Steroid Sensitive Nephrotic Syndrome – Patient Information
- How the illness affects people
Steroid Sensitive nephrotic syndrome (SSNS) is a rare kidney condition. It causes excess fluid to be retained in the body which causes swelling (oedema). This is most common in the face and eye lids, and the ankles. Facial swelling is usually worse in the mornings and ankle swelling worse in the evenings or after standing.
The swelling in Nephrotic Syndrome is soft and puffy so that if you press on it lightly it leaves a dent. With more extensive fluid retention, swelling may affect the abdomen and hands, and cause a feeling of tightness. Excess fluid inside the abdomen is known as ascites. The extra fluid in the body increases body weight.
Nephrotic Syndrome makes people vulnerable to infections while they are swollen. It can also increase their risk of developing blood clots (thrombosis), mostly affecting the veins of the legs.
People with SSNS get complete or partial remission when treated with immunosuppressive medicines such as prednisolone. The excess water in the body is cleared by the kidneys and the swelling resolves. It is extremely rare for people with Steroid Sensitive Nephrotic Syndrome to switch to other forms of the syndrome. Overall the outlook in well managed patients is good and kidney function is preserved in the long run.
- What can be done about it?
The first step is to confirm the diagnosis. There are many reasons for fluid to be retained in the body to cause swelling. In Nephrotic Syndrome it is due to very heavy losses of proteins such as albumin in the urine (proteinuria, albuminuria). A urine test will confirm this.
Because SSNS is by far the most common presentation of Nephrotic Syndrome in children after the age of 1 and before adolescence, it is common practice to be given treatment with cortico-steroids such as prednisolone. If the proteinuria is switched off and the swelling goes away the diagnosis is as good as confirmed. Patients are usually asked to test their urine every morning to show when remission occurs.
There may be relapses of the disease. These are often triggered by a head cold or infection. Two thirds of patients will have relapses and for some of these the relapses are frequent and may require alternative treatments.
- Other peoples’ experiences
The NephCure website contains many personal stories, including those from patients with SRNS.
Patient Information Days
The latest Nephrotic Syndrome Patient Information Day was held in Bristol on the 29th March 2017.
Previous events were held in Manchester (2016) and Birmingham (2015) For further details of these events please visit the Nephrotic Syndrome Trust.
- Patient support group
The Nephrotic Syndrome Trust (NeST) aims to raise money for research into the causes, treatment and hopefully a cure for Nephrotic Syndrome. They have set up a number of closed Facebook groups to help those with Nephrotic Syndrome, their family members and carers to support each other in their local area.
The latest news from NeST can be found here.
Nine groups have been set up so far:
- (NeST) South West Support Group
- Nephrotic Syndrome Friends – NeST UK South East
- North East (UK) NeST Nephrotic Syndrome Support Group
- NeST Support Group North Wales, Chester, Merseyside
- Greater Manchester NeST
- NeST – East Anglia
- NeST Midlands Support Group
- NeST- Thames Valley
- Bradford Nephrotic Syndrome Support Group NeST
More information can be found on the NeST website.
The NephCure Foundation educates the public and raises awareness of Nephrotic Syndrome around the world.
- How the disease works
In SSNS extra fluid accumulates in the tissues of the body. Normally the tiny blood vessels of the body allow water from the blood stream to move out into the tissues and back again. There is a fine balance to this process.
The watery fluid of the blood stream (plasma) is rich in proteins, especially albumin. If you know that an egg white is chicken albumin dissolved in water, you get the picture of plasma containing human albumin. When the kidneys filter the plasma they normally keeps the albumin and other proteins in the blood stream and don’t let them cross over into the urine. In Nephrotic Syndrome this fails to happen so albumin pours into the urine and is lost. The body tries to make more albumin and other proteins but it is never enough, and the level of albumin in the blood falls.
In the little blood vessels of the body, albumin helps to prevent fluid from leaking out of the plasma and into the body’s tissue. So when the level of albumin is low, fluid leaks out into the soft tissues of the body where it builds up and causes swelling.
The kidneys respond to this by retaining even more water. This is simply the kidneys doing their job to ensure that the volume of fluid in the blood stream doesn’t reduce as water is shifted into the tissues. This makes the swelling worse.
The reason why the kidneys suddenly allow albumin to be lost in the urine rather than retained in the plasma is partly known. The kidney filters (glomeruli) maintain a barrier to proteins while allowing water to flow freely into the tubules and ultimately into the urine. Why the barrier changes its performance is not known.
- What’s new? Opportunities for research and development
The Nephrotic Syndrome Rare Disease Group (RDG) is working with international partners with the aim of finding new and improved treatments, and to empower patients. To do this the RDG is registering patients with this condition in the National Renal Rare Disease Registry (RaDaR). The database will be used to find suitable participants for future research trials into the effectiveness of new treatments. If you are interested in finding out more about the registry (RaDaR) or the activity of the RDG please visit the Nephrotic Syndrome RDG page.
Researchers are currently investigating the causes of Nephrotic Syndrome . They are looking for better forms of diagnosis and treatment. The research uses new cell and molecular technologies to investigate the particular cell (podocyte) that is responsible for the kidney’s ability to filter proteins, under the National Study of Nephrotic Syndrome (NephroS). For more information please contact the Nephrotic Syndrome RDG.
Written by the Nephrotic Syndrome Rare Disease Group