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When drinking milk just doesn't help.

Most of us develop healthy bones by drinking enough milk but this doesn't help aperson with fibrous dysplasia whose bones can shatter in asplit second

THOSE OF US who tend to take our body's framework for granted would find it hard to imagine life without healthy bones. While most of us have healthy bones due to drinking milk and receiving ample nutrition from the time of our births, those who suffer from a rare bone disorder called fibrous dysplasia have bones that can shatter in a split second. The lives of such people are fraught with the risk of fractures and breaks. While bones do grow fragile and brittle with old age, this may happen in young people too. 11- year-old Angad Munshi was born with bones as fragile as glass.

Bones don't develop properly

FIBROUS dysplasia causes a young person's developing and immature skeleton to become abnormal. Usually this disease manifests itself in childhood before the age of 15 in the form of sudden fractures and extreme pain. Instead of normal bone cells, fibrous tissue develops within the bone instead. " As the bone starts growing, this fibrous tissue expands bloats and weakens the bone. This causes the affected bone to deform and become brittle," says Dr Ramneek Mahajan, senior consultant orthopaedic, Orthonova Hospital. As a result of bloating up, the bone starts bending under pressure. Just like a stick will snap when it is bent to its extreme point, the bone will also ultimately bend till it snaps if it is not treated. This disease can be detected by the abnormal position of the limbs of the patient-- 90 per cent of the time, the lower limbs are affected the most. If the problem is in the upper limbs then it is more difficult to detect as this area is less prone to injury.

" Running and jumping and even tripping can cause a break or a fracture in the lower limbs," says Dr Mahajan. " Fibrous dysplasia can affect any bone in your body.

Most people with the disorder have only one affected bone -- a form called monostotic fibrous dysplasia. When more bones are affected it is termed polyostotic fibrous dysplasia," says Dr Rajeev Thukral, orthopaedician, Max Healthcare. In some milder cases, however, the disease can remain undetected till adulthood.

Many effects

ANGAD had polyostotic fibrous dysplasia which meant that a number of bones in his body were affected. He first manifested signs of the disease at the age of four.

" When Angad initially complained of aching legs and arms, we thought he was experiencing growing pains, as all children do," says his aunt Madhavi Sharma. But, when the pain became intense enough to keep the child in tears all day, his worried parents rushed him to the doctor. X- rays revealed the presence of this disease. The bones commonly affected in this condition are the thigh and shin bones, the pelvic bones, ribs, skull and facial bones and the bones of the upper arm. " In rare cases, fibrous dysplasia may be associated with abnormalities in the hormoneproducing glands of the endocrine system such as the pituitary gland," says Dr Mahajan.

Surgery only when required

IN ANGAD'S case, the treatment was multi- faceted. Initially the diseased bones were causing excruciating pain, but since they had not splintered or broken doctors did not operate. " We only operate on the area that needs immediate attention. If all the bones are worked upon the child will be bed ridden for a long time. This inactivity will render the bones even weaker, even if they have been straightened, making a person prone to osteoporosis later," says Dr Mahajan.

Relief from pain was obtained by painkillers and massage. The femur bones of both Angad's legs, the tibia of both his arms and some bones of his ribs were affected. When he was eight, his right femur splintered and had to be operated upon. The diseased portions of the bone were removed through surgery and the remaining hollow was packed with grafted bone particles.

Then the bent bone was straightened and steel rods fixed to keep it in place. " This is the usual procedure of surgery and is quite effective," says Dr Thukral. Subsequent operations were required for his ankle after which no other breaks have happened till date.

Any pain should be reported

ONE OF the biggest problems of condition -- especially if only one bone is affected -- is that it often escapes diagnosis. In fact, diagnosis is often just incidental.

Therefore if a child complains continually of aches and pains, these complaints should be taken seriously as they might be symptoms of this bone disorder. Typically X- rays, CT scans and MRIs are done to discern the extent of damage suffered by the bone. A bone scan can also be done in extreme cases if the doctor believes the damage is extensive.

Besides bone fractures, fibrous dysplasia can also cause other serious complications. In certain cases, the brittle bones may even turn cancerous and if such a suspicion arises, a biopsy needs to be performed to confirm this.

Various complications

IF THE bones of the spine are affected then a person can even develop scoliosis or an abnormally curved spine later on in life. The weakened area of an affected bone causes the bone to be bent out of shape curving the limbs. " Early arthritis of the leg and pelvic bones is a common problem in these patients. Because this is a disease which can affect almost any bone in the body, it can also cause problems like vision and hearing loss as the nerves to the eyes and ears may be surrounded by affected bone," says Dr P K Dave, HOD orthopedics, Rockland Hospital. Deformed bone in the skull can cause cranial pressure and abnormalities of the brain can be the result of this.

Deformity of facial bones can lead to loss hearing and vision as well.

" While there is no cure for fibrous dysplasia, this is a selflimiting disease. When the skeleton is fully matured, and the bones do not grow any more, surgery is sought to rectify any lasting damage successfully," says Dr Mahajan. Calcium and vitamin D intake along with other prescribed medicines can help make the bones stronger as can bone strengthening exercises.

However, sports like karate can cause bone fractures and should be avoided.

rituparna. mukherjee @ mailtoday. in

Soy saviour

Many soy foods are naturally high in calcium as this mineral is added as a coagulating agent. This inhibits the breakdown of bones. Soy protein is also more effective in bone formation than milk protein.

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Publication:Mail Today (New Delhi, India)
Date:Oct 20, 2009

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