Treatment involves resolving the underlying condition where possible to prevent or minimize further pancreatic damage and managing the symptoms. Those affected may be given oral preparations of pancreatic enzymes as a supplement to make up for the decreased production and to aid digestion. They may also be given vitamin supplements, especially fat-soluble vitamins A, D, E, and K, since absorption of fats is frequently impaired, and may be put on a diet low in fat and high in protein and calories to help them gain and maintain weight.
In Shwachman-Diamond Syndrome (SDS), unlike in cystic fibrosis, pancreatic lipase secretion often increases with age. The reasons for this are not yet known, but about 50% of children with SDS will experience improvements in pancreatic function and fat absorption and will no longer require enzyme replacement therapy.
For more on other less common conditions involving the pancreas, see Pancreas.org: Rare syndromes.