HELLP Syndrome in APS and Lupus

I had never heard of the HELLP syndrome until after having my first baby. My symptoms went into remission and the pregnancy went smoothly. I did go into labor 4 weeks early, but it turned out to be a blessing and probably saved both my life and my baby’s. HELLP stands for Haemolysis, Elevated Liver enzymes, and Low Platelets. The HELLP syndrome is a rare life threating complication of pregnancy. Even though the hallmark sign of the HELLP syndrome is a low platelet count, the HELLP syndrome is actually a clotting problem. Something triggers the clotting cascade and the platelet count drops as multiple clots are formed throughout the body including the liver, which ultimately shreds the red blood cells leading to anemia. The only way to stop HELLP and save the mother’s life is to deliver that baby no matter if the baby is able to survive on its own or not. Patients with APS or Lupus are at a higher risk for developing HELLP. Lovenox and aspirin are given to help prevent the HELLP syndrome in Lupus patients. Even on both medications, I still developed the HELLP syndrome. Usually patients developed pre-eclampsia with elevated blood pressure and protein in the urine before developing the HELLP syndrome. I was an unusual case in that my blood pressure was a perfect 120/80 and I didn’t even have a trace of protein in my urine. It’s important for APS patients to be screened using blood work to catch complications before they go undetected too far.

The HELLP syndrome in the antiphospholipid syndrome: retrospective study of 16 cases in 15 women.

HELLP-like syndrome associated with hepatic necrosis in a patient with systemic lupus erythematosus.

Invalid INR tests in APS patients

Most doctors don’t realize that INR tests may not be accurate in patients with APS or Lupus patients positive for Lupus anticoagulants. It was a nightmare to regulate my INR levels while I was on Coumadin for a DVT. I had to take a fairly large dose of Coumadin to get my INR within therapeutic ranges. Just when everything would seem stable, my INR would drastically spike to a high value. My blood work was like a rollercoaster and I had to have my INR checked weekly. After I was diagnosed with APS, the doctor explained that the antibodies themselves can cause the INR test to read too high or too low. Sometimes the finger stick machines will actually display an error when testing a patient with APS. Looking back, I was probably the patient that was really supratherapeutic (blood was thinned too much) even though the tests showed that I was subtherapeutic. This is another example of how lab tests are not always correct and if doctors are not aware the consequences could be dangerous. Below are some journal articles explaining the INR test issues. Their results showed that the chromogenic factor X and prothrombinproconvertin time assays were the most accurate methods to monitor coagulation in APS patients.

Monitoring Warfarin Therapy in Patients with Lupus Anticoagulants

INR Monitoring in Patients with Antiphospholipid Antibodies with Finger Stick INR Devices

Chilblains Lupus

Chilblains (sometimes called perniosis) are extremely painful sores that appear on the extremities such as fingers, toes, and ears. The red swollen sores usually occur during very cold yet humid conditions. If the sores last for extended periods of time or occur during warm weather, the Chilblains are more than likely caused by a connective tissue disease. Chilblains are most commonly associated with Lupus but are also seen in patients with undifferentiated connective tissue disease and Raynaud’s.

Clinical and histopathologic features and immunologic variables in patients with severe chilblains. A study of the relationship to lupus erythematosus.

Chilblain lupus erythematosus (lupus pernio): clinical review of the Mayo Clinic experience and proposal of diagnostic criteria.

Below are some pictures of my feet with Chilblains sores. It is extremely painful and I wanted to cut my toes off. When the sores start to heal the pain is replaced with intense itching. Not fun. Chilblains Lupus is a rare symptom of Lupus and most Rheumatologist have never seen a case. Three Rheumatologist saw my feet in person and another three saw the pictures. None of them could identify the sores. Trental and Plaquenil are suggested as treatment for Chilblains Lupus in medical journal articles. Prednisone instantly healed my sores and Procardia helped keep them from returning.

Elevated Factor VIII in Liver Disease

I had lots of coagulation blood work drawn after being diagnosed with a DVT (deep vein thrombosis). One of those tests happened to be a Factor VIII test which ended up being elevated. The test was actually ordered on accident and the doctor could not explain anything about the test. I started research Factor VIII and decided most doctors really don’t know much about this coagulation factor.

Interestingly I discovered some articles that explain Factor VIII is actually increased in liver disease when most all the other clotting proteins and factors are reduced due to impaired liver function. This sounds like a contradiction but scientists have discovered that the cirrhotic liver in unable to produce adequate amounts of a protein called low density lipoprotein receptor-related protein (LRP). One of the functions of LRP is to control the amount of Factor VIII in the blood stream by metabolizing excess amounts of Factor VIII. Another contributing factor to the increase of Factor VIII in liver disease is the increased production of von Willebrand factor (vWF) in cirrhosis. Just as LRP helps regulate the levels of Factor VIII by destroying the extra Factor VIII, vWF helps preserve Factor VIII. Therefore extra vWF and low levels of LRP would lead to higher levels of Factor VIII.


Factor VIII expression in liver disease

There is yet one more reason for increased levels of Factor VIII in liver disease. The liver is composed of tons of special cells found only in the liver called sinusoidal endothelial cells. They are very permeable and allow the liver to directly interact with the blood and perform its life essential functions. In autoimmune hepatitis (AIH) and other autoimmune liver conditions, antibodies attack these sinusoidal endothelial cells and turn them into normal capillary endothelial cells found throughout the body, which are not permeable. These special sinusoidal endothelial cells have some other important properties. One of which is they do not make Factor VIII, which normal capillary endothelial cells do. Therefore Factor VIII increases when lots of sinusoidal endothelial cells are replaced with Factor VIII producing normal endothelial cells.

Capillarization of Hepatic Sinusoid by Liver Endothelial Cell-Reactive Autoantibodies in Patients with Cirrhosis and Chronic Hepatitis

Doctors Who Apologize

As illustrated in my last couple posts, autoimmune patients can be treated pretty poorly by medical professionals. The next couple articles show how a sincere apology from a doctor can actually heal both the doctor and the patient. As a patient who has been unfairly judged, I would like to think those doctors who have hurt me in the past realized what they did was wrong but just couldn’t bring themselves to apologize. Whether it was because of fear of admitting wrong and being sued or just plain pride, I’ll never know.


The Healing Power Of Apologies

“For moral reasons, physicians should be held to the same standards as others; that is apologies should be offered for “offenses,” not for predictable and expected mistakes.”


A Doctor's Apology Can Heal

"For me, the biggest piece of this conversation was her offering me forgiveness, you know … And it still sends a chill down my spine. Forgiveness goes both ways. It helps both sides."

“Nine years ago, no one ever blamed my mother's doctor for her death from a disease that ravaged her nonsmoker's lungs. But the memory of his apology and his earnest attempt to learn from what had happened still comforts me. There are times when "sorry" is the only thing to say. And sometimes, a doctor's apology helps everyone to heal.”


A Doctor's Apology, An Intensely Moving Experience

“The idea of a doctor apologizing to a patient was unheard of as few as 5 years ago. I first came upon the concept shortly after my misdiagnosis was resolved, through a piece run by CBS news. I promptly printed it off and mailed it to the doctors involved in my misdiagnosis. Then in 2007, CBS aired still another piece that talks about the physician's relief once the apology is made.”


Saying sorry is good for your health

“Physicians in particular may find it hard to apologize. Dr. Leape says this has to do with the culture of medicine. ‘First, our training leaves us with emotional baggage,” he says. “Physicians are selected for and socialized to expect perfect performance. A serious failure, such as an error that results in a major injury or death, can be so devastating to a doctor’s self-image that it may be overwhelming.’”

“In addition, he says, ‘Most physicians receive no training or support in offering apologies. They don’t know how to do it; they may feel ashamed and worry about being sued. So, it’s not a big surprise that apologies don’t always happen or are offered in the wrong way.’”

Doctors Feel Threatened By Invisible Illnesses

While this article deals with Fibromyalgia, it will ring true with anyone who is suffering from an autoimmune disease. Almost everyone with an autoimmune disease has had at least one rude encounter with a doctor, friend, or loved one questioning their pain.

Some Doctors, Friends, Relatives Dismiss Sickness

"I know in my case I just had the idea if you got sick you went to a doctor and they treated you," Matallana said. "I never, ever imagined someone would question my pain and my inability to function. That was to me almost as bad as enduring the physical symptoms.”

“The transformation of dynamic people into suffering wrecks, without any apparent cause, prompts confusion and questioning. Fibromyalgia patients say even friends, relatives and spouses find it hard to accept the reality of what has been called an invisible illness.”

“Dr. Patrick Wood, an assistant professor of medicine at Louisiana State University, decided to specialize in fibromyalgia … I don't think it makes me popular with my colleagues, but we're trained to be little gods and anything that challenges our god-like capacity we dismiss," Wood said. "(A doctor) could say, 'There's nothing to objectively demonstrate you're really sick. How do I know you're sick?' You have to trust the patient's report, which we're often not willing to do when it comes to pain."

“Some doctors dismiss patients with vague complaints of pain as hypochondriacs or malingerers, lazy people looking for an excuse not to work. It doesn't help matters that he and other doctors regularly encounter true malingerers, about whom they must make judgments to determine government disability payments.”

"Every doctor is faced with being a useful idiot, being a tool for somebody who wants to get out of work," said Lubin, who trained at Yale and Harvard. "I'd rather you fooled me once and then I could say, 'Shame on you,' rather than try to prevent my ego from ever being bruised by assuming everyone whoever asked me for a pill for their pain is a drug-seeker. We can't lose our humanity just because we're afraid of being hoodwinked."

ANCA Antibodies Found in Autoimmune Hepatitis

My positive CANCA test puzzled all the doctors so I started researching ANCA antibodies in light of my other symptoms and test results. I had just recently been diagnosed with Autoimmune Hepatitis (AIH) which is an autoimmune disease where the immune system attacks the liver. I found a couple articles written by German scientists who discovered that AIH patients can be positive for CANCA as well as PANCA. Even more interesting they discovered that the target antigen responsible for creating the CANCA test result was smooth muscle. Anti Smooth Muscle Antibody (ASMA) is the commonly known antibody associated with AIH. I also tested positive for ASMA so the positive CANCA could probably be explained by ASMA.

Actin is a target antigen of anti-neutrophil cytoplasmic antibodies (ANCA) in autoimmune hepatitis type-1.

Characterization of target antigens from anti-neutrophil cytoplasmic antibodies in autoimmune hepatitis type-I.

Anti-neutrophil cytoplasmic antibodies in type 1 and 2 autoimmune hepatitis.

High-titer antineutrophil cytoplasmic antibodies in type-1 autoimmune hepatitis.