Other Health Questions


Other Health Questions


  1. Should I restrict calcium if I am affected by PXE?
  2. How harmful is alcohol to someone with PXE?
  3. Does PXE affect joints and cartilage?
  4. Does PXE affect the lifespan of an individual?
  5. Can PXE cause scarring in your lungs?
  6. Is a magnesium supplement for leg cramps safe for people with PXE?
  7. Are Fosamax and Evista safe for women with osteoporosis and PXE?
  8. Is it safe for someone with PXE to take Forteo for osteoporosis?
  9. Is smoking cessation therapy safe for someone with PXE?
  10. Is it safe for someone with PXE to take allergy shots?
  11. Can breast lumps in young males be related to the calcification occurring in PXE?
  12. What is the association between PXE and testicular microlithiasis?
     

1. Should I restrict calcium if I am affected by PXE?


NO! It is not advisable for PXEers to restrict calcium in the hopes it will slow or stop the progression of your PXE. There is no good scientific evidence either in animals or humans that calcium restriction alters the course of PXE. This idea came from a single nutritional survey published in 1984 (but never confirmed) that high calcium intake in adolescence might be associated with more severe PXE later. There is no clear-cut evidence that either calcium restriction or high calcium intake affect PXE. In addition, restricting calcium may lead to future osteoporosis.

Calcium is critical to muscle and cellular function, and the body has a large reservoir of calcium stored in the bones. By restricting your dietary intake of calcium, you trigger the body to “mine” this stored calcium. If the restriction is severe and long term, osteoporosis can result.

Our Medical Advisory Board recommends that in the absence of any evidence to the contrary people with PXE consume the U.S. Recommended Dietary Allowance (RDA) of calcium per day, factoring in your dietary consumption. You can estimate your dietary intake of calcium by reading the nutritional content label for the foods you eat. The RDA of calcium for adults varies with gender and age, but is currently 800 – 1200 mg/day, with 300 – 400 mg magnesium. If dietary intake of calcium is low, calcium citrate is a very absorbable form of calcium. In the absence of good experimental and clinical data on the effect of high calcium intake on PXE, it would be prudent to limit calcium intake to an amount approximating the RDA, along with sufficient vitamin D.   [July 2009]

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2. How harmful is alcohol to someone who has PXE?


PXE International is not aware of alcohol having any specific effect on the disease process in PXE. However, accidents and falls are certainly a greater risk with heavy alcohol consumption, and this might increase the risk of retinal hemorrhage. Heavy alcohol consumption might predispose to gastric bleeding due to its effects on the lining of the stomach.  [March 2006]

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3. Does PXE affect joints and cartilage?


Long ago, PXE was thought to be a connective tissue disorder. The discovery of the gene helped scientists understand that PXE is a metabolic disease. PXE does not affect cartilage or joints. Read about the things it does affect [March 2006]

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4. Does PXE affect the life span of an individual?


People with PXE have a normal life span. However, because PXE can involve premature and faster progression of heart disease and macular degeneration, a person with PXE should diligently avoid the factors that increase risk in these areas - smoking, poor diet, and lack of exercise. It is recommended that you follow a heart healthy diet, do not smoke, and get moderate regular exercise. The PXE General Bulletin has a good overview of proactive steps you can take to optimize your health.  [March 2006]

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5. Can PXE cause scarring in your lungs?


There have been minor radiological changes in the lungs reported in PXE. There can be changes in the lung tissues, but these changes would not be true scarring, such as when the body's tissues heal from an injury.  [March 2006]

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6. Is a magnesium supplement for leg cramps safe for people with PXE?


Our nutritionist recommends increased magnesium in general for people with PXE. There is almost no possibility of toxicity with too much magnesium, but it can give you diarrhea. If you take more than your body can absorb, the excess will be excreted in the bowels. Consult your physician or a nutritionist for appropriate dosage.

Magnesium rich foods include whole grains, nuts, fruits, and green leafy vegetables. Grains include buckwheat, millet and rye. Seeds and nuts include almonds, filberts, peanuts, pecans, English walnuts and sunflower seeds. Fruits include boysenberries, pineapple and raspberries. Vegetables and legumes include Jerusalem artichoke, beet greens, Brussels sprouts, Swiss chard, tofu, green beans and leeks. [March 2006]

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7. Are Fosamax and Evista safe for women with osteoporosis and PXE?


Fosamax is not contraindicated in PXE. The class of drugs it belongs to inhibits bone resorption and does not affect mineralization of elastic tissue. The medication can cause gastric and esophageal irritation because it must be taken on an empty stomach, but PXE International is not aware of any problem with this in patients with PXE.

Evista is a selective estrogen receptor modulator. It is an estrogen that does not affect estrogen receptors in the breast, so there is no increased risk of breast cancer. It is associated with a three-fold increase in the risk of venous thromboembolism (clots in veins), but PXE International is not aware of any particular risk in PXE.

The decision can be made based on underlying risk factors and the known need for treatment of fairly significant osteoporosis - which is associated with a high risk of spinal compression, wrist, and hip fracture.  [March 2006]

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8. Is it safe for someone with PXE to take Forteo for osteoporosis?


Forteo is a new type of osteoporosis medication that stimulates the formation of osteoblasts, cells that make bone. The medication is injected daily for up to two years. PXE International is not aware of any contraindication to its use in PXE. Some patients do complain of gastric side effects, even though it is injected. It is approved only for patients at significant risk of fracture.  [March 2006]

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9. Is smoking cessation therapy safe for someone with PXE?


PXE International is not aware of smoking-cessation therapy that would cause specific problems for people with PXE. As with any treatment, ask whether this is a medically recognized treatment that has been found to be safe in the general population. Also, anyone receiving medication for smoking cessation should be screened by an ophthalmologist to ensure that they are not at risk for angle closure glaucoma.  [March 2006]

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10. Is it safe for someone with PXE to take allergy shots?


PXE International is not aware of any issue regarding allergy shots and PXE. Allergy shots are injections of minute and steadily increasing concentrations of allergens given to induce tolerance to an allergen. If your ENT specialist thinks it will be of help, and your symptoms warrant it, then you can receive the shots.  [March 2006] 

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11. Can breast lumps in young males be related to the calcification occurring in PXE?Unilateral gynecomastia or breast enlargement is common in boys going through puberty, and is unrelated to PXE. It is transient and nothing to worry about, although embarrassing to the sufferer. Your primary care physician should evaluate this.  [March 2006]

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12. What is the association between PXE and testicular microlithiasis?


An article recently published in the medical journal Radiology confirmed the association of PXE and testicular microlithiasis1. Testicular microlithiasis is a condition in which very small mineral deposits (microliths) are distributed randomly in the testicles. The condition is asymptomatic (causes no symptoms), so it is typically discovered during testing for other testicular anomalies. According to the study, testicular microlithiasis does not appear to be the risk factor for testicular cancer for males with PXE that it may be for the males in the general population. There is no known association between PXE and testicular cancer. Further, it may be that testicular microlithiasis merely represents another manifestation of PXE. The authors recommend that males with PXE with confirmed testicular microlithiasis remain under long-term observation by ultrasonography and physical examination. This finding has been confirmed by other authors.

Read the Patient Summary of this article in our Published Peer Reviewed Literature section of the web site. [March 2006]

1Bercovitch RS, Januario JA, Terry SF, Boekelheide K, Podis AD, Dupuy DE, Bercovitch LG. Testicular Microlithiasis in Association with Pseudoxanthoma Elasticum. Radiology. 2005 Nov; 237(2):550-4.

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Disclaimer


These are replies to general and specific questions which have been submitted to us in the past. Our responses may not apply to any particular individual´s situation and are not a substitute for medical advice given by a physician who is familiar with the individual´s case and who has examined the patient. In addition, the responses are updated on a periodic basis but may not be current.