Paget’s Disease Of Bone, abbreviated as PDB, is a degenerative disorder arising at the cellular level and characterised by the abnormal structure of one or more bones in the body. Also termed as osteitis deformans and Paget’s disease, this illness invariably leads to unbearable aching in affected bones, in the skull, as well as hip, thigh, leg regions – the pelvis, femur, tibia, like in the case of hip dysplasia.
This ailment is named after the recognised surgeon and pathologist from England, Sir James Paget, who first described the deformative phenomenon in the arrangement of bones, in the year 1877. People above the age of 40 are at a higher risk of acquiring Paget’s disease and the condition is more common in men than women. Also, this bone-related sickness, which usually does not maim the entire skeleton or spread from one part of the body to another, tends to occur in persons with a family history of the illness.
This malady gives rise to faster replacement of old osteocytes, tissues, with new cells due to rapid regeneration and remodelling, invariably resulting in fragile and weak bones. Paget’s disease manifests in very severe forms at a rather slow pace and hence can be diagnosed, treated properly, provided the symptoms are reported to a healthcare professional at once. The more prominent complications of PDB are osteoarthritis, bowed knees and broken bones i.e. fractures. However, in rare instances, osteitis deformans can also result in neurological complaints, heart problems and bone cancer.
Causes Of Paget’s Disease Of Bone
Medical researchers are yet to establish the exact causes of Paget’s disease of bone. However, laboratory studies have demonstrated that PDB is possibly triggered by hereditary factors due to aberrant genetic material and is more often witnessed in members of the same family who have at least one affected individual. The genes responsible for the initiation of wounded bones have been figured out as SQSTM1 and RANK, which regulate the structure and function of osteoclasts – a type of bone cell.
Furthermore, Paget’s disease of bone may also be prompted by underlying infectious diseases such as the measles virus, but this theory has not yet been decisively proven. Scientists are still investigating the factors behind PDB and conducting experiments to showcase a clear mechanism of progression of this bone disorder.
In numerous cases, PDB presents with no obvious indications. The distinguishing symptoms of Paget’s disease of bone develop in the vicinities of afflicted bones and comprise the following:
- Agonizing pain in the hips, due to PDB in the pelvis
- Piercing headaches or even hearing loss, owing to PDB in the skull
- Discomfort, tingling sensation and numb feeling in the arms, legs, because of PDB in the spine that hurts the nerve roots
- Aching in the lower leg, knee, leading to distended bones in the area
- Osteoarthritis of the hips and knees
The doctor conducts a thorough physical examination, to look for any noticeable external signs of inflammation in the affected bones. Then blood samples of the patient are collected, to determine if a specific enzyme – alkaline phosphatase, is present in higher than normal concentrations.
X-ray scans are taken of the patient, which display any irregularities in bone structure, besides highlighting the portions of bone reabsorption, enlargement, to identify the exact spot affected by Paget’s disease. Moreover, radioactive assays of bone scans are also performed, to detect the zones in the bones that have undergone maximum damage due to PDB.
Once the diagnosis of Paget’s disease of bone is confirmed in the patient, the foremost method of treatment is utilizing prescription medications – bisphosphonates, that are primarily recommended for osteoporosis. These are advised either to be taken orally or administered by the physician via injections into the bloodstream.
In seldom circumstances, surgery may be necessary, to correct for the structural defects in the bones, assist in repairing fractures, repositioning malformed bones, replacing deteriorating joints owing to severe arthritis, or lowering the force and stress on nerves.
Additionally, the medical expert advises the patient to adhere to a wholesome, balanced, diet, take calcium, vitamin D supplements and exercise every day. This offers relief from excruciating pain in bones and other symptoms of Paget’s disease, guarantees complete recovery and restores bone health of the patient.