The Reluctant Donor

I’m pretty sure I’ve mentioned the exceedingly personable folks I met at the kidney disease think tank and then the AAKP National Patient Meeting earlier this year. Actually, you’ve already heard from one from them. This past July, Cindy Guentert-Baldo guest blogged about being a PKD patient. Today’s guest blog by Suzanne F. Ruff looks at the other side of same kidney disease. Ms. Ruff is no stranger to spreading awareness of kidney disease as you can see by her credentials:

author of The Reluctant Donor

Freelance writer for The Charlotte Observer

Executive Board of Directors American Association of Kidney Patients (AAKP)

Living Donor Council of The National Kidney Foundation (NKF)

Published in Chicken Soup for the Soul: Grieving & Recovery & Say Hello to A Better Body

Before you start reading Suzanne’s guest blog, I feel it only fair to warn you it left me in tears.

Why am I called The Reluctant Donor?  A simple answer is because I cried and whined all the way into the operating room to donate a kidney to my sister.  But it’s really not simple.  It’s complicated.

I really didn’t like my sister.  Okay, okay, I know.  If you have a sibling, you probably know what I’m talking about . . . siblings can drive you crazy.  If you don’t have a sibling, well, it’s complicated.  That’s part of the reason I titled my book, The Reluctant Donor, but not quite the whole reason.

On my journey to become a living kidney donor to a sister I didn’t like, I learned a lot of things.  Probably the most important thing is that although I may not have liked my sister, I discovered how much I love her. When I didn’t like her, it was because she was crabby grouchy and scared.  I learned something from that, too.  My sister was crabby and grouchy because she was ill…very, very ill.  That’s what happens when you don’t feel well, when your kidneys fail, and when you’re scared, terrified and afraid: you are not yourself.

I also learned denial is a powerful thing.  My sister was in denial.  Kidney disease does that to you; my sister and I should know.  We were born into a family chockful of people with kidney disease. Polycystic kidney disease or ADPKD (Autosomal Dominant Polycystic Kidney Disease) to be exact. This is a hereditary disease that causes cysts to grow around both kidneys causing the kidneys to fail.  If one of your parents carries the gene (our mother did), you have a 50% chance of inheriting the disease.   My sister, along with my other sister, inherited that gene from our mother.  Our mother, along with Mom’s two brothers and two sisters, inherited that gene from their mother.

Yes, sirreee, we were chockful of kidney disease. Over twenty-three family members now have or had the disease. We’ve had ten deaths from kidney disease, including our mother.

I did not inherit the gene that causes the disease.  Many people ask me if I feel guilty, sort of like survivor’s guilt, because my sisters have the disease and I don’t. I don’t feel guilty.  A person has no power over what genes they inherit.  But, I do feel a tremendous responsibility to do what I can to eradicate the disease that has ravaged my family.  So, I wrote my book. 

There is no cure for PKD.  Growing up I learned I was named after my grandmother who died of polycystic kidney disease before I was born.  When her kidneys failed, the doctors told her there was nothing the doctors could do for her. Mom described my grandmother’s death: Mom, a teenager then, her father, her brothers and sisters were gathered around my grandmother’s hospital bed, when my grandmother sat straight up and said, “Here I am, Lord!” and died.

The disease then hit five of my grandmother’s six children, including my mother. Through their suffering and deaths, I have learned courage and faith.  One of my aunts diagnosed with PKD in the 1960’s was one of the first to be able to try the new-fangled machine called dialysis. But, alas! There were not enough dialysis machines!!!!!  My aunt was a Roman Catholic nun.  She offered to give up her spot on the waiting list and died a few months later. She was 45 years old.

Presently, my three cousins, all brothers, suffer from polycystic kidney disease.  Their eldest brother, John, passed away from polycystic kidney disease (PKD) in 1996. Two of the three brothers are on dialysis and the other brother will need dialysis soon.    Their sister has offered to be a living donor to one of them, but each of them insists the other brother accept her kidney. A stalemate … as the disease progresses.

I have other stories about my magnificent family, but this blog is near its end.  You might even say none of this explains why I cried, kicked and screamed my way into the operating room to donate one of my kidneys to my sister.

Plain and simple: I was afraid.  I don’t like hospitals.  I hate them.  Growing up, the people I loved most died in hospitals.  I don’t like needles. I don’t like blood.   I was afraid I would die, afraid the surgery wouldn’t be a success, afraid my life would change because I donated.  I was always afraid of polycystic kidney disease as one by one, people I loved suffered and died.

Something happened to me, though, when my sister collapsed in kidney failure.  My faith kicked in and I stepped up.  We are blessed.  The surgery was a success. My sister is now a grandmother. Life is so precious!

Having gained both another son-in-law and my first grandchild this year, I can only agree with Suzanne… and life was precious for me before. I’m reading her book now and enjoying it. Should you decide to read Suzanne’s book (and any and all of mine), be sure to leave a review. Those are what get our books recognized… and in Suzanne and my cases, spreads awareness of kidney disease.

Until next week,

Keep living your life!

 

Cindy Tells All

On June 11th of this year, I wrote about Polycystic Kidney Disease after having met Cindy Guentert-Baldo at a kidney event. She has a type of kidney disease that I had no clue about until she started explaining it. What she had to say caught my attention, so I asked her if she would be willing to guest blog. I knew she had a family and is both a lettering artist and YouTube creator. That’s a lot of busy, especially if you’re dealing with a chronic illness. Luckily for us, she was able to work a guest blog into her busy schedule.

*****

In some ways, I live the typical middle class American mom life. I have a middle schooler and a high schooler. I work from home, my husband works a 9-5 in an office. The kids go to school, do their homework, go to activities. I have coffee dates with friends and dinner out with family, we go to the movies, we stay home and do yard work. Same routine, same rhythm as so many other families we know.

This picture doesn’t tell the whole story: I have polycystic kidney disease. I am currently in Stage 4, with my eGFR hovering around 25. My kidneys, at last measurement, were 27 and 25 cm in length.

Part of my daily rhythm is taking 10 different medications to control my blood pressure, manage other symptoms of being in Stage 4 of kidney disease and to help with my pain levels. Another part is having to take breaks when my energy flags or my pain levels get high enough to make sitting at a desk impossible. My kids have learned to read my body language so they know when Mom’s having a bad pain day. They’ve also learned to not hug me around my stomach, as my kidneys are so large that a loving hug could send me to bed for a few days.

I’ve burst a cyst making my bed, tying my shoe, twisting at the waist. I currently have a cyst the size of a healthy kidney underneath my left ribs that is a constant reminder that I am sick.

Aside from the physical problems that come with ADPKD (Let me help Cindy out here with a definition from emedicine at https://emedicine.medscape.com/article/244907-overview: “Autosomal dominant polycystic kidney disease (ADPKD) is a multisystemic and progressive disorder characterized by cyst formation and enlargement in the kidney … and other organs (eg, liver, pancreas, spleen). Up to 50% of patients with ADPKD require renal replacement therapy by 60 years of age.”), there is also the emotional baggage I carry.

This disease is genetic – I have multiple family members in different stages. In some ways, I am grateful to have people to talk to who understand without my having to explain. On a recent vacation my sister (who is in Stage 5) and I lay next to each other and just let out our frustrations and difficulties, knowing we had someone listening who understood. Our grandmother is in her 15th year with her transplant – she has impressed upon us how crucial it is to be informed about the disease in general and our health specifically.

I carry a lot of emotional, painful baggage due to this disease. Our father passed away from a brain stem aneurysm at age 40, brought on due to high blood pressure and PKD. My sister and I were diagnosed shortly afterwards. These days, as I approach 40, I live with a certain amount of terror. What if I die and leave my kids the way my dad left me? I’m aware of how unreasonable of a fear that is – my father died because he was unable to get health insurance and, thus, was unable to care for himself as his kidney disease progressed. I have learned from that.

That doesn’t change the deep fear inside me.

I also live with the guilt that I may have passed this disease to one or both of my children. Was I selfish becoming a parent knowing the kids themselves could wind up with PKD? I was healthy when I had them. I had no idea what I would be feeling like as my kidneys grew and began to fail. Make no mistake; I adore my children, and the world is a better place with them in it. But that doesn’t make the guilt go away.

I worry about having access to healthcare. I worry about dialysis with kids still in school. I worry about something happening to me the way it did to my dad. I worry about something happening to my sister the way it did to our dad. I struggle with my body image as my kidneys grow and I look more and more pregnant. I fight with my expectations of what I think my body should be able to do, and what I am actually able to do. I fight against the idea that I am a sick person.

Despite ALL of this, I love my life. I love my family. I love my friends. I live a mundane, repetitive, fantastic, beautiful life of a mom, a wife, a sister, a friend, an artist, a woman.

I am not PKD. I am a person with PKD…

And I am so much more.

*****

I have to admire Cindy for her honesty here. She would be having these feelings whether or not she shared them with us, but the fact that she did may just make it easier for other PKD patients to speak about their own fears.

By the way, The American Kidney Fund’s next webinar, Advocating for a rare disease, is on Thursday, July 26, 2018 from 2:00 – 3:00 p.m. EDT. The speakers will be Angeles Herrera, Holly Bode, You can register at https://register.gotowebinar.com/register/7986767093922676227.

In other news, the SlowItDownCKD book series now includes SlowItDownCKD 2011, 2012, 2013, 2014, 2015, 2016, and 2017, all available from Amazon.com and B & N.com. I had contemplated changing the title of What Is It and How Did I Get It? Early Stage Chronic Kidney Disease to SlowItDownCKD 2010 but rejected the idea. I like that title; don’t you? Of course, expect SlowItDownCKD 2018 early next year. These books were written for those of you who have requested the blogs in print form for those family members and friends who are either not computer savvy or don’t have easy access to a computer. It’s my pleasure to comply with that request. Oh, I still have one desk copy each of the retired The Book of Blogs: Moderate Stage Chronic Kidney Disease, Parts 1 & 2 if you’ve not received a free book from me before and would like one of them. Just respond with a comment so I know you were the first to ask.

Until next week,

Keep living your life!

PKD: That’s News to Me

For the last eight years, I’ve pretty much stuck to writing about Chronic Kidney Disease with an exception here or there. When I was at a pharmaceutical think tank to help the company understand how they could be more helpful to kidney patients, I met a woman with polycystic kidney disease (PKD).

I’d heard of it and knew it had to do with multiple cysts on the kidneys, but that’s all I knew. That got me to thinking. Why didn’t I know more and what more should I know about it? So I did what I do best: decided to write about it.

Right now, the former English teacher in me is begging to come out. Indulge me, please. Poly is a prefix meaning many. Cyst means an abnormal sac in the body which contains air, fluid, or a semi-solid substance. Thank you What Is It and How Did I Get It? Early Stage Chronic Kidney Disease for the definition of cyst. Ic is simply a suffix meaning of or about. Aren’t you glad I studied English at Hunter College of the City University of New York all those years ago?

Seriously now, I turned to PKD Info at https://www.pkdinfo.com/ only to discover there are two different kinds of polycystic kidney disease. Let’s start with a simple definition of the term PKD.

“PKD describes a group of genetic diseases that cause cysts to form and grow in the kidney. Genetic diseases are the result of changes, or mutations, in a person’s DNA, and can be passed from parent to child. In PKD, cysts are filled with fluid. Over time, they expand, making the kidneys grow larger. This makes it hard for the kidneys to function normally and can lead to kidney failure.”

As for the two different kinds, the PKD Foundation at https://pkdcure.org/what-is-pkd/ tells us:

“There are two types of PKD: autosomal dominant (ADPKD) and autosomal recessive (ARPKD). ADPKD is the more common type and affects more than 600,000 Americans and 12.4 million people worldwide. ARPKD is a rare form of the disease that occurs in 1 in 20,000 children worldwide.

A typical kidney is the size of a human fist and weighs about a third of a pound. PKD kidneys can be much larger, some growing as large as a football, and weighing up to 30 pounds each. The number of cysts can range from just a few to many. The size of the cysts can range from a pinhead to as large as a grapefruit. Although the primary sign of PKD is cysts in the kidneys, there are other symptoms that can occur in various areas of the body.”

I needed more information, especially about how the two types of PKD differ so I turned to my old standby The National Institute of Diabetes and Digestive and Kidney Diseases at https://www.niddk.nih.gov/health-information/kidney-disease/polycystic-kidney-disease/autosomal-dominant-pkd  and found the following information:

“’Autosomal dominant’ means you can get the PKD gene mutation, or defect, from only one parent. Researchers have found two different gene mutations that cause ADPKD. Most people with ADPKD have defects in the PKD1 gene, and 1 out of 6 or 1 out of 7 people with ADPKD have a defective PKD2 gene….

Health care providers can diagnose people with PKD1 sooner because their symptoms appear sooner. People with PKD1 also usually progress more quickly to kidney failure than people with PKD2. How quickly ADPKD progresses also differs from person to person.”

Symptoms? What symptoms? The Mayo Clinic at https://www.mayoclinic.org/diseases-conditions/polycystic-kidney-disease/symptoms-causes/syc-20352820 answered that question:

“Polycystic kidney disease symptoms may include:

  • High blood pressure
  • Back or side pain
  • Headache
  • Increase in the size of your abdomen
  • Blood in your urine
  • Frequent urination
  • Kidney stones
  • Kidney failure
  • Urinary tract or kidney infections”

Whoa! I’ve got at least four of those symptoms, so how do I know I don’t have PKD? Remember those wonderful people who elected SlowItDownCKD as one of the six best kidney blogs two years in a row? You’re right, it’s Healthline at https://www.healthline.com/health/polycystic-kidney-disease#diagnosis. As they explained:

“Because ADPKD and ARPKD are inherited, your doctor will review your family history. They may initially order a complete blood count to look for anemia or signs of infection and a urinalysis to look for blood, bacteria, or protein in your urine.

To diagnose all three types of PKD, your doctor may use imaging tests to look for cysts of the kidney, liver, and other organs. Imaging tests used to diagnose PKD include:

  • Abdominal ultrasound. This noninvasive test uses sound waves to look at your kidneys for cysts.
  • Abdominal CT scan. This test can detect smaller cysts in the kidneys.
  • Abdominal MRI scan. This MRI uses strong magnets to image your body to visualize kidney structure and look for cysts.
  • Intravenous pyelogram. This test uses a dye to make your blood vessels show up more clearly on an X-ray.

Did I just read THREE types of PKD? I did. Maybe I’d better find out what the third one is. To do so, I turned to News Medical at https://www.news-medical.net/health/Polycystic-Kidney-Disease-vs-Acquired-Cystic-Kidney-Disease.aspx.

“The cause of ACKD is not fully known, and contrary to PKD, it tends to develop after a patient has had chronic kidney disease for some time – most commonly when they are undergoing renal dialysis to clean the blood (for example, in end stage renal disease). The cysts are created by the build-up of waste products and the deteriorating filtration in the kidneys.”

ACKD is Acquired Cystic Kidney Disease. It seems I have nothing to worry about at this point in my CKD, but I’m wondering how many of you know if there is PKD in your family history. Maybe it’s time to find out. Notice none of the tests are invasive. You know, of course, that we’ve just scratched the surface of PKD information today, right?

I did have cysts show up in both of my kidneys and my liver, but they were very small despite some growth being noticed and there were very few of them. I feel like I’ve dodged a bullet.

How are you beating the heat this summer? I’m hiding in my air conditioned office separating The Book of Blogs: Moderate Stage Chronic Kidney Disease, Part 2 into two less unwieldy books each with larger print and an index. I’ll let you know when SlowItDownCKD 2013 and SlowItDownCKD 2014 are available. Surely you’ve noticed that The Books of Blogs: Moderate Stage Chronic Kidney Disease, Part 1 is no longer for sale. That’s because it has now been separated into SlowItDownCKD 2011 and SlowItDownCKD 2012.

Until next week,

Keep living your life!