Antiphospholipid Syndrome (APS) - It's In The Blood

Life with Antiphospholipid Syndrome (APS) – my new-ish diagnosis. Here’s what I’ve learned so far since my diagnosis in July 2023. Readers of this site know that writing out a list of questions when I receive new health information is a standard course of action for me. I’ve been doing this since 2009. My wish is that these basic questions help other readers of this site.

I’ve made a point of publishing this page just before Rare Disease Day on February 28th. APS is rare. Only 1 in 2,000 people have it. Figures I’d get it along with all my other autoimmune problems.

An autoimmune disease patient doesn’t expect to fly for the first time in four years and land with a life-threatening diagnosis. But that’s what happened to me in the summer of 2023. I puffed up after my flight everywhere and had visible edema running through my legs and in my upper body and face. At first I thought it was flight related, but my face and body were tight and shiny for ages after my flight and it became apparent that something was wrong when livedo reticularis showed up in my legs and my feet started puffing up with fluid.

I don’t have healthcare in Canada so I treated it as best as I could and tried to enjoy my time at home.

Being home was great. I’m so grateful for that time! Words can’t describe how good it was to wrap my arms around my mom and dad and to be home in my most favorite place in the world. I’m also unspeakably proud of myself for flying solo but those days are now at an end. I am not allowed to fly now for at least six months after ‘landing my new APS diagnosis’.

I worked so hard to keep active during my 16 hour journey in the air. It was not enough and I’m not going to beat myself up about it. The inflammation and edema was not fully under control when I left Taiwan. In fact, I’ve had edema throughout my legs for over a year. I’m used to seeing inflammation in my knee joints, but this looked different. There was fluid build-up above my knees and running down my shins. It was definitely not arthritis.

Flying made it worse. And it has heightened my angst and anxiety of flying disabled considerably.

How was I to know that would happen? Of course, I was thorough about packing an emergency flight kit for my flight. The only thing I forgot was the thing I needed most: aspirin. You’ll learn more about why that’s important later.

When my doctor delivered the news, I was shocked. I was familiar with APS because of my friend Sheryl,. Everything I’ve read about APS indicates that it happens mostly in lupus patients.

Do I have lupus? Nope! But my doctor says APS is more common in inflammatory arthritis patients than we think and that’s because it’s an autoimmune disorder.

So let’s get to know my new ‘not so best friend’ today with my Antiphospholipid Syndrome (APS) FAQ page.

Here are some key points to know about this condition:

What is Antiphospholipid Syndrome (APS)?

Antiphospholipid syndrome (APS) is an autoimmune disorder that can cause blood clots in arteries and veins. With APS, your immune system sees normal proteins in your blood, called phospholipids, as foreign substances.

Your immune system makes antibodies to fight what it sees as foreign substances and this causes your blood to become sticky or form clots. Patients with APS can experience blood clots, including heart attacks, strokes, and miscarriages. The formation of blood clots is life-threatening and it’s imperative that you keep a close eye on your blood.

APS is also known as APLS and APLA. You might also know it as Hughes Syndrome or ‘Sticky Blood’.

I’ve said many times that once a person has an autoimmune disease, they keep collecting autoimmune diseases like they’re going out of style. I’ve now got at least four autoimmune diseases and disorders that we know about. My primary diseases are axial spondyloarthritis and Psoriatic Arthritis.

Banner image: 5 Things You Should Know About APS (Antiphospholipid Syndrome). This image features an illustration of legs with blood clots in them with the following text: 1. APS is a rare autoimmune blood disorder. The odds of getting APS are 1 in 2,000. It is commonly seen in patients with autoimmune disease. 2. APS is characterized by recurring blood clots. (Thromboses) 3. APS is the number one cause of stroke in young people. 4. 20% of blood clots in large veins are due to APS. 5. 75-90% of people who have APS are women.

Questions About Antiphospholipid Syndrome (APS)

Here are some questions you may consider asking healthcare professionals about antiphospholipid syndrome (APS):

What Are the Symptoms of APS?

Antiphospholipid syndrome can present with a variety of symptoms.

Some common symptoms of APS include:

blood clots
livedo reticularis (a net-like rash on the skin)
neurological symptoms such as headaches, migraine, and seizures
joint pain
heart palpitations
gastrointestinal symptoms such as abdominal pain or diarrhea
Thrombocytopenia. (Some people with APS may have low levels of platelets in their blood (thrombocytopenia), which can lead to an increased risk of bleeding or bruising.)

Livedo Reticularis is a distinctive pattern of discoloration on the skin. It looks like a lace-like network of reddish-blue lines or patches. It’s caused by impaired blood flow in the small blood vessels of the skin. You’ll know it when you see it!

Now I know I’ve had APS since at least 2019 as I had livedo reticularis showing up regularly on my legs when I flew home that summer too. I remember showing my mom and she thought it was because my skin was too warm. Now I know it’s not and my suspicion that something was wrong was right.

What kind of doctor should I see for APS?

APS is a complex condition. Some patients might only see a rheumatologist for it, while others might need several kinds of healthcare providers including rheumatologists, hematologists, and obstetricians.

How is APS Diagnosed?

Doctors use a combination of clinical symptoms and criteria as well as lab tests to diagnose APS. Please refer to the section above to check APS symptoms.

However, APS clinical criteria include the presence of specific types of blood clots or pregnancy complications along with positive lab tests for antiphospholipid antibodies.

What Types of Antiphospholipid Antibodies Are Present?

Knowing which antibodies are present can impact the severity and treatment approach.

There are different types of antiphospholipid antibodies, including anticardiolipin antibodies, lupus anticoagulant, and anti-beta-2 glycoprotein I antibodies. The presence of these antibodies is a key diagnostic feature. The most common blood tests for APS are:

Anticardiolipin antibodies: IgG, IgM, and IgA
Lupus anticoagulant: This panel of blood clotting tests might include dRVVT (dilute Russel Viper venom time), lupus aPTT, mixing studies, hex phase phospholipid test, and platelet neutralization procedure
Antibodies to b2-glycoprotein I (IgG, IgM, IgA)

The severity and type of symptoms can vary among individualsIt’s important to note that not everyone with APS will experience all of these symptoms, and the severity of symptoms can vary widely among individuals.

Additionally, some individuals with APS may be asymptomatic and only discover the condition after experiencing complications such as blood clots or recurrent miscarriages.

How Can I Manage the Increased Risk of Blood Clots?

One of the primary features of APS is the increased risk of thrombosis, which is the formation of blood clots in both arteries and veins.

This can lead to serious complications such as deep vein thrombosis (DVT), stroke, pulmonary embolism, and heart attacks. Make sure you talk to your doctor about how to manage your increased risk of blood clots.

For me, I’ve found that moving to compression sleeves has helped with pain and swelling with APS. They have also improved my blood flow. Essentially, I put my flight compression socks on in July and I’ve never taken them off. I wear them every day now to help with blood circulation.

A pink blog banner for MySeveralWorlds.com with an image of hot pink leg compression sleeves and several graphics of blue compression socks. The text on the image says How Do Compression Socks help with pain and blood clots. Compression socks work to improve circulation and reduce swelling and edema.

What Are the Treatment Options for APS?

The primary goal of treatment is to prevent blood clots. You may need to take anticoagulant medications (blood thinners) such as warfarin or heparin to reduce the risk of blood clots.

Managing your medication regimen, including remembering to take your medication as prescribed and attending regular check-ups to monitor your blood clotting function, can become a part of your daily routine. It’s really important to remember to take your blood thinners at the same time each day!

Pregnant women with APS may also be prescribed low-dose aspirin.

How Will APS Affect My Daily Life?

Individuals with APS may be advised to make lifestyle changes to reduce the risk of blood clots.

Make sure you ask about diet as it’s more than likely you’ll need to start paying strict attention to your vitamin K intake. Vitamin K is usually in green, leafy vegetables by the way. This vitamin affects your blood’s ability to clot and it can interfere with how certain APS medications work.

In my case, I was allowed to stay on my raw diet as I eat green leafy vegetables every day and my doctor did not put me on heparin or warfarin. He put me on daily Enoxaparin injections to thin my blood instead. The reason he chose this option is because he felt it was the safest option and I wouldn’t have to worry about diet modifications.

Maintaining a healthy weight, NO SMOKING, and staying active to keep your blood moving are also recommended. However, contact sports seem to be a no-no, especially if you take blood thinners. People who take blood thinners bruise easily.

Again, ASK YOUR DOCTOR about what kind of lifestyle modifications you should think about if you get an APS diagnosis. This includes modifications you should make with day to day activities, work, and relationships.

Are There Specific Considerations for Women Planning Pregnancy?

APS is also associated with pregnancy complications, such as recurrent miscarriages, stillbirths, and preterm births. Women with APS may require special monitoring and treatment during pregnancy to reduce these risks.

If applicable, discussing pregnancy planning, risks, and management strategies is important.

How Often Should I Have Follow-Up Appointments?

Regular monitoring is essential for individuals with APS, especially those on anticoagulant therapy. Your appointments will involve routine blood tests, unfortunately. Blood tests help your rheumatologist assess the effectiveness of the treatment.

What Should I Do About Traveling When I Have APS?

Obviously this is a huge question for me since my ASP flared while I was traveling. Right now, I’m not allowed to fly anywhere, but my doctor has told me that when/if that time comes, he’ll let me know how to handle the 16-hour flight back to Canada. More than likely, it will involve doing blood thinner injections during my flight.

Individuals with APS, especially those who are taking anticoagulant medications, may need to take precautions when traveling to reduce their risk of blood clots during long flights or periods of immobility.

This may include staying hydrated, wearing compression stockings, and taking breaks to move around and stretch during travel. (All of which I’ve been doing each day simply because it feels right to do it!)

What is the Emotional Impact of APS?

Obviously living with APS is terrifying. It is, by far and away, my most immediately life-threatening diagnosis. I was not expecting to be so scared or anxious after my diagnosis and it had a huge impact on me emotionally and psychologically. You may experience stress, anxiety, or feelings of uncertainty about your health and future.

The emotional impact of life with APS, thankfully, is discussed online by many other patients. Reading their thoughts made me feel less alone. I was so scared when I got my APS diagnosis, especially at night when pain gets worse and your thoughts become darker.

Seeking support from healthcare professionals, support groups, or mental health professionals can be helpful in managing these emotional challenges.

Remember, open communication with healthcare professionals is key, and it’s essential to seek clarification on any concerns or uncertainties you may have about antiphospholipid syndrome.