really like our pediatrician, however she does not have kids. If she did, she would know better.

There's always that moment too when they listen to their heart and spend a few extra seconds at the same spot, or go back over a spot for a second or a third time. You just hold your breath for a few moments, hoping that thats just the routine, that they don't hear anything wrong, that your baby is just fine. And she is. Shes 13lbs 3oz, and for anyone keeping track, that makes Drew BIGGER than her!! He weighed in at 13lbs 7oz yesterday. My boy is growing and his sister is doing great, small head and all.

And any doctors out there who catch wind of this blog, you don't have to sugar coat it for us, but how about not starting with the worst case scenario? Us parents have a hard enough time just being parents. Thanks.

Monday, July 12, 2010

Here we go again...

We're heading back to the hospital tomorrow for two weeks. AHHHGGGHH!!!!! I don't wanna go. I just wanna be at home and live our lives and not be bothered with CF and all of the nonsense that comes along with it.

A couple of weeks ago Drew got his first cold. I thought he was getting better, then he started to sound bad again. They put him on antibiotics about 10 days ago and expected that to clear things up, but he still sounds terrible. Hes happy as can be - eating fine, no fevers, happily playing - but sounds like hes got a permanent frog in his throat. So we took him back to the doctor today and they decided that they wanted to get rid of this once and for all and that IV antibiotics would be best to do that.

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.

Another option that was discussed was a bronchioscopy where they would put a tube down his throat to collect some mucus and culture it to see exactly what bacteria there is and treat that specific bacteria aggressively. Without doing this, they won't know exactly what's down there but they are hoping to aggressively treat with a strong mixture of antibiotics that would attack and kill what could likely be causing th problem. I'm hoping this plan works because if it doesn't then they will do a bronchioscopy and that requires time in the OR and a breathing tube so that they can collect what they need from deep down in his lungs and i'm hoping we don't have to do the whole "kiss goodbye before surgery" ever again.

I asked the doctor if this is an indicator of things to come and the answer was most decidedly no. She said that sometimes kids (even kids without CF) get colds the come with a cough and the cough just lingers. That may very well be what this is. It doesn't mean that hes gonna be in the hospital again next time he gets a cold, but it also doesn't mean he won't. We are just going to treat aggressively because the longer we can go with healthy clean lungs the better things will be. It was a good meeting with the doctor today, even though we didn't want to hear that we will be heading back to the hospital for 2 weeks.

I'll post an update when I have a chance. Say some prayers for a speedy recovery, and a cold-free and healthy future.

Friday, July 9, 2010

A Rant

Its been a long week. Its been a long couple of weeks. Drew got his first cold about 2 weeks ago and we're still struggling through it. Hes taking antibiotics and we've increased his CPT to 3-4 times a day, after using a nebulizer and Albuterol and saline. It takes close to an hour to do all of that...3-4 times a day. And the poor guy is only awake for about 2 hour at a time. He takes between 15-30 min to eat, so when we have to add the CPT process he simply wakes up, eats, takes meds, gets his chest therapy, and goes back to sleep. I hate it.

I've spent hours (yes, literally hours) on the phone with insurance this week trying to get everything with our mail order pharmacy sorted out, and I still don't think its right. There are 10,000 things I could have used that time for and I'm bitter that I'll never get that time back.

I talked to another CF mom and another friend with CF (just turned 49!!) this week about my obsession with germs lately. I just can't seem to figure out or be settled with how i'm going to deal with germs. I wanna ask people to wash their hands when they come into my house. I wanna ask people not to touch him when we're out in public (which is very infrequently). But I haven't gotten that comfortable with that yet. And I think its because I don't want people to think hes weird or different or that there's something wrong with him. But I also don't want him to get sick. There is incessant hand washing and sanitizer use. Our doctor said that since we are his handlers, our hands are most important right now, but when he starts crawling that we will just need to try to make sure his toys are clean and we wash his hands a lot. I know hes gonna get sick, and colds and stuff are in a way good for his immune system. He needs to build up some resistance. But this routine of caring for him when hes sick suck. And I'm sure that this isn't as bad as its gonna get. But they gave me some good perspective and told me that I will figure out what works for us and I know I will. I'm just ready to know how its gonna be

I shouldn't complain. There are a million people who have it a lot worse than I do. But I just wanna complain. I'm tired of this already and its just starting. I'm tired. I'm scared. I'm annoyed. I'm broke. I'm hopeful. I'm scared. I'm hopeful.

Wednesday, July 7, 2010

2010 Cystic Fibrosis Foundation Commemorative Bracelet

As I had mentioned a few weeks ago, we were paired with 3 of the nominees for the Cincinnati Finest Young Professionals Fundraiser. Basically, all nominees are charged with raising at least $2500 for CF and whoever raises the most at the end of 3 months wins the title of Cincinnati's Finest Young Professional.

One of the people that we have been honored to share our story with is a man named Jimi Merk. He is a custom jewelry designer and he has created a stainless steel charm for the CFF Bracelet and will be donating all proceeds to the Cystic Fibrosis Foundation. The bracelet and charm are available on his website for a $20 donation - jimeyedesigns

Other events that Jimi will be hosting in the Cincinnati area to raise money for CF include
  • 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
I will post more information about these events as they get closer, or if you are interested in attending any of them and have questions, feel free to email me and I will get you the information that you need.


I'm so annoyed. Everything I've read and everyone I've talked to related to CF has told me that dealing with insurance is a nightmare. We haven't had any problems...until now. We got a notification in the mail yesterday that told us that Drew's meds weren't being covered by insurance anymore unless we started to use Mail Order. No problem! I'd be happy to get a 90 day supply and not have to worry about it but 4 times a year.

So this morning I called the Mail Order Pharmacy to discuss what I needed with them. On Friday, our CF Clinic sent in a 90 day order for his Creon (enzymes). I was finally able to get a hold of someone today after 4 days of trying to get through, and they apparently never received the script. They said they would call the doctor and get it all sorted out and that I would have my 90 day supply in about 2 weeks. Thats gonna be a problem as we only have enough to get us through the rest of this week. However, if I try to get them at CVS down the street I have to pay full cost for them because insurance is only going to cover them if they are mail ordered. After some screaming and yelling, I got them to put an "override" in the system so that we could get a 30 day supply at CVS to hold us over until the 90 day supply arrives.

Then we started to discuss the kids heartburn medicines that we also received letters about. Lily is on it for reflux, but Drew takes it because it neutralizes stomach acid so that the enzymes aren't broken down in his system before they do what they need to do. Drew's is refrigerated, and apparently compounded at Children's. So my options are to either switch medications to something that comes in a liquid form, or have the mail order place break it down for me from pill to powder and mix it myself at home. I'm annoyed because the stuff we get from Children's is so easy and convenient and I don't wanna switch meds and I also don't wanna add another step to my day! I finally agree to just call my doctor to discuss what the best option is. But then the discussion of how I'm going to get this before 2 weeks and I'm on hold again while the man on the other line tries to get override approval for this as well so I can get 1 more 30 day supply at Childrens.

Lily's meds are available, it will just take 2 weeks to get them. Hopefully we have enough to last us that long.

I'm just annoyed because the convenience of CVS and Children's being right at my fingertips is being taken away from me. I know mail order can be convenient, but TWO FREAKING WEEKS to get a prescription?! I am just going to need to be careful to recognize and remember when we're about 2 weeks from running out. Also equally annoying is that with the Creon, he takes a similar amount every day, but not always the same, and its regularly adjusted. So for example, right now, every time Drew has a bottle, he takes 2 pills of Creon. However, as he gets bigger and the calorie content or the quantity goes up, he may take 2 and a half pills or 3 pills with every bottle. The problem is that if the prescription is written for like 10 pills a day and we get enough for 90 days, when he starts to take more pills he will use up the supply, but when I go to reorder they will tell me that its to early to reorder. SO ANNOYING!

Another pain in my ass is that I have a Creon rebate card, where the manufacturer of Creon will refund you $50 every month for 12 months for your prescription, however the mail order pharmacy will not accept secondary insurance, which is what they consider this. My copay for the Creon is less than $50 for a 1 month supply through mail order, so I could get it for FREE if they accepted the card, but they don't accept it. They said I could call the manufacturer to see if they could issue the rebate. That sounds like a reasonable solution, if I didn't have 3 kids and 1000 things I was already managing to make sure that everyone stays happy and healthy. I'm just so annoyed.

When I hung up the phone with the man, who was actually very nice, I looked at the call timer and it was 1 hour and 23 minutes. SO ANNOYED!!! If there was no CF there would be none of this BS nonsense. So FUCF, FU.

Saturday, July 3, 2010

Lily - 0; Drew - 1

Drew gets the award for first to roll over! He did it this afternoon shortly after waking up from his nap. I was sure that we were going to need PT to help him with things like rolling over and sitting up, just because he is a little weaker than Lily from the time spent in the hospital and the surgeries to his abdomen. But it sure doesn't look like that's going to be the case and that's delightful! He went from belly to back, and i'm pretty sure it scared the sh*t out of him because he started screaming at the top of his lungs after it happened. Then Lily started screaming, but I think mostly because she knew all of our attention was on Drew and shes a bit of an attention hog :). But I was so happy, so proud. It helps me to see that hes going to do all of the same things the girls do, that he going to be "normal". Love you, boy!

Thursday, July 1, 2010

First of Many

Drew got his first cold this week. I knew it was coming so we just embraced it rather than freaking out. The girls got colds too, so rather than panic and call the doctor right away, we decided that we should just let it run its course.

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.

The cold started on maybe Thursday or Friday. I got nervous, but we just increased his CPTs to 3 times a day and kept an eye on him. He had a clinic appointment scheduled for Monday so I figured that they would be able to check him out then if things hadn't gotten any better. By Sunday he was sounding pretty rough and his breathing seemed labored so I was glad our appointment was at 8am.

The doctor listened to his lungs and said they sounded clear. He honestly sounded better on Monday morning than he did all day Sunday. We decided that it was probably just a cold, not anything worse, and that we would skip antibiotics. [clarifier - antibiotics wouldn't do anything for a viral cold, however the increase in mucus provides the perfect breeding ground for bacteria that would be killed off by the antibiotics] However, it was also decided that we should start using a nebulizer before CPT to moisten his airways and help to loosen the mucus. They gave us saline and Albuterol. We spend 10-15 minutes using the nebulizer now before we do his 30min of CPT 3 times a day. Thank God he is such a laid back kid. He doesn't seem to mind the mask on his face and it sure does make him sound better. It does kind of suck that we have to use it now but if it helps him thats whats important.

They also did a throat culture. They do these ever 3 months to see whats going on in his lungs. So far, there's only staff. It sounds horrible to me, but they say they expect it in CF kids. Bad things they could find include Pseudomonas. Its is a type of bacteria that is of concern for persons with CF. It causes frequent lung infections, which leads to damage to the lungs. B.cepacia is the really bad one. B.cepacia bacteria are often resistant to common antibiotics. B.cepacia poses little medical risk to healthy people. However, people who have certain health problems like weakened immune systems or chronic lung diseases, particularly cystic fibrosis (CF), may be more susceptible to infections with B.cepacia. B.cepacia is a known cause of infections in hospitalized patients. The effects of B.cepacia on people vary widely, ranging from no symptoms at all, to serious respiratory infections, especially in patients with CF.
Presumably nothing new was found with this culture. They said they would call by the end of the week if they saw anything, and we haven't heard from them which works for me.

He seems to be doing better. Hes still coughing a little more than normal but its always hard to tell what his normal is because its always going to be changing. But hes doing better so I'm happy.