This article is written by Lou Antosh, one of NephCure Kidney International’s co-founders and current Advisory Committee Member. It was originally posted in a magazine in conjunction with NephCure’s New York Countdown to a Cure event in 2008. Lou’s daughter was diagnosed with FSGS in 2000, when she was 16 years old. 

It wasn’t a Big Bang that started NephCure. More like rising whimpers from hurting families scattered about in Michigan (The Smoklers), Seattle (The Ortons), and Philadelphia (The Stewarts and Antoshes). We found each other out of desperation, shocked and angered to hear that Nephrotic Syndrome and Focal Segmental Glomerulosclerosis (FSGS) were researched too little.

When Brad Stewart, my wife and I went to Bethesda, MD, to ask the National Institute of Health
(NIH) why so little was being done, the Feds encouraged us to be advocates. We can’t lobby Congress, they said, but you can. They told us of a few other individuals we might want to contact. As a result, I first heard the voice of Irv Smokler on the phone. He and Brian Orton had chartered an organization. We needed to form a board, to talk about our stores, about fighting for the kids.

There is no NephCure without Irv, whose inner fire raged despite his son’s remission. Long before us, he befriended kidney researchers at the University of Michigan and elsewhere, supported them, learned the lingo. Irv understood our fears. He always asked sincerely how our kids were doing. He knew we needed to find allies in Washington, DC. He asked always “What can I do?” He did a lot.

Very early on, we watched as top FSGS researchers met at the NIH to discuss the Nephrotic Syndrome/FSGS problem and possible battle plan. Irv understood more than me, but one fact was clear: these distinguished doctors didn’t know nearly enough.

The life of a baby is cute in the watching, but boring in the telling. Our infancy had mistakes, stops and starts. The only money for a long time was Irv’s. We held our first ptient education day in Philadelphia on the day our daughter was married. I helped set up and left, only to hear later from Brad that one benefactor had handed us a check for $10,000. It seemed a sacred moment, like God telling us: “This is a good thing.”

We moved headquarters from Michigan to a tiny office in New Jersey and searched for an executive director. Henry Brehm showed up an after that, no mistakes were big enough to stop us. I can’t figure out why his phone ear is normal sized. Passion, intensity, endurance and more, all in a gentlemanly package. A dynamo whose staff is every bit as amazing as the chief.

The branches sprouted. Melanie, Chrissy and Autumn testified before Congress. Our truly caring advisory board gave us street cred when we exhibited. We had a patient day in Seattle and Brian Orton eventually popped an idea—a motorcycle event that has yielded huge funds for NephCure. The Stewarts threw a beef and beer and others followed suit. It would take thousands of words to list all of you who built NephCure with pain and walk-a-thons and checkbooks. It grew, and the NIH officials watched and said, yes, this is good.

Since, we have dedicated over $6 million towards research—an astounding amount considering where we started, and our growth continues.

Recently, our biggest breakthrough came in the form of NEPTUNE, the Nephrotic Syndrome Research Study Network. We partnered with research institutions and with $6.25 million from NIH, this five-year, $10.25 million study hopes to open doors to the answers we are looking for.

Until the disease is cut off at its roots, NephCure will grow because it is fed by the desperation and belief and hope of all of you who read this.

Your whimpers shall never be in vain.

 

If you or a loved one suffer from Nephrotic/Nephritic Syndrome, FSGS, IgAN, or other protein-spilling kidney diseases, please consider joining one of the 25+ clinical trials in the glomerular disease space. To find the trial right for you or your loved one, visit KidneyHealthGateway.com

NephCure is proud to announce one of its Scientific Advisory Board members, Dr. Jochen Reiser is the recipient of the 2019 Hero of Medicine Halo Award. This award celebrates scientists for their tireless pursuit of cures and treatments that help people live better lives.

Dr. Reiser will be honored with the award Saturday, October 12, 2019 at the 2^nd Annual Halo Awards celebration at the Museum of Science and Industry in Chicago, IL. For more information about the Halo Awards, please visit www.halocures.com/translation/2019-halo-awards

An avid kayaker, runner, and all-around outdoorsman, Robert Gyurjan is turning his passions into a way to raise awareness about focal segmental glomerulosclerosis (FSGS).  Originally from Debrecen, Hungary, Robert moved to the United States in 2000.

It wasn’t until eight years later that he realized something wasn’t quite right with his health. He was ultimately diagnosed with FSGS at the age of 30. Rather than let the disease take control of his life, he has found a way to use what he loves to raise critical funds to help find a cure; even creating the “R U Kidneying Me?” team.

NephCure: How did you first get involved with NephCure Kidney International?

Robert: I was diagnosed with FSGS in 2008. My proteinuria was somewhat under control for years, then a test at the end of 2017 showed that things might be going downhill. So, I wanted to search for and reach out to other patients looking for doctors and resources. A Google search led me to NephCure and I was hooked from there on out!

What are some goals you are setting for yourself?

I started fundraising for NephCure last year and I’m continuing my efforts this year. While the obvious goal is to raise more money each year, I have realized that raising awareness is just as important. I’m trying to reach more people and educate them on FSGS through various type of marketing tools such as social media and newspaper articles. I was even able to put up a billboard encouraging folks to donate to my team “R U Kidneying Me?”

What are some of your successes to date?

In 2018, I was able to raise a little over $4,200. This year, as part of Countdown to Cure in Boston and a New England champion, I have been able to raise about $3,500 so far; those numbers are increasing as you read this!

Are there any challenges you have faced?

Since I use the Josh Billings Triathlon as a ‘vehicle’ for my fundraising efforts, there is always a personal challenge to complete this 27-mile bike, 5-mile kayak and 6-mile run competition. Last year I did all three myself, but this year I was a kayaker as part of a team. On the other hand, while the local hospital has a great nephrology department, it seems like there is never enough doctors and having a difficult time with replacements.

Why is it so important for you to get involved with NephCure and our mission?

Most of the volunteers are either related to patients or patients themselves. There is nothing more driven, dedicated than a heart of a volunteer. I have only met/talked with a handful of NephCure friends, but I have always felt a special connection from the first minute I got involved with them. Whether it was discussing a project or talk about the disease, it is truly like a big family.

Is there anything else you’d like to mention?

With some much-appreciated help, I have created my own promo and team name, “R U Kidneying Me?”, with a weblink www.TeamRUK.comthat is linked to my NephCure fundraising page for easy access. I am also a single father of a wonderful 10-year-old son, Sammy. Hockey and baseball are his passions!

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This past weekend kicked off NephCure’s successful ‘fundraising season’ with two first-year events, Rocky Mountain Pig Jig and Philadelphia Countdown to a Cure, as well as an outstanding New York Walk. Combined, the three events raised roughly $115,000to support our mission of finding better care and new treatments for those battling rare protein-spilling kidney diseases, like Nephrotic Syndrome.

On Thursday, September 19, the Inn at Villanova in Wayne, PA transformed into the 2019 Philadelphia Countdown to a Cure. The tables were set, auction items displayed, and dinner was served. Over 80 guests showed up to the evening dedicated to funding research and finding a cure for our community battling rare kidney diseases.

“This is the first time we’re honoring those specifically in the medical field, I think that’s very special,” Deb Pollock, NephCure’s National Director of Advancement said. “It was heartwarming to see how involved and invested each physician, researcher, and medical professional, who attended Countdown, was.”

One of NephCure’s original co-founders, Lou Antosh, proudly took to the podium to explain the history and significance the organization plays in patients and physician’s worlds alike. He went into great detail, making sure the crowd thoroughly understood the dedication and work Dr. Lawrence Holzman, the 2019 Philadelphia Countdown to a Cure Honoree, has put into figuring out what exactly the disease is.

Just days after, on September 21^st, over 1,700 miles away in Colorado Springs, CO, another first-year event was underway: the Rocky Mountain Pig Jig. A simply beautiful afternoon to invite hundreds of people out for the day comprised of live music, dancing, games, prizes, and a BBQ competition.

“From the moment you stepped foot into the parking lot, the overwhelming and delicious smells from the BBQs surrounded you. I was there at 8am and even then, I thought ‘Wow, I’m already hungry!’” Pollock recalled.

With the BBQ smell wafting throughout UCHealth Park, the live music billowed throughout the stadium. Several bounce houses, cornhole boards, and craft tables resembled paradise for the children at Rocky Mountain Pig Jig. While their parents lingered around their version of paradise– the beer garden.

The event not only welcomed those from Colorado Springs, but also those from neighboring states and even across the country.

Meanwhile in New York, on the very same day, nine teams helped raise more than $40,000 for our organization at the region’s walk held in East Meadow, NY. Dozens of volunteers, patients,and their families rallied together to share their personal stories and walk side by side for a cause that affects their loved ones.

Our regional leader, Marlene Botta, accepted an Emerging Leader Award from the New York State Assembly on the behalf of her daughter, Jackie. Marlene and other NY Walk committee members put in an immense amount of work to organize a fantastic event for those affected by rare kidney diseases.

The combined $115,000 raised for NephCure’s mission of finding better care and new treatments for those battling rare forms of kidney disease could not have come at a more crucial time. After decades of work, the hope of new therapies is now very near. We have the rare opportunity to bring the first treatments ever for Nephrotic Syndrome into the hands of patients in the next several years. Thank you for all who attended the 2019 Philadelphia Countdown to a Cure, Rocky Mountain Pig Jig, and New York Walk. Upcoming NephCure events include Silicon Valley Pig Jig and New Jersey Countdown to a Cure, to find more events in your area visit give.nephcure.org.

Just like any typical 14-year-old boy, Rocco Murdocca was growing and changing. On the outside, nothing set him apart or caused alarm for illness.

The changes were normal, gradual, and slight enough that as an outsider, you wouldn’t be able to tell the difference. In fact, his parents, Julia and Rocco Sr., had a hard time realizing anything was wrong. The only sign that sparked concern was his weight gain.

“Over the course of a year, he gained almost thirty pounds! I thought he was getting fat and made him join the gym. He kept saying, ‘Ma, everyone at the gym says my legs are jacked.’ But they weren’t muscular, they were just building up with fluid,” Julia explained.

Confusion engulfed the Murdocca family, as they tried to piece together what could possibly be happening to Rocco’s body.  “There were a lot of signs that were there, but we just never knew,” she recalled.

After a counterproductive trip to urgent care, Julia instinctively knew her son needed to see another doctor. Rocco’s pediatrician was the first to notice the spilling protein.

“The pediatrician then told us to go to the emergency room and that’s where they told us he has Nephrotic Syndrome,” Julia said.

During his stay 4 day stay in the hospital Rocco received several rounds of albumin infusions to improve his renal function. Over that short time, he lost the thirty pounds he had gained over the previous twelve months.

Just shy of his 15^thbirthday, Rocco was initially given a rigorous course of steroids, but Julia demanded he receive a biopsy. It was that biopsy that confirmed their fear: focal segmental glomerulosclerosis (FSGS).

“I felt like I was in a dream. We all thought, we have a diagnosis, he’s going to take medicine, it’s going to get better. This happens to other people, this doesn’t happen to us,” Rocco’s mother said.

To their dismay, the steroids did not work, and Rocco was then given a concoction of alternative second line drugs.

“They did more damage than help for him. He wasn’t responding to any medication,” Julia noted.

Almost immediately after Rocco’s diagnosis, she saw a shift in her family. Rocco Sr. and her three older daughters kept their emotions, thoughts, and words to themselves.

“We were all just numb. We were afraid to discuss our feelings. My children were afraid to come to me and say, ‘I had a bad day.’ It was weird,” Julia said.

Despite the abounding hospital visits, medication, and side effects, she came to understand the importance of not allowing the disease take control of her and her family’s life.

“Don’t focus on the disease. You have to focus on your whole family because when you neglect other members of the family, that’s when you start to fall apart. That’s when you let the disease control your life,” Julia advised.

“Pay extra attention to the other children in your family that are healthy because although what they’re going through is not as serious as your child who is affected, what they’re going through is just as important.”

In the beginning of 2019, Rocco’s kidneys rapidly declined, losing nearly 90% of their function. This propelled him into dialysis only a month later, with a kidney transplant on the horizon.

Ultimately, Julia and Rocco Sr. made the decision to delay the transplant so that their son could enjoy his senior year of high school to the fullest.

“He wanted to go to prom, he wanted to go to on his senior trip, and graduate. So, we did dialysis until he graduated high school in June,” Julia noted.

In the meantime, both parents were tested and ended up being a donor match for Rocco. With Rocco Sr. being a stronger match, he agreed to give one of his kidneys to his son. And so, the preparation began. First came Rocco’s double nephrectomy on July 5, 2019.

The transplantation date was scheduled for just over a month later, on August 13^th. Julia and her family remained steadfast, mentally preparing for the transplant without letting it consume their entire world.

As the transplantation date arrived, Rocco and Rocco Sr. courageously took on the disease together. Unfortunately, just four days after receiving his father’s kidney, it was confirmed Rocco’s FSGS came back. “We were absolutely devasted,” Julia declared.

Since August 17^th, Rocco has been undergoing plasmapheresis and rituximab infusions in hopes of achieving remission.

“My son is resilient; I wish I had half of the strength that he has,” Julia expressed.

The Murdocca’s focus now remains on bringing Rocco back to full health.

Although, Rocco currently has a lot of unknowns, one thing he’s certain about is his love of all thing’s music. While he’s not positive where he’ll go to college, he knows his passion for playing instruments and songwriting will drive him to great success.

With a diverse background in medicine, our new Director of Clinical Outreach, Kristen Hood, will bring more to NephCure Kidney International than just her 15 years of nursing knowledge. Kristen is a mother, and her 10-year-old son, Logan, was diagnosed with Nephrotic Syndrome back in 2016.  

Kristen’s unique personal and professional experience with these glomerular diseases gives her invaluable insight on how to engage, educate, and mobilize patients into clinical trials through KidneyHealthGateway.com.

If her name sounds familiar, it’s because Kristen has been an active volunteer for NephCure for over 3 years! Learn more about her and her family’s story and see what she’s looking forward to during her time with our organization.

As the Director of Clinical Outreach, what does your role consist of at NephCure?

Professionally, I have been a nurse for over 15 years. In my position with NephCure, I will be using my nursing and medical knowledge to engage, educate, and mobilize patients into clinical trials through Kidney Health Gateway.

Can you tell us a little about your family’s story and how you became involved with NephCure in the first place?

Our son Logan was diagnosed with Nephrotic Syndrome in July 2016.  He had been ill for several months. He had 2 misdiagnoses, then while on vacation became acutely ill and was rushed to ER.

When we received Logan’s diagnosis, we began scouring the internet for information and support. NephCure was the first website that popped up. I recall while registering on the NephCure website, a friend from college reached out and asked if she could share my information with a mom who had a daughter with a similar diagnosis. Turns out, she was referring to NephCure’s own Kelly Helm! NephCure and Kelly Helm provided more support and education to our family than we ever imagined.

How has the shift from volunteer to full-time employee been for you?

This shift from volunteer to full-time employee has been great! I have been an active volunteer with NephCure for 3 years, so I have gotten to know quite a few of the staff through different events: Advocacy Day, CDTAC, NLS, walks, etc.

Transitioning to an employee, the staff have been very welcoming and seem excited for their new team member! I feel blessed to work with the most amazing people. You can tell that everyone who works at NephCure truly believes in and works toward the mission and goals of the organization.

What are you most looking forward to in your position?

I am really looking forward to learning about our patients that have registered into KidneyHealthGateway.com. I look forward to finding out what they need and how I can help them make the decision to enter one of the listed clinical trials. I want my work to be purposeful and meaningful by bringing about better treatments, which eventually will be accessible to all.

What do you hope to share with others during your time at NephCure?

What I hope to share with others is the power and promise we have at our fingertips for better treatments, and possibly a cure, through Kidney Health Gateway clinical trials. I hope to educate and empower our patients and patient families to reach out and participate in a clinical trial. Our patients and families hold the promise for a better future…and I want them to know how important and appreciated they are, as well as supported.

How has NephCure impacted you and your family?

As a patient family, NephCure has given our family the opportunity to move away from a devastating diagnosis and the looming unknown of Logan’s future. NephCure gave us an opportunity to actively change our focus through advocacy and volunteerism.

We did not want to sit around and wait to see what would happen next…rather, NephCure gave us active ways to promote hope, positivity, and contribute to a better future for Logan and others like him.  With NephCure’s help, we turned Logan’s devastating diagnosis into purposeful action for a better and brighter future.

Why is now so important for individuals to get involved with NephCure and clinical trials, specifically?

We have, at our fingertips, the world’s most renowned and respected nephrologists and researchers, working hard to find better treatments—and maybe a cure! —to these rare protein-spilling diseases that affect the patients and caregivers of the NephCure community.

We have met with the top members of the FDA; we have leveraged our weight on behalf of the research and clinical trials for our patients and families. We now need them to engage and participate. The future of new medications, better treatments, and better quality of life is in their hands.

What is some advice you would give to parents of a recently diagnosed child?

My best advice is to take everything one day at a time. When faced with a devastating diagnosis or situation, it is easy for us to constantly be looking ahead into the unknown. We know there will be inevitable challenges, but I would caution looking too far ahead.

Keep perspective to a smaller scale and initially, just get through the days, one by one. I would also encourage parents to not forget to take care of themselves, physically and mentally. Take daily walks outside, pray, meditate, and eat healthy. You must take care of yourself too.

When you’re not at work, what can you usually be found doing?

My family loves traveling, fishing, camping, and playing sports. All of our kids play sports, so their athletic schedules keep us very busy! I am also in a doctorate program, so I am very busy with my own schoolwork!

 

Born an identical twin, Josie Dolena’s allergies set her apart from her sister, Violet, at a very young age. While both girls were originally born with dairy allergies, Violet outgrew hers as a toddler; Josie’s lingered.

Their parents, Heather and John, grew to know Josie as their sensitive child; not in the way of her feelings, but rather her sensitive immune system.

Because Josie was prone to reacting to certain foods and products, Heather acted quickly the moment she noticed changes to the then 4-year-old’s appearance.

“[Josie] woke up one morning to go to preschool and her older sister and I noticed that the one side of her face, especially her eye area, was pretty puffy,” Heather recalled.

“If it were one of my other daughters, I probably would have let it go and just kind of kept an eye on it, but my husband and I thought it was odd since it was the first thing in the morning and she hadn’t eaten anything yet to react like that.”

The couple, who both have medical backgrounds, couldn’t figure out a reasoning for the reaction and ultimately decided to take her to the doctor.

By the time the Dolena’s got to the pediatrician, Josie’s swelling went down. Her seemingly ‘normal’ physical appearance stumped her pediatrician, who requested a urine sample as a precaution.

But the swelling came back the next morning with a vengeance and getting the urine sample was next to impossible. That’s when Heather instinctively knew something was not right with her daughter.

“I kept giving her water and lemonade, it took multiple cups to get her to be able to go,” Heather said.

After numerous tests, the lab work results confirmed the worst: Josie had Nephrotic Syndrome.

The suggested treatment plan started with high dose prednisone for roughly six months, but being a child and adolescence psychiatrist, John was very hesitant about his daughter undergoing such strong medication. He has seen first-hand the mental and physical effects prednisone has on young children.

After meeting one-on-one with Josie’s doctor, John reluctantly decided to go forward with the steroids, as there is no other option available to those with Nephrotic Syndrome or other protein-spilling kidney diseases.

They were pleased when Josie’s protein spillage levels considerably dropped within just days of starting the medication.

“The biggest challenge through all these years has always been the prednisone. The nephrologist said, ‘there’s going to be side effects, it’s a tough drug to take.’ I’m a nurse, I knew it was going to be a tough drug to take, but I was not prepared for daily life with prednisone and how it affects everything, every day, all day long. We’re blessed that she reacts to it, but the side effects are just brutal. It’s a double-edged sword,” Heather explained.

After her first round of prednisone, Josie went into remission and stayed in it for 15 months.

“We were thinking maybe she’ll be one of those kids who only has the initial flare up and they never relapse again. I knew it was a small number, but we were almost getting to that,” Heather expressed.

Unfortunately, that hope quickly vanished as Josie experienced her first relapse after eating something that contained and/or touched dairy. She was quickly put back on prednisone again.

“Mentally the prednisone was tough on her. When she was very young, she would say ‘I feel sad and I don’t know why.’ She’d say, ‘I’m worried I’m going to have to start taking thatmedicine again,’” Heather remembered.

Amid the side effects, Josie responded quickly to prednisone. But just as she was weaned off, she started spilling about a month later. That’s when the brigade of relapses began.

“There was a period of time, for about 8 to 12 months, where she just would come off [prednisone] and within 4 to 8 weeks would relapse again. So, we got to the point, with the third relapse, where we started talking about moving to a second line drug,” Heather said.

After her eigth relapse, the Dolena’s added Cytoxan into Josie’s treatment plan. Finally, after some scares during the bad flu season, the now nearly 11-year-old is in remission!

“We’re at about 18 months now. We’re kind of coming up on the period where it seems like the last few relapses happened— that 15 to 22-month mark. I’m hoping to just keep going,” Heather said.

Although Josie is doing well at the moment, Heather knows the drug will cause anxieties to linger, “she associates any medicine with what prednisone has done to her and she tries to avoid medicine at all costs.”

The disease also causes anxieties to surface for Heather as well.

“I wonder, are we trying to make things as normal as they can be for her? I don’t want her to focus on the illness, I don’t want that to define her. I tend to overthink as a parent,” she said.

Turning to dance and swim as physical outlets, Josie recently competed in the county championship for butterfly stroke. As far as Heather and John’s outlets go, they turn to NephCure.

 

“You’re not alone,” Heather confirmed. She encourages other parents going through the same thing to, “Talk to other parents. Chances are there’s a ton of them that have a ton of experience going through what you are.”

Heather recently became the Patient and Engagement Volunteer Lead for the Pennsylvania and New Jersey region. She hopes to help as many of those affected by Nephrotic Syndrome as she can, making it her mission to remind them to seek treatment from a specialist, as well as get a second opinion.

“With the new patients I come in contact with I try to tell them to make sure they’re seeing a NephCure Specialist. A lot of nephrologists aren’t necessarily a specialist. I refer them to KidneyHealthGateway.com. There might be clinical trials that they’re eligible for,” she advises.

Diagnosis & Treatment of Pediatric Nephrotic Syndrome

 

Diagnosis

 

The initial diagnostic work-up includes taking a detailed medical and family history along with a general physical exam followed by common laboratory test. Additional test may be used to further classify the specific type of NS.

 

History & Physical

 

Taking a detailed medical history will allow the physician to learn about potential familial patterns or medication use that may be relevant to the presenting symptoms. During the physical your physician will use a common physical exam technique known as percussion. This is when your physician taps on certain areas on the body, paying close attention to the sound made. Normally, certain areas of the body produce a resonant sound, however dull or thud-like sounds can be heard if the air is full of fluid. Edema, or fluid buildup in tissue, is one of the major clinical features associated with NS. Another classic sign of edema is the formation of a pit on the skin when pressure is applied. This is due to the shifting water just underneath. It is important to note that several diseases can also result in edema. In order to confirm a diagnosis of NS a physician will proceed to order urine and blood tests.

 

Laboratory Tests

 

A urine test is one of the most common methods of medical diagnosis. Your kidneys play an important role in removing various waste products and excreting them through your urine. When your kidney is not functioning properly several other molecules may end up being excreted. These molecules are important and can help your physician hone in on the diagnosis. In the case of pediatric NS, the two most important values are the presence of albumin in your urine and the urine albumin-to-creatinine ratio. The presence of albumin is found by dipping a chemically treated strip of paper into the urine sample. The strip then changes color if albumin is present. Another important value to look at is the Urine albumin-to-creatine ratio. A high ratio indicates that the kidneys are leaking large amounts of protein into the urine. This is known as proteinuria and is a key clinical feature of NS.

 

Blood tests also serve as a critical test in the confirmation of pediatric NS. Two important diagnostic factors to consider are the circulating levels of cholesterol, triglycerides, and albumin. High levels of cholesterol and triglycerides as well as low levels of circulating albumin serve to further confirm the diagnosis of NS. A diagnosis of Pediatric Nephrotic Syndrome is confirmed by urinary protein excretion greater than 50 mg/kg per day and low levels of albumin detected in blood. For a detailed description of the cause behind these values, please click here to learn more.

 

Additional Test

 

Other tests that may be performed include an ultrasound of the kidney as well as a kidney biopsy. The ultrasound is a medical device that uses sound waves to visualize the underlying structures. The sound waves are harmless to the child and this examine is typically performed in an outpatient setting. In a kidney biopsy a health care provider takes a small portion of kidney tissue and then examines it using high power microscopes. This procedure can usually be done with a local anesthetic; however, some cases may require the use of general anesthesia. There are several different subclassifications of pediatric NS that can be differentiated this way. Genetic studies should also be done before starting any treatment regiments.

 

Treatment

 

Treatment regimen is dependent on which type of NS – Primary, Secondary or Congenital – a child is diagnosed with. Briefly,

• Primary NS – is NS in the absence of systemic disease
• Secondary NS – NS associated with a systemic disease
• Congenital NS – NS as a result of a genetic defect

 

Please click here for a more detailed description of the different types of NS.

 

Primary Nephrotic Syndrome (PNS)

 

PNS treatment revolves around symptom relief focusing primarily on immune system suppression, removal of extra fluid, and lowering patient’s blood pressure. The main drug of choice to subdue the overactive immune response is prednisone – a corticosteroid. Corticosteroids work by binding to genetic elements and preventing the production of inflammatory immune proteins. Studies have shown that 90% of patient achieve symptom control after 6 weeks of daily corticosteroid use. Unfortunately, many children suffer from recurrent relapses and medication use is altered accordingly. In the case that a child stops responding to glucocorticoids other immune suppressing medication can be prescribed. To address the fluid overload a physician may prescribe a diuretic, which act on different segments of the kidney’s filtration system to prevent the re-uptake of sodium. The sodium in the kidney tubules attracts water in our circulatory system. Once in the kidney tubules the water is expelled in the urine. In addition, other common blood pressure medication may be used in combination with the diuretics.

 

Secondary Nephrotic Syndrome (SNS)

 

In SNS treatment focuses on resolving the underlying primary illness this in turn will relieve the stressor causing the kidney damage. SNS can develop as a result of bacterial or viral infections as well as exposure to certain chemicals and medications. Other disease processes can also lead to the development of NS. A physician may prescribe antibiotics, adjust or change medications in order to treat SNS. In addition to these changes, a physician will also address symptom relief with similar medications used to treat PNS.

 

Congenital Nephrotic Syndrome (CNS)

 

Treatment of CNS again follows the same initial steps to control symptoms relief. The aggressive nature of this type of NS requires more aggressive treatment such as dialysis, kidney removal, or transplant may be performed. Unfortunately, many cases are fatal within the first year.

 

References

 

Niaudet, Patrick. (2019). Etiology, clinical manifestations, and diagnosis of nephrotic syndrome in children. T. Mattoo & M. Kim (Eds.) UptoDate. Available from https://www-uptodate-com.ezproxy.med.ucf.edu/contents/etiology-clinical-manifestations-and-diagnosis-of-nephrotic-syndrome-in-children?search=nephrotic%20syndrome%20children&sectionRank=2&usage_type=default&anchor=H8&source=machineLearning&selectedTitle=1~150&display_rank=1#H8

 

Niaudet, Patrick. (2019). Symptomatic management of nephrotic syndrome in children. T. Mattoo & M. Kim (Eds.) UptoDate. Available from https://www-uptodate-com.ezproxy.med.ucf.edu/contents/symptomatic-management-of-nephrotic-syndrome-in-children?search=nephrotic%20syndrome%20diagnosis&source=search_result&selectedTitle=5~150&usage_type=default&display_rank=5

End-Stage Renal Disease Life Expectancy

Renal diseases can have varying lengths of impacts on you as an individual, including shortening your life expectancy. End-stage renal diseases mean that your kidneys performance is deficient or not performing at all. It means that they are not able to get rid of different wastes from the body and you have to use other means to get rid of the wastes.

End-stage renal disease or ESRD diagnosis for a patient means that the kidney functionality is below 10% of the standard operation rates. Since one doesn’t start at the top, kidney diseases also function the same way; they gradually stop working until you reach the ESRD stage. In between, you still have your ten to twenty years of life expectancy level.

GFR or Glomerular Filtration Rate

It is a measure indicating the performance of your kidneys at any specific time. The test aims at identifying the possibility of kidney disease infections. It determines the rate at which it has advanced and the possible medication or treatment options available for the patient.

The GFR measurement is ml/min/1..73m2. With the GFR equal to or greater than 90, it means that you have healthy functional kidneys. However, the symptoms are starting to show, which is also described as stage 1. At stage 2, the GFR is to 89 functionality reduction is slight.

Stage 3A and 3B marks the full-blown kidney disease, which is now quite noticeable. The GFRs for the two is 45 to 59 and 30 to 44 respectively. Stage 4 is characterized by a 15 to 29 GFR rate.  Stage 4 paves the way to ESRD, which translates to total kidney failure with a GFR of less than 15.

Life Expectancy

Chronic kidney diseases affect old people differently from the young adults. Thus, life expectancies decrease with age when the diagnosis occurred. If diagnosed at the age of 30, you have a possibility of living 10 to 20 years. However, due to the improvement in renal treatment and technology, the lifespan may go up with a few years.

According to research studies conducted by NCBI in 2017, individuals with a GFR of 60 and above at the time of diagnosis and aged between 30 and 50 years have a life expectancy of between 24 years and 12 years. For those facing stage 4, life expectancy also increases with 20 years and seven years, respectively.

For the elderly, the life expectancy shortens even further, once they reach the end-stage renal disease. Individuals aged 60 years to 85 years have a life expectancy of 6 years and one and a half or one year, respectively.

Dialysis and ESRD Life Expectancy

Dialysis not only improves your agility against ESRD, but it also increases your life expectancy. The youngest diagnosis of zero to fourteen years, the life expectancy is 24 years for males and 22 years for females. The life expectancy reduces with age. For individuals aged 30 to 35 years, their life expectancy is 14 years for males and 13 years for females. Those aged between 70 and 75 years have a life expectancy of 4 years for both males and females.

Kidney Transplants and ESRD Life Expectancy

If lucky and have to undergo a kidney transplant, your life expectancy improves even further. You can live till you die of natural causes if the transplanted kidney are compatible with your body. For zero to 14 years of age, you can live for 59 and 61 more years after the transplant for males and females respectively. If 30 to 35years of age, you can acquire 34 and 36 years more for males and females respectively. If aged between 70 and 74 years of age, you have 9 and ten more years to live. Transplants are advised against if aged 80 and above.

Conclusion

Reaching the end-stage, renal disease is not the end. You can still live for a few more years with dialysis and kidney transplants. Even without these options, you always get a few years before time is up. Therefore, you should aim at living a lifestyle that does not provoke an increase in the deterioration rate. Aim to improve your GFR rate by following the right lifestyle for individuals suffering from kidney diseases.