I had already started this Newsletter when I talked on the phone with Cliff Webster, the Grandfather of a child with MSUD. I mentioned that Jessica, Peter Shaffer's daughter, had been hospitalized for vomiting which did not respond to IVs. I mentioned the good response to a specially modified Total Parenteral Nutrition (TPN) treatment for MSUD that she and other children have experienced. He encouraged me to print more about this treatment in the Newsletter in laymen's language.
In the April '91 Newsletter under Resources we listed the paper titled "Branched-Chain Amino Acid-Free Parenteral Nutrition in the Treatment of Acute Metabolic Decompensation in Patients with Maple Syrup Urine Disease" by Gerard T. Berry, M.D., et.al. from the Children's Hospital in Philadelphia (CHOP) and reprinted from the New England Journal of Medicine 324:175-179 (January 17, 1991).
That column in our '91 issue was submitted by Alice Mazur, R.N., P.N.P. from CHOP. The description of the paper is given thus. "This report explains the treatment of acutely ill children using a new intravenous nutritional therapy. It involves nine episodes of illness in five patients with MSUD at CHOP. A mixture of complete nutrients, except the branched chain amino acids is administered intravenously. This proved effective in reducing the plasma branched chain amino acid (BCAA) levels when patients were not eating or were vomiting."
Dr. Morton mentioned the use of this type of hyperalimentation (more than normal nourishing) in his article, "MSUD News From the Clinic for Special Children," in the Dec '92 issue of the Newsletter. In the Dec. '93 issue, Glenda Groff told how the TPN was instrumental in helping her son, Jordan, recover from a severe illness. In the same issue, Dr. Richard Allen (from the University Hospitals in Ann Arbor, Michigan) called TPN a great advance in treatment, avoiding the need for dialysis that had previously been used during acute bouts of illness. Dr. Allen, Dr. Morton and CHOP have used this treatment very successfully on newborns with elevated levels of BCAA. I do not know how many other medical centers are using this treatment at this time.
Rather than reprint a technical article on TPN for MSUD, I decided some personal testimonies from parents may help to verify the importance of this apparently lifesaving treatment. It is very important that you and your doctor are familiar with TPN and have a plan for its use before it is needed.
I called three families who had children treated with TPN. I asked them to write a short account of their experience and fax it to me as soon as possible. All three faxes arrived within twelve hours. I call that real support! Hopefully these reports will help this treatment receive the recognition it deserves.
What is TPN?