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Kenneth
H Neldner MD, Texas Tech University Health Sciences Centre Lubbock,
Texas
Childhood to the adolescent years
Ages 20 to 45 years
Age 45 and over
Eye care
Osteoporosis
Age 80 and over
Major unsolved problems in the management of PXE
Childhood to the
Adolescent years
- Low-calcium
diet (600 to 800 mg/day calcium)
- Avoid head
trauma sports (soccer, boxing, rugby, etc) others alright
- 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)
- 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
- 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 over concern.
Age 45 years and over
- Exercise
program is becoming more important (Use It or lose It!)
- Weight control
- a must
- Continue
surveillance of blood lipids and blood pressure
Eye
Care
- Be more careful
to avoid trauma or very heavy lifting
- Continue
surveillance of blood lipids and blood pressure.
- Use Amsler
grid regularly
- Get second
or third opinions on laser therapy if retinal haemorrhage occurs.
- If central
vision is lost, 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 dog or has used Braille. Peripheral vision always
remains.
- Continue
surveillance of blood lipids and blood pressure
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).
- 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.
- Chelation
- Alpha interferon
- Topical creams
and lotions
- Antacids
(Tagamet, Zantac, Propulsid)
- High-dose
vitamins/minerals (do not overdose)
- Cortisone
(must regulate carefully) (can use only for short periods)
- Acupuncture
- Herbal remedies
- Fosamax (for
osteoporosis)
- Surgery (surgical
excision of vessels and scar tissue from under the macula)
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. Alpha interferon injections for retinal haemorrhages
were recommended a few years ago but are no longer felt to be
of value.
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