It’s an odd disease and pretty obscure. There are only around 100 cases in the United States and fewer than 200,000 worldwide.
According to Rareshare, a site launched in July 2008 to provide resources on rare diseases, an unusual acquired disorder officially known as Systemic Capillary Leak Syndrome (SCLS) is also commonly called Clarkson’s disease. The syndrome was first identified in 1960 by researchers B. Clarkson, D. Thompson, M. Horwith and E.H. Luckey.
The Washington Post reports that the condition is marked by sudden attacks that set off a rapid drop in blood pressure as the body’s capillaries start to leak. SCLS can lead to multiple organ failure and even death within a matter of hours. Rareshare states that attacks frequently last several days, necessitate emergency care and are sometimes fatal.
Clarkson’s disease typically occurs in adults. Its appearance in children is very unusual. The condition is not hereditary and has no known causes. Medical literature points to exercise, upper respiratory infections and even hormones as possible factors. Some patients report that stress affects SCLS.
Because patients typically present with a handful of symptoms common to other conditions, it’s sometimes difficult to get a timely diagnosis. Common symptoms include swelling, muscle pain, a runny nose, dizziness, lightheadedness and abnormally low blood pressure. An individual suffering from Clarkson’s might experience a decrease in the fluid content of his blood along with an increased concentration of formed elements.
SCLS patients also experience low levels of protein in the blood, nausea, excessive thirst and generalized edema. Their clinical condition sometimes declines markedly within a matter of hours. They might also experience cold limbs with sweating, rapid swelling of all limbs accompanied by compartment syndrome and decreased urine output. A runny nose and flu-like symptoms sometimes precede an acute attack. The patient might suffer from cramps, frequently loose stools and vomiting caused by severe nausea.
Several tests are available for a definitive diagnosis. Clarkson’s disease can be identified when symptoms are coupled with an elevated hemoglobin level. Decreased serum albumin and an increase in creatine kinase (CPK) levels with significant edema and compartment syndrome are also indicators. Although a chest X-ray might show some fluid retention, the lungs of most patients are not significantly affected unless they are overagressively hydrated. Overall, most victims of this illness show monoclonal gammopathy of unknown significance (MGUS).
Several treatments are available for SCLS. Many of them utilize intravenous (IV) boluses and drips, coupled with frequent lab work. Physicians might prescribe phenylephrine or norepinephrine for hypotension. Hydrocortisone is sometimes administered via IV, and consultations are often scheduled with a doctor specializing in orthopedics because of increased limb pressures.
Each Clarkson’s attack has two phases, according to Rareshare. The first phase often lasts several days. Known as the resuscitation phase, it’s a time when physicians focus on controlling the capillary leak and maintaining the patient’s blood pressure. The second stage is typically referred to as the recruitment phase, when tissue reabsorbs albumin and fluids. The goal of this phase is to try to maintain the patient’s blood pressure at a level just high enough to ward off permanent organ damaged. Diuretics could be required, and many SCLS deaths occur during this phase.
One of the most important aspects for patients living with Clarkson’s disease is not feeling alone. Since the disease is so rare, they’re unlikely to live within commuting distance of anyone else suffering from it. So far, more than 1,000 individuals suffering from obscure medical conditions have registered with Rareshare to discuss their experiences and support each other.