Causes, complications and risk factors that worsen Myasthenia Gravis!

Causes of Myasthenia Gravis:

1 –  Antibodies

Your nerves communicate with your muscles by releasing chemicals (neurotransmitters) that fit precisely into receptor sites on the muscle cells at the nerve-muscular junction.

In myasthenia gravis, your immune system produces antibodies that block or destroy many of your muscles’ receptor sites for a neurotransmitter called acetylcholine (as-uh-teel-KOH-leen). With fewer receptor sites available, your muscles receive fewer nerve signals, resulting in weakness.

Antibodies can also block the function of a protein called a muscle-specific receptor tyrosine kinase (TIE-roh-seen KIE-nays). This protein is involved in forming the nerve-muscular junction. Antibodies that block this protein can lead to myasthenia gravis.

2- Thymus Gland

The thymus gland is a part of your immune system situated in the upper chest beneath your breastbone. Researchers believe the thymus gland triggers or maintains the production of the antibodies that block acetylcholine.

Large in infancy, the thymus gland is small in healthy adults. In some adults with myasthenia gravis, however, the thymus gland is abnormally large. Some people with myasthenia gravis also have tumors of the thymus gland (thymomas). Usually, thymomas aren’t cancerous (malignant), but they can become cancerous.

3 – Other Causes:

Some people have myasthenia gravis that isn’t caused by antibodies blocking acetylcholine or the muscle-specific receptor tyrosine kinase. This type of myasthenia gravis is called antibody-negative myasthenia gravis. Antibodies against another protein, called lipoprotein-related protein 4, can play a part in the development of this condition.

Rarely, mothers with myasthenia gravis have children who are born with myasthenia gravis (neonatal myasthenia gravis). If treated promptly, children generally recover within two months after birth.

Some children are born with a rare, hereditary form of myasthenia, called congenital myasthenic syndrome.

Factors that can worsen Myasthenia Gravis:

  • Fatigue
  • Illness
  • Stress
  • Some medications — such as beta blockers, quinidine gluconate, quinidine sulfate, quinine, phenytoin,     certain anesthetics and some antibiotics
  • Pregnancy
  • Menstrual periods

Complications of Myasthenia Gravis:

Complications of myasthenia gravis are treatable, but some can be life-threatening.

Myasthenic crisis

Myasthenic crisis is a life-threatening condition that occurs when the muscles that control breathing become too weak to do their jobs. Emergency treatment is needed to provide mechanical assistance with breathing. Medications and blood-filtering therapies help people to again breathe on their own.

Thymus gland tumors

Some people with myasthenia gravis have a tumor in their thymus gland, a gland under the breastbone that is involved with the immune system. Most of these tumors, called thymomas, aren’t cancerous (malignant).

Other disorders

People with myasthenia gravis are more likely to have the following conditions:

  • Underactive or overactive thyroid. The thyroid gland, which is in the neck, secretes hormones that regulate your metabolism. If your thyroid is underactive, you might have difficulties dealing with cold, weight gain and other issues. An overactive thyroid can cause difficulties dealing with heat, weight loss and other issues.
  • Autoimmune conditions. People with myasthenia gravis might be more likely to have autoimmune conditions, such as rheumatoid arthritis or lupus.

 

 

QUOTE FOR WEDNESDAY:

“Myasthenia gravis (my-us-THEE-nee-uh GRAY-vis) is characterized by weakness and rapid fatigue of any of the muscles under your voluntary control. Myasthenia gravis is caused by a breakdown in the normal communication between nerves and muscles.”

MAYO CLINIC

What is Myasthenia Gravis?

Myasthenia GravisMyasthenia Gravis2

What is Myasthenia Gravis?

Myasthenia gravis is an autoimmune disease. Your body’s own immune system makes antibodies that block or change some of the nerve signals to your muscles. This makes your muscles weaker.

This neuromuscular disease leads to fluctuating muscle weakness and fatigue. Getting in more technical terminology in the most common cases, muscle weakness is caused by circulating antibodies that block nicotinic acetylcholine receptors at the postsynaptic neuromuscular junction. By blocking the ability of the neurotransmitter acetylcholine to bind to these receptors in the muscle, these antibodies keep motor neurons from signaling the muscle to contract (to do movement). Alternatively, in a much rarer form, muscle weakness is caused by a genetic defect in some portion of the neuromuscular junction that is inherited at birth as opposed to developing through passive transmission from the mother’s immune system at birth or through autoimmunity later in life.

Common symptoms are trouble with eye and eyelid movement, facial expression and swallowing. But it can also affect other muscles. The weakness gets worse with activity, and better with rest.

There are medicines to help improve nerve-to-muscle messages and make muscles stronger. With treatment, the muscle weakness often gets much better. Other drugs keep your body from making so many abnormal antibodies. There are also treatments which filter abnormal antibodies from the blood or add healthy antibodies from donated blood. Sometimes surgery to take out the thymus gland helps.

For some people, myasthenia gravis can go into remission and they do not need medicines. The remission can be temporary or permanent.

If you have myasthenia gravis, it is important to follow your treatment plan. If you do, you can expect your life to be normal or close to it.

Common signs or symptoms of the disease are:

Painless, worsening muscle weakness usually seen at the end of the day or after exercise

Blurred or double vision

Drooping eyelids and facial muscles

Slurred speech

Fast heartbeats

Shortness of breath or trouble breathing

How it is diagnosed:

During a physical exam, your health care provider may observe signs such as a droopy eyelid, difficulty holding your arms out at shoulder length for a reasonable length of time, or a weak grasp.

Blood tests may reveal the presence of acetylcholine-receptor or muscle-specific tyrosine kinase-seropositive (MuSK) antibodies. Specialized tests use electricity to stimulate muscles, and at the same time, measure the strength of muscle contraction.

If you have myasthenia gravis, muscle strength will decrease predictably over the course of testing. You may be given certain medications — edrophonium or neostigmine — as part of a diagnostic exam. In a patient with myasthenia gravis, these drugs will significantly and noticeably improve muscle strength for a short time. This helps to further confirm the diagnosis.

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Because other conditions sometimes occur along with myasthenia gravis and can interfere with treatment, your health care provider may order other tests. You may need a CT scan or an MRI to check for a thymoma. You’ll be checked for high blood pressure and glaucoma, and you’ll probably have your blood tested to see if you have thyroid disease, other autoimmune diseases (such as rheumatoid arthritis or systemic lupus erythematosus), diabetes, kidney problems, or any infections.

What Are the Treatments for Myasthenia Gravis?

There is no cure for myasthenia gravis, but it is treated with medications and sometimes surgery. You may be put on a drug called pyridostigmine (Mestinon), that increases the amount of acetylcholine available to stimulate the receptors.

The disorder is most commonly treated with the steroid prednisone and the the drugs azathioprine (Imuran),  mycophenolate (CellCept), and cyclosporine (Neoral) to suppress the immune system.

In severe cases, you may need to have your blood sent through a special machine that removes the antibody-containing plasma and replaces it with antibody-free plasma. This is called plasmapheresis. You may also be given a preparation called intravenous immunoglobulin (IVIg) through a needle in a vein.

If you have a thymoma, you will need surgery to remove your thymus (thymectomy). In fact, your doctor may recommend that you undergo this surgery even if no tumor is present because removal of the thymus seems to improve symptoms in many patients.

Research continues on and even T cells being looked at for putting the patient back in remission which has been reached in research labs on mice and other animals.  Technology and medical research are a step away from knowing how to put a patient in remission for a long time if not forever.  Hopefully its around the corner and history keep showing cure for one disease after another.  We do have hope in that this disease will be under complete control one day.

 

QUOTE FOR TUESDAY:

“Scoliosis and other spine disorders in children such as Scoliosis, kyphosis, spondylolysis, spondylolisthesis, torticollis can be idiopathic (of unknown origin), congenital (present at birth) or neuromuscular (related to nerves and muscles).   Scoliosis – a sideways curvature of the spine that may affect in any one or a combination of its three major sections:  cervical spine (neck) or thoracic spine (chest and upper back region) or lumbar spine (lower back).

John S. Blanco, MDShevaun Mackie Doyle, MDRoger F. Widmann, MD for Hospital of Special Surgery in Manhattan, NY.

What is Scoliosis?

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Scoliosis is a problem with the spine where the spine is curved instead of straight, with the upper back being rounded and the lower back having a “swayback,” or inner curved problem, reports WebMD.

According to the Scoliosis Research Society, 85 percent of all scoliosis causes are idiopathic, meaning the cause is unknown. The remaining causes of scoliosis include birth defects, such as vertebrae that form abnormally before birth, and certain disorders such as cerebral palsy, Marfan’s syndrome, muscular dystrophy and Down syndrome. Infections and spinal fractures can also cause scoliosis.curvature of the spine during surgical correction of this condition. Screws and rods are placed in order to stabilize and straighten the spine.

What You Should Know About Adult Scoliosis

Scoliosis is defined as a curve of the spine of 10 degrees. Adult scoliosis is broadly defined as a curve in your spine of 10 degrees or greater in a person 18 years of age or older. Adult scoliosis is separated into 2 common categories:

  • Adult Idiopathic Scoliosis patients have had scoliosis since childhood or as a teenager and have grown into adulthood.  We do not yet know the cause of idiopathic scoliosis, but there is a lot of genetic work going on in an attempt to answer this question.
  • Adult “De Novo” or Degenerative Scoliosis develops in adulthood. Degenerative scoliosis develops as a result of disc degeneration. As the disc degenerates, it loses height. If one side of the disc degenerates more rapidly than the other, the disc begins to tilt. As it tilts, more pressure is placed on one side of your spine and gravity tends to cause the spine to bend and curve. The more discs that degenerate, the more the spine begins to curve.

Scoliosis is more common in girls than in boys, and the diagnosis is usually made after a child reaches 10 years of age. A doctor performs a physical examination and may take X-rays to definitively diagnose the disease. An X-ray tells if there is any growth left in the growth plates of the femur or humerus, and scoliosis can become worse if the patient has more growing to do, states MedicineNet. Serial X-rays are performed to track the changes of the spinal curve, which helps determine the best course of treatment.

Types of idiopathic scoliosis are categorized by both age at which the curve is detected and by the type and location of the curve.

When grouped by age, scoliosis usually is categorized into three age groups:

  • Infantile scoliosis: from birth to 3 years old
  • Juvenile scoliosis: from 3 to 9 years old
  • Adolescent scoliosis: from 10 to 18 years old

This last category of scoliosis, adolescent scoliosis, occurs in children age 10 to 18 years old, and comprises approximately 80% of all cases of idiopathic scoliosis. This age range is when rapid growth typically occurs, which is why the detection of a curve at this stage should be monitored closely for progression as the child’s skeleton develops.

Terms Used to Describe Spinal Curvature

Scoliosis curves are often described based on the direction and location of the curve. Physicians have several detailed systems to classify specific curves, but here are some common terms used to describe scoliosis:

Terms that describe the direction of the curve:

  • Dextroscoliosis describes a spinal curve to the right (“dextro” = right). Usually occurring in the thoracic spine, this is the most common type of curve. It can occur on its own (forming a “C” shape) or with another curve bending the opposite way in the lower spine (forming an “S”).

Severe scoliosis can lead to heart and lung problems if not treated, as the ribs press against the chest, making breathing more difficult, states Mayo Clinic. Adults who had scoliosis as a child may experience more back pain throughout their lives as compared to people without scoliosis.

Symptoms of scoliosis include an uneven waist, uneven shoulders, disjointed hip and a protruding shoulder blade, according to Mayo Clinic. The spine also curves or twists in acute cases, and the disease can cause one side of the ribs to protrude more than the other. Severe cases also induce labored breathing and back pain.

Diagnosis

Scoliosis can be recognized and diagnosed with a clinical exam, but xrays are necessary to fully evaluate the magnitude and type of scoliosis present. For a proper scoliosis evaluation, full length, whole spine xrays need to be performed. An MRI may also be recommended if there are symptoms of leg pain that may be associated with stenosis or if there is concern about possible spinal cord compression or abnormalities.

Treatment

The treatment of adult scoliosis is very individualized and based on the specific symptoms and age of the patient. Many patients have scoliosis and have very minor symptoms and live with it without treatment. Patients with predominant symptoms of back pain would typically be treated with physical therapy. Patients with back pain and leg pain may receive some benefit from injection treatment to help relieve the leg pain.  If lumbar stenosis (narrowing of the spinal canal) is present and is unresponsive to non-surgical treatment, then a decompression( removal of bone and ligaments pressing on the nerves) may be recommended. If the scoliosis is greater than 30 degrees, a fusion procedure will most likely be recommended along with the decompression. The fusion is recommended to prevent the curve from progressing when the spine is destabilized by the bone removal that is necessary to  decompress the nerves. Fusions are usually accompanied with metal rod and screw placement into the spine to help correct and stabilize the scoliosis and help the bone heal or fuse together. The length of the fusion, or the number of spine levels included, depends on the type of scoliosis and the area of the spine involved. The goal of adult scoliosis surgery is to first remove pressure on the nerves, and second to keep the scoliosis from progressing further.

QUOTE FOR MONDAY:

“Every year countries around the world celebrate World Blood Donor Day (WBDD). The event serves to raise awareness of the need for safe blood and blood products and to thank voluntary, unpaid blood donors for their life-saving gifts of blood.

A blood service that gives patients access to safe blood and blood products in sufficient quantity is a key component of an effective health system. The global theme of World Blood Donor Day changes each year in recognition of the selfless individuals who donate their blood for people unknown to them.”

World Health Organization WHO

Why we need blood donor’s!

 

Why do we need blood donor’s?

We need to make sure that we have enough supplies of all blood groups and blood types to treat all types of conditions.

By giving blood, every donor helps us meet the challenge of providing life-saving products whenever and wherever they are needed.

The American Red Cross states the following facts:

  • Every two seconds someone in the U.S. needs blood.
  • Approximately 36,000 units of red blood cells are needed every day in the U.S.
  • Nearly 7,000 units of platelets and 10,000 units of plasma are needed daily in the U.S.
  • Nearly 21 million blood components are transfused each year in the U.S.
  • The average red blood cell transfusion is approximately 3 pints.
  • The blood type most often requested by hospitals is type O.
  • Nearly 21 million blood components are transfused each year in the U.S.
  • It is estimated that sickle cell disease affects 90,000 to 100,000 people in the U.S. About 1,000 babies are born with the disease each year. Sickle cell patients can require frequent blood transfusions throughout their lives.
  • The number of whole blood and red blood cell units collected in the U.S. in a year: 13.6 million
  • The number of blood donors in the U.S. in a year: 6.8 million
  • Although an estimated 38 percent of the U.S. population is eligible to donate blood at any given time, less than 10 percent of that eligible population actually do each year.
  • Blood cannot be manufactured – it can only come from generous donors.
  • Type O negative blood (red cells) can be transfused to patients of all blood types. It is always in great demand and often in short supply. Type O is the “Universal Blood Donor”
  • Type AB positive plasma can be transfused, it’s the “Universal Blood Recepient”.

This allows pt’s diagnosed with this illness to experience:

  • Bleeding due to lack of platelets
  • shortness of breath due to lack of RBC’s carrying oxygen (02) to the tissues of the body.
  • Dizziness again due to lack of 02 carried to the brain with anemic, & bleeding causing your B/P to be low (orthostatic b/p-changing your position anemic and blood dropping from the brain to cause dizziness) also.
  • cognitive impairment due to lack of 02 to the brain since RBC count is low in the body.
  • Bruising due to low platelets.
  • petachiae (small red/purple spots on the skin)
  • susceptibility to infections

Conditions for needing blood donors:

Aplastic anemia occurs when bone marrow stops making enough blood forming stem cells. In all three blood lines; red blood cells, white blood cells and platelets, patients with aplastic anemia have a low blood count. The bone marrow is found to be aplastic which means there is a low growth of blood forming stem cells.

Cancer of all types –  Where with or without the treatments of chemo or radiation in the end cancer itself kills all good cells that are created by our bone marrow.  Chemo or radiation kill the bad cells = cancer cells and good cells = RBCs, WBCs, Platelets.  So blood transfusion commonly needed in cancer patients.

Anemia– Lack of RBCs in the body.

Conditions causing bleeding in the body like GI bleed, hemmoragic stroke, endometriosis, hemophilia, and simple patients in the OR that bleed and need blood transfusions in the OR and ICU and even possibly on the Med/Surg or Telemetry unit.  I could go on with types of conditions the deciding factor that makes the doctor order the blood transfusion is obviously heavy bleeding occurring right in front of the surgeon’s or ER MDs eyes or checking the Complete Blood Count called a CBC looking at the hemoglobin (Hmg)-think of it at the fluids in the bloodstream and looking at the Hematocrit (Hct)-think of it as the solids in the bloodstream and if the Hg is critical 6-7 than one or two blood transfusions are ordered.

Treatments:

  • Treatments vary on a case by case basis. Age is often the determining factor for which treatment to use. Stem cell transplantation may be used for individuals younger than 30 years and who have a matched sibling donor=Blood Donor Needed.
  • Stem cell transplantation is a procedure which replaces defective bone marrow with healthy cells. Around 80% of patients make a complete recovery using stem cell transplantation.
  • For older patients with aplastic anemia, immune suppressing therapy with anti-thymocyte globulin(ATG) and cyclosporin is typically used. Around 70-80% of aplastic anemia patients respond to this treatment.

QUOTE FOR THE WEEKEND:

“The facts about cataracts are clear. Once they start to develop, they will get progressively worse. You cannot reverse cataracts, which is why it is important to seek medical attention when symptoms appear.  The first modern cataract surgery was performed in 1747.  Nine out of ten patients regain twenty-twenty vision or better after laser cataract surgery. ”

 

 

Part II Cataract Awareness Month – help prevent blindness

Medications that can cause Cataracts:

Certain medications are well-known causes of cataracts, and some drugs can also accelerate their development. Steroid medications – whether pills, injections, or eye drops – are most frequently associated with cataract formation. If you are taking steroid medications to manage a long-term condition, it is important to note any visual changes and to have your ocular health managed by a qualified ophthalmologist. The medications that can cause cataracts include:  Corticosteroids, Eye drops containing steroids, Glaucoma medications, Certain antipsychotics and antidepressants, Certain medications for autoimmune conditions, and Medication to control heart arrhythmia

Signs and Symptoms of Cataracts:

You may not have any symptoms at first, when cataracts are mild. But as cataracts grow, they can cause changes in your vision. For example, you may notice that:

  • Your vision is cloudy or blurry
  • Colors look faded
  • You can’t see well at night
  • Lamps, sunlight, or headlights seem too bright
  • You see a halo around lights
  • You see double (this sometimes goes away as the cataract gets bigger)
  • You have to change the prescription for your glasses often

These symptoms can be a sign of other eye problems, too. Be sure to talk to your eye doctor if you have any of these problems.

HOW YOU CAN PREVENT CATARACTS:

Maintaining healthy eating habits can help to prevent cataracts in two ways. First, a good diet will control your weight, thus eliminating one of the significant risk factors. Second, increasing your antioxidant intake can also inhibit the oxidation process. In a 2013 Swedish, researchers observed 30,000 women over age 49, and found that those who consumed the highest amounts of antioxidants had a 13 percent lower chance of developing cataracts than those who consumed the least amounts.

To reduce your risk for cataracts, be sure to eat plenty of foods high in antioxidants.

TREATMENTS FOR CATARACTS:

Surgery is the only way to get rid of a cataract, but you may not need to get surgery right away. 

Home treatment. Early on, you may be able to make small changes to manage your cataracts. You can do things like:

  • Use brighter lights at home or work
  • Wear anti-glare sunglasses
  • Use magnifying lenses for reading and other activities

New glasses or contacts. A new prescription for eyeglasses or contact lenses can help you see better with cataracts early on.

Surgery. Your doctor might suggest surgery if your cataracts start getting in the way of everyday activities like reading, driving, or watching TV. During cataract surgery, the doctor removes the clouded lens and replaces it with a new, artificial lens (also called an intraocular lens, or IOL). This surgery is very safe, and 9 out of 10 people who get it can see better afterwards.

Talk about your options with your doctor. Most people don’t need to rush into surgery. Waiting to have surgery usually won’t harm your eyes or make surgery more difficult later. Remember these tips:

  • Tell your doctor if cataracts are getting in the way of your everyday activities
  • See your doctor for regular check-ups
  • Ask your doctor about the benefits and risks of cataract surgery
  • Encourage family members to get checked for cataracts, since they can run in families

Latest research on cataracts:

Scientists are studying what causes cataracts and how we can find them earlier and treat them better.

 

 

 

QUOTE FOR FRIDAY:

“Cataracts are the most frequent cause of vision loss in people over the age of 40 and the leading cause of blindness worldwide. There are actually more cases of cataracts worldwide than there are of glaucoma.”

Omni Eye Specialists – https://www.omnieye.com