Lifelong Management of PXE

Annual Check List for PXE Patients:

Kenneth H Neldner MD, Texas Tech University Health Sciences Centre Lubbock, Texas

Childhood to the Adolescent years:

Low-calcium diet (600 to 800 mg/day calcium)
Avoid head trauma sports (football, boxing, rugby, etc)
Avoid tobacco in any form (important)
Watch for stomach upset or bleeding (black stools). Use Hematest if in doubt
Avoid excessive aspirin or NSAID group of analgesics. Occasional use is alright, though

Ages 20 to 45 years:

Maintain strict weight control
Continue low-calcium diet (not over 800 mg/day)
Continue to avoid excessive use of aspirin and NSAID group of pain pills
Develop a regular exercise program (30 to 45 minutes three times/week) Swimming and walking are very good.
Avoid tobacco in any form
Limit pregnancies. Keep dietary calcium at about 1000 mg/day during pregnancy
Observe general eye care
Avoid occupational eye hazards with high risk of head trauma, high lifting, or vibration. Do not lift anything more than 20lbs in weight as it is stressful on the eyes.
Avoid recreational eye hazards (head trauma sports, shooting firearms)
Avoid excessive rubbing of eyes
Learn to use an Amsler grid
Find an ophthalmologist with PXE experience
Watch for signs of Gl bleeding or chronic stomach upset – this is a medical emergency.
Check blood lipids (cholesterol, triglycerides, LDH, HDL) annually
Control with diet and exercise if possible; medication if necessary
Follow low fat diet. Eat 5 servings of fruit and vegetables each day.
Watch blood pressure. Manage with exercise and weight control, if possible; medications if necessary.
Use dietary supplements, especially antioxidants (vitamins A, zinc, selenium, and copper). Start Ocu-Vite vitamin/mineral pill (one/day) at about age 40 years.
If intermittent claudication, intensify exercise program. Try Trental.
Live a normal life, with concern for your PXE but not overconcern.

Age 45 years and over:

Eye Care:

Be more careful to avoid trauma or very heavy lifting DO NOT lift anything over 20lbs in weight as it is adding stress to your eyes. If you already have angioid streaks very close to the macular where the central vision is stored, the added stress could make things far worse and even cause a weakness which could result in the possibility of having a retinal haemorrhage.
Continue surveillance of blood lipids and blood pressure.
Use Amsler grid regularly
If you experience any sight changes, distortion, things that should be straight appear to be wavy etc it is likely that it could be the start of a retinal haemorrhage. You must go to Eye Casualty immediately.
If you start to experience any central vision loss you will need to be checked over by an Eye Specialist who knows about PXE and will most likely have to have Lucentis or Avastin eye injections until the sight becomes stable. Then you will be seen on a monthly, 3 monthly or 6 monthly basis for eye check ups.
If you have the eye problems, it is important that you do not carry out a job where you are on a computer all day, if so you should seek employment that does not require 20/20 vision.
If poor central vision, there are many low-vision devices available to improve vision.
Remember that no one with PXE has ever gone completely blind and no one has needed a guide dog or has used Braille. Peripheral vision always remains.

Osteoporosis:

Frequency or severity in PXE is unknown.
A bone scan will tell you if you have osteoporosis. (New tests and methods are available).
If definite osteoporosis on bone scan, increase dietary calcium to 1000 mg/day.
If no osteoporosis, continue lower calcium intake (800 mg/day).
Estrogen and exercise are best for prevention and treatment of osteoporosis.
Intermittent claudication. Try Trental. Increase your exercise program (especially exercise of your legs – the best exercises are walking and swimming)
Be happy. Don’t worry! – It will only make you worse.

Age 80 and over:
Please help any of us who do not have PXE and are still around!

Alternative Therapy:
No proven benefit but not thoroughly tested.

Major unsolved problems in the management of PXE:

Dietary calcium intake. Aggravation of PXE during childhood and adolescence by excess dietary calcium is fairly well proven. The value of continuing a low-calcium diet throughout adult life is less well proven. (I continue to recommend an 800 mg/day calcium diet throughout adult life.)
Osteoporosis in post-menopausal women is of unknown significance in PXE. The actual incidence is unknown, hence the need for larger doses of calcium is unknown. My recommendation: Get a bone scan to make an absolute diagnosis of the presence and severity of osteoporosis. If present and severe, increase calcium intake to 1000 mg/day. If not, stay at 800 mg/day.
Supplemental estrogen and progesterone pills present another dilemma. If estrogen aggravates PXE in early life, will extra estrogen aggravate it in later life? No one knows the answer. My recommendation (until more is known) is to take estrogen supplements if there is evidence for significant osteoporosis and avoid it if there is no osteoporosis. The proven helpful treatments for osteoporosis are estrogen and exercise. The amount of calcium in the diet is the least helpful; ie you can’t cure osteoporosis with a high-calcium diet alone.
The value of the alternate therapies listed above is unknown. Chelation therapy has been reported to be helpful in some, but certainly not in all.