About My Condition

by Andrea Hoole

I am currently stage 5, and my EGFR (what measures total kidney function) is roughly 8%. My function has rapidly declined from 15% to 8% in just 2 short months. I have also been diagnosed with Polycystic Liver disease, which is where most of my complications come from in addition to my kidneys failing. Recently, during routine transplant testing, I was diagnosed with a blood clotting disorder as well as fluid building up in the outer lining of my heart, due to kidney failure. I have tried to avoid dialysis as long as possible, but it is no longer possible. I have had 3 major surgeries to remove and drain huge cysts, have had sepsis twice due to cyst ruptures and have been on the transplant list at Tufts Medical Center since 03/25/2019. The wait list for a deceased donor with O blood type is over 10 years in this region. I am blood type O positive (positive or negative DOES NOT MATTER). My best chance at survival is living donor transplant by another blood type O.  If someone would be willing to donate that is NOT blood type O….my transplant coordinators (Katie Ottariano, NP) will coordinate with the potential donor to do a paired kidney exchange or “kidney swap”  A paired kidney exchange, also known as a “kidney swap” occurs when a living kidney donor is incompatible with their recipient, but does match another person on the transplant waitlist. Two live donor transplants would then occur. Suppose there were two donor/recipient pairs, Donor and Recipient 1 and Donor and Recipient 2:  

Donor 1 would give a kidney to Recipient 2.

Donor 2 would then give a kidney to Recipient 1.

My transplant coordinator can be reached at 617-636-8096 for any questions someone may have about the donation process.  She is off on Fridays but can be reached M-Th 8am to 4pm.  OR questions can be emailed to [email protected].

Polycystic Kidney Disease (PKD)

What is it?

There are two types of PKD:

ADPKD  <———THIS IS WHAT OUR FAMILY HAS

autosomal dominant

ADPKD is the more common type of PKD and is estimated to affect more than 600,000 Americans and 12.4 million people worldwide.

ARPKD

autosomal recessive

ARPKD is a rare form of PKD that occurs in 1 in 25,000 children worldwide.

Autosomal dominant polycystic kidney disease (ADPKD) is one of the most common, life-threatening genetic diseases. In ADPKD, fluid-filled cysts develop and enlarge in both kidneys, eventually leading to kidney failure. It is the fourth leading cause of kidney failure and more than 50 percent of people with ADPKD will develop kidney failure by age 50. Once a person has kidney failure, dialysis or a transplant are the only options.

ADPKD is a painful disease that impacts quality of life. The average size of a typical kidney is a human fist. Polycystic kidneys can get much larger, some growing as large as a football, and weighing up to 30 pounds each.  There are 5 stages of kidney disease.  Stage 5 is near or at total renal failure.

Unlike some genetic diseases, ADPKD does not skip a generation meaning it often affects many people in one family. Approximately 10 percent of the people diagnosed with ADPKD have no family history of the disease, with the disease developing as a spontaneous (new) mutation. Once a person has ADPKD, even through a spontaneous mutation, they have a 50 percent chance of passing it on to each of their children. PKD can affect organs other than the kidneys. The following list of potential problems may look long and overwhelming, but it is important to remember that most people do not have all of these problems. If you have PKD, you and your family should be aware of the following possibilities so you can play an active role in understanding and managing your own healthcare.

More than eighty percent of patients with PKD develop liver cysts during their lifetime. Liver cysts can occur in those under the age of 30, but are usually small and detectable only by MRI scanning. The liver can remain normal in size with a few cysts or can become enlarged. Even with increased liver size from PKD, the amount of functional liver tissue remains more than adequate. This means it is highly unlikely that patients with severe polycystic liver disease (PLD) would develop liver failure.

Although not common, severe PLD can present with symptoms due to a “mass effect” (i.e. abdominal fullness, pain, early satiety (feeling full), ankle swelling and fluid accumulation within the abdomen). In the severe and symptomatic cases, cyst decompression may be needed. When a few very large cysts are present, additional surgical intervention may be recommended to take care of these cysts. Partial liver resection may be considered in selected cases. This should only be performed at specialized centers with experienced surgeons.

Liver cysts occur more often in women than men. Women develop liver cysts at a younger age and have more and larger cysts than men.   In addition to cysts developing on other organs from PKD and PLD (polycystic Liver Disease), there are several other ailments associated with PKD that patients must be monitored regularly for.  These include: Mitral valve prolapse, brain or abdomen aneurysms, both inguinal and umbilical hernias, diverticulosis and diabetes.

The PKD Foundation

PKDcure.org

The PKD Foundation is the only organization in the U.S. solely dedicated to finding treatments and a cure for polycystic kidney disease (PKD) and to improving the lives of those it affects. The PKD foundation hosts an annual walk in various locations all over the United States to help fund research to find a cure. 100% of all donations made to the walk for PKD will go directly to research to find a cure. 

To get more information on the PKD Foundation you can visit www.pkdcure.org

Become A Kidney Donor!

If you are interested in becoming a kidney donor, please visit www.livingdonortufts.org for more information and to complete the online health history questionnaire.

Once you have completed the online form, your info will be reviewed by a living donor coordinator. If you are a possible candidate, you will  be contacted. If you have questions at any time, you can contact a living donor team member at [email protected].

2022 SaveAndrea.org