Enos Hoover, the father of Edith Hoover, a 16 year old with MSUD, has been instrumental in starting the Clinic for Special Children. As a member of the board of directors, he represents families with MSUD children. Following is Enos and his wife, Anna Mae's recent experience with the Clinic's services.
We have found the services of the Clinic a big help in keeping Edith's condition under control. We started using the 2,4-DNPH test regularly, and learned that her leucine was often slightly elevated (5 to 10 mg/ml). Also ph was low (5 to 7). So we started using sodium bicarbonate (20 gr/day) which keeps the urine ph at 7 to 9, and keeps the DNPH clear more often. We also cut back on formula to 14 oz./day and give 24 cal./oz. when levels are low.
When we have a blood test done at the Clinic we get results in 1 1/2 hrs. at a fraction of the previous cost. Our new grandson's negative diagnosis was made at 26 hrs. after birth! Most of the midwives in Lancaster County are now using the DNPH on all their newborns. So I believe we made progress with MSUD in the past year.
The new building is ready at this time. Much of the labor and some materials were donated. I know we would have used a local care center such as this earlier, but I suppose the time was not right yet. It is really an answer to our prayers, and we are sure it will also be a help to many younger parents and special children.
Anne Fredericks began taking her son to the Clinic and I asked her for her comments. This is from her letter.
As to your question about Dr. Morton, I feel that he has been an answer to our prayers. He is such a knowledgeable man but also a very caring man, and Jeff really likes him. We have had many problems, and he has been so supportive. We now use DNPH and check ph as well as ke-tones. I just feel I have some better ways to keep on top of his levels. He takes so much time to explain things to us and to Jeff. We're all so thankful that Dr. Morton has moved to Lancaster.
Peter Shaffer and his wife Sharon from Kentucky have used DNPH for 9 years. Peter explains how they use it.
Ever since Jessica was diagnosed 9 years ago we have used DNPH testing. DNPH is a chemical reagent that is mixed with urine. When it is negative it is a clear yellow. When it is positive, it becomes a cloudy yellow. The more positive the more cloudy. We rate our readings negative or 1, 2, 3 or 4. When we reach the 4 level the DNPH test looks almost like mustard. It is so thick you cannot see through it.
We use the DNPH as an indicator of how Jessica is doing. We do not use it alone. We also utilize keto-sticks. However, when we hit a 4 DNPH, we are also hitting the highest reading on the keto-stick.
We also pay close attention to neurological symptoms such as the loss of balance, slurred speech, drowsiness. In Jessica's case we have seen high DNPH's with no neurological symptoms (very rare) and even occasionally severe neurological symptoms, but low DNPH. Like most children, just when you have them figured out, they change.
I would like to share my experiences. (The Editor)
At the symposium in Montreal last year we learned that Dr. Charles Scriver's patients used DNPH for years. Concerning his use of the test for home monitoring he wrote, There are many ways of looking after a patient with MSUD. This is just one of them. I say this because two other major centers don't subscribe to the monitoring of the keto acid, (and there may be no difference in outcome). I think the use of the test in the home gives the parent some sense of control.
Dr. Scriver's last statement pinpoints the major advantage of DNPH as a tool in home monitoring. I have found it so helpful in my 26 years of caring for children with MSUD. Frequent blood tests were not feasible as we lived 160 miles from our medical center.
Correlating blood tests with DNPH reactions in two children over a period of time helped me learn to judge their amino acid levels quite accurately. This took much of the guess work out of daily monitoring. I could implement diet changes at the first sign of elevated levels and our children seldom needed to be hospitalized for illnesses. It also aided me in varying their diet.
I was given keto-sticks to use twice that I can remember when our children were sick in the hospital. I was disgusted because they were obviously sick, but the keto-sticks showed no reaction. Later I did get some to try at home but never saw a positive reaction. They were totally useless for us. In contrast to Peter's report, we have seen a very positive reaction with DNPH frequently without neurological symptoms, usually caused by stress or from an extra gram of protein eaten that day. The test changes rapidly, sometimes in an hour on those occasions. Evidently there is a difference in how some children respond to the different tests.
For many years I used two test tubes each time I tested urine. I would put 20 drops of urine in each and then add the reagent to only one of them. If the urine was cloudy to start with, I could watch both to see if the reagent was causing it to become more cloudy. I never had a problem with this method as taught to me by our doctor, Dr. Allen. I will always appreciate him introducing me to DNPH.
Shayla is 21 yrs. old and has been responsible for monitoring her diet for several years. I would be very uncomfortable allowing her this independence if she did not use DNPH. She cannot tell if her leucine levels are elevated by how she feels physically and has shown no distinct neurological signs with high levels for years.
Our friends, Leon and Dianne Kennedy have recently taken their second foster child with MSUD into their hearts and home. I asked her how she feels about using DNPH. Without a moment's hesitation she answered emphatically, I wouldn't even consider taking an MSUD child without DNPH! For some of us, it is a necessity.
A word of caution on DNPH
DNPH is an acid and must be handled with care and kept out of reach of children. I've been told one can get an acid burn from it. Though we've never experienced that problem in over 23 years of use, I do advise caution. Do clean up any spills immediately, as it stains easily. It should be kept in a dark bottle out of bright light, as light tends to weaken the solution.
DNPH is not a miracle worker but a very helpful aid in caring for persons with MSUD. It would be great if all parents would have the opportunity to try it if they are interested. My thanks to Dr. Morton for his efforts in investigating 2,4- DNPH as a tool in home monitoring.