FDA Approves Drug to Treat Rare Autoimmune Disease

The U.S. Federal Drug Administration (FDA) has approved a drug manufactured by ChemoCentryx to treat a rare group of autoimmune diseases, called anti-neutrophil cytoplasmic autoantibody-associated vasculitis, or ANCA-AAV for short. According to ANCA Vasculitis News, ANCA-AAV causes inflammation and damage to the body’s small blood vessels. This inflammation is the result of antibodies that bind to certain cells of the immune system, called neutrophils, and overly activate them.

Since small blood vessels are found throughout the body, ANCA-AAV causes a variety of symptoms, including:

  • Poor kidney function, leading to kidney failure
  • Severe respiratory problems, including shortness of breath, hoarse voice, cough with blood or mucus, and chest pain
  • Neurological symptoms, including tingling, burning, numbness, and weakness
  • Central nervous system (CNS) involvement, such as headaches, cognitive impairment, and memory deficits. In severe cases, seizures, paralysis or loss of consciousness may also result.
  • Ear, nose, and throat (ENT) symptoms, like sinusitis, nasal discharge, rhinitis, and tinnitus (ringing in the ears)
  • Eye symptoms, including eye pain, vision impairment, and vision loss
  • Joint pain, muscle pain, and muscle loss
  • Skin lesions, including rashes, sores, ulcers, bumps, and bleeding underneath the skin
  • Digestive problems, like vomiting, diarrhea, nausea, abdominal pain, and blood in one’s stool
  • And more…

Due to the numerous symptoms ANCA-AAV causes, it can often be fatal for patients; in fact, ChemoCentryx states that the first year mortality rate for patients living with the disease is between 11 and 18 percent. Current ANCA-AAV treatments on the market include steroids and immunosuppressant medications which compromise the body’s ability to fight off infections. That’s why having a new treatment on the market that works via a different mechanism may be a saving grace for many ANCA-AAV patients.

The new drug, called avacopan, will be sold by ChemoCentryx under the brand name Tavneos. It works by blocking a protein called C5a receptor that is responsible for causing numerous inflammatory diseases. The drug’s wholesale price will be an astronomical $150,000-$200,000 per patient per year. However, it could be the life-saving treatment that the 40,000 ANCA-AAV patients in the US need.

The drug is currently being tested for use on other conditions as well, including the autoimmune skin disease Hidradenitis Suppurativa (HS). The drug has already received approval for commercialization in Japan, and is expected to be approved in Europe by the end of 2021.

Martina, a 29-year-old mother-of-two from Stuttgart, Germany, describes what it was like being diagnosed with this challenging autoimmune condition:

“It began rather unspectacularly, with flu-like symptoms, fever, headaches, and joint pain. So anyone would think, ‘It’s just a normal flu.’ Then it got worse over the course of a week and I went to the hospital.”

She was eventually diagnosed with ANCA-AAV after undergoing a series of blood tests. Unfortunately, she had to give up her career as an educator in the process, since she didn’t have a strong enough immune system to be interacting with children while taking steroids and immunosuppressants to control the disease. Patients like Martina are the ones that could potentially stand to benefit from new drug therapies like avacopan.

Martina, 29, appears in an ad to raise awareness for vasculitis conditions.

To find resources for those living with ANCA-AAV and their caretakers, visit: https://www.myancavasculitis.com/living-with-aav/

The Importance of Sleep with a Chronic Illness: Top 3 Reasons

Getting adequate sleep is important to maintain vital health- an especially so for chronic illness patients. Image courtesy of the Sleep Foundation.

If you have a chronic illness, you’ll know all too well the feeling of going about your day when you’ve had poor sleep. That feeling of mental grogginess, accompanied by the physical aches…it’s not fun. Sleep performs several essential functions beyond just improving your mood, memory, and mental clarity. Sleep actually has important physiological impacts on your body as well. Read on to learn the top three reasons why sleep is especially important for chronic illness patients.

1. Decrease Inflammation

According to the Division of Sleep Medicine at Harvard Medical School, there is a link between lack of sleep and the risk of developing certain diseases and health problems. For example, sleep deprivation studies have shown that when healthy research study participants were deprived of adequate sleep levels, they experienced increased blood pressure and inflammation levels, in addition to impaired blood glucose control. These symptoms actually mimic the impact of increased stress on the body. Other studies have found that prolonged sleep deficiency can lead to chronic, body-wide low-grade inflammation and is associated with various diseases that have an inflammatory component, such as diabetes.

As many of you know, increased inflammation and an abnormal inflammatory response are what underlie many chronic illnesses, including autoimmune disease. As a result, it’s important to get enough sleep to ensure that your inflammation levels stay in-check.

2. Prevent Weight Gain & Hormone Imbalances

In addition to increasing your inflammation, studies have shown that a lack of sleep can also lead to weight gain. For instance, people who habitually sleep less than six hours per night are much more likely to have a higher than average body mass index (BMI), and people who sleep eight hours have the lowest BMI. For this reason, sleep deprivation is now considered a possible risk factor for obesity.

Poor sleep increases cortisol levels, a stress hormone that can increase visceral (mid-section) body fat storage. It is associated with increases in insulin as well; insulin is a hormone that regulates glucose processing and also promotes fat storage. A lack of sleep can also be the culprit for lower levels of leptin, a hormone that alerts the brain that it has had enough to eat, and higher levels of ghrelin, a biochemical that stimulates one’s appetite. This means that those with poor sleep may have intense food cravings, and feel hungry despite consuming enough calories.

3. Strengthen Immune Memory

According to the Sleep Foundation, sleep helps to strengthen immune memory, which is the immune system’s ability to remember how to recognize and react to dangerous antigens. With autoimmune disease, patients’ immune systems incorrectly attack their own healthy tissues and cells. As a result, autoimmune patients have immune systems with poor immune memory. Getting adequate sleep levels can help to strengthen your immune system’s ability to differentiate between your own tissues and foreign invaders.

Although the exact reasons why sleep helps your body to improve its immune memory are unknown, there are several hypotheses. For example, it’s believed that because breathing and muscle activity slow down while you’re asleep, your body now has freed up the energy for the immune system to perform these critical immune-memory tasks.

Another way that sleep helps your immune system is through the production of melatonin, a sleep-promoting hormone. Melatonin is known to have anti-inflammatory effects by scavenging toxic free radicals, which cause tissue destruction during an inflammatory reaction. Melatonin also reduces the over-expression of a variety of pro-inflammatory cytokines, which can cause a cytokine storm in one’s body. There is also some evidence that melatonin inhibits the production of adhesion molecules, which are responsible for causing inflammatory white blood cells to stick to endothelial cells in one’s connective tissue.

How Much is Enough?

By now, you may be convinced that you need more sleep…but how much shut eye is really enough? According to the Sleep Foundation’s guidelines, adults aged 18-64 need between 7 to 9 hours of sleep every night. Adults over the age of 65 are recommended to get a similar amount of sleep – between 7 to 8 hours – each night. Infants, children and teens need even more hours of sleep to sustain their growth and development.

Sleep Problems and Chronic Illness

Having a chronic illness like an autoimmune disease can directly impact your ability to get quality sleep. For instance, many autoimmune patients suffer from chronic pain, which makes it challenging to fall asleep or to stay asleep. In fact, two-thirds of patients with chronic pain conditions report experiencing sleep disorders like insomnia.

I myself have Sjogren’s Syndrome, which, in addition to chronic joint pain, causes severe dry eyes and dry mouth. In my earliest days of living with Sjogren’s, I had difficulty staying asleep, since I would constantly wake up every few hours to chug bucketloads of water to relieve my chronically dry mouth, go to the bathroom (as a result of all the water I was drinking!) and put in eyedrops to relieve my severely dry eyes. Fortunately, I was able to find relief for my dryness symptoms through prescription and over-the-counter products, which made it possible for me to get a good night’s rest, without having to constantly wake up.

Chronic illnesses can also result in mood disorders like depression and anxiety, which can in turn make falling asleep difficult. If you’re staying up late at night due to incessant worrying about your health problems, it’s important to get treatment for your mental health conditions from a provider who understands the realities of living with a chronic illness.

Thanks for stopping by the Autoimmune Warrior blog! Do you have difficulty sleeping with your chronic illness? Let us know in the comments below!

Christina Applegate Reveals Multiple Sclerosis Diagnosis

Actress Christina Applegate has revealed that she has MS, a neurological autoimmune disease. Photo courtesy of Mike Coppola via CNN.

49-year-old actress Christina Applegate revealed on Twitter this week that she has been diagnosed with multiple sclerosis (MS), an autoimmune disease affecting the central nervous system. Applegate says she was diagnosed “a few months ago” after experiencing symptoms of the disease.

Commenting on her diagnosis, she said: “It’s been a strange journey. But I have been so supported by people that I know who also have this condition. It’s been a tough road…but as we all know, the road keeps going.”

According to John Hopkins Medicine, multiple sclerosis occurs when the immune system attacks nerve fibers and the myelin sheath – a fatty substance which insulates healthy nerve fibers – in the brain and spinal cord. This attack causes inflammation, which destroys nerve cell processes and myelin, altering electrical messages in the brain.

There are different types of MS, the most common of which is relapsing-remitting multiple sclerosis, which affects 90% of those diagnosed. Symptoms of a multiple sclerosis relapse include: fatigue, numbness, tingling, blurred vision, unsteady gait, and weakness.

Worldwide, more than 2.3 million people live with MS, including almost 1 million adults in the United States alone, according to the National MS Society. The neurological autoimmune disease can be disabling, although the MS Society states that the majority of people with the condition do not become severely disabled. Two-thirds of people who have MS remain able to walk, though they may need a mobility aid, such as a cane, and some will use a scooter or wheelchair because of fatigue, weakness, balance problems, or to assist with conserving energy. 

Since coming out as newly diagnosed with MS, Applegate has received an outpouring of support from fans and other celebrities with the disease. Fellow actress Selma Blair, who co-starred with Applegate in a romantic comedy in 2002 and also has multiple sclerosis, tweeted: “Loving you always. Always here. As are our kids. Beating us up with love.” Talk show host Montel Williams, who also has MS, also tweeted his support: “We have MS – it will never have us unless we let it. Tara and I are sending hope and light your way.”

MS isn’t the first health battle Applegate has faced. In 2008, the star revealed that she had had a double mastectomy after testing posting for the BRCA gene, pre-disposing her to breast cancer. Facing her new MS diagnosis, Applegate has requested “privacy…as I go through this.”