Thursday, February 10, 2011
Multiple Intelligences
Dr. Thomas Armstrong
The theory of multiple intelligences was developed in 1983 by Dr. Howard Gardner, professor of education at Harvard University. It suggests that the traditional notion of intelligence, based on I.Q. testing, is far too limited. Instead, Dr. Gardner proposes eight different intelligences to account for a broader range of human potential in children and adults. These intelligences are:
•Linguistic intelligence ("word smart")
•Logical-mathematical intelligence ("number/reasoning smart")
•Spatial intelligence ("picture smart")
•Bodily-Kinesthetic intelligence ("body smart")
•Musical intelligence ("music smart")
•Interpersonal intelligence ("people smart")
•Intrapersonal intelligence ("self smart")
•Naturalist intelligence ("nature smart")
Dr. Gardner says that our schools and culture focus most of their attention on linguistic and logical-mathematical intelligence. We esteem the highly articulate or logical people of our culture. However, Dr. Gardner says that we should also place equal attention on individuals who show gifts in the other intelligences: the artists, architects, musicians, naturalists, designers, dancers, therapists, entrepreneurs, and others who enrich the world in which we live. Unfortunately, many children who have these gifts don’t receive much reinforcement for them in school. Many of these kids, in fact, end up being labeled "learning disabled," "ADD (attention deficit disorder," or simply underachievers, when their unique ways of thinking and learning aren’t addressed by a heavily linguistic or logical-mathematical classroom. The theory of multiple intelligences proposes a major transformation in the way our schools are run. It suggests that teachers be trained to present their lessons in a wide variety of ways using music, cooperative learning, art activities, role play, multimedia, field trips, inner reflection, and much more (see Multiple Intelligences in the Classroom). The good news is that the theory of multiple intelligences has grabbed the attention of many educators around the country, and hundreds of schools are currently using its philosophy to redesign the way it educates children. The bad news is that there are thousands of schools still out there that teach in the same old dull way, through dry lectures, and boring worksheets and textbooks. The challenge is to get this information out to many more teachers, school administrators, and others who work with children, so that each child has the opportunity to learn in ways harmonious with their unique minds (see In Their Own Way).
The theory of multiple intelligences also has strong implications for adult learning and development. Many adults find themselves in jobs that do not make optimal use of their most highly developed intelligences (for example, the highly bodily-kinesthetic individual who is stuck in a linguistic or logical desk-job when he or she would be much happier in a job where they could move around, such as a recreational leader, a forest ranger, or physical therapist). The theory of multiple intelligences gives adults a whole new way to look at their lives, examining potentials that they left behind in their childhood (such as a love for art or drama) but now have the opportunity to develop through courses, hobbies, or other programs of self-development (see 7 Kinds of Smart).
How to Teach or Learn Anything 8 Different Ways
One of the most remarkable features of the theory of multiple intelligences is how it provides eight different potential pathways to learning. If a teacher is having difficulty reaching a student in the more traditional linguistic or logical ways of instruction, the theory of multiple intelligences suggests several other ways in which the material might be presented to facilitate effective learning. Whether you are a kindergarten teacher, a graduate school instructor, or an adult learner seeking better ways of pursuing self-study on any subject of interest, the same basic guidelines apply. Whatever you are teaching or learning, see how you might connect it with
•words (linguistic intelligence)
•numbers or logic (logical-mathematical intelligence)
•pictures (spatial intelligence)
•music (musical intelligence)
•self-reflection (intrapersonal intelligence)
•a physical experience (bodily-kinesthetic intelligence)
•a social experience (interpersonal intelligence), and/or
•an experience in the natural world. (naturalist intelligence)
For example, if you’re teaching or learning about the law of supply and demand in economics, you might read about it (linguistic), study mathematical formulas that express it (logical-mathematical), examine a graphic chart that illustrates the principle (spatial), observe the law in the natural world (naturalist) or in the human world of commerce (interpersonal); examine the law in terms of your own body [e.g. when you supply your body with lots of food, the hunger demand goes down; when there's very little supply, your stomach's demand for food goes way up and you get hungry] (bodily-kinesthetic and intrapersonal); and/or write a song (or find an existing song) that demonstrates the law (perhaps Dylan's "Too Much of Nothing?").
You don’t have to teach or learn something in all eight ways, just see what the possibilities are, and then decide which particular pathways interest you the most, or seem to be the most effective teaching or learning tools. The theory of multiple intelligences is so intriguing because it expands our horizon of available teaching/learning tools beyond the conventional linguistic and logical methods used in most schools (e.g. lecture, textbooks, writing assignments, formulas, etc.). To get started, put the topic of whatever you’re interested in teaching or learning about in the center of a blank sheet of paper, and draw eight straight lines or "spokes" radiating out from this topic. Label each line with a different intelligence. Then start brainstorming ideas for teaching or learning that topic and write down ideas next to each intelligence (this is a spatial-linguistic approach of brainstorming; you might want to do this in other ways as well, using a tape-recorder, having a group brainstorming session, etc.). Have fun!
Tuesday, November 23, 2010
Life After Life
In Life After Life Raymond Moody investigates case studies of people who experienced “clinical death” and were subsequently revived.
This classic exploration of life after death started a revolution in popular attitudes about the afterlife and established Dr. Moody as the world’s leading authority in the field of near-death experiences.
The extraordinary stories presented here provide evidence that there is life after physical death, as Moody recounts the testimonies of those who have been to the “other side” and back — all bearing striking similarities of an overwelming positive nature.
These moving and inspiring accounts give us a glimpse of the peace and unconditional love that await us all.
Tuesday, November 2, 2010
What is Hyperbaric Oxygen
"Hyper" means an increase in the quantity or quality of something; "baric" means pressure. Combined with "oxygen," these two terms add up to one of the most exciting new developments in medicine: hyperbaric oxygen therapy (HBOT). Using pure oxygen under increased pressure, the body's natural ability to heal from traumas, diseases and other afflictions is enhanced - and in many cases, is accelerated.
A Brief History
HBOT has been in use since the mid-1800s. It began when an anesthesiologist postulated that by increasing the levels of oxygen in operating rooms, patients would be able to heal faster. Unfortunately, while there were some modest benefits, HBOT began to be touted as a universal cure-all, and more. It was promised to do everything from restoring men's hair to enlarging women's breasts - yet it failed to deliver. This was the start of the "bad press" that HBOT received, some of which carries on to this day. The more accepted uses of HBOT through most of this century have been in relation to saving the lives of SCUBA divers stricken with decompression sickness, or "the bends" (a potentially fatal condition, that occurs when the diver returns to the surface too quickly).
HBO Today
During modern HBOT, the patient breathes pure, 100% oxygen under increased atmospheric pressure. The air we normally breathe contains only 19-21% of this essential element; via HBOT, the concentration of pure oxygen dissolved into the bloodstream is dramatically increased (up to 2,000%), with virtually no energy expenditure. In addition to the blood, all body fluids - including the vital lymph and cerebrospinal fluids - are infused with the healing benefits of this molecular oxygen. This oxygen can then: (a) reach bone and tissue which are inaccessible to red blood cells, (b) enhance white blood cell function, and (c) promote the formation of new capillary and peripheral blood vessels. The result is increased infection control, and faster healing of a wide range of conditions.
HBOT requires a prescription, and is approved by the American Medical Association (AMA), the Food & Drug Administration (FDA), and Medicare. It is typically used as part of an overall medical treatment plan, for various diseases or injuries associated with hypoxia, or a lack of oxygen on a cellular level. It is at this cellular tissue level where all life takes place. While HBOT is sometimes used as a primary emergency treatment, it is more often applied as a cost-effective adjunctive or enhancement therapy.
When administered by accredited physicians and highly trained technicians, HBOT is extremely safe and effective. New profit-oriented centers, however, have been increasing in number, and often do not have trained technicians or medical physicians on site. These centers should be avoided. While HBOT's popularity is increasing in the United States, it is used much more extensively in Europe and the Orient. In fact, in some areas of Italy, a physician may actually have his or her license revoked for neglecting to utilize HBOT!
Oxygen, The Basis of Life
Human beings can survive without food for weeks, and without water for days - but only minutes without oxygen. Oxygen is the basis of life. Used appropriately, it can mean the difference between life and death, coma and mental alertness, paralysis and movement, illness and health.
It has long been understood that healing cannot be achieved without sufficient oxygen levels in the tissues, where most illnesses and injuries occur and often linger. Hyperbaric oxygen therapy can provide this oxygen, naturally and virtually risk free.
"The positive powers of hyperbaric oxygen are really a modification of God's gift to man." - Dr. Richard A. Neubauer, M.D., Medical Director, Ocean Hyperbaric Neurologic Center
How is HBOT Administered?
A patient undergoing HBOT spends a prescribed amount of time in one of several types of enclosed delivery units: (1) Monoplace, which are cylindrical, body-length chambers, or (2) Multi-Person Chambers, which can accommodate up to 36 adults. In each type of unit, pure oxygen is administered while atmospheric pressure is increased, and controlled under closely monitored conditions.
HBOT dosage, which is prescribed by the attending physician for each patient's particular needs, consists of the following measures: (1) Pressure (one to three atmospheres absolute), (2) Duration of each treatment (60-120 minutes), and (3) Frequency of treatments.
Oxygen inhalation treatments are non-invasive and painless, and side effects are rare and minimal. Fewer than 5% report slight discomfort from ear pressure, similar to that experienced during air travel. During treatment, the patient can rest comfortably, listen to music, or watch television.
There is no recovery period with HBOT, so patients can resume their daily activities almost immediately. As overnight stays are not required, all treatment is on an outpatient basis.
How is the Effectiveness Measured
Most people are familiar with MRI (magnetic resonance imaging) and CAT (computerized axial tomography) scans, which are superb at depicting structural anatomy. However, neither is designed for or is capable of measuring the brain activity.
A specialized tool, the SPECT (single photon, emission-computed tomography) scan, has been proven effective in this task - and it is the primary tool OHNC employs to objectively measure the effectiveness of HBOT on patients. Specifically, SPECT scanning show actual brain functioning, in visual terms. It can help doctors to see how blood is flowing through different areas within a patient's brain, visualize brain metabolism, and make a better diagnosis of his/her condition.
During SPECT scanning, a radioactive "tracer" agent is injected into a vein in the hand or arm. The tracer localizes in an area of the brain where it can then be "photographed." Only viable tissue can absorb the tracer, which breaks down harmlessly within a few hours. A special gamma camera aimed at the head pinpoints the position and energy of photons emitted, as the tracer disintegrates. As inert (dead) cells do not absorb the tracer at all, SPECT scanning can distinguish between living and dead (necrotic) tissue. SPECT scanning can also identify between recoverable brain cells (referred to as sleeping cells, idling neurons, or the ischemic penumbra). If the living brain tissue is determined to be recoverable, or in an electrically inactive or idling state, HBOT may substantially and/or permanently revive them.
Friday, October 15, 2010
WHAT IS ABR?
ABR stands for Advanced BioMechanical Rehabilitation.
ABR is a unique biomechanically based rehabilitation approach for children and young adults with brain injury that brings predictable recovery of musculoskeletal structure and motor functions.
ABR is the method of structural correction of musculoskeletal deformities. It is a hands-on method performed by the parents who learn the ABR technique and receive individual prescription of applications from the ABR professional staff.
• ABR is a method that re-builds even the most severely distorted musculoskeletal structure
• ABR redefines "rehabilitation" – ABR improves musculoskeletal structure so significantly that normal motor functions recover spontaneously, making special training and management for "motor disabled" unnecessary.
We use no pharmaceuticals, no electrical instruments and no surgeries - ABR is a hands-on method of manual applications to the child's body, based purely on biomechanical principles.
ABR biomechanical reconstruction of the musculoskeletal system follows the path of normal motor development - starting from the neck and trunk and later descending to the periphery (arms and legs).
ABR provides planned progress of the musculoskeletal structure and function through predictable stages. Results are predicted in numbers of hours of exercises done and changes in the alignment, mobility, size, tone and strength of the child's body – specifically in the chest, abdomen and the pelvis.
ABR is more than just a new rehabilitation method; ABR is a comprehensive philosophy of the child's recovery. The cornerstones of the ABR philosophy are fundamental biomechanical principles of the human body's growth and development. Respectively ABR takes bio-electrical and bio-chemical factors into account only through their biomechanical manifestations.
This biomechanical approach allows us to have exact guidance for every single movement. Every single ABR application to a child's body is precisely calculated and adjusted for each individual patient.
ABR opens a new dimension in the treatment of the mildest to the most severe motor dysfunctions in the sense that it gradually brings changes to the mechanical and electrical structure of the muscles, thus allowing spontaneous developments of motor function.
ABR emphasises 3 essential concepts in describing the functional role of smooth muscles or internal myofascia with respect to the body's biomechanical structure:
• Hydraulic (or hydraulic/pneumatic) skeleton. This concept highlights the structural roll of the smooth muscles - in comparison to the classical definition of the «hard», bony skeleton as being that which carries the sole responsibility for the body's structure. Smooth muscles and other structures of internal myofascia are the membranes that maintain the shape of the body, with an effect similar to the whitish-coloured membranes within a grapefruit. These tissues envelop and ensheath the major bodily cavities (cranial, thoracic, abdominal etc.), enwrap each of the bodies individual organs (lungs, liver, stomach etc.) and sustain the smallest compartments within each of the bodies system. It is the hydraulic quality of the smooth muscles that administers inner strength and form. The term 'hydraulic skeleton' is used most frequently by ABR for convenience.
* A normal "hydraulic skeleton" provides normal alignment of the bony skeleton and normality of skeletal muscles - allowing normal motor function.
* Am abnormal, weakened "hydraulic skeleton" results in the collapse of the musculoskeletal system and impossibility of motor function.
• Visceral skeleton (visceral core) is the name used for the observation and discovery that the structures comprising the hydraulic skeleton serve as the body's core and define the mechanical foundation of the internal organs for the body's structure.
• Hydraulic/pneumatic capacity characterizes the "strength" and volume of the hydraulic/ pneumatic skeleton and the quality of «hydraulic support» that it gives to the musculoskeletal system.
The term pneumatic capacity summarizes several main characteristics:
• The level of pressure necessary for the development of sufficient internal volumes.
• The size of internal volumes.
• The level of strength of the myofascial membranes that is necessary to achieve the normal internal pressure/volume ratio. This is in turn required to sustain the challenges of gravity and of the external atmospheric pressure.
The objective of ABR is to restore proper tone to the smooth muscles/internal myofascia, which in a cascade effect restores proportions and alignment of the skeleton. During this process, the muscle tone is normalized and the arms and legs develop increasing muscle mass, normal range of mobility, and finally: sufficient strength - so that the children can develop their movements in normal spontaneous ways. All other approaches address the skeletal muscles directly. Nevertheless, such a direct approach proves to give limited results. ABR sees the direct approaches as the ones addressing the "tip of the iceberg". The underwater part is the smooth muscles.
What are the smooth muscles?
The smooth muscles are the ones that make up the internal organs, such as the liver, lungs, kidneys, intestines, etc.
Why address the smooth muscles?
ABR considers the smooth muscles as the "primary victims" of the brain injury. As their tone significantly drops, following brain injury, the secondary victims are the structures of musculoskeletal system, which distort as a result causing cerebral palsy as the disorder of posture and movement.
The total surface of the lining of internal organs is enormous in comparison to their respective volumes. For example the total active surface of the lungs is about 900 sq. feet (80 sq.m.) in relation to a volume that is approximately 3 litres. This huge surface is necessary for efficient metabolism. On the other hand, it seals the internal hydraulic volume that composes the walls that hold the shape of the bodily cavities.
ABR recognizes the importance of the strength of this lining made of smooth muscles (internal myofascia) for the proper development of a human body and, particularly of the musculoskeletal system, which is supported by this internal "hydraulic skeleton".
ABR considers the smooth muscles to be the primary victims of a brain injury. As their tone drops following a brain injury, the secondary victims are the structure of the musculo-skeletal system, which in turn collapses - changing the normal alignment of muscles and bones and shifting the distances between points of muscular attachments. Such shift of attachments in turn causes the skeletal muscles imbalance: i.e. spasticity and contractures. Finally, such muscular imbalance makes normal movements impossible. The resulting pathological diagnosis is cerebral palsy, as a disorder of posture and muscle control.
This is why ABR proposes a unique technique to administer kinetic input directly to smooth muscles.
ABR is the first and the only approach that recognizes the fundamental role of smooth muscles in the development of the musculoskeletal system.
Unfortunately, in traditional medicinal practice the internal organs are looked upon only from the viewpoint of chemical metabolism, while, on the other hand, the biomechanical approach does not normally focus on anything other than the classical skeleton comprised of «muscles and bones».
Strengthening of the smooth muscles induces gradual growth of internal pneumatic capacity, which in a cascade effect restores:
• volume, shape and strength of the neck and trunk,
• normal alignment of the joints of the limbs, eliminating spasticity and contractures,
• normal volume and then strength of weakened skeletal muscles,
• normal alignment of the shoulder girdle and arms as well as pelvis and legs - allowing normal "insertion" of arms and legs and thus making proper movements possible.
Moreover, restoration of muscular skeletal structure re-establishes normal metabolism (flow of blood and oxygen supply) of 'defective', atrophied muscles and, in addition, normal electrical ascending activity to the brain, opening wide the "back door" to function.
ABR targets the core structures of human body: the smooth muscles of internal organs. To be precise we target the entire complex of internal myofascia (mucosa, smooth muscles, serosa etc. and their sublevels).
• that are directly related to the quality of the general metabolism - regulating proper breathing, swallowing, digestion, evacuation, etc. and being responsible for the general health of a child.
• that we call the hydraulic skeleton of the human body - providing essential "hydraulic support" to the superficial structures of a "classically" defined musculoskeletal system (muscles, ligaments, bones etc.). ABR states that this "hydraulic skeleton" defines the proportions and the alignment of the bony skeleton and the quality of the skeletal muscles.
Normalization of the musculoskeletal system must come first.
• Nervous activity flowing from the muscular skeletal system «ascending» to the brain plays a vital role in the development of normal signals emanating from the central nervous system «descending» to the musculoskeletal system.
• When the musculoskeletal structure is profoundly distorted, any training is narrowed down to quite a limited scope: "trying to put poor structure to some better functional use".
• Without normalization of the musculoskeletal structure, any functional progress of a brain-injured person would always remain significantly limited and largely unpredictable.
Bio-electrical plasticity of the brain
ABR shows evidence that irreversibility of initial structural brain damage does not make motor function recovery intrinsically hopeless. The brain damage is not a critical obstacle for successful biomechanical reconstruction, as long as the musculoskeletal system is addressed in an effective biomechanical manner. There is no critical need to «repair» the brain before initiating the restoration of the biomechanical system.
The brain damage is not a critical obstacle for successful bio-mechanical reconstruction as long as the musculoskeletal system is addressed in a bio-mechanical proper way. There is no critical need to "repair" the brain first.
ABR approach makes restoring the mechanical structure of the musculoskeletal system its first and primary goal. Why? We support the following concept:
Mechanical transformation of the musculoskeletal elements (muscles, joints, etc.) by ABR automatically changes the parameters of their electrical charge. This respectively, changes the electrical activity of these elements (for instance, the skeletal muscles) and then translates into a transformation of the ascending signals sent to the brain, which in turn creates an adequate base for forthcoming descending signals to the muscles.
ABR states that even an injured brain still has enough reserves to rearrange its electrical connections in order to integrate biomechanical structural improvements of the musculoskeletal system, provided that the structural improvements are significant enough.
ABR in a nutshell
Everyone believes that a CP child has poor functions because his/her brain is too damaged to be able to control normal movements. Respectively CP children are considered incurable because the brain damage is irreversible.
ABR has a different philosophy. We believe that even the badly injured brain has enough electrical plasticity to allow control of normal motor functions, however, for this plasticity to become activated, a child's musculoskeletal structure has to be improved to a sufficient level - to the so-called plasticity threshold.
Existing treatment methods fail to achieve recovery of motor functions. This failure is then blamed on the brain damage.
We believe that the answer lies differently. Existing treatment methods fail, not because of insufficient «reserves» of the damaged brain, but because they fail to provide sufficient structural improvements to the musculoskeletal system. As a result, the injured brain has "too little of a good musculoskeletal structure to work with" and cannot utilize its remaining plasticity (reserves) for control of motor functions.
Musculoskeletal structure reconstruction must come first!
ABR approach makes restoring the mechanical structure of the musculoskeletal system its first and primary goal.
• full range of movements (ex.? the head being able to move unrestrictedly in all directions)
• proper alignment (ex.: legs in respect to the pelvis; arms in respect to the shoulders, etc.)
• muscular mechanical response (proper muscular balance)
In turn, «quality of mechanical performance» requires «bio-mechanical capacity» of the musculoskeletal system, which implies:
• Proper volume, tone and strength of the skeletal muscles
• Proper volume, mobility and alignment of the joints
• Equilibrium of strength and length between reciprocal muscular groups (ex: biceps, triceps)
• Adequate proportions between size and strength of centre (head, neck, trunk) and periphery (arms and legs)
• Cascade of muscular interactions (centre to periphery)
ABR re-establishes - unit-by-unit - proper skeletal and muscular structure to permit proper unrestricted performance of movement.
Saturday, August 22, 2009
About CMV
Cytomegalovirus, or CMV, is found universally throughout all geographic locations and socioeconomic groups, and has infected between 50% and 85% of adults in the United States by 40 years of age. CMV is also the virus most frequently transmitted to a developing child before birth.
Each year in the United States approximately 30,000 children are born with congenital CMV causing an estimated 400 deaths and leaving approximately 8,000 children with permanent disabilities such as hearing or vision loss, or mental retardation. It is estimated that 1 in every 150 children are born infected with congenital CMV. More children are adversely affected by congenital CMV than by several better-known childhood diseases or syndromes such as Down Syndrome, Fetal Alcohol Syndrome and neural tube defects.
High-risk Groups
CMV infection is important to certain high-risk groups, especially pregnant women. Major areas of concern are the risk of infection to the unborn baby during pregnancy and the risk of infection to pregnant women who work with children, including mothers who have already had children.
Approximately 40-50% of college-educated, white women of childbearing age in this country currently have no protective immunity to CMV, making this group most vulnerable to contracting a primary CMV infection while pregnant and passing the disease on to an unborn baby. Young, American women of the middle and upper socio-economic classes have lower rates of natural CMV immunity because many have had minimal contact with small children prior to becoming pregnant themselves.
Transmission
CMV can be transmitted to the unborn child of a mother with a primary or a recurrent CMV infection. When a baby catches CMV prior to birth it is known as a congenital CMV infection. Approximately 90% of all infants who are infected with CMV prior to delivery are born without symptoms of the virus; however, the remaining 10% will have varying degrees of abnormalities.
Transmission of the virus is often preventable because it is most often transmitted through infected bodily fluids that come in contact with hands and then are absorbed through the nose or mouth of a susceptible person. Therefore, care should be taken when handling infants, small children and items like diapers. Simple hand washing with soap and water is effective in removing the virus from the hands.
Effects on Pregnancy
The incidence of primary (or first) CMV infection in pregnant women in the United States varies from 1% to 3%. When infected with CMV, most women have no symptoms and very few have symptoms resembling mononucleosis. It is their developing unborn babies that may be at risk for congenital CMV disease. CMV remains the most common cause of congenital (meaning from birth) viral infection in the United States. For infants who are infected by their mothers during pregnancy, two potential problems exist:
- Generalized infection may occur in the infant, and symptoms may range from moderate enlargement of the liver and spleen (with jaundice) to fatal illness. With supportive treatment, most infants with CMV disease usually survive. However, 80% to 90% will have complications within the first few years of life that may include hearing loss, vision impairment, and varying degrees of mental retardation.
- Another 5% to 10% of infants who are infected but without symptoms at birth will subsequently have varying degrees of hearing and mental or coordination problems.
Wednesday, July 30, 2008
Understanding Osteopetrosis
1150 17th St., NW, Suite 500, Washington DC 20036
202-223-0344 or 800-624-BONE
TTY (202) 466-4315
E-Mail: orbdnrc@nof.org
One of the less common metabolic bone disorders is osteopetrosis. Osteopetrosis is an inherited disease caused by a defect in bone resorption--the process in which old bone is broken down and removed so that new bone can be added to the skeleton. Osteoclasts are the cells responsible for bone resorption. In osteopetrosis the osteoclasts do not perform normally, either because there are too few of them or because they are ineffective in removing bone. This flaw in bone resorption results in bones that are abnormally dense, yet are easily broken. Men and women are equally affected by the disease.
The increased bone mass in individuals with osteopetrosis can limit the amount of available bone marrow space. Since the bone marrow is responsible for the production of blood cells, impaired bone marrow function can produce several clinical problems. For example, interference with red blood cell production can lead to anemia. Impairment of white blood cell production can limit the body's ability to fight infection. When platelet production is suppressed, individuals are prone to bleeding since platelets are essential for clotting blood. Anemia, infection, and bleeding are just some of the symptoms that individuals with osteopetrosis can experience--blindness, deafness and even stroke can occur when the skeletonis so dense that blood vessels and nerves cannot pass through the bones.
Three major types of osteopetrosis have been identified in humans:
Malignant infantile form. This type of osteopetrosis is usually discoveredin the first months of life. In addition to having many of the above symptoms of the disease, children with this condition often have significant delays in psychomotor and tooth development. The malignant infantile form of the disease can be severe and frequently results in death from infection or bleeding. In fact, thirty percent of children with this form of the disease will die during the first ten years of life. This form of the disease is inherited as an autosomal recessive trait—there by affecting some brothers and sisters.
Adult form. The adult form of osteopetrosis is milder than the infantile form. This type of the disease is benign and generally does not alter life expectancy. Many of the individuals with the adult form of osteopetrosis have few or no symptoms. It is inherited from generation to generation as an autosomal dominant trait.
Intermediate form. This type of the disease is found in children younger than ten years of age. It tends to be less severe than the malign antinfantile form, but more severe than the adult form. Individuals with this type of osteopetrosis generally do not have a reduced life expectancy.
The diagnosis of osteopetrosis is usually made when dense bones are discovered on x-rays. A bone biopsy can confirm the presence of the disease, while supplemental tests are usually performed to evaluate any potential complications. Confirming the specific subtype of the disease is important so that individuals can receive the most appropriate treatment.
Various therapies have been used in the treatment of osteopetrosis. Bone marrow transplantation (BMT) allows the abnormal osteoclasts to be replaced with normal cells. BMT is the only approach that has resulted in a cure of the malignant infantile form of the disease. Because of its high failure rate and because of its side-effects, BMT tends to be reserved for only the most severe cases of osteopetrosis. According to the Paget Foundation, survival rates after BMT in children with osteopetrosis range from 40-70%.
High doses of calcitrol (the active form of vitamin D) have been used tostimulate osteoclast function in individuals with osteopetrosis. Calcitrolhas been shown to significantly reduce symptoms in people who have mild or severe forms of the disease, though it is not approved by the Food and Drug. Administration for this purpose. Similarly, experimental use of interferon gamma 1-b treatment has been reported to decrease infection rate and improve blood abnormalities in severely affected patients. Another type of medication that may be used to treat patients with osteopetrosis are glucocorticoids. Large doses of glucocorticoid drugs (such as prednisone) may be given for short periods of time to patients with impaired red blood cell or platelet production.
Research efforts are underway in an attempt to identify the gene defects that cause osteopetrosis.
Two documents on osteopetrosis have recently been developed by the Resource Center and the Paget Foundation: a patient information brochure and an annotated bibliography for professionals. For further information on these educational resources, please contact the ORBD~NRC at 202-223-0344.