Paget Disease of Bone, Rickets & Osteomalacia

Pharmacology

-Anti-osteoclastic therapy- reduces bone turnover, reverses the course of the disease, relieves pain, and improves mobility. -Bisphosphonates-Cornerstone-stabilizes rapid bone turnover. -Plicamycin-Antibiotic used to possibly stabilize the disease

Diagnosis

ALP - alkaline phosphatase Rachitic Rosary is a row of beadlike prominences at the junction of a rib and it's cartilage(enlarged costochondral joints), resembling a rosary Harrisons sulcus is an indentation on the chest roughly along the 6th rib, which is usually bilateral but can also occur unilaterally.

Note: Paget Disease of Bone

Bone turnover is the cycle of bone breakdown by osteoclasts and bone rebuilding by osteoblasts, which continues through life. Paget disease (osteitis deformans) is a localized rapid bone turnover disorder, most commonly affecting the skull, femur, tibia, pelvic bones, and vertebrae. The disease occurs in about 2% to 3% of the population older than 50 years. The incidence is slightly greater in aging men than in women. A family history has been noted, with siblings often developing the disease. The cause of Paget disease is not known

Pathophysiology

Initial (lytic) phase - bone resorption faster Second (mixed lytic & blastic) phase - increased bone formation (abnormal new bone) to balance rapid bone breakdown. Third (sclerotic) phase - Disorganized, weaker bone formation Infiltration of fibrous connective tissue & blood vessels Hyper vascular bone

COLLABORATIVE CARE

•Medical management •Physical Therapy •Weight management •Surgery is not a great option because soft bones do not make ideal surgical sites

NURSING INTERVENTIONS

•Pain Relief - Ibuprofen or Acetaminophen •Prevention of Pressure Injury -Skin Care -Positioning •Safety -Fall Prevention -Use of Devices •Encourage pt. verbalize feelings & concerns •Encourage diet with calcium & Vitamin D •TED hose / SCD (prevent DVT) •Administer Medications •Education

Management

•Physical, psychological, & pharmaceutical - Reduce Pain & Discomfort •Kidney Disease - Calcitriol(activated form of Vit D) •Malabsorption - Calcium with Vit D •Exposure to sunlight •Diet •Orthopedic Deformities -Braces or surgery (osteotomy to correct long bone deformity).

Clinical Manifestations

Paget disease is insidious(gradual, sneaks up, ambushes). Some patients don't have symptoms but only have a skeletal deformity. Found most times on an x-ray done for unrelated problems. Skull deformity(thickened skull) causing impaired hearing. Femur/tibia tends to cause bowing that produces one to "waddle." Due to increased vascularity, it may create a warm feeling or tenderness. Pain can be mild to moderate and is increased with weight bearing.

Nursing Interventions

•Assess posture, gait, & ability to walk with or without aid. •Check bony prominence •Assess shapes of bones •Administer prescribed diet. •Rich in Calcium •Rich in Vitamin D (Liver, Processed Milk, Tuna, Salmon, Mackerel) •Discuss purpose of physical therapy. •Administer Calcium & Phosphorus supplements •Administer prescribed analgesics. •Gentle back massage. •Instruct regarding home safety. •Teach client on the signs of fracture.

Paget Disease of Bone

•Disorder of localized rapid bone turnover •Excessive bone resorption followed by replacement of bone marrow with vascular, fibrous connective tissue (bone is larger, disorganized, weaker) •The skull, femur, tibia, pelvic bones, & vertebrae. •2% to 3% of >50 years. •Men >women. •Family history •Unknown cau

DIAGNOSIS

•Elevated serum ALP (Alkaline Phosphatase) •Urinary hydroxyproline (type of collagen) •Normal blood calcium levels. •X-rays - Demineralization & bone overgrowth •Bone scans - Extent of disease. •Bone biopsy differential diagnosis

Pathophysiology

•Failed Ca Absorption (GI Disorders, Lack of Vit. D) •Excessive Loss of Ca (Renal) •Renal Failure - CA used to combat Acidosis-causing Hypocalcemia •PTH Stimulate Ca from bones to blood • Hyperparathyroidism-Leads to skeletal decalcification •Prolonged use of anticonvulsant (Phenytoin, Phenobarbital) Note: The major defect in osteomalacia is a deficiency of activated vitamin D, which promotes calcium absorption from the GI tract and facilitates mineralization of bone. The supply of calcium and phosphate in the extracellular fluid is low and does not move to calcification sites in bones.

COMPLICATIONS

•Fractures •Arthritis •Hearing Loss

Risk factors

•Limited exposure to sunlight •Strict vegetarian diet or lactose intolerance •Exclusive breastfeeding (MOM vit. D deficiency) •Dark-pigmented skin •Prematurity •Malabsorptive GI disorder •Chronic renal disease •H/O fractures or bone pain Note: Observe for dental deformities and bowlegs. Also may have decreased muscle tone.

OSTEOMALACIA

•Metabolic bone disease •Inadequate mineralization of bone. S/S •Soft & weak skeleton •Skeletal Pain, tenderness to touch, bowing of the bones, fractures. Physical Exam •Skeletal deformities (spinal kyphosis, bowed legs) •At risk for falls & pathologic fractures

OSTEOMALACIA/RICKETS

•Osteomalacia is what we call this disorder in adults. •Rickets is what we call this disorder in children. Picture of a 4-year old girl with Rickets. Bowing of the legs.

RICKETS

•Softening or weakening of the bones. •Cause - -Nutritional deficiencies -Calcium and phosphorus regulation disturbances (CKD). -GI disorders - fat absorption altered (Crohn's disease, celiac disease, cystic fibrosis) •Calcium laid down - Third Trimester-premature babies miss this period of accumulation of calcium •Rickets of prematurity •Most likely to occur during periods of rapid growth •Diagnostics - -low ca & po4(Calcium and phosphate levels) -high alkaline phosphatase levels. -Radiographs

Diagnosis

•X-ray -Demineralization of bone -Compression fracture •Laboratory studies -Low serum calcium and phosphorus -Elevated ALP. -Urine excretion of calcium & creatinine is low. •Bone biopsy - Increased osteoid Note: On x-ray studies, generalized demineralization of bone is evident. Studies of the vertebrae may show a compression fracture with indistinct vertebral end plates. Laboratory studies show low serum calcium and phosphorus levels and a moderately elevated ALP. Urine excretion of calcium and creatinine is low. Bone biopsy demonstrates an increased amount of osteoid, a demineralized, cartilaginous bone matrix that is sometimes referred to as prebone.