QUOTE FOR THE WEEKEND:

Jul25

“Acromegaly (called gigantism in children) is a rare condition in which the whole body or parts of the body, particularly the hands and feet, grow larger than normal. More than 95 percent of all cases of acromegaly are the result of a growth hormone–secreting pituitary adenoma.

Growth hormone–secreting pituitary adenomas begin in the somatotropic cells of the pituitary gland. Also called GH, the growth hormone secreted by these tumors triggers the liver to produce insulin-like growth factor 1, or IGF-1. IGF-1 is involved in bone and tissue growth, metabolism, and other processes of the body. When the body produces too much IGF-1, it leads to a range of symptoms known as acromegaly.”

Memorial Sloan Kettering Center (Acromegaly (Growth Hormone–Secreting Pituitary Adenomas) | Memorial Sloan Kettering Cancer Center)

Gigantism, also knows as Acroemegaly – learn the s/s, diagnostic tests and treatment!

Jul25

 

Gigantism is described as accelerated growth during childhood from the production of excess growth hormone. By definition, gigantism must occur during childhood before the growth plates in the long bones of the body (for example, the femur or humerus) have closed. In adults, the condition is called acromegaly.

Gigantism is most often caused by a benign tumor on the pituitary gland called a pituitary adenoma. However, it can also be caused by the following disorders:

  • Neurofibromatosis
  • McCune-Albright syndrome (MAS)
  • Carney complex
  • Multiple endocrine neoplasia type 1

Sign and Symptoms of Gigantism:

  • Abnormally tall stature
  • Abnormal growth of the face, hands and feet
  • Thickened facial features
  • Irregular menstrual cycle
  • Excessive perspiration with slight activity
  • Delayed puberty
  • Double vision
  • Deafness
  • Headache

How Gigantism is Diagnosed:

Magnetic resonance imaging (MRI) is used to determine the size and location of your child’s tumor if it is suspected that the disease is caused by a pituitary adenoma.

Furthermore, several blood tests can provide a diagnosis. High levels of prolactin or increased amounts of insulin growth factor-1 (IGF-1) can suggest acromegaly, as can high levels of growth hormone in the blood after oral administration of a large dose of glucose. Low levels of cortisol, thyroid hormone, testosterone (in boys), and estradiol (in girls) can also suggest involvement of the pituitary gland.

Treatment for Gigantism:

Surgery

Surgery is the best form of treatment and cures 80 percent of the cases of gigantism. Your child’s surgeon will gain access to your child’s pituitary gland using the transsphenoidal approach—so named because the route crosses, or transects, the sphenoid bone. This bone is located behind your child’s nose, mostly within their skull.

Using precise surgical instruments, the surgeon will make an incision through your child’s nasal cavity to create an opening in the sphenoid bone. Once the surgeon gains access to your child’s sphenoid sinus (the air-filled area behind the sphenoid bone), further incisions will be made until a hole is created in the sella turcica—the bone that cradles and protects the pituitary gland.

After your child’s pituitary gland is in the operative field, removal of the tumor can proceed. Your child’s surgeon will use high magnification to readily distinguish normal pituitary tissue from the tumor.

Once the tumor has been removed, your child’s surgeon will clean the tumor cavity and seal it.

At Barrow Neurological Institute at Dignity Health St. Joseph’s Hospital and Medical Center, our surgeons specialize in two types of surgery for adenomas:

  • Microsurgery uses a powerful operating microscope to help your child’s surgeon distinguish between tiny structures in and around the pituitary gland.
  • Endoscopic surgery uses small tubes and a tiny camera to help your child’s surgeon remove the tumor in small pieces.

Most patients are able to return home the day after their surgery for removal of a pituitary adenoma, and nasal packing is seldom required.

Pharmacological Treatment and Hormone Therapy

Treatment with a prescription medication is possible if surgery does not cure your child’s gigantism or if surgery is not recommended. Medicines are also sometimes prescribed before surgery to improve the likelihood of a good outcome.

  • Octreotide or lanreotide are synthetic forms of the hormone somatostatin and stop the release of growth hormone. They are often effective for the long-term control of gigantism, but they can only be administered by injection every two to four weeks. If your child’s tumor is particularly large, these drugs may be administered before surgery. Due to the side effects of these drugs and their expense, surgery to achieve a long-term cure is preferable.
  • Bromocriptine and cabergoline are from a class of drugs called dopamine agonists. They can lower IGF-1 and growth hormone levels in about half of the people treated with them (although your symptoms could improve even if your IGF-1 and growth hormone levels do not decrease). Although not as effective as the synthetic hormones octreotide and lanreotide, they are less expensive and more convenient to administer, because no injection is required. They can be combined with octreotide in children with no adverse effects on long-term health.
  • Pegvisomant is a recently developed drug that blocks the action of growth hormone in your body, thereby lowering IGF-1 levels. It must be administered by subcutaneous (beneath the skin) injection daily. It is another option if your child does not respond to surgery or other medications, or if your child cannot tolerate these treatments for other reasons.

Gamma Knife

Gamma Knife radiosurgery is a highly advanced form of radiotherapy that is used to achieve similar results to the traditional surgical techniques described above. However, with Gamma Knife it can take several years for growth hormone and levels to return to normal, rather than days or weeks as with traditional surgery. It is typically a treatment of last resort in patients with gigantism.

The ‘knife’ in this surgery is actually made up of many small beams of radiation focused on a single point. Each individual beam too weak enough to damage healthy tissue, but at the point where the beams converge they deliver a dose of radiation that is lethal to the tumor.

Gamma Knife is an outpatient procedure, does not involve any incisions, and requires only brief sedation under general anesthetic.

However, there are additional considerations for pediatric Gamma Knife surgery. These are best discussed with your child’s neurosurgeon.

 

QUOTE FOR FRIDAY:

Jul24

“Approximately 220,000 children and adolescents had arthritis during 2017–2021, representing 305 per 100,000. Prevalence increased with age and was highest among those aged 12–17 years, non-Hispanic Black or African American children and adolescents, children and adolescents with anxiety or depression, those who were physically inactive, had overweight or a heart condition, or lived in a food-insecure or smoking household.

Self-management interventions, physical activity or weight control, screening and linking to mental health services, and equitable access to therapies might improve arthritis outcomes in children and adolescents.”

Center for Disease Prevention and Control (Arthritis Among Children and Adolescents Aged 18 Years — United States, 2017–2021 | MMWR)

 

 

Part II Juvenile Arthritis (JIA) – How its diagnosed and treated!

Jul24

How Juvenile Arthritis (JIA) is Diagnosis:

Diagnosis of juvenile idiopathic arthritis can be difficult because joint pain can be caused by many different types of problems. No single test can confirm a diagnosis, but tests can help rule out some other conditions that produce similar signs and symptoms.

1. Blood tests

Some of the most common blood tests for suspected cases include:

  • Erythrocyte sedimentation rate (ESR). The sedimentation rate is the speed at which your red blood cells settle to the bottom of a tube of blood. An elevated rate can indicate inflammation. Measuring the ESR is primarily used to determine the degree of inflammation.
  • C-reactive protein. This blood test also measures levels of general inflammation in the body but on a different scale than the ESR.
  • Antinuclear antibody. Antinuclear antibodies are proteins commonly produced by the immune systems of people with certain autoimmune diseases, including arthritis. They are a marker for an increased chance of eye inflammation.
  • Rheumatoid factor. This antibody is occasionally found in the blood of children who have juvenile idiopathic arthritis and may mean there’s a higher risk of damage from arthritis.
  • Cyclic citrullinated peptide (CCP). Like the rheumatoid factor, the CCP is another antibody that may be found in the blood of children with juvenile idiopathic arthritis and may indicate a higher risk of damage.

In many children with juvenile idiopathic arthritis, no significant abnormality will be found in these blood tests.

2. Imaging scans

X-rays or magnetic resonance imaging may be taken to exclude other conditions, such as fractures, tumors, infection or congenital defects.

Imaging may also be used from time to time after the diagnosis to monitor bone development and to detect joint damage.

Juvenile Arthritis (JIA) Treatment:

Treatment for juvenile idiopathic arthritis focuses on helping your child maintain a normal level of physical and social activity. To accomplish this, doctors may use a combination of strategies to relieve pain and swelling, maintain full movement and strength, and prevent complications.

1. Medications

The medications used to help children with juvenile idiopathic arthritis are chosen to decrease pain, improve function and minimize potential joint damage.

Typical medications include:

  • Nonsteroidal anti-inflammatory drugs (NSAIDs). These medications, such as ibuprofen (Advil, Motrin, others) and naproxen sodium (Aleve), reduce pain and swelling. Side effects include stomach upset and, much less often, kidney and liver problems.
  • Disease-modifying antirheumatic drugs (DMARDs). Doctors use these medications when NSAIDs alone fail to relieve symptoms of joint pain and swelling or if there is a high risk of damage in the future.DMARDs may be taken in combination with NSAIDs and are used to slow the progress of juvenile idiopathic arthritis. The most commonly used DMARD for children is methotrexate (Trexall, Xatmep, others). Side effects of methotrexate may include nausea, low blood counts, liver problems and a mild increased risk of infection.
  • Biologic agents. Also known as biologic response modifiers, this newer class of drugs includes tumor necrosis factor (TNF) blockers, such as etanercept (Enbrel, Erelzi, Eticovo), adalimumab (Humira), golimumab (Simponi) and infliximab (Remicade, Inflectra, others). These medications can help reduce systemic inflammation and prevent joint damage. They may be used with DMARDs and other medications.Other biologic agents work to suppress the immune system in slightly different ways, including abatacept (Orencia), rituximab (Rituxan, Truxima, Ruxience), anakinra (Kineret) and tocilizumab (Actemra). All biologics can increase the risk of infection.
  • Corticosteroids. Medications such as prednisone may be used to control symptoms until another medication takes effect. They are also used to treat inflammation when it is not in the joints, such as inflammation of the sac around the heart.These drugs can interfere with normal growth and increase susceptibility to infection, so they generally should be used for the shortest possible duration.

2. Therapies

Your doctor may recommend that your child work with a physical therapist to help keep joints flexible and maintain range of motion and muscle tone.

A physical therapist or an occupational therapist may make additional recommendations regarding the best exercise and protective equipment for your child.

A physical or occupational therapist may also recommend that your child make use of joint supports or splints to help protect joints and keep them in a good functional position.

3. Surgery

In very severe cases, surgery may be needed to improve joint function.

***Parents or caregivers help limit the arthritis in your children by doing the following:

  • Getting regular exercise. Exercise is important because it promotes both muscle strength and joint flexibility. Swimming is an excellent choice because it places minimal stress on joints.
  • Applying cold or heat. Stiffness affects many children with juvenile idiopathic arthritis, particularly in the morning. Some children respond well to cold packs, particularly after activity. However, most children prefer warmth, such as a hot pack or a hot bath or shower, especially in the morning
  •  Eating Well. Some children with arthritis have poor appetites. Others may gain excess weight due to medications or physical inactivity. A healthy diet can help maintain an appropriate body weight.Know adequate calcium in the diet is important because children with juvenile idiopathic arthritis are at risk of developing weak bones due to the disease, the use of corticosteroids, and decreased physical activity and weight bearing.

 

QUOTE FOR THURSDAY:

Jul23

“Juvenile arthritis (JA), also known as pediatric rheumatic disease, isn’t a specific disease.

JA is an umbrella term for various inflammatory and rheumatic diseases affecting children under 16, with juvenile idiopathic arthritis (JIA) being the most common type.  These conditions affect hundreds of thousands of kids and teens in the United States.

Most kinds of JA are autoimmune or autoinflammatory diseases. That means the immune system, which is supposed to fight against foreign invaders like viruses and germs, instead releases inflammatory chemicals that attack healthy cells and tissue. In most JA cases this causes joint inflammation, swelling, pain and tenderness, but some types of JA have few or no joint symptoms or only affect the skin and internal organs.

The exact causes of JA are unknown, but researchers believe that certain genes may cause JA when activated by a virus, bacteria or other external factors. There is no evidence that foods, toxins, allergies or lack of vitamins cause the disease.”

Arthritis Foundation (Juvenile Arthritis: Symptoms, Diagnosis, and Treatment | Arthritis Foundation)

 

Part I Juvenile Arthritis (JA)-What is actually is vs Adult Arthritis, the several types of JA, and if left untreated what can happen!

Jul23

What is Juvenile Arthritis (JIA) and how different is it than adults with a type of arthritis?

Like adults, children can develop arthritis. The most common type of chronic, or long-lasting, arthritis that affects children is called juvenile idiopathic arthritis (JIA). JIA broadly refers to several different chronic disorders involving inflammation of joints (arthritis), which can cause joint pain, swelling, warmth, stiffness, and loss of motion. The various forms of JIA have different features, such as the pattern of joints involved and inflammation of other parts of the body besides the joints. JIA may last a limited time, such as a few months or years, but in some cases it is a lifelong disease that requires treatment into adulthood.

JIA is “idiopathic,” meaning that its origins are not understood. While the exact causes of JIA are unknown, it begins when the immune system becomes overactive and creates inflammation.

With treatment, most children achieve periods of wellness (remission), and sometimes the disease goes away permanently with no further need for medications. It is important to see a doctor early if your child has swollen or stiff joints because delaying therapy can lead to joint damage, a lesser response to treatment, and other problems.

Who Gets Juvenile Idiopathic Arthritis (JIA)?

By definition, JIA begins in children and adolescents before the 16th birthday. Most types of the disease are more frequent in girls, but enthesitis-related JIA, a form of the disease that involves inflammation of the places where ligaments and tendons (flexible bands of tissue) attach to bones, is more common in boys. Systemic JIA, a rare type of JIA that features fever and rash, affects boys and girls equally. Children of all races and ethnic backgrounds can get the disease.

It is very rare for more than one member of a family to have JIA, but children with a family member with chronic arthritis, including JIA, are at a slightly increased risk of developing it. Having a family member with psoriasis is a risk factor for a form of JIA called psoriatic JIA.

There are many types of Juvenile Arthritis (JIA)with distinct features:

Generally, they all share arthritic symptoms of joint pain, swelling, warmth, and stiffness that last at least 6 weeks.

The types of JIA:

  • Oligoarticular juvenile idiopathic arthritis. This is the most common and mildest form, affecting four or fewer joints. It is considered persistent if symptoms continue for 6 months or longer, and extended if five or more joints become involved after 6 months of illness. Commonly affected joints are knees or ankles. A form of eye inflammation called chronic (long-lasting) uveitis can develop in children with this form of JIA. About half of children with JIA have this type.
  • Polyarticular juvenile idiopathic arthritis–rheumatoid factor negative. This is the second most common type, affecting five or more joints in the first 6 months. Tests for rheumatoid factor are negative. The rheumatoid factor blood test checks for autoimmune disease, especially rheumatoid arthritis, which is an adult form of arthritis. Some of these children develop chronic uveitis.
  • Polyarticular juvenile idiopathic arthritis–rheumatoid factor positive. A child with this type has arthritis in five or more joints during the first 6 months of the disease. Tests for rheumatoid factor, a marker for autoimmune disease, are positive. It tends to occur in preteen and teenage girls, and it appears to be essentially the same as adult rheumatoid arthritis.
  • Enthesitis-related juvenile idiopathic arthritis. This form of JIA involves both arthritis and enthesitis. Enthesitis happens when inflammation occurs where a ligament or tendon attaches to a bone. The most common locations for enthesitis are the knees, heels, and bottoms of the feet. Arthritis is usually in the hips, knees, ankles, and feet, but the sacroiliac joints (at the base of the back) and spinal joints can also become inflamed. Some children get episodes of acute anterior uveitis, a sudden onset of inflammation of the front of the eye. Unlike most other forms of JIA, enthesitis-related JIA is more common in boys.
  • Psoriatic juvenile idiopathic arthritis. Children with this type have psoriasis, a skin condition, as well as inflammation of the joints. The skin condition usually appears first, but sometimes painful, stiff joints are the first sign, with the skin disease occurring years layer. Pitted fingernails and dactylitis (swollen fingers or toes) are also signs of the disease.
  • Systemic juvenile idiopathic arthritis. Systemic means the disease can affect the whole body, not just a specific organ or joint. Systemic JIA usually starts with fever and rash that come and go over the span of at least 2 weeks. In many cases, the joints become inflamed, but sometimes not until long after the fever goes away, and sometimes not at all if treatment is started quickly. In severe forms, inflammation can develop in and around organs, such as the spleen, lymph nodes, liver, and linings of the heart and lungs. Systemic JIA affects boys and girls with equal frequency.
  • Undifferentiated arthritis. This category includes children who have symptoms that do not fit into any of the other types or that fit into more than one type.

If left untreated, uveitis can lead to eye problems such as cataracts, glaucoma, and vision loss, so it is important for children with JIA to have frequent eye exams.

  • Skin changes. Depending on the type of JIA a child has, he or she may develop skin changes. Children with:
    • Systemic JIA who have fevers can get a light red or pink rash that comes and goes.
    • Psoriatic JIA can develop scaly red patches of skin. Psoriatic JIA can also cause pitted nails and dactylitis (swollen fingers or toes).
    • Polyarticular JIA with rheumatoid factor can get small bumps or nodules on parts of the body that receive pressure, such as from sitting.
  • Fever. Patients with systemic JIA typically have daily fevers when the disease begins or flares. The fever usually appears in the evening, and the rash may move from one part of the body to another, usually happening with the fever. Patients with other types of JIA do not generally develop fevers.
  • Growth problems. Inflammation in children with any type of JIA can lead to growth problems. Depending on the severity of the disease and the joints involved, bones near inflamed  joints may grow too quickly or too slowly. This can cause one leg or arm to be longer than the other, or can result in a small or misshapen chin. Overall growth also may be slowed if the disease is severe. Growth normally improves when inflammation is well-controlled through treatment.

Tune in tomorrow on the causes, how its diagnosed and treated!

QUOTE FOR WEDNESDAY:

Jul22

“The causes of fibromyalgia are not known. But certain things can increase someone’s risk of developing it.

Risk factors or triggers

Risk factors or triggers may include:

  • Stressful or traumatic events, accidents, or injuries.
  • Repeat injuries, such as repeated joint stress from sports or physical jobs.
  • Illness, including viral infections.

Some people may be more likely to develop fibromyalgia

These include:

Center for Disease Control – CDC (facts about fibromyalgia – Search)

Part III Fibromyalgia – the treatment!

Jul22

How is Fibromyalgia treated?

There is no cure for fibromyalgia. However, symptoms can be treated with both non-drug and medication based treatments. Many times the best outcomes are achieved by using multiple types of treatments.

Non-Drug Therapies: People with fibromyalgia should use non-drug treatments as well as any medicines their doctors suggest. Research shows that the most effective treatment for fibromyalgia is physical exercise. Physical exercise should be used in addition to any drug treatment. Patients benefit most from regular aerobic exercises. Other body-based therapies, including Tai Chi and yoga, can ease fibromyalgia symptoms. Although you may be in pain, low impact physical exercise will not be harmful.

Cognitive behavioral therapy is a type of therapy focused on understanding how thoughts and behaviors affect pain and other symptoms. CBT and related treatments, such as mindfulness, can help patients learn symptom reduction skills that lessen pain. Mindfulness is a non-spiritual meditation practice that cultivates present moment awareness. Mindfulness based stress reduction has been shown to significantly improve symptoms of fibromyalgia.

You can also try other complementary therapies such as:

  • Massage therapy.
  • Movement therapy.
  • Chiropractic therapy.
  • Acupuncture.
  • Diet supplements.

Other complementary and alternative therapies (sometimes called CAM or integrative medicine), such as acupuncture, chiropractic and massage therapy, can be useful to manage fibromyalgia symptoms. Many of these treatments, though, have not been well tested in patients with fibromyalgia.

It is important to address risk factors and triggers for fibromyalgia including sleep disorders, such as sleep apnea, and mood problems such as stress, anxiety, panic disorder, and depression. This may require involvement of other specialists such as a Sleep Medicine doctor, Psychiatrist, and therapist.

Medications: The U.S. Food and Drug Administration has approved three drugs for the treatment of fibromyalgia. They include two drugs that change some of the brain chemicals (serotonin and norepinephrine) that help control pain levels: duloxetine (Cymbalta) and milnacipran (Savella). Older drugs that affect these same brain chemicals also may be used to treat fibromyalgia. These include amitriptyline (Elavil) and cyclobenzaprine (Flexeril). Other antidepressant drugs can be helpful in some patients. Side effects vary by the drug. Ask your doctor about the risks and benefits of your medicine.

The other drug approved for fibromyalgia is pregabalin (Lyrica). Pregabalin and another drug, gabapentin (Neurontin), work by blocking the over activity of nerve cells involved in pain transmission. These medicines may cause dizziness, sleepiness, swelling and weight gain.

It is strongly recommended to avoid opioid narcotic medications for treating fibromyalgia. The reason for this is that research evidence shows these drugs are not of helpful to most people with fibromyalgia, and will cause greater pain sensitivity or make pain persist. Tramadol (Ultram) may be used to treat fibromyalgia pain if short-term use of an opioid narcotic is needed. Over-the-counter medicines such as acetaminophen (Tylenol) or nonsteroidal anti-inflammatory drugs (commonly called NSAIDs) like ibuprofen (Advil, Motrin) or naproxen (Aleve, Anaprox) are not effective for fibromyalgia pain. Yet, these drugs may be useful to treat the pain triggers of fibromyalgia. Thus, they are most useful in people who have other causes for pain such as arthritis in addition to fibromyalgia.

For sleep problems, some of the medicines that treat pain also improve sleep. These include cyclobenzaprine (Flexeril), amitriptyline (Elavil), gabapentin (Neurontin) or pregabalin (Lyrica). It is not recommended that patients with fibromyalgia take sleeping medicines like zolpidem (Ambien) or benzodiazepine medications.

Always confirm with your doctor which RX is best and not contraindicated for you, for your safety!

QUOTE FOR TUESDAY:

Jul21

“Fibromyalgia affects an estimated 3.4% of women and 0.5% of men in the United States, or 3 million to 6 million Americans. It most commonly affects women of childbearing age or older. In fact, some estimates suggest that more than 7% of women in their 70s have fibromyalgia. Many people with fibromyalgia also have psychiatric problems such as depression, anxiety, or eating disorders, although the relationship between fibromyalgia and mental health disorders remains unclear.

While not a deadly disease, fibromyalgia can significantly impact one’s emotional well-being and overall quality of life. It strikes people of all ages, but it’s more common in women than men. It also runs in families. The risk of developing fibromyalgia is much higher if a first-degree relative, such as a parent or sibling, also has the condition.”

Harvard Health Publishing (Fibromyalgia – Harvard Health)

Part II Fibromyalgia – The symptoms and how its diagnosed!

Jul21

 

Signs and Symptoms of Fibromyalgia:

Fibromyalgia is a physical disorder, not a psychological condition. The most common constellation of Fibromyalgia symptoms (widespread chronic muscle pain, sleeplessness, relentless fatigue, cognitive dysfunction, and a host of other overlapping conditions like TMJD, IBS, migraine, interstitial cystitis, metabolic syndrome, endometriosis, and vulvodynia) can wax and wane over time.

Symptoms include:

  • Tenderness to touch or pressure affecting muscles and sometimes joints or even the skin
  • Severe fatigue
  • Sleep problems (waking up unrefreshed)
  • Problems with memory or thinking clearly

Some patients also may have:

  • Depression or anxiety
  • Migraine or tension headaches
  • Digestive problems: irritable bowel syndrome (commonly called IBS) or gastroesophageal reflux disease (often referred to as GERD)
  • Irritable or overactive bladder
  • Pelvic pain
  • Temporomandibular disorder – often called TMJ (a set of symptoms including face or jaw pain, jaw clicking, and ringing in the ears)

How Fibromyalgia is diagnosed:

A doctor will suspect fibromyalgia based on your symptoms. Doctors may require that you have tenderness to pressure or tender points at a specific number of certain spots before saying you have fibromyalgia, but they are not required to make the diagnosis (see the Box). A physical exam can be helpful to detect tenderness and to exclude other causes of muscle pain. There are no diagnostic tests (such as X-rays or blood tests) for this problem. Yet, you may need tests to rule out another health problem that can be confused with fibromyalgia.

Because widespread body pain is the main feature of fibromyalgia, health care providers will ask you to describe your pain. This may help tell the difference between fibromyalgia and other diseases with similar symptoms. Other conditions such as hypothyroidism (underactive thyroid gland) and polymyalgia rheumatica sometimes mimic fibromyalgia. Blood tests can tell if you have either of these problems. Sometimes, fibromyalgia is confused with rheumatoid arthritis or lupus. But, again, there is a difference in the symptoms, physical findings and blood tests that will help your health care provider detect these health problems. Unlike fibromyalgia, these rheumatic diseases cause inflammation in the joints and tissues.