Sepsis, Septicemia, SIRS, and Septic Shock

This possepsist is not intended as medical advice, but it may suggest a topic to discuss with your child’s or loved one’s doctor. Sepsis is a difficult topic and the content may be disturbing to some readers. It is potentially life-threatening and there are no guaranteed ways of preventing or treating it, but the more that families know about sepsis, the better they can protect their family members, particularly those with MECP2 Duplication Syndrome.

September 13, 2015 is World Sepsis Day, but protecting individuals with MECP2 Duplication Syndrome from sepsis is something we need to think about every day.

What is Sepsis?

Sepsis is a condition caused by the body’s inflammatory response to an infection. Although the infection may be limited to one part of the body or system (for example, a respiratory or urinary tract infection), but sepsis affects the whole body. It can develop rapidly and become life-threatening if it can not be quickly brought under control.

Sepsis can be triggered by infections caused by bacteria, viruses, fungi, or other pathogens.

Sepsis is a medical emergency. Getting treatment quickly can greatly improve the outcome. Kumar and colleagues have demonstrated that each hour of delay in getting appropriate treatment significantly decreases the chances of survival.

Sepsis is sometimes called (SIRS), systemic inflammatory response syndrome. Sepsis, however, is always caused by an infection, while SIRS can be caused by other factors such as physical trauma or burns. Sepsis is also sometimes called septicemia, although this term is generally no longer used because it is sometimes used for a different condition, creating some confusion. Clinicians generally classify episodes of sepsis into three categories based on severity: Sepsis, severe sepsis, and septic shock.

Sepsis is a major cause of severe disability and death. In many parts of the world that lack access to advanced medical facilities, sepsis is the leading cause of death. Worldwide, approximately 8 million people die each year from sepsis, In the United States, it causes more than 200,000 deaths each year, far more than the combined total for breast cancer, prostate cancer, and HIV infections. In the US, more than 1 million patients are hospitalized with sepsis each year, but this is believed to be a very low estimate of the real number because sepsis is a complication of infections that are likely to be identified as the primary reason for hospitalization. It is also estimated that approximately 25% of all Intensive Care Unit admissions are patients with sepsis. Those who survive sepsis may have permanent organ damage or loose limbs.

Sepsis and MECP2 Duplication Syndrome

As a group, children and adults with MECP2 Duplication Syndrome have an increased risk for sepsis. Bauer and colleagues found that 5 of 27 individuals (median age of participants was 10.5 years) with MECP2 Duplication Syndrome that they studies had had at least one episode of sepsis. Because they have weakened immune systems and most have more frequent and more sever infections, the risk of sepsis is increased. Other factors may also increase the risk of sepsis in MECP2 Duplication Syndrome. For example, individuals with MECP2 Duplication Syndrome have been shown to have fewer pathogen-specific responses to infectious organisms. This may result in the body attempting to compensate with an increased general inflammatory response that brings about sepsis.

In addition, sepsis may be more difficult to recognize in individuals with MECP2 Duplication Syndrome, because symptoms of sepsis can be masked tr overshadowed by other features of the syndrome.

Managing the Risks for Individuals with MECP2 Duplication Syndrome

Unfortunately, there is no way of completely eliminating the risk of sepsis , but there are some things that can be done to manage and minimize risks. Here are a few things that can be done:

1. Discuss appropriate immunizations with your child’s physician. Vaccines against pneumonia and influenza can be very useful. Pneumococcual vaccines may need to be administered more frequently than they would be for individuals who do not have the syndrome.

2. Some individuals with the syndrome, who are particularly vulnerable to infections, may require a regimen of preventive antibiotics.

3. Seek medical treatment as soon as an infection is suspected.

4. Make sure that the physician who assesses or treats the individual with MECP2 Duplication Syndrome for a known or suspected infection understands that this patient has increased risk for sepsis.

5. Learn to recognize signs of sepsis, if you suspect sepsis, seek medical treatment immediately. Because sepsis is a medical emergency, it is better to err on the side of caution.

Click this link for signs of sepsis. It is important to remember that not all of these signs are present in every case, and in children and adults these may be more difficult to identify. Therefore, if you suspect sepsis may be developing, it is best to have the individual evaluated by a healthcare professional.

  • Rapid breathing.  Typically more than 20 times per minute, but for children and adults with MECP2 Duplication Syndrome “normal breathing” may be fast. It is a good idea to get baseline measures when your child is healthy so you can recognize when breath rate is increased for the individual
  • Change in body temperature. Sepsis can produce fever but also sometimes lowers body temperature. Know what is normal for the individual in your care. If your child has an unusual “normal” body temperature.
  • Rapid Pulse. Heart rate increases as body tries to compensate.
  • Low or dropping blood pressure. Many individuals with MECP2 Duplication Syndrome have low normal blood pressure. It is a good idea to find find out what is normal for the individual so illness-related changes can be identified.
  • Poor Appetite. At the same time, the individual may be thirsty.
  • Rash or changes in skin coloration. Pictures of sepsis rashes vary widely and many examples found on the web are from more advanced stages. In the early stages there may be blotchy areas of skin (not usually raised) or areas of skin (for example, hands or feet) may have a dark red coloring as circulation becomes poor.
  • Edema. There may be some swelling of hands or feet as circulation of blood becomes less efficient,
  • Dropping Oxygen Saturation. If you have a oximeter at home, oxygen saturation in the blood drops in sepsis. Some research has also suggested that oximetry readings during sepsis tend to be a little higher than more accurate blood gas measures, so actual saturation could be a few percent lower than the oximeter reading.
  • Lethargy or change in consciousness.

References

Bauer, M., Kolsch, U., Kruger, R., Unterwalder, N., Hameister, K., Kaiser, F. M., et al. (2015). Infectious and immunologic phenotype of MECP2 duplication syndrome. Journal of  Clinical Immunology, 35(2), 168-181.

Kumar A, Roberts D, Wood KE, et al. (2006). Duration of hypotension before initiation of effective antimicrobial therapy is the critical determinant of survival in human septic shock. Critical Care Medicine, 34, 1589-1596.

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