From The Trentonian, Trenton, New Jersey, by Jeff Edelstein
Sheri Gould is terrified.
It’s not necessarily because she’s going to have to begin kidney dialysis soon. It’s not necessarily because she knows a lifetime of dialysis is going to be difficult on her body and mind. It’s not necessarily because for many people, dialysis is just a weigh station to the end of the line.
Nope. She’s terrified because she’s seen what happens to people with Polycystic Kidney Disease (PKD) who go on dialysis. Specifically, her own mother.
“I saw my mom struggle,” Gould, 50, of Lawrence told me. “I don’t want to go through that.”
She spoke of infections that would make your skin crawl. Extreme fatigue. Quality of life going down the tubes.
Her mother died at age 70 after 14 years on dialysis.
So Gould — like many sufferers of PKD, a hereditary disease that affects roughly 1 in 10,000 people worldwide — is seeking to do something about it.
Sheri Gould is in the market for a kidney.
Got one to spare?
If so, reach out to Sheri at her website KidneyForSheri.com.
And don’t worry — you don’t need to be a perfect match, as through the Living Kidney Donor Network, your kidney might go to someone you match with say, in California, while Gould would get someone’s else’s kidney. Think of it like four first graders swapping snacks at lunchtime. You get Greg’s Snickers, Jane gets your Twix, Paul gets Jane’s potato chips, etc. You get the picture.
Only one snag to this whole scenario though, and Gould knows it: “Yes, of course,” she said. “If I had two good kidneys, I’d be afraid to give one away.” [Read more]
Since Jan. 6, the chain has bounced across the country, including stops at MedStar Georgetown Transplant Institute in Washington, D.C.; Walter Reed National Military Medical Center in Bethesda, Md.; University of Virginia Hospital in Charlottesville, Va.; and two bouts at the University of Maryland Medical Center in Baltimore, Md.
LaTwanya Goslee, 43, tried to donate a kidney to her brother, 41-year-old Charles Muse, for several years. Their mother, Edith Summers, had donated a kidney to Muse in 2003, after he suffered from kidney failure due to hypertension. But that kidney eventually failed; he went on dialysis, and needed another transplant.
Goslee and Muse hoped the second transplant would be as simple. And initially, they were a match. But after Muse was diagnosed with cancer in one of his native kidneys, they were no longer compatible. And so, they entered the Paired Kidney Exchange Program, through the National Kidney Registry.
So, even though Goslee couldn't donate directly to Muse, by donating a kidney to a stranger, her brother would be eligible to receive a kidney from one of his matches in the system. It turns out his new kidney came from Harley Florez, a man from Colombia. He donated in South Carolina, so that his aunt living there with Polycystic Kidney Disease could receive a kidney, too. And the kidney Goslee donated at the University of Maryland Medical Center was driven by courier immediately to Allegheny General Hospital in Pittsburgh, Pa. to be transplanted into a 65-year-old retired heavy equipment operator named Gary Watson. [Read more]
I'm 47 years old and people are still asking me if I am pregnant. I try to take it as a compliment about how young I must look, but when people first started asking me, I would turn very red and uncomfortable. Then they would also turn red and it was embarrassing for both of us. I have the dominant form of polycystic kidney disease (ADPKD) and polycystic liver disease (PLD), causing my kidneys and liver to develop multiple fluid-filled cysts. As a result of cyst growth due to ADPKD, kidneys become significantly enlarged and, over time, kidney function deteriorates. ADPKD is one of the most common life-threatening genetic diseases, affecting approximately one in 500 people.
Only once, while buying a decaf coffee, did I say "yes" when asked by another customer if I was pregnant, and what a big mistake that was! I tried answering her first question, "when are you due?" and was in the middle of trying to calculate my imaginary due date when she was already on to the next: "Is it a boy or a girl?" After escaping, I looked at my husband and said that I would never pretend to be pregnant again. About 10 minutes later, someone else asked me, and we both burst into laughter.
ADPKD is a genetic disease -- and a parent carrying the gene has a 50 per cent chance of passing it on to their children -- but I mutated the disease, which means my parents don't have it, and I hadn't even heard of it until I was diagnosed about 13 years ago. At that time, our third child was about a year old when I noticed protruding lumps in my stomach and my waistline getting bigger. My doctor sent me for an ultrasound where I learned both of my kidneys and my liver were filled with cysts. I was then referred to a nephrologist who diagnosed me with both ADPKD and PLD.
I have three hernias in my abdomen from the weight and pressure of my liver. I have to wrap bandages around my abdomen every day to hold in hernias. The surgeons told me that they can't operate and I will need a liver transplant eventually, probably before a kidney transplant.
Recently, my worst fears about ADPKD came true: both of our daughters, now 20 and 17 years old, have ADPKD. Our 17-year-old daughter already has over 30 cysts in each kidney and is taking blood pressure medication. Our 14-year-old son has not been tested yet. It has been a roller-coaster journey full of disappointing news and challenges, but also opportunities and evaluating the most important things in life. Here are some of the things I've learned along the way:
Get involved with the patient community (and your local community!)
After being diagnosed, I started researching, and found out that the PKD Foundation of Canada has a chapter in Toronto and it hosts annual Walk for PKD events in September. That year, I held a bake sale in my small town of Vankleek Hill, got Scotiabank to match my fundraising, and ended up bringing over $8,000 to Toronto. The next year I started my own Walk for PKD in Vankleek Hill and 11 years later, we have raised over $170,000 for the PKD Foundation of Canada from my little community alone. It wasn't easy at first being so forthcoming about my disease, but the support from my community has been more positive than I could have ever imagined.
If you're living with a chronic illness or know someone who is, be proactive and get involved with a foundation or patient community. Knowing you're not going through it alone and have a whole network of support relieves a lot of stress and questions.
Learn to love your body
[Read more]
From Air Force Global Strike Command, by Senior Airman Stephanie Morris, Minot Air Force Base Public Affairs, North Dakota
One old adage of marriage is the vow to be there for each other, for better or for worse in sickness and in health. For many couples, this is a line they recite at their ceremony and store in the back of their minds for many years. However, for one couple, that vow was tested only a couple years after they made it.
Essence Minter was diagnosed at age 18 with polycystic kidney disease while serving in the United States Navy. Both her mother and grandmother also had the disease, but they carried mutation one -- Essence was host to mutation two.
This meant she was at a higher risk of needing a kidney transplant in the future and may even need to be placed on dialysis one day.
Essence's husband, Airman 1st Class Karlton Minter, 5th Medical Operations Squadron emergency medical technician, didn't let this news keep him from meeting her at the end of the aisle.
"I knew about the disease when I met her," Karlton said. "I wasn't afraid of it."
So Karlton and Essence exchanged vows one year after he left for basic training.
Upon graduating from Lackland Air Force Base in San Antonio, Texas, Karlton and Essence found themselves at his first duty station -- Minot Air Force Base, North Dakota.
Unfortunately, it wasn't long after they arrived before they received devastating news. Essence's kidney function was barely above 20 percent, and she had no alternative but to get a transplant.
"The kidney center at Sanford will only do a transplant if the kidney is below 20 percent function," Essence explained while exchanging a pained look with her husband.
Because of this, the two would have to wait, and Karlton was forced to stand by as his wife's health waned.
"As a husband and a man, I felt it was my duty to take care of her," Karlton said. "I was just focused on my wife and on finding a way to help her."
He began speaking to his leadership and the staff at Sanford, trying to find a way to replace his wife's ailing kidneys with one of his own.
Karlton was able to garner advice on donating his kidney to his wife from a co-worker who had recently gone through a similar situation with their mother.
Gaining permission from his leadership and the Air Force was only the first of many hurdles Karlton would face.
The staff at Sanford conducted blood work, echocardiograms and a battery of other tests on Karlton, all of which finally culminated in an extensive interview.
"They must have asked me 500 questions on that survey," Karlton remarked, sharing a laugh with his wife. "They had to make sure I wasn't being pressured to donate my kidney and I was there of my own free will."
With a blood type of O positive, and test results that showed compatibility, Karlton was finally given the green light to go under the knife to save the love of his life.
"I knew the surgery was going to be successful," Karlton said. "It was something I felt in my body, in my bones. I felt spiritually that we met for a reason and this was it."
Many hours later, Essence and Karlton awoke from the first surgery either had ever undergone surrounded by hospital staff and family members. The procedure was a success. [Read more]
Managing autosomal dominant polycystic kidney disease: an interview with Dr Richard Sandford, University of Cambridge
Please can you give a brief introduction to autosomal dominant polycystic kidney disease (ADPKD)?
Autosomal dominant polycystic kidney disease (ADPKD) is one of the most common inherited kidney diseases. It's also one of the most common reasons that people require dialysis or transplantation.
European-wide figures suggest that about 10% of people receiving dialysis or transplantation have ADPKD. Therefore, there are many more people being followed-up with this condition or who remain undiagnosed because they haven't yet developed symptoms or kidney failure.
ADPKD is a condition that is often diagnosed in adulthood and the typical features are very easy to see on a kidney ultrasound scan. The scan usually reveals enlarged kidneys that contain multiple cysts. Liver cysts are also common.
Sometimes, the kidneys can become so enlarged that they each weigh several kilograms and occupy most of the abdomen or tummy.
In the majority of cases, there is also a progressive decline in kidney function.
As a genetic condition, it typically affects subsequent generations of the same family, so many families that we see will have several family members who are affected.
Please can you explain what “autosomal dominant” means in the context of ADPKD?
Autosomal dominant refers to the inheritance pattern of ADPKD. “Autosomal” means that the ADPKD genes are carried of chromosomes apart from the sex chromosomes: therefore it affects men and women equally.
“Dominant” means that, of the two copies of each polycystic kidney disease genes we have, only one copy contains a genetic alteration or mutation. This mutation is sufficient to cause the disease.
Anybody who has ADPKD and therefore a PKD gene mutation has a 50/50 chance of passing the mutation onto their children. If a child inherits the gene they will then develop the disease later in life.
How is ADPKD currently managed and how much variation is there of clinical standards throughout Europe for ADPKD?
As there are no widely adopted, disease-specific guidelines about managing this condition, ADPKD is generally managed in the same way as any other potentially progressive forms of chronic kidney disease. This involves paying attention to the control of symptoms and blood pressure, as well as the management of cardiovascular risk.
The ‘translating science into policy’ report, the first publication of the European ADPKD Forum (EAF) has been developed to help improve the management of autosomal dominant polycystic kidney disease (ADPKD) throughout Europe. One of the objectives of the EAF initiative is to recommend strategies to improve ADPKD care within the context of health policy development at the European and national levels. [Read more]
Weight loss in a patient with polycystic kidney disease: when liver cysts are no longer innocent bystanders
Abstract
Objective and importance:
Autosomal dominant polycystic kidney disease (ADPKD) is the most frequent inherited kidney disorder, and liver involvement represents one of its major extra-renal manifestations. Although asymptomatic in most patients, polycystic liver disease (PLD) can lead to organ compression, severe disability and even become life-threatening, thereby warranting early recognition and appropriate management.
Clinical presentation:
We report the case of a 56-year-old woman with ADPKD and severe weight loss secondary to a giant hepatic cyst compressing the pylorus. Partial hepatectomy was required after failure of cyst aspiration and sclerotherapy, and patient's condition improved rapidly.
Discussion and conclusions:
We discuss the presentation and classification of compressing liver cysts, and the available therapeutic alternatives for this potentially severe complication of ADPKD.
Essence Minter was diagnosed at age 18 with polycystic kidney disease while serving in the United States Navy. Both her mother and grandmother also had the disease, but they carried mutation one -- Essence was host to mutation two.
This meant she was at a higher risk of needing a kidney transplant in the future and may even need to be placed on dialysis one day.
Essence's husband, Airman 1st Class Karlton Minter, 5th Medical Operations Squadron emergency medical technician, didn't let this news keep him from meeting her at the end of the aisle.
"I knew about the disease when I met her," Karlton said. "I wasn't afraid of it."
So Karlton and Essence exchanged vows one year after he left for basic training.
Upon graduating from Lackland Air Force Base in San Antonio, Texas, Karlton and Essence found themselves at his first duty station -- Minot Air Force Base, North Dakota.
Unfortunately, it wasn't long after they arrived before they received devastating news. Essence's kidney function was barely above 20 percent, and she had no alternative but to get a transplant.
"The kidney center at Sanford will only do a transplant if the kidney is below 20 percent function," Essence explained while exchanging a pained look with her husband.
Because of this, the two would have to wait, and Karlton was forced to stand by as his wife's health waned.
"As a husband and a man, I felt it was my duty to take care of her," Karlton said. "I was just focused on my wife and on finding a way to help her."
He began speaking to his leadership and the staff at Sanford, trying to find a way to replace his wife's ailing kidneys with one of his own.
Karlton was able to garner advice on donating his kidney to his wife from a co-worker who had recently gone through a similar situation with their mother.
Gaining permission from his leadership and the Air Force was only the first of many hurdles Karlton would face.
The staff at Sanford conducted blood work, echocardiograms and a battery of other tests on Karlton, all of which finally culminated in an extensive interview.
"They must have asked me 500 questions on that survey," Karlton remarked, sharing a laugh with his wife. "They had to make sure I wasn't being pressured to donate my kidney and I was there of my own free will."
With a blood type of O positive, and test results that showed compatibility, Karlton was finally given the green light to go under the knife to save the love of his life.
"I knew the surgery was going to be successful," Karlton said. "It was something I felt in my body, in my bones. I felt spiritually that we met for a reason and this was it."
Many hours later, Essence and Karlton awoke from the first surgery either had ever undergone surrounded by hospital staff and family members. The procedure was a success. [Read more]
Autosomal dominant polycystic kidney disease (ADPKD) is one of the most common inherited kidney diseases. It's also one of the most common reasons that people require dialysis or transplantation.
European-wide figures suggest that about 10% of people receiving dialysis or transplantation have ADPKD. Therefore, there are many more people being followed-up with this condition or who remain undiagnosed because they haven't yet developed symptoms or kidney failure.
ADPKD is a condition that is often diagnosed in adulthood and the typical features are very easy to see on a kidney ultrasound scan. The scan usually reveals enlarged kidneys that contain multiple cysts. Liver cysts are also common.
Sometimes, the kidneys can become so enlarged that they each weigh several kilograms and occupy most of the abdomen or tummy.
In the majority of cases, there is also a progressive decline in kidney function.
As a genetic condition, it typically affects subsequent generations of the same family, so many families that we see will have several family members who are affected.
Please can you explain what “autosomal dominant” means in the context of ADPKD?
Autosomal dominant refers to the inheritance pattern of ADPKD. “Autosomal” means that the ADPKD genes are carried of chromosomes apart from the sex chromosomes: therefore it affects men and women equally.
“Dominant” means that, of the two copies of each polycystic kidney disease genes we have, only one copy contains a genetic alteration or mutation. This mutation is sufficient to cause the disease.
Anybody who has ADPKD and therefore a PKD gene mutation has a 50/50 chance of passing the mutation onto their children. If a child inherits the gene they will then develop the disease later in life.
How is ADPKD currently managed and how much variation is there of clinical standards throughout Europe for ADPKD?
As there are no widely adopted, disease-specific guidelines about managing this condition, ADPKD is generally managed in the same way as any other potentially progressive forms of chronic kidney disease. This involves paying attention to the control of symptoms and blood pressure, as well as the management of cardiovascular risk.
The ‘translating science into policy’ report, the first publication of the European ADPKD Forum (EAF) has been developed to help improve the management of autosomal dominant polycystic kidney disease (ADPKD) throughout Europe. One of the objectives of the EAF initiative is to recommend strategies to improve ADPKD care within the context of health policy development at the European and national levels. [Read more]
From Maney Online
Objective and importance:
Autosomal dominant polycystic kidney disease (ADPKD) is the most frequent inherited kidney disorder, and liver involvement represents one of its major extra-renal manifestations. Although asymptomatic in most patients, polycystic liver disease (PLD) can lead to organ compression, severe disability and even become life-threatening, thereby warranting early recognition and appropriate management.
Clinical presentation:
We report the case of a 56-year-old woman with ADPKD and severe weight loss secondary to a giant hepatic cyst compressing the pylorus. Partial hepatectomy was required after failure of cyst aspiration and sclerotherapy, and patient's condition improved rapidly.
Discussion and conclusions:
We discuss the presentation and classification of compressing liver cysts, and the available therapeutic alternatives for this potentially severe complication of ADPKD.
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ReplyDelete