Potter's syndrome, or more appropriately, Potter's sequence is a term that describes the effects of Oligohydramnios (insufficient amniotic fluid) resulting from any number of renal (kidney) abnormalities or a rupturing of the amniotic sac. It is a sequence rather than a syndrome because it describes a condition which can be the result of various different root causes. There are five distinct types of Potter's syndrome which are distinguished by different root kidney problems leading to Oligohydramnios. These include the Classic Potter's syndrome, which is the result of bilateral renal agenesis (BRA), or the absence of both kidneys. Classic Potter was the probable diagnosis we were given after our first ultrasound with the specialist, Dr. G.
Oligohyramnios is a dangerous and most often lethal condition because amniotic fluid is crucial for lung development. The fluid helps expand the airways and also delivers a vital amino acid (proline) as it is swallowed continuously. Ideally, the fluid cycles through the baby's system and is released back into the amniotic sac as urine (although not waste, since the mother's kidneys take care of filtering waste). Without functioning kidneys there is no urine, and therefore insufficient amniotic fluid which inhibits proper lung development. Additionally, since the amniotic fluid acts as a cushion which protects the baby from being compressed by the mother's uterus, babies with Potter's often have characteristic facial deformities and/or lower extremity anomalies.
In cases where Oligohydramnios is due to a rupture of the amniotic sac (called PROM), the tear can heal, and babies can survive. There are numerous testimonies of such cases on blogs and support websites such as benotafraid.net. However, Classic Potter's syndrome resulting from BRA is known to be 100% lethal in all recorded cases. While kidney failure is itself a lethal condition, for Potter's babies the primary cause of death is pulmonary hypoplasia (underdeveloped lungs).
Jason Clarke from the Patrick Brophy Research Lab at the University of Iowa has written a helpful and informative document on Potter's sequence which includes a long list of useful definitions of medical terminology. The document was prepared for the National Potter Syndrome Support Group and can be found here. The national support group's website was built by a family who lost a daughter to Potter's. In addition to some useful information including a Potter's FAQ (also written by Jason Clarke), there you will find a link to an online support forum.
Oligohyramnios is a dangerous and most often lethal condition because amniotic fluid is crucial for lung development. The fluid helps expand the airways and also delivers a vital amino acid (proline) as it is swallowed continuously. Ideally, the fluid cycles through the baby's system and is released back into the amniotic sac as urine (although not waste, since the mother's kidneys take care of filtering waste). Without functioning kidneys there is no urine, and therefore insufficient amniotic fluid which inhibits proper lung development. Additionally, since the amniotic fluid acts as a cushion which protects the baby from being compressed by the mother's uterus, babies with Potter's often have characteristic facial deformities and/or lower extremity anomalies.
In cases where Oligohydramnios is due to a rupture of the amniotic sac (called PROM), the tear can heal, and babies can survive. There are numerous testimonies of such cases on blogs and support websites such as benotafraid.net. However, Classic Potter's syndrome resulting from BRA is known to be 100% lethal in all recorded cases. While kidney failure is itself a lethal condition, for Potter's babies the primary cause of death is pulmonary hypoplasia (underdeveloped lungs).
Jason Clarke from the Patrick Brophy Research Lab at the University of Iowa has written a helpful and informative document on Potter's sequence which includes a long list of useful definitions of medical terminology. The document was prepared for the National Potter Syndrome Support Group and can be found here. The national support group's website was built by a family who lost a daughter to Potter's. In addition to some useful information including a Potter's FAQ (also written by Jason Clarke), there you will find a link to an online support forum.
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3 comments:
We recently found out that a friend of ours is carrying a baby with no kidneys. Your story I think will help her in her grief, and eventually through the process of healing. Thanks for sharing.
I had a little boy born with Potters syndrome who was a footling breech dry birth.He had no kidneys at all but managed to hang on for 1.5 hours long enough to be christened in Hospital .He was born on remembrance sunday he had low set ears all his limbs were bent as I carried no fluid at allbut he was the easiest of my children to give birth to .Time does heal a little but never one day goes by when I don't think about him xxx
My son Devin was born with Potter's 28 yrs ago. He was 6 lbs and 11oz and looked like a normal beautiful baby boy. I was so Blessed he was born alive, when he heard my voice he managed to turn his head and look at me. I will hold his memory close to my heart he will never be forgotten!
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