Cystic Fibrosis Related Diabetes

Cystic fibrosis-related diabetes (CFRD) is a type of diabetes that affects individuals who have Cystic Fibrosis. CFRD is an entity unto itself, having several aspects that make it different from other forms of diabetes.

Cystic Fibrosis (CF) is one of the most common genetic defects among the Caucasian population, and it is a devastating, systemic disease. When CF was first being diagnosed, children with this disorder rarely lived to reach their teens]; now the average life expectancy  of an individual with CF is around 36 years. Still horrifically short, but better. The fact that people with CF are living longer means they acquire other disorders, including a type of diabetes. It has been shown that with increasing age in the CF population there is increasing incidence of diabetes mellitus.  Roughly 20% of adolescents with CF have diabetes and about 50% of adults with CF have CFRD (1).

CFRD is not as straight-forward to diagnose as type 1 and type 2 diabetes, so it’s important for laboratory professionals to be aware of this disease. People with CF who have diabetes may not always have hyperglycemia. Also hemoglobin A1c (Hgb A1c) values, which is a test recommended by the ADA for diagnosing diabetes, may not be elevated in these patients. The oral glucose tolerance test (OGTT) is recommended for diagnosis of CFRD, and yet even these results may be equivocal in CFRD patients (2). Nonetheless, the ADA/CFF guidelines suggest that all CF patients over 10 years of age should be screened yearly for CFRD using the OGTT. In addition, at least one study in the literature has found that when performing an OGTT on CFRD patients, a glucose level at the 1 hr time point correlates best with the patient’s lung function (3). Thus, if your lab performs OGTT on individuals with suspected CFRD, the physician requesting the test may want the glucose value on a one hour time point as well as the standard 2 hour OGTT.

Individuals with CF who get CFRD tend to have weight loss, protein catabolism, worsened lung function and significantly increased mortality compared to CF individuals without diabetes. The increased mortality is directly related to decreased pulmonary function, rather than to the atherosclerotic vascular disease seen in other types of diabetes. Insulin therapy is the recommended therapy for CFRD.

-Patti Jones

References:

  1. Moran A, Brunzell C, Cohen RC, Katz M, Marshall BC, Onady G, Robinson KA, Sabadosa KA, Stecenko A, Slovis B. Clinical care guidelines for cystic fibrosis-related diabetes.  Diabetes care 33(12):2697-2708. 2010.
  2. Rana M, Munns CF, Selvadurai H, Donaghue KC, Craig ME. Cystic fibrosis-related diabetes in children – gaps in the evidence? Nature Reviews: Endocrinology, 6:371-378. July 2010.
  3. Brodsky J, Dougherty S, Makani R, Rubenstein RC, Kelly A. Elevation of 1-hour plasma glucose during oral glucose tolerance testing is associated with worse pulmonary function in cystic fibrosis. Diabetes Care, 34:292-205. 2011.

I’ll Have Pasteurized Milk, Please

Recently officials determined the cause of death of a young mother in Las Vegas: tuberculosis. The family Mycobacteriaceae contains several pathogenic species, including the most famous, M. tuberculosis. While none of the articles I read mention the species, they do mention the patient consumed unpasteurized dairy products, which leads me to believe she died of the zoonotic organism M. bovis.

Since these organisms are recovered infrequently, clinical microbiologists should brush up on the basics of these organisms on occasion. The CDC has some general information on Tuberculosis; the illustrious contributors at Wikipedia go a bit more in depth. The best sources for information are reference textbooks such as the Manual of Microbiology. It’s important to remember that mycobacteria can infect any region of the body, not just the respiratory system, so it’s important to keep an open mind. It’s also helpful to know that some species are rapid growers and may present on blood agar in a routine culture.

-Kelly Swails

 

“Light Bulb” Moments During Residency

After four months on CP rotations, I am now on a 2-month surgical pathology rotation at the VA hospital where we have a 2-day grossing schedule. While it is not as busy as the two community hospitals I will rotate in surgical pathology at in 2014, the time away from anatomic pathology brings some trepidation as I feel I’ve lost some expertise in this area. Use it or lose it. But again, it helps to have a great support staff that makes life easier by helping me out and providing me with daily laughs to make the day go by faster and almost feel like I’m not at work

While I remember being stressed when I started my 3-month “intro to surgical pathology” rotation last year as a PGY-1, a lot has changed in a short year. Last year, I felt as if there was so much that I did not know but eventually a time came, without my even realizing it, when I got most of my diagnoses correct. Clinical pathology rotations were inherently easier for me due to my research and grad school background and my comfort level in the lab setting. But since I am in an AP/CP track, its important to maintain perspective as well as skills in both disciplines.

To accomplish these goals, I approach service duties on each rotation with the same diligence. I don’t play favorites even with those rotations that I find easier, more comfortable, or more likely to be my future choice of subspecialty. There is always something I can learn and I give each rotation and every patient that same respect. Next, I learn by performing my duties with as close to the same responsibility level as my attending as I can. I find that I learn more by “doing” than by just studying. This is especially true if I interact with all members through the clinical care process – from technicians to attendings to primary care physicians and other subspecialists, not just to deliver diagnoses but to help influence healthcare decisions. This was especially true on my lab medicine rotations. But I understand that this learning style may not be the same for others.

For whatever reason, PGY-2 feels as if it has flown by more quickly, probably because I have more responsibilities and also cover night/weekend calls. But whatever what advice I or another senior gives their junior, people will only listen when they are ready to hear and have their “light bulb” moment. I know it took me a while to understand the significance of much of what I was told last year…Are you ready for your “light bulb” moment?

I will leave tomorrow to attend CAP HOD and to present a poster at the CAP conference (where I probably won’t get to visit Disneyworld). I’ll let you know how it goes in my blog post next week!

-Betty Chung

 

How the Government Shut Down Affects Your Health

The shutdown has far-reaching implications for your health.

The government-funded Centers for Disease Control employs detectives that investigate foodborne illnesses, infectious disease outbreaks, and influenza viral patterns.  They work hard to keep us healthy and productive. You know what happens when the government shuts down? They stop detecting. Development of next year’s flu vaccine gets delayed. Flu outbreaks aren’t tracked. Right now, there’s a Salmonella outbreak that isn’t being investigated as thoroughly as it would be if the CDC were open for business.  (If you’re interested in the CDC’s role in outbreak investigation, that link is here.)

The Superbug blog has a great post about the government shutdown and your health. 

-Kelly Swails

 

Under the Hood

I like to keep some humor in the lab so when I see a technologist with a panel off a machine trying to troubleshoot an issue I will say “Uh-Oh, why do you have the hood up?” It’s a little tension breaker, especially if they are stressing about having their instrument down. It also acts as a little reset button so I can go through the troubleshooting steps with them. As technologists, we are modern day mechanics. We use instruments much more than we perform manual testing, and we are expected to be able to troubleshoot instruments that are more complex than the current day automobile.

Acquiring new instrumentation can be a lab changing experience. Each instrument has its quirks and special requirements. The vendors usually offer on site or even off site training for staff once the instrument is purchased. Who you send to these training sessions is just as important as the quality of training they receive. These sessions are where your staff will learn maintenance, operation, and most importantly troubleshooting. When your shiny new analyzer goes down, and it will, the time it takes to get it back up and running affects productivity, turnaround time, and staff morale. Nothing is more detrimental to a staff’s morale then coming into work and the first thing they hear is that the instrument they are on that day is already down. Having experienced that exact thing I can tell you it takes the wind right out of you.If it happens consistently you will see a decreased engagement by staff.

Whom should you send for analyzer training? You should have a good mix of talent and maybe some of the lower performing staff. This assures that you are keeping your talented staff engaged and shows weaker performers that you are invested in building them into a top performer. The question becomes, how do I make sure that the people I send get the most out of their experience? Let them know they will be responsible for presenting the material they learned to the rest of the staff once they get back from training. If any of your staff have an issue with that they are not the ones you should send. These small presentations will help with team building as well as solidifying the information for the key operator.

As leaders we must pick our key operators very carefully. When these choices become important is most likely when we won’t be in the office. Observe the staff that likes to troubleshoot instruments or that keep a level head once instruments are down. You want to make sure that once the hood goes up you have the best mechanic for the job.

-Matthew Herasuta

Lab Product Friday-UniCel DxH800

The common maxim when buying laboratory equipment is “Fast, accurate, or cheap; pick two.” The perfect analyzer would have all three qualities, but as the saying suggests, it’s hard to find those instruments. Enter Beckman Coulter. Their website suggests the UniCel DxH800 is designed to meet these demands by improving productivity, decreasing turnaround time and reducing overall cost.

Recently Lab Medicine published a paper evaluating the performance of Beckman Coulter’s Unicel DxH800. The authors of the paper found the instrument to be accurate and efficient. They also commented that for larger facilities, this analyzer could improve productivity and turnaround times when compared to the older model (LH 750). Notably, the authors don’t mention cost, quality control, or maintenance concerns.

Does your laboratory have the DxH800? Is the maintenance easy to perform? Has this analyzer improved turnaround times in your lab? Let us know in the comments.