Waterhouse-Friderichsen syndrome is a relatively rare disease, but it poses an enormous threat to the life of the affected person. For this reason, it is enormously important that – once diagnosed – immediate intensive medical treatment is given.
What is Waterhouse-Friderichsen syndrome?
The medical profession describes as Waterhouse-Friderichsen syndrome a state of shock caused by bacterial toxins (toxins), which is primarily caused directly by the bacteria. Subsequently, there is an enormous consumption of coagulation factors (so-called consumption coagulopathy), so that parts of the tissue (hemorrhagic necrosis) of the adrenal cortex die off. The death results in bacterial blood poisoning (meningococcal sepsis). At the first sign of a possible Waterhouse-Friderichsen syndrome, a physician must be contacted immediately. If treatment is delayed or not received at all, the mortality rate is one hundred percent.
Causes
Waterhouse-Friderichsen syndrome is caused due to a tremendous release of toxins; the toxins are produced by bacteria. Predominantly, Waterhouse-Friderichsen syndrome results from meningococci; however, Haemophilus influenzae as well as pneumococci can also be responsible for Waterhouse-Friderichsen syndrome on occasion. Due to the release of toxins, coagulation factors are subsequently activated. Thus, numerous thrombi are formed, which subsequently occlude the blood vessels. Furthermore, massive hemorrhages also occur; especially those in the skin, the internal organs as well as directly on the mucous membranes are visible. The patient goes into shock due to this blood loss. Sometimes, endotoxin shock also occurs in the context of Waterhouse-Friderichsen syndrome, which affects the functions of the kidneys, adrenal glands, liver as well as lungs.
Symptoms, complaints, and signs
Classic symptoms are massive hemorrhages directly into the skin; the medical profession describes this condition as petechiae. Furthermore, intravital death spots (livid, cold areas of skin where stagnation of blood occurs) and mucosal hemorrhages also occur. Subsequently, the physician also observes classic symptoms of shock. The kidneys stop working; the patient complains of very little or sometimes no urine output. Furthermore, the affected person suffers from shortness of breath, so that subsequently the lungs go into shock. The patient becomes jaundiced due to the liver going into shock. The body gradually reduces the performance of the organs as part of the Waterhouse-Friderichsen syndrome. If the thrombi obstruct any cerebral vessels, neurological abnormalities appear. In particular, somnolence and convulsions are possible. The symptoms of Waterhouse-Friderichsen syndrome usually appear within a few hours. A medical professional must be contacted immediately at the first signs!
Diagnosis and course of the disease
The clinical picture is the most important clue that it could be Waterhouse-Friderichsen syndrome. Waterhouse-Friderichsen syndrome must be treated as soon as possible. For this reason, if any minor bleeding occurs and is visible on the skin or if diarrhea and fever occur, the patient should be immediately thought of as having Waterhouse-Friderichsen syndrome. At the first signs, a medical professional must be contacted immediately. The physician determines an abnormality by means of different fibrinolysis as well as coagulation tests; numerous coagulation factors can be evaluated as massively depleted. Furthermore, there is an enormous reduction in leukocytes (white blood cells); a further indication is also reduced platelets. Waterhouse-Friderichsen syndrome often ends with the death of the patient. If the latter is treated too late or not at all, there is no chance of survival. For this reason, immediate medical attention is essential.
Complications
Waterhouse-Friderichsen syndrome represents a life-threatening condition. If the condition is not treated promptly with intensive medical care, serious complications can occur, including death of the patient. The thrombi that occur as a result of activated clotting factors can cause blood to back up within the affected limb. If the clot penetrates to the lungs, pulmonary embolism may occur.In addition, sepsis and/or permanent venous insufficiency may occur. Furthermore, WFS can cause massive bleeding associated with anemia and deficiency symptoms. Accompanying the blood loss, shock occurs, associated with cardiovascular problems and further complications. If a so-called endotoxin shock occurs, this can disrupt the functions of the internal organs and cause, for example, liver failure and a kidney infarction. Due to respiratory distress, the lungs may go into shock. In the final stage of the disease, neurological deficits, convulsions and strokes occur. Drug therapy with cefotaxime and penicillin can cause side effects. Artificial respiration carries the risk of developing respiratory infections. Infusions, such as those given to provide fluids and nutrients, can also cause infections and other complications.
When should you see a doctor?
The affected person is always in need of medical evaluation and treatment for Waterhouse-Friderichsen syndrome. In this regard, early detection and treatment of the disease always has a very positive effect on the further course and can prevent further complications and complaints. Since it is also not possible for the disease to heal itself, the affected person should contact a doctor at the first symptoms and complaints of Waterhouse-Friderichsen syndrome. A doctor should be consulted if the affected person suffers from bleeding under the skin. In most cases, the skin also appears very cold. Similarly, severe shortness of breath may indicate Waterhouse-Friderichsen syndrome if it occurs without any particular reason and does not go away on its own. Yellowing of the skin may also indicate the disease and must be examined immediately by a physician. If the syndrome causes acute and life-threatening symptoms, an emergency doctor should usually always be called or the hospital visited. In general, however, the disease can be recognized by a general practitioner. The further treatment itself depends on the exact manifestation of the symptoms. The life expectancy of the affected person is often limited by Waterhouse-Friderichsen syndrome.
Treatment and therapy
Therapy must be started as soon as possible. After the diagnosis, the physician starts an antibiotic therapy; within the framework of this, the treating physician uses the active substances cefotaxime as well as penicillin G. These ensure that the infection is fought. Thus, antibiotic treatment is part of the treatment of the cause of Waterhouse-Friderichsen syndrome. In addition to antibiotic therapy, the physician must also treat the patient’s state of shock; in that case, it is symptom control of Waterhouse-Friderichsen syndrome. Purely combating the causes or symptoms does not lead to success. In the context of symptom control, the physician takes care to treat primarily the signs of the shock. First and foremost, this means that the affected person needs fluid, which must enter the body directly through the veins. In this way, the physician can compensate for the patient’s lack of volume. Subsequently, the patient is ventilated; this is the only way that the acid-base balance as well as the electrolyte balance can be balanced. Hydration and ventilation are the most necessary indications, so that the patient’s chance of survival increases. If the physician detects massive bleeding, fresh plasma and platelet concentrates can be administered. No other treatment options are currently available. The earlier the diagnosis is made, the better the patient’s chances of survival. If not treated promptly, the syndrome ends in death.
Prevention
To date, there is no vaccine that prevents against the meningococcal group. That group represents the main causative agent of Waterhouse-Friderichsen syndrome. However, antibodies against the meningococci are formed later in life, so that the body is protected against an “invasion” of the bacteria. For this reason, Waterhouse-Friderichsen syndrome is relatively rare. There is a vaccine against hemophilus influenzea and pneumococci that can prevent Waterhouse-Friderichsen syndrome. This is a so-called 6-fold vaccine, which can already be injected at the 3rd month of life. Other preventive measures are currently not known.
Aftercare
In the case of Waterhouse-Friderichsen syndrome, the options and measures for direct aftercare are significantly limited in most cases, and in some cases they are not even available to affected individuals. Therefore, the affected person should ideally see a doctor at an early stage and also initiate treatment to prevent the occurrence of other symptoms and complications. No independent healing can occur, so that the affected person is always dependent on treatment by a doctor. A doctor should therefore be consulted at the very first signs. In most cases, Waterhouse-Friderichsen syndrome can be well alleviated by the measures of physiotherapy or physiotherapy. In doing so, sufferers can also perform many of the exercises in their own homes to prevent the onset of other symptoms and speed healing. In most cases, aftercare also includes taking various medications. Patients should ensure that they take the medication regularly and in the correct dosage to avoid any side effects. In many cases, the syndrome also limits the life expectancy of the affected person.
What you can do yourself
Waterhouse-Friderichsen syndrome is a medical emergency. Activities or measures of self-help are extremely low in these situations. Harmonious cooperation with an experienced medical team is enormously important for the relief of symptoms. The relationship of trust between doctor and patient is therefore to be built up and supported to the best of one’s ability. Normally, the patient himself is not sufficiently responsive. Therefore, relatives are responsible for making important decisions, taking into account the patient’s wishes. If there are open questions, they should be asked in order to be best prepared for the situation and further development. In addition, if there is a lack of knowledge, the responsibility for obtaining information should not be placed solely on the physician. Relatives should research at the same time and maintain a close exchange among themselves so that all concerned have the same level of knowledge. Conflict or interpersonal complications should be avoided at this stage. Since premature death is imminent, taking appropriate precautions is advisable. According to current scientific knowledge, the chances of survival for the patient are very low. If the relatives therefore feel emotionally overwhelmed in coping with the circumstances, they should seek help. Otherwise, undesirable developments may occur that lead to problems in retrospect.
