Care in Illness

We've now discussed what MCD is, how you get it and who gets it, how to diagnose it, what the disease looks and acts like and how to treat the specific issue causing the disease.  Now let's talk about getting the whole body system back in good working condition.

In order to successfully treat MCD, the symptoms must be managed and overall health of the patient must be stabilized.  Below are some of the symptoms of nephrotic syndrome in MCD and the way in which they are treated:

Symptom	Nursing Intervention	Rationale
Swelling (Edema) (especially around the eyes, feet, ankles, abdomen)	Administer water pills (Diuretics) as ordered	This will allow the patient's body to release excess water and salt that the body is holding on to due to the excretion of protein.
Foamy Urine (excess protein in the urine)	Administer ACE inhibitors (angiotensin converting enzyme inhibitors), ARBs (angiotensin II receptor blockers) as ordered	These two medications both interfere with the renin-angiotensin-aldosterone cycle.  This cycle is the communication between the liver, the lungs, and the kidneys to help regulate blood pressure.  It tells the kidneys when to get rid of certain minerals and when to maintain them.
Poor appetite	Assist patient with diet regulation	The patient must remain nourished and healthy while the underlying problem is treated.
Weight gain	Administer water pills (Diuretics) as ordered	Weight gain is often due to excess fluid.
High blood pressure	Administer ACE inhibitors and ARBs as ordered	See above description for drugs
High cholesterol	Assist patient with diet regulation	The patient must remain nourished and healthy while the underlying problem is treated.

As we've discussed previously, the major problem in MCD is the malformation of the foot processes of the podocytes.  The treatment for this issue is summarized below:

Problem	Nursing Intervention	Rationale
Malformed foot processes of podocytes	Administer oral steroids (often Prednisone) as ordered	Corticosteroids give a great prognosis in both children and adults.  90% of patients will respond positively.  Minimal change disease will often completely remit in children within a few weeks.  About 50% of adult patients will relapse but steroids can be utilized again.

References:

http://kidney.niddk.nih.gov/KUDiseases/pubs/childkidneydiseases/nephrotic_syndrom/#sec10

      This website provides clear detail surrounding symptoms and treatment of primary childhood nephrotic syndrome...the illness that is caused by minimal change disease.  It is very thorough and easy to understand.

 http://umm.edu/health/medical/ency/articles/minimal-change-disease

http://umm.edu/health/medical/ency/articles/nephrotic-syndrome

http://www.unckidneycenter.org/kidneyhealthlibrary/minimalchange.html

Treatment

MCD is a disease that is capable of causing some serious adverse affects to the afflicted person and it affects people of all ages, races, and genders.  However, once it is discovered, it can be easily treated and kept under control.  Glucocorticosteroids are the drug of choice.  Simply taking these will lead to remission in 85% - 90% of cases.


Natural glucocorticoids are hormones or signaling molecules.  Hormones are released by glands in the body and travel through the circulatory system to other organs and tell them to do something.  In the case of glucocorticoids, they are secreted by the adrenal cortex, which sits on top of each of our kidneys and they work on many different areas throughout the body.

Hulk = anabolic; Bruce Banner = catabolic

These hormones are steroids...but not the kind that body builders take to bulk up their muscles.  These steroids are catabolic, which break things down, and in this case 'break down' or inhibit the immune system, which is believed to cause the issues with the foot processes of the podocytes on the glomeruli.




They are widely used in treatment for minimal change disease but the specific benefits are not understood very well.  Some theories suggest that steroids help by inhibiting effects of both the innate immune system (the one you are born with ) and the acquired immune system (the one that you build up when you get sick.)  Steroids inhibit the inflammatory response of the innate immune system and inhibit t-cell activity in the acquired immune system.  (T-cells are the white blood cells that attack infection that they recognize and may play a role in attacking podocytes in minimal change disease.)

When an adult patient starts on a treatment plan of glucocorticosteroids, experts expect the disease to be gone (or in complete remission) in 8-12 weeks.  With pediatric patients, remission is expected within 4-6 weeks.  Treatment will typically continue for another six weeks following remission to allow the patient to slowly taper off of the glucocorticosteroids.

Some adverse effects of steroid treatment include -

• A compromise of the immune system due to inhibition.
• Hyperglycemia, or increased blood sugars.  These steroids can actually cause diabetes in people.
• Increased bruising of the skin.
• A calcium imbalance in the system, which can be bad for your heart and bones
• Weight gain.
• Impaired memory and attention deficit.

If any of these side effects become too severe, treatment with a particular drug will be halted and a different glucocorticosteroid will be tried.  Prednisone is a common oral steroid that is prescribed.

Minimal Change Disease is relatively easy to treat once it has been discovered.  A glucocorticosteroid is given to suppress the immune system and allow the podocytes time to heal/realign.  Often times, the disease may result in a relapse later in life.  The same course of action may be taken with a relapse.


References:

www.uptodate.com/contents/treatment-of-minimal-change-disease-in-adults

web.standford.edu/group/hopes/cgi-bin/wordpress/2010/06/glucocorticoids/

http://medicine.med.nyu.edu/nephrology/files/med_nephrology/attachments/MCD.ppt.

Signs and Symptoms

A patient suffering from Minimal Change Disease will not experience any symptoms until the disease progresses to nephrotic syndrome and too much of the protein in their body is being lost into the urine.  Some of the major symptoms that a patient will notice once their body is in nephrotic syndrome and may cause them to seek treatment are as follows:

• Swelling.  You may hear the phrase 'pitting edema.'  Edema refers to the retention of fluid and swelling of the body.   'Pitting' refers to the way that the swelling is measured.  When a healthcare worker presses into the swollen area, their fingertip will leave a 'pit' that will not immediately disappear.

• Stretch marks (known medically as skin striae [STRY-ay]) are bands or lines on the skin that are due to a rapid growth.  The skin stretches to allow more space in the body.

• They can't pee!  Or at least the patient is not urinating very often.  This is called 'oliguria.' 

• Shortness of breath and chest pain.  Some of the fluid that is being kept in the body may get into the space between the lung and the lung cavity.  In a healthy person, this space is a negative pressure area which keeps the lung expanded.  Too much fluid in the potential space will cause the lung to collapse resulting in shortness of breath and pain in the chest.

• Often times, the fluid that is being retained will result in swelling specifically in the abdomen.  This is known as ascites (Uh-SIGH-tees) and it will look something like this...

• There may be an  unexplained, general weight gain.

• The individual may complain of a headache, fatigue and/or be irritable.

• Meuhrcke's lines - these lines are white bands that run parallel to one another and perpendicularly across the fingernail.  They are a result of low protein in the body.

• Anorexia.  This is just a general term for a loss of appetite or an inability to eat.

• Foamy or frothy urine.  This is caused by an excessive amount of protein being leaked into the urine. 

Signs of MCD

The main sign that medical professionals will be looking for is the presence of protein in the urine, otherwise known as proteinuria.  Healthy people will excrete a small amount of protein daily, however, once that amount is above a certain number (150mg for adults and 29-238mg for children) it is a medical problem and indicative of nephrotic syndrome.  The tentative diagnosis is then confirmed through a kidney biopsy.



References:

 http://web.b.ebscohost.com.offcampus.lib.washington.edu/ehost/pdfviewer/pdfviewer?vid=4&sid=15a322f7-c41c-48cc-8fc6-90a0fdbcaed7%40sessionmgr115&hid=125

https://www.kidney.org/atoz/content/nephrotic

http://www.merckmanuals.com/professional/genitourinary_disorders/glomerular_disorders/overview_of_nephrotic_syndrome.html

Diagnosis

Early diagnosis of this disease is extremely challenging.  Typically, the disease has already caused nephrotic syndrome and the patient is experiencing symptoms: frothy urine, weight gain from fluid retention, and edema (swelling due to fluid retention.) Once doctors are looking for reasons for the cause of nephrotic syndrome, the kidneys are biopsied (a small piece is removed to be viewed under a microscope.)

Diagnosis of this disease is essentially how the disease gets its name.  The third layer of the glomeruli, the podocytes, are visible under a regular standard light microscope, but it is nearly impossible to see detail and the way that the foot processes fit together.  So under this microscope, all elements are seen and the glomerulus shows minimal change.

 Glomerulus under a light microscope

However, if the same cells are put underneath an electron microscope, which will magnify cells to one million times their normal size, you can see the podocytes and their foot processes clearly.  It is very easy to see the way that they should uniformly fit together to form filtration slits..and as shown below, and the way that they look when they don't.

 Normal glomerulus under an electron microscope

A - close up of normal foot processes under electron microscope

B - close up of fused, raised and thinned foot processes under electron microscope

So once the symptoms start occuring the biopsy is taken and viewed and the abnormal podocyte fusion is discovered.  At this point, all symptoms can be attributed to MCD.  It is important to state here that MCD can be put into remission fairly easily once it is discovered.

However, biopsying a kidney is a fairly invasive procedure, so researchers are currently working on different methods of diagnosing MCD including the presence of certain proteins in urine, specifically transmembrane proteins that are present in large numbers in podocytes and low molecular weight proteins.  However, these diagnosis techniques are still in the early stage of research and kidney biopsy is currently the best way to make a certain diagnosis.

References:

 http://link.springer.com.offcampus.lib.washington.edu/article/10.1007/s00467-014-2915-3/fulltext.html

http://europepmc.org/abstract/med/10878397

What happens to the filter? (Pathophysiology)

The cause of this disease is still unknown.  They refer to it as 'idiopathic' which is a term they use to refer to any disease in which the cause is not known.  There are active studies being conducted now to determine why the foot processes of the podocytes don't fit together properly.



Here are a few factors that scientists believe could be potential contributing factors:

• Allergic reactions
• NSAID use
• Tumors
• Vaccinations
• Viral infections

Because scientists don't know why the disease happens, it is hard to gauge the progression of the disease.  Typically, it won't be picked up on until it has already progressed to nephrotic syndrome and there are symptoms visible in or on the body.  However, basically, the podocytes (the third layer of the glomerular filtration system) become effaced, which means soften, shorten and grow thinner and some fuse together and lift while too much space is left between others.  Protein, which is a molecule that is needed in large amounts for our bodies to move, fight off infection, balance our pH levels and many, many more things, is lost into the urine.


Here is how the glomerulus work in a normally functioning kidney:

 

Recall that the glomerulus is a three-layered filter.  In a person who is afflicted with minimal change disease, the third and outermost layer (foot processes of the podocytes) have fused together, thinned and raised and are no longer evenly spaced surrounding the capillaries, which makes it impossible for their foot processes to fit together properly and tightly.  They now allow some protein molecules to seep out and into the filtrate (the liquid that is the first stage of urine.)

When protein is lost, your body cannot regulate the processes that need protein.  You may end up suffering from:

• swelling
• shortness of breath
• needing to urinate more often
• hiccups
• fatigue
• trouble sleeping
• nausea and vomiting
• dry, itchy skin.

Eventually your body will begin to completely shut down.  However, once diagnosed, MCD is manageable.  


References:

 http://web.a.ebscohost.com.offcampus.lib.washington.edu/ehost/pdfviewer/pdfviewer?sid=07f2944c-cf4a-4486-a156-1b0c1d133dc2%40sessionmgr4002&vid=5&hid=4214

http://library.med.utah.edu/WebPath/RENAHTML/RENAL102.html

http://www.nlm.nih.gov/medlineplus/ency/article/000496.htm

Prevelance and Distribution (Epidemiology)

Who can get MCD?

Anyone can get Minimal Change Disease.  It is the number one cause of nephrotic syndrome* in children.  Most of the time, the disease affects children less than five years old and boys twice as often as girls.  However, adults can acquire MCD as well.  The average age of onset in adults is 40 years and the prevalence in both genders is equal.

*Nephrotic syndrome is another name for a disease of the kidney that includes edema (weight gain due to too much fluid in the body) and loss of protein into the urine.


MCD accounts for:

     70% - 90% of nephrotic syndrome in children under 10
     50% of nephrotic syndrome cases in children ages 10 - 18
     10% - 15% of primary nephrotic syndrome in adults

How does a person get MCD?

The cause of MCD is unknown.  It is a rare disease that was first identified in the early 1900s.  It was originally called lipoid nephrosis because of the amount of lipids in the tubules and urine. 

This disease may be related to the following:

• Allergic reactions
• NSAID use
• Tumors
• Vaccinations
• Viral infections

However, more research is currently being done to understand why the podocytes of the glomeruli fuse together and become unequally spaced.

How many people are afflicted with MCD?

MCD is more common in Asia than in North America and/or Europe, although it is not understood if this is due to more cases or diagnosis methods/biopsy practices.  2-7 new cases per 100,000 children are diagnosed annually.  The current prevalence is approximately 15 per 100,000.

References:

http://www.rarediseasesnetwork.org/neptune/patients/learnmore/MCD/

http://www.slideshare.net/avalliant?utm_campaign=profiletracking&utm_medium=sssite&utm_source=ssslideview

http://www.nlm.nih.gov/medlineplus/ency/article/000496.htm

http://emedicine.medscape.com/article/243348-overview#a0199

What is Minimal Change Disease?

Minimal Change Disease (MCD, for short) is a kidney disease in which large amounts of protein are lost in the urine.  It is the number one cause of nephrotic syndrome in children and a leading cause of nephrotic syndrome in adults worldwide. Nephrotic syndrome is a group of symptoms that include protein in the urine, low blood protein levels, high cholesterol levels, high triglyceride levels, and swelling.

Your kidneys are responsible for cleaning your blood and filtering out waste products.  To do this, all the blood is filtered in the functional units of the kidneys call nephrons.  The nephrons contain two parts, glomeruli (multiple glomerulus) and tubules.  The glomerulus (one single unit) of each nephron is the first filter.  Waste products of the blood are pushed out of the glomerulus through holes.  Larger and important parts of the blood, red blood cells and protein, are kept inside the glomerulus and go back into circulation.

Here is a short video on how the parts of your kidneys function to keep your blood clean.  He talks about the kidney, nephron and glomerulus from 3:44 - 4:48.

The system in this video is normally functioning.  In MCD, the third layer of the glomerulus filter, the podocytes, have foot processes that are not shaped properly.  Typically, they are very symmetrical and fit together perfectly (kind of like interlacing your fingers together tightly) so as to not allow anything important to pass through and leave our blood stream.  MCD presents in foot processes that don't fit together, are too thin and are slightly elevated. 









The protein is filtered out of the glomerulus, lost to the blood and voided from the body in urine.  The presence of protein in the urine is called proteinuria.  The picture to the right shows the holes in the glomeruli, and more specifically the slits between the foot processes of the podocytes, allowing protein to pass through along with the waste products. 




References:

http://www.unckidneycenter.org/kidneyhealthlibrary/minimalchange.html

http://www.nature.com/ki/journal/v83/n3/full/ki2012412a.html#close

http://www.edren.org/pages/edreninfo/minimal-change-disease.php