From NBC Channel 10, Philadelphia, PA, By Megan Holmes
A secret search for a kidney is the journey Savannah Lewis and her mom Melissa Lewis are on. This mother-daughter duo is using social media to get the word out that Savannah’s father needs a new kidney without him knowing their plan.
Jason “Jay” Lewis, a 39-year-old father of six and a husband of 10 years to Melissa, 38, has stage five kidney failure due to polycystic kidney disease. Jay discovered he had polycystic kidney disease when he was 16. The disease is genetic and Melissa says unfortunately each kid has a 50-50 shot of getting it.
"I always pray that none of mine (kids) have it," she said.
Recently Savannah, 8, one of the couple's six children, made a video aiming to get “one million likes and shares” hoping to spread the word of the family's search for a "gift of life" as they call it.
The Scranton, Pennsylvania-based family has two different Facebook platforms they are using to get the word out, and Melissa’s husband still doesn’t know they’re “on the search for a living kidney donor.”
Originally, Melissa began the Kidney For Jay Give the Gift of Life Today Facebook page in March 2017. Her daughter Savannah wanted to help out. Savannah created her own Facebook page called Savannah’s Secret Gift of Life Search.
"We are searching the world for an extra special amazing person to give my husband his life back and be able to have the energy to watch our six kids grow up,” Melissa said.
Jay's has type B blood but can accept from an O. He is on transplant list at The Hospital of the University of Pennsylvania.
Although their search is a secret for now, Melissa was willing to risk it to get the word out there.
“For a great cause and if it will get the word out so it helps us to find a living kidney donor, I say go for it," she said.
Jay's mother, Cate, is also in need of a kidney transplant.
"I asked his mother if I can do the same (search through Facebook) for her and she wants to hold off on herself until her son gets one," Melissa said. "We all work for Davita dialysis -- me, my mother-in-law, and my husband."
Jay has been a biomed tech for over 20 years and has worked his way up.
“All of the units that Jay goes to... everybody at work has managed to keep it a secret," Melissa said.
While secret-keeping is doing well at home, there are plans to publicize even more out of view from Jay. Melissa's brother Scott Brunnenmeyer is putting up a billboard in Philadelphia off the turnpike in mid-June.
“It’s hard trying to keep one of the biggest secrets of a lifetime from your best friend," Melissa said. "But, so far, the world and I aren’t doing such a bad job at it.”
PKD Research
From Cleveland Clinic Journal of Medicine
Jason “Jay” Lewis, a 39-year-old father of six and a husband of 10 years to Melissa, 38, has stage five kidney failure due to polycystic kidney disease. Jay discovered he had polycystic kidney disease when he was 16. The disease is genetic and Melissa says unfortunately each kid has a 50-50 shot of getting it.
"I always pray that none of mine (kids) have it," she said.
Recently Savannah, 8, one of the couple's six children, made a video aiming to get “one million likes and shares” hoping to spread the word of the family's search for a "gift of life" as they call it.
The Scranton, Pennsylvania-based family has two different Facebook platforms they are using to get the word out, and Melissa’s husband still doesn’t know they’re “on the search for a living kidney donor.”
Originally, Melissa began the Kidney For Jay Give the Gift of Life Today Facebook page in March 2017. Her daughter Savannah wanted to help out. Savannah created her own Facebook page called Savannah’s Secret Gift of Life Search.
"We are searching the world for an extra special amazing person to give my husband his life back and be able to have the energy to watch our six kids grow up,” Melissa said.
Jay's has type B blood but can accept from an O. He is on transplant list at The Hospital of the University of Pennsylvania.
Although their search is a secret for now, Melissa was willing to risk it to get the word out there.
“For a great cause and if it will get the word out so it helps us to find a living kidney donor, I say go for it," she said.
Jay's mother, Cate, is also in need of a kidney transplant.
"I asked his mother if I can do the same (search through Facebook) for her and she wants to hold off on herself until her son gets one," Melissa said. "We all work for Davita dialysis -- me, my mother-in-law, and my husband."
Jay has been a biomed tech for over 20 years and has worked his way up.
“All of the units that Jay goes to... everybody at work has managed to keep it a secret," Melissa said.
While secret-keeping is doing well at home, there are plans to publicize even more out of view from Jay. Melissa's brother Scott Brunnenmeyer is putting up a billboard in Philadelphia off the turnpike in mid-June.
“It’s hard trying to keep one of the biggest secrets of a lifetime from your best friend," Melissa said. "But, so far, the world and I aren’t doing such a bad job at it.”
PKD Research
From Cleveland Clinic Journal of Medicine
Vinod Krishnappa, MBBS
Department of Nephrology, Cleveland Clinic Akron General/Akron Nephrology Associates, Akron, OH
Poornima Vinod, MD
Cleveland Clinic Akron General, Akron, OH
Divya Deverakonda
Biomolecular Science, NYU Tandon School of Engineering, Brooklyn, NY
Rupesh Raina, MD, FAAP, FACP, FASN, FNKF
Consultant Nephrologist, Adult-Pediatric Kidney Disease/Hypertension, Department of Nephrology, Cleveland Clinic Akron General and Akron Children’s Hospital, Akron, OH
Address: Rupesh Raina, MD, Cleveland Clinic Akron General, 1 Akron General Avenue, Akron, OH 44307; rraina@chmca.org; raina@akronnephrology.com
Autosomal dominant polycystic kidney disease and the heart and brain
ABSTRACT
Autosomal dominant polycystic kidney disease (ADPKD) has numerous systemic manifestations and complications. This article gives an overview of hypertension, cardiac complications, and intracranial aneurysms in ADPKD, their pathophysiology, and recent developments in their management.
KEY POINTS
Hypertension and left ventricular hypertrophy are common complications of ADPKD.
Cardiovascular disease is a major cause of morbidity and death in ADPKD.
Early diagnosis and aggressive management of high blood pressure, specifically with agents that block the renin-angiotensin-aldosterone system, are necessary to prevent left ventricular hypertrophy and progression of renal failure in ADPKD.
Timely screening and intervention for intracranial aneurysm would lessen the rates of morbidity and death from intracranial hemorrhage.
Autosomal dominant polycystic kidney disease (ADPKD) has significant extrarenal manifestations. Hypertension is a common complication, arises early in the course of the disease, and is implicated in the development of left ventricular hypertrophy. Patients with ADPKD are also at risk of other cardiovascular complications (Table 1).
This article reviews the timely diagnosis of these common ADPKD complications and how to manage them.
ADPKD ACCOUNTS FOR 10% OF END-STAGE RENAL DISEASE
ADPKD is a genetic condition characterized by multiple renal cysts.1Progressive enlargement of these cysts leads to a gradual decline in kidney function and eventually end-stage renal disease by the fifth or sixth decade of life.2 Worldwide, about 12.5 million people have ADPKD, and it accounts for about 10% of cases of end-stage renal disease.1,3,4
ADPKD has a variety of clinical presentations, including (in decreasing order of frequency) hypertension, flank pain, abdominal masses, urinary tract infection, renal failure, renal stones, and cerebrovascular accidents.2
Extrarenal complications are common and include hepatic cysts, hypertension, left ventricular hypertrophy, valvular heart disease, intracranial and extracranial aneurysms, pancreatic cysts, and diverticulosis.1–5
Less-common complications are dissection of the aorta and the internal carotid, vertebral, and iliac arteries6–10; aneurysm of the coronary, popliteal, and splenic arteries11–14; atrial myxoma15; cardiomyopathy16; pericardial effusion17; intracranial arterial dolichoectasia18; arachnoid cysts2; and intraoperative inferior vena cava syndrome (normally in ADPKD patients, pressure on the inferior vena cava results in compensatory sympathetic overactivity to maintain blood pressure), which occurs due to reduced sympathetic output under the influence of epidural or general anesthesia.19
Cardiovascular complications, especially cardiac hypertrophy and coronary artery disease, are now the leading cause of death in patients with ADPKD, as renal replacement therapy has improved and made death from end-stage renal disease less common.20,21
HYPERTENSION IN ADPKD
Hypertension is the most frequent initial presentation of ADPKD, occurring in 50% to 75% of cases and usually preceding the onset of renal failure.2,22 Hypertension is more common in male ADPKD patients, begins early in the course of the disease, and is diagnosed around the fourth decade of life.21
In a study in 2007, de Almeida et al23 used 24-hour ambulatory blood pressure monitoring early in the course of ADPKD and found significantly higher systolic, diastolic, and mean 24-hour blood pressures in ADPKD patients who had normal in-office blood pressure than in normotensive controls. In addition, nighttime systolic, nighttime diastolic, and nighttime mean blood pressures were significantly higher in the ADPKD group.
Hypertension is strongly associated with an accelerated decline in renal function to end-stage renal disease, development of left ventricular hypertrophy, and cardiovascular death.20,24
Although a prospective study25 showed a strong association between renal stones and hypertension in ADPKD, the relation between them is not clear. The incidence of renal stones is higher in hypertensive than in normotensive ADPKD patients, although evidence has to be established whether nephrolithiasis is a risk factor for hypertension or the other way around.25
Hypertension in ADPKD is multifactorial (Figure 1). The major factors associated with its development are increased activation of the renin-angiotensin-aldosterone system (RAAS); overexpression of endothelin receptor subtype A (ET-A) in cystic kidneys; increased production of endothelin 1 (ET-1); and sodium retention.26–31
The renin-angiotensin-aldosterone system
Activation of the RAAS plays a major role in the development and maintenance of hypertension in ADPKD. This is thought to be mainly due to progressive enlargement of renal cysts, which causes renal arteriolar attenuation and ischemia secondary to pressure effects, which in turn activates the RAAS.26,30,32–34 Two studies in patients with normal renal function found that cyst growth and increasing kidney volume have a strong relationship with the development of hypertension and declining kidney function.35,36
Ectopic secretion of RAAS components in polycystic kidneys has also been implicated in the development of hypertension, whereby renin, angiotensinogen, angiotensin-converting enzyme (ACE), angiotensin II, and angiotensin II receptors are produced in the epithelium of cysts and dilated tubules in polycystic kidneys.37–39 Proximal renal cysts and tubules produce ectopic angiotensinogen, which is converted to angiotensin I by renin in distal renal cysts. Angiotensin I is converted to angiotensin II by ACE in distal tubules, which in turn stimulates angiotensin II receptors, causing sodium and water retention in distal tubules.37 This may be responsible for hypertension in the initial stages; however, RAAS hyperactivity due to renal injury may predominate during later stages.37
Increased RAAS activity also increases sympathetic output, which in turn raises catecholamine levels and blood pressure.34 A study showed higher levels of plasma catecholamines in ADPKD hypertensive patients irrespective of renal function than in patients with essential hypertension.40
ET-A receptor and ET-1
A few studies have shown that in ADPKD patients, increased density of ET-A receptors and overproduction of ET-1, a potent vasoconstrictor, play a significant role in the development of hypertension and gradual loss of kidney function due to cyst enlargement and interstitial scarring.28,29Ong et al29 found that expression of ET-A receptors is increased in smooth muscle cells of renal arteries, glomerular mesangial cells, and cyst epithelia in ADPKD.
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