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John and Miriam Shirk had been blessed with five healthy children before Ammon was born. They are Old Order Mennonites living in Liberty, Kentucky. When reading the following story, keep in mind that they choose to have no phone or electricity. They do not drive cars, but use horse-drawn vehicles or ride with other people.

Ammon's treatment was started in Kentucky when he was 17 days old and continued by Dr. Holmes Morton in Pennsylvania from the 19th day on. Details about the treatment of MSUD are of great interest to me and I presume of interest to others also. So I have included a few notes of information (in italics) that were supplied at my request by Dr. Morton's Clinic.

At one o'clock Saturday morning, September 10, 1994, baby Ammon made his appearance. He was our sixth child. The first few days it seemed to me that he was a little more restless than our other babies had been, but people said, "You just forgot how new babies can be."

At two days of age we took him to the doctor to be circumcised. The doctor examined him and mentioned he was slightly yellow. He told us to watch his color. At about four or five days old, he started clamping his mouth instead of feeding. He cried shrilly while arching his back. Amazingly he slept better at times, which made people think he wasn't sick. As happened often with our other children in years gone by, we wondered, "Should we take him to the doctor?"

At ten days we decided something had to be done. I called our family doctor. There were no appointments available until two days later, so we would have to go to the emergency room to do something that day. I then called the local hospital. When they heard our situation, they told us to go to our family doctor and, "They will work you in."

After a long wait in the waiting room, the doctor finally examined baby Ammon. He didn't respond when the doctor slammed both hands on the cushion on which Ammon was lying. After consulting another doctor, he sent us to Russell Springs Hospital for a host of tests. By this time it was our usual bedtime. We decided I would go home and my wife, Miriam, would stay with Ammon. Miriam's sister was staying with our other children.

Ammon's temperature, blood count and heart rate were all low. An IV was hooked-up and heat and oxygen were turned on. Next a CAT scan was done, although it was not very clear.

By noon of the next day, Sept. 21, Ammon was transferred via ambulance to the University of Kentucky in Lexington, some 80 miles away. We followed several hours later by taxi. There they did more tests, and we needed a lot of patience and did a lot of waiting. Some tests were sent all the way to Texas. The results would not be available until after the weekend, and this was only Thursday. Family members and friends visited daily.

Thursday and Saturday night we slept at a Ronald McDonald House, which is provided for people who have a family member in the hospital. Things seemed kind of at a standstill, so Miriam went home on Friday night. I stayed alone that night.

On Friday night Ammon was moved out of the ICU, but by Saturday morning he was moved back because of vomiting and choking. At this point Ammon wasn't fully conscious and, of course, he was on medication, thus he didn't know whether I was there or not. I had wandered away for a few hours, and when I came back, Ammon was on a ventilator. The doctors said his breathing had dropped to six breaths a minute (approximately 1/10 of normal). How I wished Miriam would be here with Ammon, since he was gradually losing out. I now called the neighbors to let the family know the baby's condition and waited for Miriam and some others to arrive. Those faces certainly were a welcome sight when they finally came.

On Sunday, the baby still seemed to be losing out, but now I had Miriam and also my sister for support. Ammon's blood pressure dropped and some seizure activity was suspected. On Monday, Sept. 26, test results revealed maple syrup urine disease. As this was only the third known case in Kentucky, the doctor was unfamiliar with it. He called different doctors, who he thought might know about MSUD, but he didn't learn much. We finally got him to call Dr. Morton in Pennsylvania.

Dr. Morton asked our doctor if any of the other doctors had told him what to do. Our doctor said, "Not really." So Dr. Morton made some suggestions, but the University of Kentucky Hospital didn't have the special formula Ammon needed. So we contacted Leroy Zimmermans from the western part of the state. They have a boy who has MSUD. Arrangements were made to have formula delivered yet that night by a local driver. According to the driver, they made the trip in record time.

It seemed to me it took the medical team a long time to figure out just how to mix the stuff. Finally, early Tuesday morning, Ammon was started on the formula. The lack of equipment to test the levels daily was the next obstacle which seriously slowed the treatment. Cincinnati, Ohio was the nearest place at which they could check the levels.

Now Dr. Morton recommended baby Ammon be moved again, this time by plane to the Lancaster General Hospital in Pennsylvania where Dr. Morton had facilities to care for him. Dr. Morton specializes in genetic disorders. Ammon was transferred by plane from Lexington, KY to Lancaster, PA Thursday evening, Sept. 29, the ninth day of doctoring. It took approximately three hours. On the same night Ammon arrived in Lancaster, Dr. Morton had the formula adjusted and Ammon off the ventilator. (On arrival Ammon's leucine level was 22.3 mg/%, down from a high of 32 mg/%.)

On Friday we traveled by van to PA to my sister Lena's in Bowmansville. That night Ammon vomited and drew some fluid into his windpipe, so once again he was put on the ventilator. By Sunday, he came off the ventilator for the last time. At this time his levels were normal. (On arrival at the Lancaster hospital, Ammon was given Ketonix 2, MSUD Powder, and isoleucine and valine through an NG tube. More fats and calories were administered through an IV. In 48 hours his leucine blood levels had dropped to 2.8 mg/%.)

Then Ammon got a yeast infection from his IV catheter, causing his temperature to go up. His IV was changed to his other hand and treatment begun. The IV was removed Oct. 7, and he was free of IVs for about a day. He still tested positive for yeast infection, so an IV was placed in his left leg to administer antibiotics. A week later the cultures still tested positive for infection. The most effective antibiotic was administered by IV.

By Oct. 10 Ammon was taking all his formula by mouth - he had been on a feeding tube. I neglected my diary during the next ten days. (Ammon's leucine levels went no higher than 4.9 mg/% during the last two weeks of his hospitalization.)

On Oct. 20, Ammon left the hospital. The month of hospital life seemed long, but the Lord surely helped us in many ways. We especially appreciated the visitors and phone calls. We had been staying nights with my sister Lena and had to make arrangements each day for transportation. One older couple took us to their home for the night. That was a welcome change.

After arriving back home in Kentucky, we had to learn how to care for Ammon - 700 miles from our doctor. All went reasonably well, although during Thanksgiving week we ran out of Diet Powder. We had waited until the last minute to order, and then the holiday interfered. The night before Thanksgiving Mead Johnson sent a man from Evansville, IN to deliver the Diet Powder. He had problems finding us, but drove in with the Diet Powder the next morning before we left for church. We had given up on him and arranged for travelers to bring some along from Leroy Zimmermans.

When Ammon was a little over a year old, he had a two-day hospital stay in Russell Springs. Pneumonia was suspected, but when he improved so quickly, they decided it must have been his bronchial tubes.

Ammon was the first MSUD baby in the Casey County settlement of Old Order Mennonites. The settlement was in existence about 18 years when Ammon was born. Two months after his birth, another child with MSUD, Curvin Zimmerman, son of Ivan Ray Zimmermans, was born. Three years later in 1997, Harold Zimmerman, son of Dana Zimmermans, was born and diagnosed with MSUD. Harold and Curvin are cousins.

Ammon, now a little over three years old, is doing fairly well. We generally have to keep pushing him to take enough formula and food. He does a little toe-walking due to the delayed treatment when an infant. He started to walk at about two years. His little brother Luke, our seventh child, joined us about that time. Thankfully he did not have MSUD.

UPDATE:

This history was sent to me at the end of last year. In January 1998, when in Pennsylvania, we heard that Ammon was in the Lancaster General Hospital. His parents were with him and we visited them there. Ammon was refusing to take anything by mouth, which was delaying his release from the hospital. The Shirks said they would bring the story they wrote earlier up-to-date. Following is Miriam's account sent to me in March.

On Christmas Day, 1997, we were at my sister's for a family dinner. Ammon didn't want to eat and wanted to be held all the time. He was running a fever. In the morning he had seemed OK. He still drank a little formula. Friday evening he didn't want to drink any formula. So John called the Clinic in Pennsylvania and talked with the nurse.

On Saturday, Dec. 27, John and I took Ammon to the emergency room in Russell Springs Hospital. Dr. Miles (our family doctor) ran tests to see what was causing his fever. His lungs were clear and the doctor couldn't find what was troubling Ammon. At four in the afternoon they decided to put in an NG tube. Ammon was admitted to the hospital for the night. I went home and John stayed with Ammon, who had a restless night.

On Sunday, December 28, Ammon was not improving, so Dr. Morton wanted him in Pennsylvania. We left home at 5 p.m.. Sunday and arrived at the Lancaster General Hospital Monday morning at 4 a.m. An MRI showed quite a bit of fluid on the brain (brain edema). An x-ray showed the chest was clear. Dr. Morton still did not know what was causing Ammon's fever.

On Thursday, January 1, Ammon seemed happy and was singing "Precious Memories," but he wasn't drinking his formula. His DNPH was clear. His gums looked puffy and red, as if he had a sore mouth. He had a low sodium level and so they gave him a lot of salt. On the 4th, Sunday, they took out the IVs because they went bad. They put in an NG tube, and in that way he kept his formula down.

On January 9, Ammon was released from the hospital, and we stayed with friends in the area. He still had an NG tube, because we couldn't get him to take his formula. We had an appointment at the Clinic to see Dr. Morton on the 12th. On the 13th , with a lot of coaxing, we finally got Ammon to drink some formula from his bottle. Using a syringe also, we were getting about half his formula into him.

We started for home on evening of the 14th and arrived home the next morning. We were still depending on the NG tube to get all the formula he needed into him each day. Now (March 1998), he is doing real well. He is hungry and eating at the table. He begs for his bottle which is something he rarely did before.

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