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Marsha's Story

Having lost one baby to antiphospholipid Syndrome when she didn't feel well at 24 wk. she was dismissed as having heartburn and sent home. HELLP surfaced this time and Caleb was born at 1pound. 


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Introduction to PIH/HELLP

Pregnancy Induced Hypertension complicates approximately 7% of pregnancies in the US annually and is normally found in the second half of pregnancy. Preeclampsia is responsible for 50-70% of hypertension seen in pregnancy. PIH is the occurrence of hypertension, proteinuria, and edema, and eclampsia. PIH can range from mild to severe.  Complications of pregnancy that likely increase the chances of PIH include diabetes, multiple gestation and persistent hypertension.

The etiology of preeclampsia is not fully understood; however, dysfunction of the uteroplacental bed is thought to cause generalized vasoconstriction, platelet aggregation, and the hypercoagulable state found in preeclampsia. 

The short of what happens is this:
For some reason Vasospasms start in your body.  The blood vessels start to act like a muscle relaxing and contracting.  Like opening and closing your fist.  As blood is passed through these relaxing and contracting veins it causes damage to a special lining that is found in the veins.  As this damage occurs to your red blood cells  your platelets come to the rescue to try and heal the damage.  In the process they create clots over the damage.  Those clots get bigger and bigger and begin to stress your body as it try's to push the blood past this damage.

Your body tries to make up for the damage red blood cells, which causes your veins to become 'leaky' by increasing your body's water volume.  Your kidneys start to hold on to more water resulting in low urine output.  Swelling come from those leaky veins. The decreased blood flow only adds further strain to your heart, lungs, brain, kidneys and liver. 

In HELLP the liver is mainly affected.  It is deprived of Oxygen because the the clots. It begins to die. This begins the rise in Liver Enzymes or the pain that is often misdiagnosed as Heartburn or feeling "Flu-y".
Of course these Vasospasm that cause damage also cause damage to the vessels in and out of the Placenta.  The stress causes IUGR.

Now for the longer version:

HELLP is a variant of Pre-eclampsia. HELLP stands for Hemolysis, Elevated liver, Low platelet.  HELLP occurs in approximately 0.2 to 0.6 of all pregnancies.  It occurs in 4 to 12% of all women with pre-eclampsia, eclampsia or PIH.  In 11% of patients it occurs before 27 weeks gestation.  It presents in 69% of patients antepartume and 31% of patients postpartum occuring within 48 hours but can occur up to 7 days.  The mortality rate on HELLP is 1.1 - 2% percent.  In some case studies it has been noted to be as high as 33%*.  *http://www.imbiomed.com.mx/Medcrit/Tiv11n1/english/Zti71-1.html

Infant Mortality rates range from 20%-25% depending on the severity of HELLP in the mother.  Some case studies have noted to be as high as 60% - http://www.ahima.org/journal/coding/coding.9709.2.html
The main causes are abruption of the placenta, intrauterine asphyxia, and prematurity.

HELLP is not an entity separate from PIH but a group of sign diagnostic of the disease. HELLP syndrome may also involve Disseminated Intravascular Coagulation.   It is theorized that all patients who develope HELLP have an underlying coagulopathy that is usually undetectable. 

In some reported cases of HELLP pre-eclampsia was absent or mild. Women with HELLP are often critically ill. Their infants are frequently premature with compromised conditions. Maternal mortality related to the disease is prevalent around the world. Higher incidences of still birth and fetal growth retardation (IUGR) are apparent. Some have estimated that the maternal mortality has been estimated to be as high as 24% in cases of HELLP where there are other complications. 

Women with HELLP are also at greater risk of pulmonary edema, adult Respitory Distress Syndrome, abruptio placenta, disseminated intravascular coagulation, ruptured liver hematomas, and acute renal failure.

The reason why PIH/HELLP occurs isn’t fully understood yet. New research suggests that it starts with a failure in the placenta not being as well developed as it should be, with thinning of the blood vessels from mother’s side that invade the placenta as it grows. These changes begin as early as 18-20 weeks, even in the (usual) women who don't develop pre-eclampsia until toward the end of pregnancy. 

There are also blood clots blocking off the arteries in the placenta, reducing the amount of blood getting across to the baby. This explains the common finding of a smaller than expected baby in women who have pre-eclampsia.

Gestational thrombocytopenia is expierenced by some women during and after pregnancy.  The platelet lifespan is theorized to be shorter during pregnancy.  Some of the other maternal blood disorders that can come up are or are confused with HELLP are:

  • systemic lupus erythematosus (SLE)
  • antiphospholipid antibody syndrome (APA Syndrome)
  • thrombotic thrombocytopenic purpura (TTP)
  • idiopathic thrombocytopenic purpura (ITP)
  • hemolytic uremic syndrome (HUS)
  • hepatitis
  • acute fatty liver of pregnancy
The difference between TTP (thrombotic thrombocytopenic purpura), ITP (Idiopathic Thrombocytopenic Purpura), HUS (hemolytic uremic syndrome) and HELLP.
 
  • ITP, TTP & HUS can affect a women at any time, mainly during her childbearing years.  HELLP only affects those pregnant or just post partum.
  • HELLP is also usually the only one of these blood disorders that affects the liver. 
  • The effects on the kidneys is usually minimal in HELLP but mild to severe in TTP & HUS.  Renal failure is often associated with HUS.
  • TTP -Thrombotic thrombocytopenic purpura almost always occurs antepartum
  • HUS - hemolytic-uremic syndrome is generally a postpartum disease. Symptoms can begin before delivery, but the onset in most cases is delayed for 48 hours or more after delivery (mean about four weeks). 
  • HUS usually affects infants and children and may occur following delivery in a woman.
  • All three have in common thrombocytopenia and features of microangiopathic hemolysis, including anemia & increased bilirubin
  • Most HELLP patients present with some of the following symptoms - epigastric or right upper quadrant pain, malaise, nausea and/or vomiting, headache,  flu-like symptoms, abdominal tenderness, elevated blood pressure & edema.

  •  
In my non medical opinion the main difference is in how the platelets are destroyed that leads to what the disorder is.  When the Dr. looks under a microscope at your blood and runs the liver tests they will be able to determine the problem.  It's how the platelets are damaged, destroyed and consumed that guide them in the diagnosis. 
 
Getting to the more Medical side of HELLP
 


Hemolysis: 


significantly decreased erythrocyte life span

the breakdown of red blood cells causing the release of hemoglobin into the blood plasma. It is a normal process at the end of the life-span of each red blood cell. Hemolysis is usually slow enough for the red blood cells to be removed by the liver, spleen, and bone marrow.
A reduction in the number of red blood cells in the circulation, called anemia, is caused by excessive hemolysis. If hemolysis occurs rapidly, it may produce shivering, fever, jaundice, abdominal pains, and an enlarged spleen.

The hemolysis in HELLP syndrome is a microangiopathic hemolytic anemia  (Consequence of disseminated intravascular coagulation (DIC): fragments of red blood cells, damaged by being forced through a fibrin meshwork, are found in the circulation). The red blood cells are unable to live for their normal 120 day span.  Hemolytic anemias are usually due to defects in the red blood cells structure/function or a hostile external enviornment which they are forced to live.  When red blood cell production is unable to keep up with the red blood cell destruction anemia results.

FYI - There is extravascular hemolysis (in tissues and organs) and intravascular hemoysis (in the blood)

Endothelial damage is what results from the arteriolar (The smallest divisions of the arteries located between the muscular arteries and the capillaries) spasm that accompanies PIH/Pre-Eclampsia. 

Development of hemolytic anemia accompanied by unusual erythrocyte (red blood cell) morphology and consumption of platelets and other coagulation factors occurs more commonly. 

Red blood cells become fragmented as they pass through small blood vessels with endothelial damage (The layer of epithelial cells that lines the cavities of the heart and of the blood and lymph vessels and the serous cavities of the body, originating from the mesoderm-It is permeable to water- It transports a little bit of blood protein- It can contract to regulate flow -Damage is measured in how a vessel relaxs in response to a shear stress) and fibrin (insoluble protein formed from fibrinogen by the proteolytic action of thrombin during normal clotting of blood.  Fibrin forms the essential portion of the blood clot) deposits. 

The peripheral smear (view of your blood under a microscope) may reveal spherocytes, schistocytes, triangular cells and burr cells when HELLP is diagnosed.  This is a way for your Dr. to diagnose whether microangiopathic hemolytic anemia (Fragmented RBCs (arrowheads)result from occlusion of small arteries, arterioles, and capillaries by clots. This is accompanied by acute renal failure.)or hemolysis is present. For more information on your red blood cells click here.
 

The body will generally react to the loss of RBC (red blood cells).  It reacts by adding more water to the circulation of blood.  The body causes the kidneys to hold more water that is ingested by you.  That is why during HELLP you see the decreased urine output. As the blood continues to thin out it carrys less oxygen then normal.

At the bottom of this page you will read how PIH affects the cardiac output.  With HELLP the body progresses into increased cardiac output.  The heart pumps harder and hard to push those damaged red blood cells past the damaged firbrin.  Simply put it's like trying to push mud through a small diameter straw.  The heart has to increase it's pressure to do this.  In most situations the body can react by adapting the blood vessels.  In HELLP patients it is unable to, therefore blood pressure rises.  In this high pressure environment fluid seeps into the tissues resulting in Edema.  This swelling is normally seen in the ankles and lower back.

The body now is forced to redistribute it's blood flow.  Areas such as the skin are last to recieve oxygenated blood.  In HELLP patients the results are damage to the capillaries under the skin resulting in bruises.
Some of the symptoms of HELLP are because of what is discussed above.  Some complaints are:

  • Shortness of breathe 
  • Headaches
  • Dimmed vision
  • Nausea
  • Dizziness & Fainting
  • Edema
Those symptoms are not unique only to the Hemolysis side of HELLP.  For example High Blood Pressure can be a result of Kidney problems 

HELLP vs. DIC: Hematologic changes are more severe with DIC and
are more extensive than thrombocytopenia and hemolysis of RBC’s 

Inappropriate activation of coagulation process in which thrombin
formation and hemorrhage occur simultaneously 

There must be a large wounded area eg. Many microlesions throughout
the cardiovascular system (PIH) or a massive burn or trauma site or an
abruptio placenta or fetal demise (septicemia) in utero or an amniotic
fluid embolism 

Fibrinogen is released and causes large quantities of fibrin to be release.
Pregnancy is a time where there is an abundance of fibrin. Fibrin goes to
the massive trauma area and in the process of clot building clotting
factors are used up . Excess fibrin begins to degrade. In so doing anti
coag. factors are released from FDP’s or FSP. Hemorrhage ensues
reflected in thrombocytopenia plus prolonged PTT & PT
http://www.mc3.edu/gen/faculty/Minterr/ppts/HighRiskOBII/tsld015.htm



Low Platelets:

Platelets are the first line of defense against bleeding, plugging up little holes in capillaries (primary hemostasis).

Platelet counts range from 6k to 100k with HELLP.

Of course, they also help initiate coagulation (to solidify the blood before it gets through the bigger holes), and eventually become an important component of most clots. Thrombocytopenia is the increased consumption or destruction of platelets.

Platelets, fibrin, or both accumulate wherever the endothelial cells are damaged or lost.  Again Fibrin is what  makes endothelial cells migrate (rendering the surface permeable), causing vascular smooth muscle to proliferate.

Vasospastic HTN associated with PIH causes breakdown in vascular
endothelium. Microlacerations cause platelet adherence and fibrin
deposits. Increased responsiveness to presser effects of Angiotensin II
and deficiency of Prostacyclin in PIH are associated with vasospastic
HTN. Platelet counts usually improve in 4 to 5 days.
http://www.mc3.edu/gen/faculty/Minterr/ppts/HighRiskOBII/tsld012.htm



Elevated Liver Enzyme Levels :

HELLP syndrome impairs liver function: SGOT,SGPT 

Epigastric (right upper quadrant) pain resulting from obstruction of
blood flow in the hepatic sinusoids by intravascular fibrin deposition 

Hyperbilirubinemia
http://www.mc3.edu/gen/faculty/Minterr/ppts/HighRiskOBII/tsld011.htm

Obstruction of hepatic (liver) blood flow by fibrin deposits (Fibrin being what keeps the vessel smooth and water permeable, Red Blood Cells can get caught on large deposits of Fribrin and become damaged).  It is this obstuction that results in blocked hepatic blood flow which lead to elevated liver enzymes and liver distension.
 


Other noticeable factors in PIH/HELLP:

Cardiac output is increased 30-50% in normal pregnant women and is
not altered significantly in patients with PIH. The control of peripheral resistance in normal and PIH-complicated pregnancies is critical to understanding bloodpressure control because blood pressure is the product of cardiac output times peripheral resistance, and peripheral resistance is increased in women with PIH. This likely occurs as the consequence of an increased sensitivity of the maternal vasculature to a variety of naturally occurring vasopressors (contraction of the muscular tissue of the capillaries and arteries), such as angiotensin II (An octapeptide found in blood, it is synthesised from angiotensin I and quickly destroyed. Angiotensin II causes profound vasoconstriction with resulting increase in blood pressure).



Renal Failure:

Renal Failure is also usually associated with pre-eclampsia and HELLP Syndrome.  In addition to the other complications like TTP, ITP, HUS & DIC some other diseases that also maybe be invovled with rnal failure during pregnancy are:
RENAL CORTICAL NECROSIS:
ACUTE PYELONEPHRITIS
ACUTE FATTY LIVER OF PREGNANCY
An association with LCHAD deficiency has been implicated as in AFLP
URINARY TRACT OBSTRUCTION


Some of the information on the above paragraphs came from :
www.pathguy.com

This page is constantly changing as I learn more about the medical aspects of HELLP.  I try to present the information in the most accurate and comprehensive way that I can.  Not being a Dr. the accuracy of some of the information is only as valid as the pages I find them on.  I do most of my searching for information via the web.
 

Still looking for more HELLP information?  Signs of PIH/HELLP
will take you to the next page.  Also if you have a HELLP story to share please stop by the HELLP Syndrome Birth Stories Page and leave or read a story.  Here you will also find up to date HELLP/Pre-E net articles and books.  You wil also find WebRing information at the above listed url.


Introduction to PIH/HELLP

Signs of PIH/HELLP

Effects of HELLP on Mother & Child

Chances of PIH/HELLP in subsequent Pregnancies

Prevention

My Body After HELLP

If you have an HELLP Syndrome story please take a moment to share it with us.  Please tell us what lead up to your having HELLP and what the outcome was and how you are doing today and whether you have had any pregnancies since having HELLP.
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5/1/01