Helpful Suggestions Regarding Treatment
for Hypophosphatasia
(childhood and adult)
A SUMMARY
"As yet there is no cure for hypophosphatasia
and no proven medical therapy.
Some medications are being evaluated.
Treatment is generally directed towards preventing
or correcting the symptoms or complications. ---
Why the disease can present late in life
(or even return in adults who seem to recover
from childhood hypophosphatasia) is unexplained."
Dr. Michael Whyte
----------------
1. Severely affected infants may manifest increased levels of calcium in their blood that may be treated with Calcitonin and certain diuretics.
2. Avoid large doses of calcium, vitamin D and B6. They are okay in recommended daily allowance levels. There is a chance that kidney stones could develop if large doses are taken as the skeleton doesn't take up calcium well and could turn what is absorbed from the diet into the urine.
3. Have blood and urine calcium levels monitored every 6 months.
4. It is also advised that one receive expert dental care and physical (or water) therapy. It has been found that the splintng of loose teeth can be helpful for stability. Swimming exercises provide cardiovascular and lung exercise and generally does not threaten the skeleton. Also, weight-bearing exercises to help preserve the skeleton mass in the lower limbs is helpful. By very careful not to fall.
5. The following bisphosphonate drugs should be avoided as they decrease alkaline phosphatase-- Didronel (etidronate disodium), Fosamax (alendronate), Actonel (risedronate), Aredia (pamidronate), Zometa (zoledronic acid). These are medications suitable for patients with osteoporosis (a porous, brittle bone disease). A drug to improve the condition of soft bones has yet to be discovered for hypophosphatasia. It has also been observed that hormone replacement therapy in women does not prevent bone loss.
6. When stress fractures (pseudofractures -- slowly-forming incomplete fractures) occur in the femurs, it has been discovered that the best procedure to follow is to have a steel rod pushed through the hollow center of bones to aid in healing instead of having the plates and screws. It is advised that the orthopaedic surgeon follow up, perhaps yearly, each person's rodding procedures. (see next page)
7. It is important to have all x-rays and bone densitometry etc. stored in one place so that the assisting doctors can easily locate all past records.
[More helpful suggestions (or changes to the above information)
will be provided, in future, as they become known.]
