Bones are the organs that helps us move our body parts, gives us shape and provides a support to our body. To have strong bones we need to get enough calcium and vitamin D. Exercise is also suitable for making bones strong.
If bones are not getting there proper nutrition, they become weak. Weak bones do not function properly and they are more likely to easily break. Many people suffers from bone diseases which makes them weak. Bone diseases can range from mild to severe.
Some of the most common bone problems are:
Osteoporosis
Osteoarthritis
Osteogenesis imperfecta
- Paget's disease
Osteoporosis
Osteoporosis is a disease of bone that leads to an increased risk of fracture. In osteoporosis the bone mineral density (BMD) is reduced, bone microarchitecture is disrupted, and the amount and variety of non-collagenous proteins in bone is altered.Osteoporosis is most common in women after menopause, when it is called postmenopausal osteoporosis, but may also develop in men, and may occur in anyone in the presence of particular hormonal disorders and other chronic diseases or as a result of medications, specifically glucocorticoids, when the disease is called steroid- or glucocorticoid-induced osteoporosis
Osteoporosis can be prevented with lifestyle changes and sometimes medication. Lifestyle change includes preventing falls and exercise; medication includes calcium, vitamin D, bisphosphonates and several others. Fall-prevention advice includes exercise to tone deambulatory muscles, proprioception-improvement exercises; equilibrium therapies may be included. Exercise with its anabolic effect, may at the same time stop or reverse osteoporosis.
Signs and symptoms
Osteoporosis itself has no specific symptoms; its main consequence is the increased risk of bone fractures. Osteoporotic fractures are those that occur in situations where healthy people would not normally break a bone; they are therefore regarded as fragility fractures. Typical fragility fractures occur in the vertebral column, rib, hip and wrist.Fractures
The symptoms of a vertebral collapse are sudden back pain, often with radiculopathic pain (shooting pain due to nerve compression) and rarely with spinal cord compression or cauda equina syndrome. Multiple vertebral fractures lead to a stooped posture, loss of height, and chronic pain with resultant reduction in mobility.
Fractures of the long bones acutely impair mobility and may require surgery. Hip fracture, in particular, usually requires prompt surgery, as there are serious risks associated with a hip fracture, such as deep vein thrombosis and a pulmonary embolism, and increased mortality.
Treatment
There are several alternatives of medication to treat osteoporosis, depending on gender, though lifestyle changes are also very frequently an aspect of treatment.
Medication
Bisphosphonates are the main pharmacological measures for treatment. However, newer drugs have appeared in the 1990s, such as teriparatide and strontium ranelate.
- Bisphosphonates
In confirmed osteoporosis, bisphosphonate drugs are the first-line treatment in women. The most often prescribed bisphosphonates are presently sodium alendronate (Fosamax) 10 mg a day, risedronate (Actonel) 5 mg a day and ibandronate (Boniva) once a month.
A 2007 study suggested that in patients who had suffered a low-impact hip fracture, annual infusion of 5 mg zoledronic acid reduced risk of any fracture by 35% (from 13.9 to 8.6%), vertebral fracture risk from 3.8% to 1.7% and non-vertebral fracture risk from 10.7% to 7.6%.
Oral bisphosphonates are relatively poorly absorbed, and must therefore be taken on an empty stomach, with no food or drink to follow for the next 30 minutes. They are associated with esophagitis and are therefore sometimes poorly tolerated. Although intermittent dosing with the intravenous formulations such as zolendronate avoids oral tolerance problems, these agents are implicated at higher rates in a rare but unpleasant mouth disease called osteonecrosis of the jaw. For this reason, oral bisphosphonate therapy is probably to be preferred, and prescribing advice now recommends any remedial dental work to be carried out prior to commencing treatment.
- Teriparatide
Strontium ranelate
Oral strontium ranelate is an alternative oral treatment. It has proven efficacy, especially in the prevention of vertebral fracture. In laboratory experiments, strontium ranelate was noted to stimulate the proliferation of osteoblasts, as well as inhibiting the proliferation of osteoclasts.
Strontium ranelate is taken as a 2 g oral suspension daily, and is licenced for the treatment of osteoporosis to prevent vertebral and hip fracture. Strontium ranelate has side effect benefits over the bisphosphonates, as it does not cause any form of upper GI side effect, which is the most common cause for medication withdrawal in osteoporosis. In studies a small increase in the risk of venous thromboembolism was noted, the cause for which has not been determined. This suggests it may be less suitable in patients at risk for thrombosis for different reasons. The uptake of (heavier) strontium in place of calcium into bone matrix results in a substantial and disproportionate increase in bone mineral density as measured on DXA scanning, making further followup of bone density by this method harder to interpret for strontium treated patients.
Strontium, no matter what the form, must be water-soluble and ionized in the stomach acid. Stontium is then protein-bound for transport from the intestinal tract into the blood stream. Unlike drugs like sodium alendronate (Fosamax), strontium doesn't inhibit bone recycling and, in fact, may produce stronger bones.
Strontium must not be taken with food or calcium-containing preparations as calcium competes with strontium during uptake. However, it's essential that calcium, magnesium, and vitamin D in theraputic amounts must be taken daily, but not at the same time as strontium. Strontium should be taken on an empty stomach at night.
- Hormone replacement
Estrogen replacement therapy remains a good treatment for prevention of osteoporosis but, at this time, is not recommended unless there are other indications for its use as well. There is uncertainty and controversy about whether estrogen should be recommended in women in the first decade after the menopause.
In hypogonadal men testosterone has been shown to give improvement in bone quantity and quality, there are no studies of the effects on fractures or in men with a normal testosterone level.- Selective estrogen receptor modulator (SERM)
Nutrition
- Calcium
Calcium is required to support bone growth, bone healing and maintain bone strength and is one aspect of treatment for osteoporosis. Recommendations for calcium intake vary depending country and age; for individuals at higher risk of osteoporosis (after fifty years of age) the amount recommended by US health agencies is 1,200 mg per day. Calcium supplements can be used to increase dietary intake, and absorption is optimized through taking in several small (500 mg or less) doses throughout the day. The role of calcium in preventing and treating osteoporosis is unclear - some populations with extremely low calcium intake also have extremely low rates of bone fracture, and others with high rates of calcium intake through milk and milk products have higher rates of bone fracture. Other factors, such as protein, salt and vitamin D intake, exercise and exposure to sunlight, can all influence bone mineralization, making calcium intake one factor among many in the development of osteoporosis.
A meta-analysis of randomized controlled trials involving calcium and calcium plus vitamin D supported the use of high levels of calcium (1,200 mg or more) and vitamin D (800 IU or more), though outcomes varied depending on which measure was used to assess bone health (rates of fracture versus rates of bone loss). The meta-analysis, along with another study, also supported much better outcomes for patients with high compliance to the treatment protocol.In contrast, despite earlier reports in improved high density lipoprotein (HDL, "good cholesterol") in calcium supplementation, a possible increase in the rate of myocardial infarction (heart attack) was found in a study. If confirmed, this would indicate that calcium supplementation in women otherwise at low risk of fracture may cause more harm than good.
- Vitamin D
Exercise
Exercise combined with other pharmacological treatments such as hormone replacement therapy (HRT) has been shown to increases bone mineral density (BMD) more than HRT alone. Additional benefits for osteoporotic patients other than BMD increase include improvements in balance, gait, and a reduction in risk of falls.Osteoarthritis
Osteoarthritis (OA) is a clinical syndrome in which low-grade inflammation results in pain in the joints, caused by abnormal wearing of the cartilage that covers and acts as a cushion inside joints and destruction or decrease of synovial fluid that lubricates those joints. As the bone surfaces become less well protected by cartilage, the patient experiences pain upon weight bearing, including walking and standing. Due to decreased movement because of the pain, regional muscles may atrophy, and ligaments may become more lax. OA is the most common form of arthritis.
A common misconception is that OA is due solely to wear and tear, due to the fact that OA typically is not present in younger people. However, while age is correlated with OA incidence, this merely illustrates that OA is a process that takes time to develop. There is usually an underlying cause for OA, in which case it is described as secondary OA. If no underlying cause can be identified it is described as primary OA. "Degenerative arthritis" is often used as a synonym for OA, but the latter involves both degenerative and regenerative changes.
Signs and symptoms
The main symptom is acute pain, causing loss of ability and often stiffness. "Pain" is generally described as a sharp ache, or a burning sensation in the associated muscles and tendons. OA can cause a crackling noise when the affected joint is moved or touched, and patients may experience muscle spasm and contractions in the tendons. Occasionally, the joints may also be filled with fluid. Humid weather increases the pain in many patients.
OA commonly affects the hips, feet, spine, and the large weight bearing joints, such as the hips and knees, although in theory, any joint in the body can be affected. As OA progresses, the affected joints appear larger, are stiff and painful, and usually feel worse, the more they are used throughout the day, thus distinguishing it from rheumatoid arthritis.
In smaller joints, such as at the fingers, hard bony enlargements, called Heberden's nodes (on the distal interphalangeal joints) and/or Bouchard's nodes (on the proximal interphalangeal joints), may form, and though they are not necessarily painful, they do limit the movement of the fingers significantly. OA at the toes leads to the formation of bunions, rendering them red or swollen.
OA is the most common cause of water on the knee, an accumulation of excess fluid in or around the knee joint.
Causes
Although it commonly arises from trauma, osteoarthritis often affects multiple members of the same family, suggesting that there is hereditary susceptibility to this condition. A number of studies have shown that there is a greater prevalence of the disease between siblings and especially identical twins, indicating a hereditary basis. Up to 60% of OA cases are thought to result from genetic factors. Researchers are also investigating the possibility of allergies, infections, or fungi as a cause.
Two types
Primary
This type of OA is a chronic degenerative disorder related to but not caused by aging, as there are people well into their nineties who have no clinical or functional signs of the disease. As a person ages, the water content of the cartilage decreases due to a reduced proteoglycan content, thus causing the cartilage to be less resilient. Without the protective effects of the proteoglycans, the collagen fibers of the cartilage can become susceptible to degradation and thus exacerbate the degeneration. Inflammation of the surrounding joint capsule can also occur, though often mild (compared to that which occurs in rheumatoid arthritis). This can happen as breakdown products from the cartilage are released into the synovial space, and the cells lining the joint attempt to remove them. New bone outgrowths, called "spurs" or osteophytes, can form on the margins of the joints, possibly in an attempt to improve the congruence of the articular cartilage surfaces. These bone changes, together with the inflammation, can be both painful and debilitating.
(Primary OA in the left knee of an elderly female.)
Secondary
This type of OA is caused by other factors but the resulting pathology is the same as for primary OA:
- Congenital disorders, such as:
- Congenital hip luxation
- People with abnormally-formed joints (e.g. hip dysplasia (human)) are more vulnerable to OA, as added stress is specifically placed on the joints whenever they move. [However, recent studies have shown that double-jointedness may actually protect the fingers and hand from osteoarthritis.]
- Cracking joints—the evidence is weak at best that this has any connection to arthritis.
- Diabetes.
- Inflammatory diseases (such as Perthes' disease), (Lyme disease), and all chronic forms of arthritis (e.g. costochondritis, gout, and rheumatoid arthritis). In gout, uric acid crystals cause the cartilage to degenerate at a faster pace.
- Injury to joints, as a result of an accident.
- A joint infection, e.g. from an injury.
- Hormonal disorders.
- Ligamentous deterioration or instability may be a factor.
- Obesity. Obesity puts added weight on the joints, especially the knees.
- Sports injuries, or similar injuries from exercise or work. Certain sports, such as running or football, put undue pressure on the knee joints. Injuries resulting in broken ligaments can lead to instability of the joint and over time to wear on the cartilage and eventually osteoarthritis.
- Pregnancy
- Alkaptonuria
- Hemochromatosis and Wilson's disease
Diagnosis
Diagnosis is normally done through x-rays. This is possible because loss of cartilage, subchondral ("below cartilage") sclerosis, subchondral cysts from synovial fluid entering small microfractures under pressure, narrowing of the joint space between the articulating bones, and bone spur formation (osteophytes) - from increased bone turnover in this inflammatory condition, show up clearly on x-rays.
With MRI (magnetic resonance imaging), arthrocentesis and arthroscopy, diagnosis can be made by a careful study of the duration, location, the character of the joint symptoms, and the appearance of the joints themselves. As yet, there are no methods available to detect OA in its early and potentially treatable stages.
Radiographic diagnosis results in diagnosis of a fracture within a joint, which is not to be confused with osteoarthritis which is a degenerative pathology affecting a high incidence of distal phalangeal joints of female patients.Treatment
Generally speaking, the process of clinically detectable osteoarthritis is irreversible, and typical treatment consists of medication or other interventions that can reduce the pain of OA and thereby improve the function of the joint.
Conservative care
No matter the severity or location of OA, conservative measures such as weight control, appropriate rest and exercise, and the use of mechanical support devices are usually beneficial. In OA of the knees, knee braces, a cane, or a walker can be helpful for walking and support. Regular exercise, if possible, in the form of walking or swimming, is encouraged. Applying local heat before, and cold packs after exercise, can help relieve pain and inflammation, as can relaxation techniques. Heat — often moist heat — eases inflammation and swelling, and may improve circulation, which has a healing effect on the local area.Medical treatment
Medical treatment includes NSAIDs, local injections of glucocorticoid or hyaluronan, and in severe cases, with joint replacement surgery. There has been no cure for OA, as cartilage has not been induced to regenerate. However, if OA is caused by cartilage damage (for example as a result of an injury) Autologous Chondrocyte Implantation may be a possible treatment. Clinical trials employing tissue-engineering methods have demonstrated regeneration of cartilage in damaged knees, including those that had progressed to osteoarthritis.
Dietary
Supplements that are useful for treating OA include:
Glucosamine
A molecule derived from glucosamine is used by the body to make some of the components of cartilage and synovial fluid. Supplemental glucosamine may improve symptoms of OA and delay its progression. However, a large study suggests that glucosamine is not effective in treating OA of the knee. A subsequent meta-analysis that includes this trial concluded that glucosamine hydrochloride is not effective and that the effect of glucosamine sulfate is uncertain.
Along with glucosamine, chondroitin sulfate has become a widely used dietary supplement for treatment of osteoarthritis. A meta-analysis of randomized controlled trials found no benefit from chondroitin.
Other supplements
- Omega-3 fatty acid,a vitamin supplement comprised of important oils derived from fish.
- Boswellia, an herbal supplement known in Ayurvedic medicine. It is widely available in health food stores and online.
- Bromelain, a protease enzymes extracted from the plant family Bromeliaceae, blocks some proinflammatory metabolites.
- Antioxidants, including vitamins C and E in both foods and supplements, provide pain relief from OA.
- Hydrolyzed collagen (hydrolysate) (a gelatin product) may also prove beneficial in the relief of OA symptoms, as substantiated in a German study by Beuker F. et al. and Seeligmuller et al. In their 6-month placebo-controlled study of 100 elderly patients, the verum group showed significant improvement in joint mobility.
- Ginger (rhizome) extract - has improved knee symptoms moderately.
- Selenium deficiency has been correlated with a higher risk and severity of OA
- Vitamin D deficiency has been reported in patients with OA, and supplementation with Vitamin D3 is recommended for pain relief.
- Bone Morphogenetic Protein 6 (BMP-6) has recently been shown to have a functional role in the maintenance of joint integrity and is now being produced in an orally ingested form.
Other nutritional changes shown to aid in the treatment of OA include decreasing saturated fat intake and using a low energy diet to decrease body fat. Lifestyle change may be needed for effective symptomatic relief, especially for knee OA.
Specific medications
Paracetamol
A mild pain reliever may be sufficiently efficacious. Paracetamol (tylenol/acetaminophen), is commonly used to treat the pain from OA, although unlike NSAIDs, acetaminophen does not treat the inflammation. A randomized controlled trial comparing paracetamol with ibuprofen in x-ray-proven mild to moderate osteoarthritis of the hip or knee found equal benefit. However, acetaminophen at a dose of 4 grams per day can increase liver function tests.
Non-steroidal anti-inflammatory drugs
In more severe cases, non-steroidal anti-inflammatory drugs (NSAID) may reduce both the pain and inflammation; they all act by inhibiting the formation of prostaglandins, which play a central role in inflammation and pain. Most prominent drugs in the class include diclofenac, ibuprofen, naproxen and ketoprofen. High oral drug doses are often required. However, diclofenac has been found to cause damage to the articular cartilage. Even more importantly all systemic NSAIDs are rather taxing on the gastrointestinal tract, and may cause stomach upset, cramping, diarrhea, and peptic ulcer. Such systemic adverse side effects are normally not observed when using NSAIDs topically, that is, on the skin around the target area. The typically weak and/or short-lived therapeutic effect of such topical treatments may be improved by using the drug in more modern formulations, including or ketoprofen associated with the Transfersome carriers or diclofenac in DMSO solution.
Surgery
If the above management is ineffective, joint replacement surgery may be required. Individuals with very painful OA joints may require surgery such as fragment removal, repositioning bones, or fusing bone to increase stability and reduce pain.
Over the last years, a series of articular cartilage repair procedures have managed to effectively treat articular cartilage damage in the knee. These articular cartilage repair procedures are largely believed to at least slow down the degeneration of the joint compared to untreated cases.
Other approaches:
Acupuncture
A meta-analysis of randomized controlled trials of acupuncture for knee osteoarthritis concluded "clinically relevant benefits, some of which may be due to placebo.
Low level laser therapy
Low level laser therapy is a light wave based treatment that may reduce pain. The treatment is painless, inexpensive and without risks or side effects. Unfortunately, it may not actually have any real benefits.
Osteopathy
Osteopathy is a form of physical therapy. Osteopathic treatment focuses on reducing pain, easing swelling and improving the mobility and range of joint movements. Recent research has shown benefits of resistance therapy for patients with knee osteoarthritis.
Prolotherapy
Prolotherapy (proliferative therapy) is the injection of an irritant substance (such as dextrose) to create an acute inflammatory reaction. It is claimed to strengthen and heal damaged tissues including ligaments, tendons and cartilage as part of this reaction. The injection is painful and may cause an increase in pain for a few days afterwards. The only other significant risk is the rare possibility of infection.
Osteogenesis imperfecta
As a genetic disorder, OI is an autosomal dominant defect, most people with OI receive it from a parent but it can also be an individual mutation.
Types
There are eight different types of OI, Type I being the most common, though the symptoms range from person to person.
Type I
Collagen is of normal quality but is produced in insufficient quantities:
- Bones fracture easily
- Slight spinal curvature
- Loose joints
- Poor muscle tone
- Discolouration of the sclera (whites of the eyes), usually giving them a blue-gray color. The blue-gray color of the sclera is due to the underlying choroidal veins which show through. This is due to the sclera being thinner than normal because of the defective Type I Collagen not forming correctly.
- Early loss of hearing in some children
- Slight protrusion of the eyes
IA and IB are defined to be distinguished by the absence/presence of dentinogenesis imperfecta (characterized by opalescent teeth; absent in IA, present in IB). Life expectancy is slightly reduced compared to the general population due to the possibility of fatal bone fractures and complications related to OI Type I such as Basilar Invagination.
Type II
Collagen is not of a sufficient quality or quantity
- Most cases die within the first year of life due to respiratory failure or intracerebral hemorrhage
- Severe respiratory problems due to underdeveloped lungs
- Severe bone deformity and small stature
Type II can be further subclassified into groups A, B, C, which are distinguished by radiographic evaluation of the long bones and ribs. Type IIA demonstrates broad and short long bones with broad and beaded ribs. Type IIB demonstrates broad and short long bones with thin ribs that have little or no beading. Type IIC demonstrates thin and longer long bones with thin and beaded ribs.
Type III
Collagen quantity is sufficient but is not of a high enough quality
- Bones fracture easily, sometimes even before birth
- Bone deformity, often severe
- Respiratory problems possible
- Short stature, spinal curvature and sometimes barrel-shaped rib cage
- Loose joints
- Poor muscle tone in arms and legs
- Discolouration of the sclera (the 'whites' of the eyes)
- Early loss of hearing possible
Type III is distinguished among the other classifications as being the "Progressive Deforming" type, wherein a neonate presents with mild symptoms at birth and develops the aforementioned symptoms throughout life. Lifespan may be normal, though with severe physical handicapping.
Type IV
Collagen quantity is sufficient but is not of a high enough quality
- Bones fracture easily, especially before puberty
- Short stature, spinal curvature and barrel-shaped rib cage
- Bone deformity is mild to moderate
- Early loss of hearing
Similar to Type I, Type IV can be further subclassified into types IVA and IVB characterized by absence (IVA) or presence (IVB) of dentinogenesis imperfecta.
Type V
Same clinical features as Type IV. Distinguished histologically by "mesh-like" bone appearance. Further characterized by the "V Triad" consisting of a) radio-opaque band adjacent to growth plates, b) hypertrophic calluses at fracture sites, and c) calcification of the radio-ulnar interosseous membrane.
OI Type V leads to calcification of the membrane between the two forearm bones, making it difficult to turn the wrist. Another symptom is abnormally large amounts of repair tissue (hyperplasic callus) at the site of fractures. At the present time, the cause for Type V is unknown, though doctors have determined that it is inherited.
Type VI
Same clinical features as Type IV. Distinguished histologically by "fish-scale" bone appearance.
Type VII
- In 2005 a recessive form called "Type VII" was discovered.
Treatment
At present there is no cure for OI. Treatments are aimed at increasing overall bone strength to prevent fracture and maintain mobility.
There have been many clinical trials done with the drug, Fosamax, a drug used to treat women experiencing brittleness of bones due to osteoporosis. The FDA will not approve Fosamax as a treatment for OI because long term effects of the drug have not been studied.
Bone infections are treated as and when they occur with the appropriate antibiotics and antiseptics.
Physiotherapy
Physiotherapy used to strengthen muscles and improve motility in a gentle manner, while minimizing the risk of fracture. This often involves hydrotherapy and the use of support cushions to improve posture. Individuals are encouraged to change positions regularly throughout the day in order to balance the muscles which are being used and the bones which are under pressure.
Physical aids
With adaptive equipment such as crutches, splints, grabbing arms, and/or modifications to the home many individuals with OI can obtain a significant degree of autonomy.
Bisphosphonates
Bisphosphonates (BPs), particularly those containing nitrogen, are being increasingly administered to increase bone mass and reduce the incidence of fracture. BPs can be dosed orally (e.g. alendronate) or by intravenous injection/infusion (e.g. pamidronate, zoledronic acid).
BP therapy is being used increasingly for the treatment of OI. It has proven efficiency in reducing fracture rates in children, however only a trend towards decreased fracture was seen in a small randomized study in adults. While decreasing fracture rates, there is some concern that prolonged BP treatment may delay the healing of OI fractures.
The therapy is repeated every three to six months, and lasts for the life of the patient. Common side effects include bone pain, low calcium levels, nausea, and dizziness. According to recent results, extended periods of pamidrinate, (i.e.;6 years) can actually weaken bones, so patients are recommended to get bone densities every 6 months-1 year, to monitor bone strength.
Surgery
Metal rods can be surgically inserted in the long bones to improve strength. The placement of stainless steel rods into the intramedullary canals of the long bones to stabilize and strengthen them. His treatment proved extremely useful in the rehabilitation and prevention of fractures; it was adopted throughout the world and still forms the basis for orthopedic treatment of OI.
Spinal fusion can also be performed to correct scoliosis, although the inherent bone fragility makes this operation more complex in OI patients. Surgery for basilar impressions can be carried out if pressure being exerted on the spinal cord and brain stem is causing neurological problems.
Paget's disease
Paget's disease, known as osteitis deformans, is a chronic disorder that typically results in enlarged and deformed bones. It is named after Sir James Paget, the British surgeon who first described this disease. The excessive breakdown and formation of bone tissue that occurs with Paget's disease can cause bone to weaken, resulting in bone pain, arthritis, deformities, and fractures. Paget's disease may be caused by a slow virus infection such as measles, Canine distemper virus, and respiratory syncytial virus, present for many years before symptoms appear.Paget's disease is rarely diagnosed in people less than 40 years of age. Men are more commonly affected than women.
Symptoms
Symptoms can include:
- Bone pain is the most common symptom. Bone pain can occur in any bone affected by Paget's disease. It often localizes to areas adjacent to the joints.
- Headaches and hearing loss may occur when Paget's disease affects the skull.
- Pressure on nerves may occur when Paget's disease affects the skull or spine.
- Somnolence (drowsiness) due to vascular steal syndrome of the skull.
- Paralysis due to vascular steal syndrome of the vertebrae.
- Increased head size, bowing of limb, or curvature of spine may occur in advanced cases.
- Hip pain may occur when Paget's disease affects the pelvis or thighbone.
- Damage to joint cartilage may lead to arthritis.
- Teeth may spread abnormally.
- Fractures.
Diagnosis
Paget's disease may be diagnosed using one or more of the following tests:
- Pagetic bone has a characteristic appearance on x-rays. A skeletal survey is therefore indicated.
- An elevated level of alkaline phosphatase in the blood in combination with normal calcium, phosphate, and aminotransferase levels in an elderly patient are suggestive of Paget's disease.
- Bone scans are useful in determining the extent and activity of the condition. If a bone scan suggests Paget's disease, the affected bone(s) should be x-rayed to confirm the diagnosis.
Other medical conditions
Paget's disease may lead to other medical conditions, including:
- Arthritis: Long bones in the leg may bow, distorting alignment and increasing pressure on nearby joints. Pagetic bone may enlarge, causing joint surfaces to undergo excessive wear and tear. In these cases, pain may be due to a combination of Paget's disease and osteoarthritis.
- Loss of hearing in one or both ears may occur when Paget's disease affects the skull and the bone that surrounds the inner ear. Treating the Paget's disease may slow or stop hearing loss. Hearing aids may also help. It is believed by some that the disease was responsible for Beethoven's deafness.
- Cardiovascular disease: In severe Paget's disease, the heart works harder to pump blood to affected bones. High-output congestive failure may rarely occur.
- Kidney stones are somewhat more common in patients with Paget's disease.
- Nervous system: Pagetic bone can cause pressure on the brain, spinal cord, or nerves, and reduced blood flow to the brain and spinal cord.
- Sarcoma: Rarely, Paget's disease is associated with the development of a malignant tumor of bone. When there is a sudden onset or worsening of pain, sarcoma should be considered.
- When Paget's disease affects the facial bones, the teeth may become loose. Disturbance in chewing may occur.
- Rarely, when the skull is involved, the nerves to the eye may be affected, causing some loss of vision.
Treatment
Types of physicians
The following types of medical specialists are generally knowledgeable about treating Paget's disease.
- Endocrinologists -- Internists who specialize in hormonal and metabolic disorders.
- Rheumatologists -- Internists who specialize in joint and muscle disorders.
- Specialists -- Orthopedic surgeons, neurologists, and otolaryngologists (physicians who specialize in ear, nose, and throat disorders) may be called upon to evaluate specialized symptoms.
Drug therapy
The goal of treatment is to relieve bone pain and prevent the progression of the disease. The U.S. Food and Drug Administration has approved the following treatments for Paget's disease:
Bisphosphonates
In general, the most commonly prescribed are the three most potent bisphosphonates: Actonel, Fosamax and Aredia. Didronel and Skelid may be appropriate therapies for selected patients but are less commonly used. None of these drugs should be used by people with severe kidney disease.
- Didronel (etidronate disodium) -- Tablet; approved regimen is 200–400 mg once daily for 6 months; the higher dose (400 mg) is more commonly used; no food, beverages, or medications for 2 hours before and after taking; course should not exceed 6 months.
- Aredia (pamidronate disodium) -- Intravenous; approved regimen 30 mg infusion over 4 hours on 3 consecutive days; more commonly used regimen 60 mg over 2–4 hours for 2 or more days.
- Fosamax (alendronate sodium) -- Tablet; 40 mg once daily for 6 months; patients should wait at least 30 minutes after taking before eating any food, drinking anything other than tap water, taking any medication, or lying down.
- Skelid (tiludronate disodium) -- Tablet; 400 mg (two 200 mg tablets) once daily for 3 months; may be taken any time of day, as long as there is a period of 2 hours before and after resuming food, beverages, and medications.
- Actonel (risedronate sodium) -- Tablet; 30 mg once daily for 2 months; patients should wait at least 30 minutes after taking before eating any food, drinking anything other than tap water, taking any medication, or lying down.
Calcitonin
- Miacalcin is administered by injection; 50 to 100 units daily or 3 times per week for 6-18 months. Repeat courses can be given after brief rest periods. Miacalcin may be appropriate for certain patients but is seldom used. The nasal spray form of this drug is not approved for the treatment of Paget's disease.
Surgery
Medical therapy prior to surgery helps to decrease bleeding and other complications. Patients who are having surgery should discuss pre-treatment with their physician. There are generally three major complications of Paget's disease for which surgery may be recommended.
- Fractures -- Surgery may allow fractures to heal in better position.
- Severe degenerative arthritis -- If disability is severe and medication and physical therapy are no longer helpful, joint replacement of the hips and knees may be considered.
- Bone deformity -- Cutting and realignment of Pagetic bone (osteotomy) may help painful weight bearing joints, especially the knees.
Complications resulting from enlargement of the skull or spine may injure the nervous system. However, most neurologic symptoms, even those that are moderately severe, can be treated with medication and do not require neurosurgery.
Diet and Exercise
In general, patients with Paget's disease should receive 1000-1500 mg of calcium, adequate sunshine, and at least 400 units of vitamin D daily. This is especially important in patients being treated with bisphosphonates. Patients with a history of kidney stones should discuss calcium and vitamin D intake with their physician.
Exercise is very important in maintaining skeletal health, avoiding weight gain, and maintaining joint mobility. Since undue stress on affected bones should be avoided, patients should discuss any exercise program with their physician before beginning.
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