Rare Disease Day 28 February 2019

We are #rare!!

Celebrate You’re Rare

Facts

Osteonecrosis – Avascular Necrosis -Aseptic Necrosis-Ischemic Necrosis-Bone Infarction- has many different names and causes.In children its Legg Calves Perthes.

They all mean – A Loss of blood supply to the bone  which may lead to bone cell death and can be caused by an injury (meniscal tear -bone fracture or joint dislocation; called traumatic osteonecrosis).

There may be no history of injury (non-traumatic osteonecrosis); however, other risk factors are associated with the disease such as some medications (steroids, also known as corticosteroids), alcohol usage or blood coagulation disorders. Increased pressure within the bone also is associated with osteonecrosis.

One theory is that the pressure within the bone causes the blood vessels to narrow, making it difficult for blood to circulate through the bone. Osteonecrosis can also be associated with other disorders. The exact reason osteonecrosis develops is not fully understood for some risk factors.

rareday2019avn

Sometimes, osteonecrosis occurs in people with no risk factors (idiopathic). Some people have multiple risk factors. Osteonecrosis most likely develops because of the combination of factors, possibly including genetic, metabolic, self-imposed (alcohol, smoking), and other diseases that you may have and their treatment.

Injury:

When a joint any joint hip,knee, shoulder, ankle, elbow is injured, as in a fracture or dislocation, meniscus tear the blood vessels may be damaged.

This can interfere with the blood circulation to the bone and lead to trauma-related osteonecrosis.

Studies suggest that this type of osteonecrosis may develop in more than 20% of people who dislocate their hip joint.

Corticosteroid Medications:

Corticosteroids, such as prednisone, are commonly used to treat diseases in which there is inflammation, such as systemic lupus erythematosus, rheumatoid arthritis, inflammatory bowel disease, and vasculitis. Studies suggest that long-term, high dose systemic (oral or intravenous) corticosteroid use is a major risk factor for non-traumatic osteonecrosis with reports of up to 35 percent of all people with non-traumatic osteonecrosis.

And corticosteroids come in many versions – inhaled and ingested corticosteroids for asthma-cold-sinus problems or steroid injections into joints, topical for skin-

Patients should discuss concerns about corticosteroid use with their doctor.

Doctors aren’t sure exactly why the use of corticosteroids sometimes is associated with osteonecrosis. They may have negative effects on different organs and tissues within the body. For example, they may interfere with the body’s ability to build new bones and to break down fatty substances.

These substances would then build up in and clog the blood vessels, causing them to narrow. This then would reduce the ability of blood to flow inside a bone.

Alcohol Use:

Excessive alcohol use is another major risk factor for non-traumatic osteonecrosis. Studies have reported that alcohol accounts for about 30% of all people with non-traumatic osteonecrosis. While alcohol can slow down bone remodeling (the balance between forming new bone and removing bone), it is not known why or how alcohol can trigger osteonecrosis.

Other Risk Factors:

Other risk factors or conditions associated with non-traumatic osteonecrosis include Gaucher disease, pancreatitis, autoimmune disease, cancer, HIV infection, decompression disease (Caisson disease), and blood disorders such as sickle cell disease. Certain medical treatments including radiation treatments and chemotherapy can cause osteonecrosis. People who have received a kidney or other organ transplant may also have an increased risk.

Affected Populations

Osteonecrosis usually affects people between 30 and 50 years of age; about 10,000 to 20,000 people develop osteonecrosis each year in the United States.

Osteonecrosis affects both men and women and affects people of all ages. It is most common among people in their thirties and forties.

Depending on a person’s risk factors and whether the underlying cause is trauma, it also can affect younger or older people.

Related Disorders

Some Symptoms of the disorders listed below may be similar to those of osteonecrosis. Comparisons may be useful for a differential diagnosis:

Osteopetrosis is a combination of several rare genetically caused symptoms grouped together as one disorder. It can be inherited and is marked by increased bone density, brittle bones, and, in some people, skeletal abnormalities. Although symptoms may not initially be apparent to people with mild forms of this disorder, trivial injuries may cause bone fractures due to abnormalities of the bone. The dominantly transmitted form is milder than the recessive form and may not be diagnosed until adolescence or adulthood when symptoms first appear. More serious complications occur in the recessive form which may be diagnosed from examination of skeletal x-rays during infancy or childhood. (For more information on this disorder, choose “Osteopetrosis” as your search term in the Rare Disease Database.)

Reflex sympathetic dystrophy syndrome (RSDS), also known as complex regional pain syndrome, is a rare disorder of the sympathetic nervous system that is characterized by chronic and severe pain. The sympathetic nervous system is that part of the autonomic nervous system that regulates involuntary functions of the body such as increasing heart rate, constricting blood vessels, and increasing blood pressure. Excessive or abnormal responses of portions of the sympathetic nervous system are thought to be responsible for the pain associated with reflex sympathetic dystrophy syndrome. The symptoms of RSDS typically begin with burning pain, especially in an arm, finger(s), palm of the hand(s), and/or shoulder(s). In some individuals, RSDS may occur in one or both legs or it may be localized to one knee or hip. Frequently, RSDS may be misdiagnosed as a painful nerve injury. The skin over the affected area(s) may become swollen (edema) and inflamed. Affected skin may be extremely sensitive to touch and to hot or cold temperatures (cutaneous hypersensitivity). The affected limb(s) may perspire excessively and be warm to the touch (vasomotor instability). The exact cause of RSDS is not fully understood, although it may be associated with injury to the nerves, trauma, surgery, atherosclerotic cardiovascular disease, infection, or radiation therapy. (For more information on this disorder, choose “reflex sympathetic dystrophy” as your search term in the Rare Disease Database.)

Legg-Calvé-Perthes disease (LCPD) is one of a group of disorders known as the osteochondroses. The osteochondroses typically are characterized by degeneration and subsequent regeneration of the growing end of a bone (epiphyses). In LCPD, the growing end of the upper portion of the thigh bone (femur) is affected. The upper section of the thigh bone is known as the head or “the ball” and connects to the hip in a depression or “socket”. This is the hip joint, which is a ball and socket joint. The disorder results from an unexplained interruption of the blood supply (ischemia) to the head of the femur, which causes degeneration and deformity of the femoral head. Symptoms may include a limp with or without pain in the hip, knee, thigh, and/or groin; muscle spasms; and/or limited or restricted movement of the affected hip. The disease process seems to be self-limiting as new blood supplies are established (revascularization) and new healthy bone forms (re-ossifies) in the affected area. The exact cause for the temporary interruption of blood flow to the femoral epiphysis is not fully understood. Most times the disorder appears to occur randomly for no apparent reason (sporadically).

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Osteonecrosis-Avascular Necrosis -Broken Down

Osteonecrosis is the death of a segment of bone caused by an impaired blood supply. We all need an adequate blood supply to the bone other wise the bone can collapse and die. The blood supply to bone is delivered to the endosteal cavity by nutrient arteries, then flows through marrow sinusoids before exiting via numerous small vessels that ramify through the cortex. Reductions in vascular supply are associated with bone loss. Bones in our body are living tissue. They have their own blood vessels and are made of living cells, which help them to grow and to repair themselves. As well, proteins, minerals and vitamins make up the bone when you get to little blood flow the bone breaksdown much faster than it can repair and generate new bone.

This disorder can be caused by an injury or can occur spontaneously.

Typical symptoms include pain, limited range of motion of the affected joint, and, when the leg is affected, a limp.

The diagnosis is based on symptoms, the person’s risk of osteonecrosis, and the results of x-rays and magnetic resonance imaging.

Stopping smoking, stopping excessive alcohol use, and minimizing the use of or lowering the dose of corticosteroids reduce the risk of developing the disorder.

Various surgical procedures can be done if nonsurgical measures (such as rest, physical therapy, and pain relievers) do not relieve symptoms. Eat a healthy diet

Each year in the United States, about 20,000 people develop osteonecrosis. The hip is most commonly affected, followed by the knee and shoulder. The wrist and ankle are affected less often. Osteonecrosis does not usually affect the shoulder or other less commonly affected sites unless the hip is also affected. However, osteonecrosis of the jaw (ONJ) is a disorder involving only the jaw bone.
Causes

Osteonecrosis is not a specific disease but a condition in which death of the bone is confined to one or more specific (localized) areas. There are two general categories of osteonecrosis:Making it a rare disorder.

Traumatic (following an injury)

Nontraumatic

Traumatic osteonecrosis is the most common. The most frequent cause of traumatic osteonecrosis is a displaced fracture. In a displaced fracture, a bone breaks into two or more parts and moves so that the fractured ends are not lined up. The type of displaced fracture that causes osteonecrosis most often affects the hip (see Hip Fractures) and most commonly occurs in older people.

Another cause of traumatic osteonecrosis is a dislocation. A dislocation occurs when the ends of bones in joints become completely separated from each other, as in a hip dislocation.

A displaced fracture or a dislocation may damage the blood vessels supplying the upper end of the thighbone (the femoral head, part of the hip joint), resulting in death of this part of the bone. This death of bone occurs less often in other areas of the body.
Some Risk Factors for Osteonecrosis

Traumatic osteonecrosis- injury to bone or joint like meniscal tear -broken bones-dislocations etc…

Fractures (breaks in bones) and dislocations (when the ends of bones in joints become completely separated from each other)

Nontraumatic osteonecrosis

Excessive alcohol

Blood clotting (coagulation) disorders

Bone Marrow Edema

Chemotherapy

Corticosteroids

Cushing syndrome

Decompression sickness

Gaucher disease

High level of lipids in the blood (hyperlipidemia)

HIV infection

Liver disease

Lupus and other autoimmune connective tissue disorders

Miscellaneous conditions (such as chronic kidney disease and rare genetic mutations)

Organ transplantation

Pancreatitis

Radiation

Sickle cell disease

Smoking

Tumors

Vasculitis
Nontraumatic osteonecrosis occurs without direct trauma or injury. This type may be caused by a disease or condition that results in the blockage of small blood vessels that supply certain areas of the bone. The areas most commonly affected are the femoral head (which is part of the hip joint), the knee, and the upper arm at the shoulder. This disorder occurs most commonly among men and people between the ages of 30 and 50 and often affects both hips or both shoulders. The most common causes are

Corticosteroids (when given at high doses, for long periods of time, or both)

Chronic, excessive alcohol use (more than 3 drinks a day for several years)

A number of other causes have been identified, but these occur much less often. These other causes include certain blood-clotting disorders, sickle cell disease, liver disease, tumors, Gaucher disease, radiation therapy, and decompression sickness (which occurs in divers who surface too quickly). A number of disorders that are treated with high doses of corticosteroids (such as lupus) also may be associated with osteonecrosis. In these cases, it may not be clear whether the cause is the disorder or the corticosteroids.

In about 20% of people with osteonecrosis, the cause is unknown.

If one bone has nontraumatic osteonecrosis, the same bone on the opposite side of the body sometimes has it also, even if there are no symptoms. For example, if one hip is affected, about 60% of the time the other hip is affected.

Spontaneous osteonecrosis of the knee (SPONK or SONK) can occur in older women (occasionally men) who have no specific risk factors for the disorder. SPONK differs from other forms of osteonecrosis. SPONK is thought to be caused by an insufficiency fracture. An insufficiency fracture is caused by normal wear and tear on bone that has been affected by osteoporosis. SPONK occurs without direct trauma or injury.
Symptoms

As osteonecrosis progresses, more and more tiny fractures may occur, particularly in bones that support weight, such as the hip. As a result, the bone usually collapses weeks or months after the blood supply is cut off. Most often pain develops gradually when the bone begins to collapse. At times, however, pain may begin suddenly and could be related to increased pressure that develops in and around the affected area of bone. Regardless of how sudden, pain is increased by moving the affected bone and typically is alleviated with rest. The person avoids moving the joint to minimize pain.

If the affected bone is in the leg, standing or walking worsens the pain and a limp develops.

In osteonecrosis of the hip, pain is usually present in the groin and may extend down the thigh or into the buttocks.

Spontaneous osteonecrosis of the knee causes sudden pain along the inner part of the knee. There may be tenderness in this area, and the joint often becomes swollen with excess fluid. Bending the knee may be painful, and people may have a limp.

Osteonecrosis of the shoulder often causes fewer symptoms than osteonecrosis that occurs in the hip or knee.

Osteoarthritis (damage to the cartilage covering the joint surfaces) develops over time, often after a large part of the bone collapses.
Diagnosis

X-rays

Magnetic resonance imaging

Because osteonecrosis is often painless at first, it may not be diagnosed in its earliest stages. Doctors suspect osteonecrosis in people who do not improve satisfactorily after having certain fractures. They also suspect the disorder in people who develop unexplained pain in the hip, knee, or shoulder, particularly if these people have risk factors for osteonecrosis.

X-rays of the affected area usually show osteonecrosis unless the disorder is in its earliest stages. If x-rays appear normal, however, magnetic resonance imaging (MRI) is usually done because it is the best test for detecting osteonecrosis early, before changes appear on ordinary x-rays. The x-rays and MRI also show whether the bone has collapsed, how advanced the disorder is, and whether the joint is affected by osteoarthritis. If doctors discover nontraumatic osteonecrosis in one hip, they also examine the other hip with an x-ray or MRI.

Blood tests may be done to detect an underlying disorder (such as a blood-clotting disorder).
Prevention

To minimize the risk of osteonecrosis caused by corticosteroids, doctors use these drugs only when essential, prescribe them in as low a dose as needed, and prescribe them for as short a duration as possible.

To prevent osteonecrosis caused by decompression sickness, people should follow accepted rules for decompression during diving and when working in pressurized environments (see prevention of decompression sickness and see Diving Safety Precautions and Prevention of Diving Injuries).

Excessive alcohol use and smoking should be avoided.

Various drugs (such as those that prevent blood clots, dilate blood vessels, or lower lipid levels) are being evaluated for prevention of osteonecrosis in people at high risk.
Treatment

Nonsurgical measures to relieve symptoms

Surgical procedures

Hip replacement

blood supply long bones

Some areas affected by osteonecrosis need only nonsurgical measures to relieve symptoms. Other areas need to be treated with a surgical procedure.
Nonsurgical measures

Several nonsurgical measures are available for treating the symptoms caused by osteonecrosis. Taking anti-inflammatory drugs or other pain relievers, minimizing activity and stress (such as weight bearing for osteonecrosis of the hip and knee), and undergoing physical therapy are ways to relieve symptoms but not cure the disorder or change its course. These measures, however, may be adequate for treatment of the shoulder, the knee, spontaneous osteonecrosis of the knee, and small areas of osteonecrosis of the hip, which may spontaneously heal without treatment. Osteonecrosis heals without treatment in about 80% of people if the disorder is diagnosed early and if the affected area is small.

Spontaneous osteonecrosis of the knee is usually treated without surgery, and pain usually resolves.
Surgical procedures

There are a number of surgical procedures that slow or possibly prevent progression of the disorder. These procedures are done to preserve the joint and are most effective for treating early osteonecrosis, particularly of the hip, that has not yet progressed to bone collapse. If bone collapse has occurred, a type of joint replacement procedure may be done to decrease pain and improve function.

Core decompression, the simplest and most common of these procedures, involves drilling one or many small tracks or holes (perforations) into the area in an attempt to decrease pressure inside the bone. Core decompression often relieves pain and stimulates healing. In about 65% of people, the procedure can delay or prevent the need for total hip replacement. In younger people, core decompression may also be used even if a small amount of collapse already has taken place. The procedure is relatively simple, has a low rate of complications, and requires the use of crutches for about 6 weeks. Most people have satisfactory or good results overall. However, results for any particular person can be hard to predict. About 20 to 35% of people require a total hip replacement.

During core decompression, surgeons may inject a person’s own bone cells into the small hole or holes. This enhancement to the core decompression procedure may help heal the femoral head (which is part of the hip joint).

Bone grafting (transplanting bone from one site to another) is another procedure. For osteonecrosis of the hip, this can involve removing the dead area of bone and replacing it with more normal bone from elsewhere in the body. This graft supports the weakened area of bone and stimulates the body to form new, living bone in the affected area.

An osteotomy is another procedure designed to save the affected joint. This procedure is done particularly in the region of the hip and may be suitable for younger people in whom some degree of collapse already has occurred, which makes them poor candidates for core decompression or other procedures. Usually the osteonecrosis is in the weight-bearing area of the femoral head. An osteotomy changes the position of the bone so that the weight of the body is now supported by a normal area of the femoral head and not by the collapsed area.

Bone grafting and osteotomy are difficult procedures, however, and are not often done in the United States. They require a person to spend up to 6 months on crutches. These procedures are done only at selected centers that have the surgical experience and facilities to achieve the best results.

A total joint replacement is an effective procedure to relieve pain and restore motion if osteonecrosis has caused significant joint collapse and osteoarthritis. About 95% of people benefit from total replacement of the hip or knee (see Hip replacement). With modern techniques and devices, most daily activities can be resumed within 3 months and most joints should last more than 15 to 20 years.

In younger people with osteonecrosis, a total joint replacement may have to be revised (called revision surgery) or replaced at some later time. However, with modern devices, revision surgery has become much less common. Because total joint replacement is now so successful, there is much less need to do other procedures that replace part of the joint or remove the surface cartilage and place a cap on each bone end.

Occasionally, a partial or total replacement of an extremely painful knee or shoulder may be needed for advanced osteonecrosis that is not alleviated by nonsurgical treatment.
More Information

National Institute of Arthritis and Musculoskeletal and Skin Diseases

 

©Debla2016 Butterfly

The first image at top of page I was given special permission to tweak  it for rare disease awareness day

Published by

ChronicallyGratefulDebla

The body always knows what to do to heal itself. The challenge is listening and doing what your body needs. I was diagnosed with Osteoarthritis in 2012, Avascular Necrosis aka Osteonecrosis in 2014 and Factor V Leiden hetero, Spondylolisthesis 2016 Health Advocate for all above and more Health Activist World Changer Love photography, cooking, hiking,walking and learning to live a new normal since my diagnosis. And also Advocate for Heart Disease it's something I learned about as a kid ,both parents had it and now so does my husband. My Links Support Group Avascular Necrosis/Osteonecrosis Support Int’l https://m.facebook.com/groups/DeadBoneDiseaseAvn Facebook Main Profile https://m.facebook.com/public/Deborah-Andio Main Blog ChronicallyGratefulDebla.com YouTube Awareness Videos https://m.youtube.com/user/debbieandio New Blog on Google 3/8/2017 http://chronicallygratefuldebla.blogspot.com/2017/03/hello-i-am-debbie-andio-i-am-blogger.html Link to Mini Fact Videos http://cortanavideo.trade/user/UC1RtmEwtWKC8w9EgE2IwOFg Twitter https://mobile.twitter.com/debbiea001 Instagram https://www.instagram.com/debbiea_1962 FB Information and Awareness Pages Bone info and Joint of the Day https://m.facebook.com/AvascularNecrosisRareDiseaseDayFeb29/ ON/AVN https://m.facebook.com/Osteonecrosis-Avascular-Necrosis-Support-913679995417381/ ON/AVN https://m.facebook.com/ONAvascular-Necrosis-Knowledge-752404224891578/ Recipes https://m.facebook.com/YummyGoodness/ Facebook Link https://m.facebook.com/ChronicallyGrateful.Me/ New Morning Talk Anyone who wants to do a f b live via a guest message me. Various topics health, pain, food https://m.facebook.com/MorningTalk.Health/

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