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What is Paget's disease of bone? "Paget's disease of bone is a chronic bone disorder. Normally, there is a process in which your bones break down and then regrow. In Paget's disease, this process is abnormal." - medlineplus.gov Where does bone begin? To begin to understand how diseases of the bone develop one must first have a thorough understanding of how bones develop. All bones develop from the mesoderm. The axial skeleton develops in the paraxial mesoderm of the notochord within the neural groove. The lateral plate mesoderm is where the appendicular skeleton develops and the neural crest is the site of the craniofacial bones and brachial arch. It is here that the mesenchymal cells commit to become cartilage, by condensing into nodules. Chondrocytes then proliferate and secrete the extracellular matrix. There are genetic problems associated with the condensation of gestation such as syndactyly or polydactyly via the Homeobox gene. Here, either too little or too much mesenchyme result in dystoses. Bone formation can take the path of both intramembranous ossification, where bone develops from mesenchymal sheets and endochondral ossification, where hyaline cartilage becomes bone. In Paget's disease the bone becomes similar in consistency to chalk. It begins with osteoclast activity followed by a mixed phase of both osteoclast and osteoblastic activity with the third stage ending with osteoblast proliferation. Paget's disease affects multiple bones at least 2/3 of the time. There is an increase in interleukin 1 along with an increase in the macrophage colony stimulating factor m-CSF. Diagnosing the disease can be done by radiological methods along with lab work. The typical cotton wool patches around the bone show up on X-ray. This can be seen most clearly when the disease affects the skull. Osteosclerotic activity may also be seen on an X-ray making the bone appear more dense. The pattern appears as a mosaic. Lab values include an increase in alkaline phosphatase during the osteosclerotic phase, which can be almost three times the normal value which is between 39-117 IU/L for adults. Men More Commonly Develop Pagets Disease Paget's disease typically occurs in men over the age of 50 and may go unnoticed for years before the patient seeks treatment. The patient may find that they are having to continually increase their hat size due to the osteosclerotic phase. The patient's calcium and phosphate levels can be normal as well as their parathyroid hormone levels. However, during the osteolytic phase, the patient's calcium levels will be elevated. There is another lab test which is distinctive to Paget's disease and that is urine hydroxyproline. Normal free hydroxyproline levels in males ages 22-55 is 0.7-1.55 µg/mL and in females is 0.7-1.40 µg/mL. Any elevation in these levels should be a clue to consider Paget's disease as a differential diagnosis and to prompt the practitioner to perform further diagnostic testing. Signs and symptoms at first may be totally asymptomatic except for an elevation in alkaline phosphatase. The patient may experience bone pain, erythema over the area, there may be spinal involvement along with impaired healing, pathological fractures and increasing skull size, which may also cause neurological complications if the increase in bone puts pressure on cranial nerves. There is a 1% chance that these patients could develop osteosarcoma due to neoplastic complications. Women and Paget's Disease How would a woman present with Paget's disease? In women the disease can manifest in the pelvis, limbs or long bones and spine. The key is to do a thorough assessment with respect to pain, onset, duration, location and determine any abnormalities in specific labs and radiographs. When there is osteosclerotic activity in the long bones, it can present as, "Saber Shins", which is the bowing of the tibia and fibula due to the lack of bone tensile strength needed to support the axial skeleton. The bones of those with Paget's disease undergo remodeling from an imbalance in both osteoclastic and osteoblastic activity. In the beginnings of the disease there is proliferative osteoclastic activity where Rank(L) receptors found on the osteoblast binds to Rank receptor on the osteoclast causing bone resorption. This activity, if not halted by OPG (osteoprotogrin) , estrogen and IL-10 will cause an overactivity of bone resorption thus raising the calcium levels in the blood. Other players in bone formation include the Wnt and B Catenin receptors which act on LDL receptor LRP5 related proteins to block osteoclastic activity and increase bone mass. This proliferative bone crunching phase usually goes unnoticed in Paget's disease unless it is found incidentally through a routine physical exam or other health issue which the patient may be seeking treatment. It is also important not to forget about the job of the parathyroid gland in the regulation of calcium. The parathyroid gland is the grand regulator of calcium levels in the body. Disease of this gland is the main cause for Osteitis Fibrosa Cystica or primary hyperparathyroidism. In Paget's disease if the calcium is elevated due to osteoclastic activity the PTH may be still remain normal for the exception of the lytic bone phase where there may be a slight increase. It is important to keep in mind that during normal biological function, the parathyroid gland will release the parathyroid hormone in response to low calcium levels, where it will stimulate the liver to metabolize vitamin D which is then activated in the kidneys to its main active form of 1,25 (OH)2 Dihyroxy Vitamin D3. This is then used to make the distal ileum of the small intestine permeable to calcium so that our bodies can utilize it as the second messenger in metabolic synthesis of proteins and in energy cycles. The practitioner should be aware of the complexity of the how all these hormones and receptors interact in disease states. This knowledge will help in the diagnosis of Paget's disease as well as in many other diseases. Have you seen Paget's disease in your practice? References Kumar, V., Abbas, A. K., & Aster, J. C. (2015). Robbins and Cotran pathologic basis of disease (Ninth edition.). Philadelphia, PA: Elsevier/Saunders Paget's Disease of Bone - MedlinePlus
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The Concern: Non-licensed Health Care Workers The number of non-licensed health care workers performing clinical skills only performed by licensed medical professionals is continually widening due to shortages in the medical workforce. Hospitals are notorious for placing advanced medical tasks in the hands of those without the education and credentials so as to save money. When it comes to the average consumer, they are typically not aware of what skills can or cannot be performed by a non-licensed professional versus a registered nurse or physician. American people who have vitamin infusion therapy performed by unlicensed personnel should be concerned. Any type of IV infusion must be first ordered by a physician and then administered by a Registered Nurse, paramedic, or physician assistant. Although some medical assistants have been known to start IV’s, they are usually done in the emergency room under the supervision of a nurse. Therefore, consumers should know the qualifications of the person starting their vitamin infusion. Complications such as extravasation and infiltration can occur. The difference between these is that extravasation is caused by a vesicant fluid that causes tissue damage, whereas infiltration is caused by non-vesicant fluid which causes edema and fluid buildup under the skin. Critical Thinking There is a great deal of critical thinking involved in choosing the needle gauge, drip rate, and tubing diameter. The medical professional must also consider the age of the patient, the diameter of the vein, and the patient’s medical condition. The diameter of the angio catheter should be taken into consideration based upon the patient’s veins. A too large of bore can cause rupture of the vein and perhaps phlebitis or necrosis of the underlying skin. This constitutes a medical emergency. The person performing the infusion must watch their patient closely during their infusion to make sure that they are not experiencing any heart-related issues such as palpitations or chest pain. This can be caused by fluid volume overload. There is a lot of risk associated with something as simple as a vitamin infusion. According to several of the vitamin infusion spas, the actual process is only thirty minutes from start to finish. Many spas stated that they did not have any complications associated with their clients and most of their clients are regular customers. The medical aspects of starting an IV requires in-depth knowledge of the anatomy of the vein and the circulatory system. The practitioner must know that permanent damage can occur to a nerve when starting an IV in the hand. But there is much more to the specialization of vitamin infusion. The fluids themselves can be toxic if the patient has not first had blood work completed. There are certain vitamins that are water-soluble and those that are not. If a patient receives too much of a particular vitamin it can cause renal failure. IV Vitamin Therapy The fat-soluble vitamins are vitamins D, E, A and K which should never be transfused due to their toxicity. Each of these vitamins can be toxic in even small amounts. These vitamins are not typically transfused during an infusion due to their lethality at high concentrations. Most of the vitamins infused consist of water-soluble vitamins such as vitamin B, C, glutathione and many other combinations. When medication such as a vitamin is administered via intravenous infusion the vitamin bypasses the liver; this is known as the first-pass effect. Both dietary forms of folate and cobalamin exert their beneficial effects by being metabolized via the liver thus promoting gut health. Vitamin B 12 is also called cobalamin. This is found in foods such as red meat, chicken and dairy products. Folate or Vitamin B9 is a natural vitamin found in green leafy vegetables. Folic acid is the manmade form of the vitamin, though they both have the same effect. However, if the patient has low folate levels, then administration of Vitamin B12 could mask these low levels, which could cause neurological damage if left untreated. Our intestinal bacteria synthesize both vitamins. The upper intestinal bacteria produce folate while cobalamin requires a luminal binding protein whereas folate does not. Administration of Vitamin B 12 or hydroxocobalamin is rapidly excreted in the urine when administered via IV, thus nullifying is nutritional effects. Typically, high dosages of Vitamin B12 are given when the patient's Vitamin B12 levels are dangerously low. The importance of monitoring blood values such as hematocrit, reticulocyte count, Vitamin B12, folate, and iron will let the medical practitioner know if vitamin therapy is even necessary. Patients with impaired renal function could suffer further renal damage if not carefully monitored. This can happen at even low doses. Vitamin B12 depends on the presence of sufficient intrinsic factor and calcium ions. Vitamin B12 is bound to the intrinsic factor during transit through the stomach. Then separation occurs in the terminal ileum in the presence of calcium. Vitamin B12 then enters the mucosal cell for absorption. Patients with early Leber’s disease which is a type of hereditary optic nerve atrophy can suffer immediate optic atrophy when administered Vitamin B12. When it comes to which is better, oral vitamins or parenteral administration the jury is out, since there is no definitive data to determine which is best. While obviously it is cheaper to simply take vitamins, the parenteral route offers faster and swifter administration to the desired site, but is it worth the risk? Typically, IV infusion of cobalamin occurs when a person does not have the proper absorptive mechanisms in their gut to process and use the vitamin. While an infusion is faster, the typical route for those deficient in vitamin B12 is an intramuscular injection. Deficiencies in Vitamin B12 can cause anemia, but excess concentrations can also be harmful. While there are many other water-soluble vitamins and cocktails of vitamins infused in these clinics, it is important that customers familiarize themselves with the dangers associated with each. The best advice is to seek a physician’s advice before just showing up at an IV infusion spa. Don’t let them talk you into something that could actually be harmful, and above all make sure that the person who is starting the IV is a licensed medical professional.” In Closing People need to be aware that there are dangers associated with IV vitamin infusion and they should always consult with their physician to determine what is the best route or if it is needed. Resources Oral vitamin B12 versus intramuscular vitamin B12 for vitamin B12 deficiency Folate-Deficiency Anemia Cellsentials Vita-Antioxidant
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Great thought provoking question. I love being a nurse first and foremost. It is very rewarding. However, I can say first hand that my experiences with physicians have not been pleasant. I know that I'm preaching to the choir, but how many times have we had to correct their mistakes, reduce the IV fluid rate so our patient didn't go into cardiovascular overload or shock and have to justify with a litany of reasons for calling them at 2am just to get our patient pain medication or something to sleep. Very frustrating. Let us not forget some of the nurses aides who flatout refuse to help us. It takes a strong nurse to stay in nursing and a strong nurse to stand up to physicians. Being a patient advocate and protector is what we do. May God give us strength.