Living with Lupus: Fact #23 – Lupus nephritis and my friend

Living with Lupus: As many as 40 percent of all people with lupus, and as many as two-thirds of all children with lupus, will develop kidney complications that require treatment.  I have known several lupus patients with kidney involvement, but only one person’s story stands out above all the others as both a wonderful and horrible example.  My friend was courageous and undaunted in her battle with lupus, while her medical treatment plan was fatally flawed.

My friend had lupus nephritis

Several years ago, a wonderful woman in my church was suffering with end stage renal disease.  For years, she and her husband taught bible classes to our children and hundreds of others in the church.  She lost her husband to a pituitary tumor, and in the years that followed, her Lupus went on to destroy her remaining kidney function.  Many people prayed for her and helped her any way they could, and she kept her cell phone close, even during church services waiting for the call that a kidney had been found for a transplant.

lupus nephritis

During all her battle with lupus nephritis, her treatment never involved a rheumatologist to our knowledge.  Many times, several of us who knew her, including a couple of doctors that attended our church, encouraged her to strongly to seek out a rheumatologist to treat her lupus,   She kept telling us that her nephrologist planned to send her to one eventually, telling her, “we will treat the lupus once we get your kidneys under control.”  We encouraged her to push for a referral, or to even go see my rheumatologist, but she was passive and unwilling to push her nephrologist for a different approach.  She wouldn’t question him!

We all tried to explain to her that if lupus was what was causing her kidney failure, and they would probably never get her kidneys under control without controlling her lupus.  From what she told us about her medications and treatment plans, it was clear that her doctor never addressed her lupus!  Meanwhile, she continued to attend church services, prepare financial statements as the church treasurer, and even sang in the church choir.

kidney transplant

One day, she finally received the call and a successful kidney transplant surgery was done! For awhile, she was so much better.  But, she still did not see a rheumatologist.  Although the anti-rejection drugs she was receiving were doing a great job of preventing the organ rejection, they were not controlling her underlying lupus.   Although we continued to urge her to seek out a rheumatologist, she never did.

Eventually, she began to have problems with lupus attacking her transplanted kidney!  But, still, no rheumatologist. She was losing her transplanted kidney, not from rejection, but from lupus. 

About this time, I attended an LFA patient/doctor conference held in Alaska.  When I saw one of the physicians’ continuing medical education sessions was going to be taught by one of the world’s leading nephrologists, I decided to sit in with the doctors and listen.  I sat in the back of the conference room, thinking about my friend as I listened to discussions of the standard treatment protocols for lupus nephritis.  I realized from what my friend had told me about her medications, none of the standard treatments were tried on her.  I became more frustrated as the session progressed.

The most profound information I took away from the session what that almost without a single exception, emphatically, a lupus patient should never lose a transplanted kidney.  I spoke with the speaker after the session, and asked about the likelihood of losing a transplanted kidney.  The response?  “Once the physicians realize a patient has lupus nephritis, they know what to do to prevent loss of the transplanted kidney.  It is unheard of to lose a transplanted kidney to lupus.  That should never happen!”

But, this was not the case for our friend.  No one ever treated her lupus.  It seemed clear to me then that she might have been an unfortunate of victim of ignorance and unintended malpractice.  She never was referred to a rheumatologist, let alone ever receiving the prevailing standard of care for her lupus nephritis that I had learned about at the conference.

peritoneal dialysis

Eventually, our friend completely lost all kidney function, and began peritoneal dialysis while awaiting a second kidney transplant.  She got weaker and weaker, her skin became paper-thin covered with deep purple splotches, her hair  became sparse and fine, and her heart and lungs became weak.  She eventually became so ill, the sad day came when she received the word that she was permanently removed from the transplant waiting list.  She was never going to get another kidney.

A handful of us felt so helpless, as our many attempts over the years to convince her to be proactive about her treatment failed.  We were frustrated as we had to accept that she just didn’t have it within her to question her doctor or seek a second opinion.  Ultimately, that was her choice, and we had to respect it.  But, eventually it was too late. It was not too long after she was removed from the transplant list until we lost our dear friend.

Lupus took her kidneys, but not her joy

She was a close friend to many people near me in my life, my mother-in-law, many of my best friends and all the children whose lives she had touched over the years, including my own kids.  Through all her struggles, this amazing woman’s personal faith in God and unquenchable joy seemed to overcome all the emotional challenges of her long battle with illness and organ failure.

Remarkably, she never displayed any bitterness about her lupus and kidney failure, never considered blaming her doctors, and she patiently endured the unsuccessful treatments she received.  Through all her lupus battle, she inspired and encouraged everyone who knew her.  She never ever complained about how she felt, and kept a cheerful, sunny disposition to the end.  Our dear friend had lupus, and it took her life, but it never took away her great joy.

Even now, many years later, her name is mentioned as an inspiration and an icon of nobility and courage in the face of fatal illness.  She will not quickly be forgotten.

Family lupus awareness and "Could I possibly have lupus, too?"

Sign the Lupus Awareness Pledge

How does a person know if they have lupus?

While self-diagnosis is never a very good idea, everyone should know enough about lupus to recognize possible symptoms that call for a serious conversation with their doctor.  One out of every 150 people do have lupus, and 90% of those people are women.  We all know someone who has lupus, and some of those people may be in our own families.

First, if a woman has several symptoms that are commonly associated with lupus, it is time for a conversation with the doctor about lupus.

Second, if a woman has lupus symptoms, and finds out that other family members have lupus, or even other auto-immune diseases, its important that their doctor knows other family members have lupus or similar conditions.  Relatives of people with lupus have an increased risk for lupus, and relatives of people with any type of auto-immune disease have a higher likelihood of other auto-immune diseases.

This week, some of my out-of-town relatives dropped in for leisurely Saturday afternoon lunch.

A lovely young relative sat at my kitchen table, eating her sandwich while telling me about recurring pain she was experiencing, and problems she is having with her kidneys.

She had already started seeing a nephrologist for the kidney problems, and was considering whether to follow through on kidney needle biopsies the nephrologist ordered.  After hearing her story, I encouraged her and her mother to put their apprehensions aside, and to schedule the biopsies.

As the afternoon wore on, she told about several other symptoms that, together with kidney problems, would cause any alert family doctor to send their patient for lab work and a follow-up with a competent rheumatologist.  Her symptoms included protein in her urine, mouth ulcers, joint pain, recurring round rashes on her legs and recurring inflammation and pain in her salivary glands.

I also encouraged her to get a rheumatologist on board as soon as possible.

Over lunch we chatted about the various other members of our family with other auto-immune diseases, such as rheumatoid arthritis, auto-immune thyroid disease, fibromyalgia, auto-immune psoriasis, allergies and asthma.  Unfortunately, our family has an auto-immune rich (or poor) gene pool.

She told me that her nephrologist had already begun to suspect that her kidney problems were being caused by “something autoimmune.”  (If she is reading this post, I sincerely hope it will not embarrass her that I am anonymously mentioning her situation.)  I think telling her story may help someone else.

As I spoke with her and her mother about my lupus, it became obvious to them that she may be at risk for lupus.  She did  not previously know that her various symptoms are common to lupus.  Her parents knew something about lupus.  However, even this knowledge was not enough to help them suspect that their daughter might be at risk for lupus, also.

I can see that my mission to increase lupus awareness, even right in my family, is far from over!

So, I emailed this wonderful young relative a link to the Lupus Foundation of America website, and the link to my lupus blog, so that she can start to do a little research and learn more about lupus between now and her next doctor appointment.  Now, she has enough lupus awareness to tell her doctor about her other possible lupus symptoms, those that she had previously completely disregarded as having no possible relationship to her recurring kidney problems.

Now, she knows that all her symptoms might have a common cause.

I sincerely hope my suspicions are wrong, and I pray that she does not, in fact, have lupus.  However, if her doctors eventually decide that she does, I will not be at all surprised.  If so, it is ultimately best to find this out while she is young.   Early diagnosis and treatment would give her the opportunity to properly manage it and reduce any potentially disabling impacts it could otherwise have on her life if it went undiagnosed and untreated.

How fitting it was, that we had such an important family conversation about lupus during Lupus Awareness Month!  The knowledge she gained this week about lupus could have potentially life-changing impacts.

My lovely young relative is a classic example of the great need for improved lupus awareness.  She may not end up having lupus at all, but her symptoms include the classic list of things that should send any patient to their doctor with the important question, “Someone in my family has lupus, could I possibly have lupus, too?”

Lupus advances on swollen ankles and bathroom scales

Weight at an all-time high

Some troubling symptoms have suddenly emerged with my lupus in the past few days.  Only rarely were swollen ankles part of my past lupus experience, but suddenly my calves are thickened, my ankles have mild pitting edema, my abdomen is extremely swollen (and feels like a watermelon,) my prednisone moon face is now further exaggerated, my asthmatic lungs are heavy and labored and my skin is itching terribly.  In 3 days I gained 10 pounds, and now weigh a whopping ten pounds over my previous life-time high.

To describe my present state as uncomfortable would be a gross understatement.  I feel somewhat like the blueberry bloated kid in the Willy Wonka story, ready to burst with pressure from within.  Very few garments in my closet are loose enough for comfort, and my lower limbs are not very pretty as they stick out distended from under my skirt hem.  I look down at them and see my grandmother’s ankles!  (Please don’t confuse me with the fact that I AM a grandmother…)

I did some simple research on these new symptoms and found a couple of things to discuss with my doctor on Monday: my kidneys and my heart.  The fluid retention is a possible symptom of problems with one of these organs.

So far in this life-long battle with lupus, I have been spared lupus’ attack on my kidneys.

It has long been accepted that least 50% of lupus patients have some type of kidney involvement, and recently I heard that experts have increased this number to almost 60%.  Lupus is a leading cause of kidney failure.  Lupus may have rampaged against some of my other vital organs, liver and brain, but has until now left my kidneys alone.  These symptoms could indicate that may have changed.

My heart has a couple of problems that we know about, but up to now lupus has not been one of them.  Lupus is known to be a cause of several possible heart problems, and my new symptoms connected with heart problems in lupus patients.

I have a mild heart murmur caused by mitral valve regurgitation.  This involves the same part of the heart as better known mitral valve prolapse, but is different.  A prolapsing mitral valve doesn’t close properly and stresses the heart, making it work harder with each beat.  My regurgitating mitral valve doesn’t quite seal all the way, and a little bit of blood whooshes back through the valve.  My heart takes 10 steps forward moving the blood, and one step backward.  A little bit inefficient, but has not been  problematic for the health of my heart, so far.

Feeling like the Goodyear blimp!

Am I worried?  Maybe a little, but mostly just concerned that there is something new and different going on in my body that may be caused by lupus.  It will be extremely important to connect with my doctors next week and find out what sense they can make of these new developments.  I know better than to try to diagnose myself, but the possibilities identified in my quick research encourage me to seek the opinion of real medical doctor.

In the meantime, I feel like the Goodyear blimp, and will just have to wear my “big clothes.”

Lupus awareness: Someone we know has lupus!

Lupus Foundation of America lupus awareness poster

Someone you know has lupus!  A few years ago, the Lupus Foundation of America launched a wonderful awareness campaign using this slogan.   The statement was featured on a hauntingly beautiful poster of a young woman’s face, surrounded in a field of blue-green and butterflies.  The campaign very successfully raised awareness about lupus and the 1.5 million Americans who suffer with this chronic auto-immune disease.

I still display a copy of the attractive poster in my office at work.  Over the years, my own lupus awareness was gradually raised as I learned about people I knew who had lupus.  Eventually, my lupus awareness became very personal when I learned that I, too, had lupus.

Someone I knew had lupus

One of my childhood playmates was a little girl who grew up in the house next door.   We played hop-scotch, hula hoops and skated together around the neighborhood when we were young girls.  As a young married woman, she became mysteriously ill and developed disfiguring pigmented rashes on her face.  I hurt for her.  Julie has lupus.

Thirty years ago, my husband’s college friend was engaged to a lovely woman who suddenly became extremely ill with a painful disabling illness.  We have looked on at her health in frustration for years.  She has been unable to access appropriate medical treatment, and has suffered for nearly three decades.  All the while, never successfully managing her illness.  We prayed for her.  Liz has lupus.

A vibrant woman I knew received accommodation from her employer under the Americans with Disabilities Act, and was allowed to telecommute to work a portion of each work week.  This allowed her to better cope with overwhelming fatigue she often experienced.  Because of the success of her ADA accommodation, she was able to remain employed for many more years before eventually losing her long battle with chronic illness and cancer.  She became a professional role model for me.  Kathy had lupus.

Over the past few years a friend in my church struggled, first with the loss of her kidneys, then her transplanted kidney, and finally her life.  Her doctors never found a way to stop the progression of her severe chronic illness.  This amazing woman’s personal faith in God and unquenchable joy seemed to overcome all the challenges of her long battle with illness and organ failure.  She inspired and encouraged everyone who knew her.  Gaynel had lupus.

Someone you know has lupus!

Someone you know may have lupus.  Generally, the sooner lupus is diagnosed the more manageable it is.  It has been shown that earlier diagnosis and treatment reduces the overall severity and mortality rates of lupus patients.  Someone needs to tell people that they might have lupus.  More people need to know and be able to observe and identify the signs and symptoms of lupus in people around them.  Perhaps a well-informed family member or friend will know enough about lupus to notice they are at risk.  How else will people with lupus ever find out, unless they, or someone they know, is aware what to look for?

Everyone, and especially every woman, should realize that lupus is a very important women’s health issue.  Every person should be aware enough about lupus to guide her friend, family member, or even herself to seek medical advice and treatment if they have signs and symptoms of lupus.  We should know how to recognize these signs, so that we may warn people when we think they may have this potentially disabling and life-threatening disease.

Where might you meet someone with lupus?

I thought it would be interesting to bounce around some “real” numbers to compare to common situations and circumstances, to help illustrate the incidence and prevalence of lupus around us.  These comparisons may help underscore the importance of lupus awareness in our routine social settings.

Schools

Someone you know at school has lupus

Census figures put the average American household size between 3-4 people, and this tells us that the average school child may have one sibling and 4 cousins. This means that for every elementary school classroom of 30 students in America, at least one of the students in that class, one of her siblings, or one of her cousins has or will develop lupus.  (1:180)

Statistics about lupus also reveal that, among all the students attending any given school in America, one child out of every 5 classrooms (of 30 students) has or will develop lupus in her lifetime.  Additionally, for every 300 PTA Club members in that school, 2 of these members of that organization are likely to have lupus.  Do school nurses and teachers know what to look for?

Workplace

I have approximately 1,500 co-workers at my place of employment.  Statistics about the national incidence of lupus tell me that 9 of my co-workers are likely to have lupus.  I personally know of only two coworkers that have had lupus.  I wonder sometimes who the other 7 are, and if they even know or suspect they have lupus.  If they don’t know, they may have been struggling with illness like I did, without knowing what makes them so sick.  (1:150)

Church and community groups

The church where I worship has over 300 members, and many more frequent visitors to our local community fellowship.  Statistics can accurately help me predict that 2 members of my church are likely to have lupus.  This statistic gets very close and personal, when these 2 people are identified as me and someone else I know.  The other other person was my friend who recently died from lupus-caused kidney failure.

My own church is on a par with national averages for lupus in the general population.  It is questionable whether most pastors or other community organization leaders know enough about lupus to recognize it.  Would they recognize warning signs of lupus in a suffering member of their congregation or group?  Would they have adequate lupus awareness to be able to encourage women with signs of lupus to ask their doctors about the possibility of lupus?  (1:150)

460 fans in attendance were likely to have lupus

Sporting events

My husband is a great NASCAR fan, so I thought I would “run the numbers” for the throngs of people sitting in the grand stands when he recently attended a weekend race at Phoenix International Raceway.  Statistics let us deduce that out of the 70,000 in attendance at that racing event, over 460 of the fans were likely to have lupus!

Cities and towns

In the City of Phoenix where I reside, there are well over 1.5 million other people. The entire population of my city represents the total number of people in the United States that have lupus. Of these people who are my friends, neighbors and fellow citizens, statistics tell us that 10,000 other people living in my city also have lupus.

Because 90% of lupus patients are known to be women, we can estimate that approximately 9,000 women in Phoenix may have lupus.  Is there a high enough level of lupus awareness in my community, that they or someone who knows them would suspect that these women have lupus?  Is there someone around them who knows to encourage them to ask the right questions, and get the right answers that can result in their diagnosis and treatment?

Japanese disaster victims

3,000 displaced Japanese women with lupus

As I was considering all of these statistics, my thoughts turned to current events in Japan, and the estimated half-million people who have been recently displaced by its horrific earthquake and tsunami.  Statistics let us infer that at least 3,000 of these displaced people are likely to be women with lupus.

In addition to their homelessness, I cannot even begin to imagine the unthinkable plight of these unfortunate women with lupus.  These Japanese lupus patients are likely to be suffering greatly from medication shortages, inadequate shelter and rest, and extended exposure to damaging ultraviolet light from the sun.  These women are likely to experience many lupus problems as they go through this horrible disaster.  Some may even lose their lives because of impacts that stress they are experiencing may have on their lupus.

My heartfelt thoughts and prayers go out to women with lupus who are going through this extremely stressful time in their lives.  I pray for their protection, their encouragement, and their continued faith and hope through this difficult ordeal.

What indicates a person might have lupus?

Why is lupus awareness so important? Family doctors surveyed nationally admit that they do not understand lupus well enough to recognize it easily in their patients.  People who know the signs and symptoms of lupus can encourage others to seek medical advice, or ask their doctors about their own risks of having lupus.  The evidence of appropriate diagnostic criteria helps doctors determine if a patient might have lupus.

Each case is unique and unpredictable.  It often  requires a doctor to observe their patient over an extended period of time to identify the multiple ways their patient is affected by lupus before a doctor eventually suspects lupus.  From patient to patient, and from time to time in the same patient, lupus frequently changes in its scope, severity and which body systems it will attack.  This makes it very difficult to diagnose.

Because lupus attacks varying body systems, and it is so systemic in nature, it is easily mistaken for other health problems related to these same body systems.  Typically, a patient waits multiple years and consults with several doctors before their lupus is accurately diagnosed.

Early diagnosis and treatment are known to slow the progression of the disease and increase life expectancy and reduce mortality.  Diagnosis is the first logical key to a patient receiving the treatment they need.

Additionally, lupus is a potentially disabling and life-threatening disease with at least two under-recognized high-risk complications.  It is very easy to understand why these manifestations of lupus are so import to women’s health.

• Lupus is one of the major causes of kidney failure
• Lupus is a significant cause of heart disease in women

Someday, a life helped by your increased lupus awareness might be very close to you.  It might even be your own!