I’m often asked about #immunodeficiency–does my body fight infection like it’s supposed to? Is this baby’s immune system normal? Obviously, probably not. Fortunately, #Stevens Johnson syndrome is quite rare, but antibody deficiency is NOT rare and many strategies can be performed to improve the situation.
I often start the conversation about #immunodeficiency: “are you worried about how well you fight infection?” Since immunodeficiency comes in many flavors, there are no absolutes to checking the immune system, but here’s some pointers that are helpful. Remember, the body fights infection in compartments, so test for something in each compartment and you’ll hit a home run with the bases loaded.
This article appeared in KevinMD’s blog and has a very interesting chain of comments. From just reading the article you would think that certain #vaccines prevent #cancer. I would like to think that’s true, but nothing is so simple in medicine. The “cause and effect” relationship to what we do is always the elusive holy grail. This is somewhat of a brainiac article, so buyer beware!
You blurt out an insult to your girlfriend, the quarterback throws for an interception, I bought a stock that tanked….if only I could take back decisions I’ve made. We all feel that way at times, but how can a lab test be a bad decision? Continue reading Why Doctors Order Too Many Tests→
Normally, I like to include lab studies for evaluation of patients with chronic infections. It’s very important to find out if your body can make the right amount of antibodies to fight infection. But…there’s always the exception. In this case, IgA can be absent from your bloodstream and not cause a problem because it’s gone. In short, I call this asymptomatic IgA deficiency (you should see the long version). The link below comes from the American Academy of Allergy, Asthma, and Immunology–good stuff and easy to understand if you’re interested.
Immunodeficiency–does my child get too many infections? How can I tell? Read on in the following journal article. Author information and citation listed at the end. I’ll summarize for you. Before I begin, take a look at this 3 minute (yes really) video on immunodeficiency; it will help with background information.
April 11, 2011 — Three main warning signs may help identify children with underlying primary immunodeficiency diseases (PID), according to the results of a retrospective survey reported online April 11 and will appear in the May print issue of Pediatrics. “Children with severe, recurrent, or unusual infections may have an underlying …PID,” write Anbezhil Subbarayan, MBBS, from the Department of Paediatric Allergy and Immunology, University of Manchester, Royal Manchester Children’s Hospital in Manchester, United Kingdom, and colleagues.
“Ten warning signs have been promoted by patient support groups to help identify children with PID, but the signs have never been tested in a rigorous scientific study.”
The objective of the study was to examine the efficacy of these 10 warning signs in the prediction of defined PID among 563 children evaluated at 2 tertiary PID centers in northern England. Medical records were reviewed for 430 children with a defined PID and 133 children for whom thorough workup did not result in a diagnosis of a specific PID.
Factors most predictive of PID were
A family history of immunodeficiency disease
Sepsis treated with intravenous antibiotics in patients with neutrophil PID (primary immunodeficiency)
Failure to thrive in children with T-lymphocyte PID. In simple terms, this means you just don’t grow.
When these 3 signs were present, PID was correctly identified in 96% of patients with neutrophil and complement deficiencies and in 89% of children with T-lymphocyte immunodeficiencies.
The only warning sign that correctly identified patients with B-lymphocyte PID was a positive family history. These are patients with NO antibody production–the “bubble boy”
“PID awareness initiatives should be targeted at hospital pediatricians and families with a history of PID rather than the general public,” the study authors write. “Our results provide the general pediatrician with a simple refinement of 10 warning signs for identifying children with underlying immunodeficiency diseases.”
“Delayed diagnosis of PID may be associated with increased morbidity and mortality,” the study authors conclude. “In children presenting with infection, pediatricians should always inquire about a family history of PID, as this is the best predictor that the patient may also have an underlying immunodeficiency….In those with recurrent or severe infections, clinical features other than family history are often not helpful and a lower threshold is required for requesting antibody testing in which the diagnosis of a B-cell immunodeficiency is suspected.”
Selective antibody deficiency shows up a little bit differently in that children will often have recurrent ear infections or sinus disease. A different disease altogether!
Pediatrics. Published online April 11, 2011.
Laurie Barclay, MD
Freelance writer and reviewer, Medscape, LLC
Disclosure: Laurie Barclay, MD, has disclosed no relevant financial relationships.
WASHINGTON – The Food and Drug Administration says it has approved Pfizer Inc.‘s best-selling Prevnar 13 vaccine for use in preventing pneumococcal disease in adults age 50 and older.
The agency’s move on Friday was widely anticipated, coming a little over a month after a panel of federal health experts voted overwhelmingly to recommend the use of Prevnar 13 as a safe and effective vaccine for preventing the bacterial infection in adults.
Pneumococcal disease causes meningitis, pneumonia and ear infection.
Prevnar 13 protects against 13 strains of the disease. It’s already a standard vaccination for infants and young children.
By Mark Lennihan, AP–good job on this article!
Two key late-stage studies of Pfizer’s blockbuster pneumococcal vaccine for children show it works at least as well as a rival in adults, a big market the drugmaker wants to tap.
What does this mean for you?
If you have chronic sinus infections or pneumonias, I will check antibody levels to see how well you fight infection. If low, you will usually be immunized with Pneumovax™–0.50ml in adults, 0.25ml in children.
Now, I have a choice: use Prevnar 13 or Pneumovax. Which one is better? You would have to ask!
Studies haven’t been done with immunodeficient patients comparing the two vaccines, but the FDA now considers Prevnar 13 as effective in normal adults as Pneumovax.
How about the cost? You guessed it….the Prevnar 13 is ~twice as expensive as Pneumovax™
If you’re like most doctors, who doesn’t spend the winter months prescribing antibiotics and treating upper respiratory infections? But when is it serious enough to pull the trigger on a detailed work-up for immunodeficiency?
As with everything in medicine, when symptoms are outside of the “norm,” it’s time to intervene and start your work-up. Consider the following:
1. Severe infections such as sepsis or recurrent pneumonia are a no-brainer. Do the work-up!
2. The average number of yearly upper respiratory infections in a toddler is 8 to 12, so monthly episodes of a snotty nose aren’t that unusual. I would be suspicious if fluid accumulates behind the ears, or antibiotics are required with every upper respiratory infection.
3. Parental or patient anxiety should be included in your evaluation. Remember, checking for antibodies as a screen will go a long way in relieving that nagging question, “is my child normal?”
4. Remember, our job as physicians is to screen for immunodeficiency, not order every test available after the first encounter. Signs & symptoms of immunodeficiency evolve and change our outlook as to how much testing is appropriate.
Some suggestions for your consideration:
Categorize immunodeficiency into 5 subsets. The appropriate tests to order follow in a logical pattern thereafter.
1. Antibody deficiency. Labs for this diagnosis should include not only IgG, IgA, and IgM, but also specific antibody titers to Strep Pneumoniae. Most children now receive PCV-7 or PCV-13 and a subset of kids with chronic infections won’t demonstrate a vigorous antibody response to these immunizations. Interestingly, many of these children will respond to the adult Pneumovax™ and a good diagnostic test for antibody deficiency is to challenge with Pneumovax™ and repeat titers in one month. Want more info? This pocket guide is the best!
2. T and B cell deficiency. Infections in this category are viral infections that cause sepsis or meningitis, not self-limited colds. AIDS falls into this category, although there are many T & B cell deficiencies in addition to AIDS. Most labs will now provide panels that count the T/B and NK cell populations without having to remember each individual test. As you can imagine, this saves time and headaches. Don’t forget to order mitogen and antigen stimulation…determines how well lymphocytes respond to infection. Often the absolute cell number fails to tell the entire story.
3. Neutrophils. The PacMan of the immune system. Usually infections due to chronic granulomatous disease (CGD) involve recurrent cellulitis or persistent abscesses. You can order screens that measure the oxidative burst when a neutrophil is confronted with bacteria!
There are several prototypes of CGD depending on the missing enzyme, but most of them can’t produce superoxide anions when needed to kill the invading bug.
4. Complement deficiencies are rare, but easy to measure. Recurrent infections with Neisseria should raise the red flag. CH50, C4, and C3 will tell you if further investigation is needed.
5. Last but not least, is the INNATE arm of immunity. When I was a fellow 20 years ago, I was not taught about innate immunity because we didn’t know it existed. How else does your body recognize a “new” infection without having to wait 7-10 days to mount an antibody response first? Toll-like receptors are found on most surveillance cells that recognize nucleic components (DNA, RNA & other repeating sequences) found only on foreign invaders like bacteria, viruses, and fungi. Believe me, you don’t want to memorize the list of Toll-like receptors (TLRs), but it’s a great cure for insomnia. Labs can measure Mannose-binding protein, but other TLRs will have to wait their turn.
If you examine a patient (young or old) with chronic infections and at least think of immunodeficiency, that’s a great start and you’ve come a long way! Have fun with it–you can give someone their life back with the correct treatment. (Sounds like another post to me!)
An apple a day might keep the doctor away, but what is modern hospital medicine really like? Follow Dr. Benjamin Kirkland - a Doctor working in Australia - through the pinnacles and pitfalls of everyday hospital medicine!