Quite a few parents were aware of Jordon Groff's illness this summer. His mother, Glenda , gives an account of this very trying time for the family and Jordon's progress since then.
Today is a damp, dreary day, and it is great to be here at home with our children. As I reflect on this past summer, it almost seems like a nightmare. Since the beginning of May, Jordan has been having a problem with constant vomiting. It would go in a cycle of about 3 to 4 days. He would vomit for several days, then be fine for a few days, then we would start all over again. During these times, he would have diarrhea and be very hyper. These were days of frustration for both doctor and mother.
I started keeping a chart of all the foods he ate and all the formula he drank. I also charted his activeness and any vomiting. Dr. Morton, being concerned that Jordan had Jardia, put him on Flagil. After the medication was discontinued, Jordan would seem fine for a week or two at the most. Then we would start the cycle all over again.
The beginning of August, after starting with a low grade fever and vomiting, Jordan was admitted to Lancaster General Hospital under the care of Dr. Morton. We felt we needed to get to the root of his problem, so tests were scheduled. On Aug. 8th he had an endoscopy, to examine his throat, stomach, and small bowel. Samples of tissue were also removed for testing. That day he was started on the TPN. Little did we know what the next few weeks would hold for our family.
Over the next days, tests began coming back. The good news was that they were negative, the bad news was that we were not getting any closer to the source of our problem. That following week, Jordan began running temperatures as high as 106E. More blood work revealed he had a yeast infection in his blood stream, known as a Candida infection. At the time of the first blood culture, there were thousands of colonies in 1cc of blood. Our son was deathly ill.
He was put on a medicine know as Amphetarison, a very powerful one with some bad side effects. His kidney function and heart had to be closely watched. He was moved into a room close to the nurse's station and put on one-to-one nursing. That room became my home for the remainder of his stay. I began to feel like a dispatcher trying to get babysitters for our daughter and seeing that things were done at home.
After a few days, it was discovered that muscle pain went along with this illness. That explained why we could no longer hold him without his crying. He was the most comfortable lying on his back with his legs propped on a pillow. This was not the little boy we were used to-no smiles, no words, no more mischief dancing out of his eyes. Tests were run to find the source of the infection, but none was found. New problems cropped up. IV sites blew and become abscessed. A central line was inserted, solving that problem. Dr. Morton was able to concentrate the TPN, lessening the fluid overload that was starting to occur-another problem solved. Jordan's liver was enlarged, his pancreas inflamed, and blood had to be given.
On September 1st, Jordan was transferred to the Children's Hospital in Philadelphia. Dr. Morton wanted a second opinion on his illness. Jordan's amino acid levels had been pretty well under control until then, when it rose to 15 mg/dl. Tests were run and came back negative. We began to see a slight improvement.
Jordan's amylase was elevated, but they did not think it was high enough to have pancreatitis. They began to feed him, which made him worse again. Since they were not doing any more than Dr. Morton was doing, he was transferred back to Lancaster General Hospital. We were very grateful to have our son back under Dr. Morton's care.
Jordan definitely had pancreatitis, as he began vomiting more and more bile. His leucine level had dropped below 5 mg/dl. Dr. Morton began an IV. He needed to be on the TPN, but this was like feeding him, not allowing his pancreas to rest. He was put on a medication which aggravated his MSUD somewhat. After consulting other doctors, glutamine, alanine and aspartic acids were added to his TPN.
We began to see an improvement. Jordan began begging for a drink, but could not have any due to his constant vomiting. Finally, the day came when we were allowed to give 1/2 ounce. Once you gave him the cup there was no way you could get it back until it was empty. We gradually increased the amount to an ounce an hour.
Jordan's blood cultures were still coming back positive some of the time. He needed a stronger dose of medication. But when it was given at 1.5 mg/kg, his kidney function began to rise. He was lowered to 1 mg/kg.
By September 25th, Jordan had been weaned off the TPN, and was drinking formula. He still had an IV. Returning to the hospital the next morning, I remarked to my husband, "Maybe we can bring him home soon." Little did I know what we would find. As we walked into the room, the nurses were trying to wake him up without success. I picked him up and his eyes would not open; he held himself stiff and cried. It reminded me of when he was a week old and sick from high levels.
Dr. Morton was called from surgery. Jordan had brain edema from which so many of these children die. He was immediately taken into the treatment room and given Phenobarbital and Manatol. Glutamine, which had not been added for 2 days, was added to the formula and given by Ng tube. Another IV was started and our little boy was put back on constant care nursing. They did an MRI the following day and it revealed an abnormal patterning of the Myelin. By the end of the week, he was back to what he had been before the brain edema. Through all this his highest leucine level was 11 mg/dl.
I had begun feeding him food, and what a mess! It was like trying to feed a baby, because he had a hyper gag reflex. We started with banana baby food and gradually added other foods.
We still had the problem of positive blood cultures. Out of 28 blood cultures, 18 were positive. Dr. Morton began working on getting an experimental drug, know as ABLC. One hundred and thirty-two people in the United States had used the drug with good results. On October 11th, the new medicine was given through an IV. It seemed to be just what Jordan needed. On October 14th he was sent home, ending a 65 day hospital stay. How wonderful home and the bed looked compared to the hospital room and cot.
We returned daily to the hospital for Jordan's medication through his central line. I had to feed him every 3 hours around the clock and flush his central line. As soon as I would pull syringes out of the kitchen cupboard, Jordan would come running saying, "My flushes." On November 4th, his medication was stopped and the central line pulled. That evening, he came to me and wanted me to flush his lines. It seemed he had forgotten that it was no longer there. He now had 32 days of negative cultures.
We never really discovered where the blood infection came from. Dr. Morton thought it was the result of a nutritional problem. Jordan became malnourished, weakening the lining of his mouth and throat, causing him to be vulnerable to the Candida infection. Dr. Morton thinks there are some amino acids that, to us, are non-essential, but to these children may be essential. We are hoping that Jordan's illness will bring new light to the problems that are faced by these children.
We are returning to the clinic weekly for checkups. On November 17th, Jordan returned to Lancaster General for an MRI to compare with the previous one. Dr. Morton and the radiologist were amazed at the difference. Where previously the patterning of the myelin was abnormal, this one looked normal. There definitely had been a fluid build-up. There were a few spots on the brain stem, ones that Dr. Morton feel will clear up with time. We know God is still in the business of doing miracles.
There are times when we look back wondering how we made it through, but we know that God promises that, "They that wait on the Lord shall renew their strength, they shall mount up with wings as eagles, they shall run and not be weary, they shall walk and not faint." We are very grateful to Dr. Morton and to God for the wisdom he has given Dr. Morton.
It is hard to believe all that Jordan has gone through when we see him running around and busy playing. He had to relearn nearly everything. The only difference we see is a small limp in his left leg, which Dr. Morton feels will clear up.
We apologize to the ones who felt neglected by all the time Dr. Morton spent on Jordan. We would never wish this on anyone. I feel inadequate in writing this, so maybe Dr. Morton will at some time write a more detailed article on his discoveries. The DNPH proved again it can be depended on as a very reliable test.
Jordan is doing great in his eating, his appetite has really increased. His favorite foods are French fries and spaghetti. I do not use any low protein foods at this time. He is now on glutamine and thiamine since coming home. Some days are still frustrating, but we are grateful to still have our son with us. May God be with you all.
Family Histories - He's Our Miracle
- Written by Glenda Groff