Hughes Diseases goes by a couple of different names. Hughes Syndrome, Antiphospholipid Syndrome & Sticky Blood disease are all common names for Hughes disease. Hughes diseases is an autoimmune disease in which the body attacks the blood cells and the fat within the blood & create antibodies which result in the creation of clots. These are a certain type of phospholipids which encourage the creation of these antibodies. People with Hughes diseases are also known to have abnormalities in their cardiac valves in 30% of the time. Women are more prone to the development of Hughes Disease than men by 3 to 5 times.
The development of these clots can lead to all sorts of problems. These problems can include:
~Deep Vein Thrombosis, which are clots that form around the valves in the lower limbs and can obstruct blood flow. These can be very painful and if the clot is thrown elsewhere in the vascular system, it can result in a Stroke, Myocardial Infarction, or Pulmonary Embolism.
~Arterial Thrombosis, A blood clot in the artery
~Can cause serious complications during pregnancy. These complications include high blood pressure, preeclampsia, preterm delivery, miscarriage, or stillbirth.
~TIA, Transient Ischemic Attack
~Rash that appears to be like a purplish or reddish web, or sometimes can present with what appears to be blood blisters.
The appearance of any of these symptoms should be brought to the attention of a physician or medical professional immediately. Also unexplained bleeding from the nose or gums that last more than ten minutes and is not the result from brushing.
Less Common Symptoms of Hughes Disease include:
~Migraines of Chronic Headaches.
~Low Blood Platelet Count
~Altered Mental Status
~ Chorea, Involuntary Muscle jerks or movements
~Mental Health Problems such as Depression, Psychosis, Anxiety.
~Hearing Loss or Tinnitus
~Heart Valve Damage
~Livedo Reticularis, Purplish Rings Around Wrists and Knees.
~Blood Leaks back into Heart Chamber.
~Mitral Valve Degeneration or Thickening. This Happens in about 30%-35% of cases.
The patient is given high doses of anticoagulants (blood thinners) to break up the clots that may have formed and to prevent new ones from forming. The most commonly used anticoagulants are Warfarin, Xarelto, Heparin, Eliquis, and Pradaxa.
This is generally the treatment protocol in emergent situations. In therapeutic and maintenance treatment, lower dose anticoagulants like low dose aspirin are used. Changes in diet and lifestyle can also lower the risk of clots forming. Hughes syndrome is generally not a singular disease. When I say this, I mean that it means it usually accompanies another autoimmune disease such as lupus.
This can also be secondary to POTS, but this is not very common. And Since POTS is often secondary to EDS, that is why it is in this library. This disease needs to be managed by a team of doctors. At the minimum, a Rheumatologist and Immunologist should be part of the medical team. It wouldn't hurt to also consult a Geneticist and Hematologist if your team and insurance deems it necessary and allows it.
CAPS- Catastrophic Antiphospholipid Syndrome
CAPS is aggressive. It is the sudden formation of blood clots all over the body in the vascular system. It is also referred to as a Thrombotic Storm. It happens to a small number of patients with Hughes Syndrome. Caps can lead to multiple organ failure and in rare cases, death.
The Symptoms of Caps are as follows:
~Dark Urine, or Stool
~Altered Mental Status
~Episodes of erratic movement or fits
~Multi System Organ Failure
~Bone Marrow Necrosis
~Changes in skin color
Blood draws can be done. When the blood is tested in a lab, the lab tech or pathologist will look for certain things to confirm CAPS. These include:
~Disseminated Intravascular Coagulation
~High Fibrin Degradation Products
~Elevated Acute-phase reactants
These are not all the results they look for. For a complete list, click on this link.
They Key to managing any disease is learning what you can do proactively to avoid having an attack. Expecting your physician to do all the work is unrealistic. You have to want to help yourself. There are some things that you can do to lower your risk of Hughes Disease becoming a severe problem.
Eating a healthy diet is always a great way to reduce your risk of having health issues in general. Eating vegetables and fruits rich in Vitamin K. Foods low in Trans Fats such as poultry, fish, nuts, whole grains, and low-fat dairy. Limiting red meat intake will also reduce your risk of complications of Hughes Disease.
Avoiding Smoking, Alchohol, Energy Drinks, and Caffeine will also lower your risk of complications with Hughes Disease. Exercise is a great asset too. Now I am not talking about going to the gym and bodybuilding. a 30-minute walk every day is a great way to exercise. It will lower your risk over developing DVTs and will improve your cardiovascular health.
Talk to your physician about what treatment regimen is best for you. Each person is different and their care should be tailored for their specific needs. Take care of yourself. Help your doctor help you and chances are you will live a long and productive life.