Joseph's Story
The Reason for The Diabetes Insipidus Foundation, Inc.
So many people have asked me about why The Diabetes Insipidus Foundation, Inc. was started and about our DI child, Joseph.   I was encouraged to write his story for the website by many people. 

Here is Joseph's Story....

Joseph is our 3rd child out of 5 children. He was born in 1988. This is his story and he is why The Diabetes Insipidus Foundation, Inc. was started.

He was a planned homebirth, however our midwife did not make it in time and Joe delivered our son after after a 2 hour labor. The only problem that I had was high blood pressure during the last several weeks of the pregnancy. His labor and birth were normal, albeit quick. He was our smallest baby at the time, weighing in at 8 pounds 10 1/2 ounces. He was beautiful! Chunky and round and blonde. The epitome of perfect health. He also was never separated from me at birth or anytime there after. He went everywhere I went, including to work and meetings. He never took a bottle, pacifier or sucked his thumb. I was IT!

He took to the breast right away and loved to nurse. At the time, I was still nursing his older sister, who was just over 3 years of age. She began to complain that he "took all of the milk" and left her none as I nursed him first. I decided that she needed to be wean after a few months of this complaining from her and bribed her to stop with a Barbie doll!  I attended La Leche League meetings with him.and would mention frequently how often he nursed. I was told that babies like him are considered "barracudas." He would nurse every hour to hour and one half all day and stay latched on all night, even in deep sleep. If I took him off of the breast, he would root to get back on. (Can you imagine if he didn't sleep with us how many times in the middle of the night we would have had to get up so that he could nurse??!!) He peed so much that he soaked through 3 clothe diapers and 1 diaper doubler at one time. (A fun date was going to the grocery store and studying diaper doublers and how many we would need in a week!) We learned to keep two sets of sheets on the bed separated by a cotton rubber pad. One of us would strip the bed and the other the baby.

I would joke with my sisters about him nursing so much and peeing so much. His diapers weighed pounds and were always wet and never had the ammonia smell associated with a baby's wet diaper.  Many times Joseph would nurse until he projectile vomited. As soon as he vomited, he wanted to nurse again. He was not a colicky baby, instead he was a contented, happy child who just liked to nurse a lot!! In fact, he refused solid foods until he was 14 months old. I knew, however, that with a breastfed child who is exclusively breastfed, that he still got all of his nutrients that he needed.  I also believe that if he had not been exclusively breastfed on demand he would have suffered with dehydration.  He was 28 pounds at a year of age! (Our others were between 22 - 24 pounds at a year.)

At 29 months of age, we decided it was time to wean and potty train him. Now mind you, we already were very much aware of the fact that he nursed often and peed so much that his diapers weighed pounds! However, that first week brought great concern and some shock with it. He was asking for water every 15 minutes! Downing gallons of water in a day!   (Can't imagine the gallons of milk that I must have made in a day in order to sustain him!)  And, he was peeing every 15 minutes! Clear pee that never seemed to stop coming!

"Oh my gosh! He has diabetes!" was the first thought through our minds. We called our pediatrician. Dr. Arnold Brenner said to bring him in right away. He did a urine test and it came back negative. Then, Dr. Brenner told us about diabetes insipidus and that he thought that this is what Joseph has. And, he explained that it was NOT sugar diabetes/diabetes mellitus/diabetes that we always think of when we hear the word diabetes.  What relief was through me! No insulin! No horrors of "diabetes." He sent us home to monitor Joseph for a week or so and then had us try the DDAVP nasal spray on Joseph. It worked! No more drinking and peeing every 10-15 minutes around the clock! Joseph was actually potty trained at 2 1/2 years of age, including night time, thanks to the DDAVP. This is also the time that Joseph began to speak words other than "baby talk."  (Our other children spoke in sentences from 13 months of age to 18 months of age.)

Now the real fun begins....MRI's, water deprivation test, doctor to doctor and scheduling everything. He started at going for MRI's every 3 months, then every 6 months, then every year, now every other year (to make sure that there is no tumor growing). The water deprivation test showed a positive response for Central Diabetes Insipidus. It was the worst day of my life! Thank God, my husband was there. The child had just turned 3 years old. He cried, he screamed, he begged for "a drop of water." He got on the floor and pounded it. It was heart wrenching. I had to leave at one point because I couldn't stand the agonizing screams from him. My husband stayed. Finally, it was over! Blessed relief. He was given his DDAVP and all was right with the world!

At the age the 5 years and 2 months, the decision to place him on injectable DDAVP was made as the nasal spray had lost its efficiency for him. At 5 years and 3 months of age, he was drawing and injecting himself.  He was learning the letter in his name and recognizing the numbers from 1 to 7. Prior to this he could not identify one letter in his name or any numbers. We went under Child Find (local school system) for testing for learning problems. They said that since he could zip a zipper, tie a shoe, and button a button that there was no problem. We knew that there was. He couldn't remember anything. We were ecstatic that he was FINALLY learning!!

I had contacted Dr. Roberta Diaz Brinton, a scientist who researches vasopressin and memory on the cellular level. She was a Godsend in describing how vasopressin affects memory. She explained so much to us, including why Joseph did not dehydrate after receiving his only DPT shot at 2 months of age. He had a spiked fever, high pitched crying/screaming and slept for 4 days, only rousing to nurse occasionally. I had mastitis from him not nursing much.

We went to see Dr. Gary Robertson that June. Joseph was 5 years and 4 months of age. He was taken off his DDAVP for several days of testing. He was diagnosed as an "idiopathic (no known cause) severe central DI." Several of our family members were tested and no one is a carrier. So, Joseph was either born with it or developed it in early infancy. No one knows for sure.

Dr. Robertson got his dose adjusted to just about half of what he was on. But, during this time of being off Joseph had lost his ability to recall what he had learned. After he was put back on his needles, he started to be able to recall what we had taught him.  When we got home, Joseph was having behavioral problems that had been alleviated with the injections and still some memory difficulties.  Three weeks after we got home, we had to increase his dose a little and within five minutes of that increase we had a totally different child.  Calm, cool and collected. We were joyous! Not only was his drinking and urination under better control, so were his behavior and memory.

He entered Kindergarten that fall. The school district did not want to deal with either the learning problems or his DI. They said that they had no means of providing ice water for him and the teacher and staff really did not understand diabetes insipidus. They had a very cavalier attitude about it. He went the first day and then was put on Home and Hospital instruction (a teacher came to the house at the school district's expense). That worked out great as he got to use the bathroom and get water as he needed it without any argument.  He went through academic testing and the school said that there were no learning disabilities with Joseph. They had withheld test results that showed that there were. We decided on homeschooling Joseph for 1st grade.

After one year of homeschooling Joseph, and the stark realization of the fact that he was remembering what he had been taught and was having great difficulty learning to read, I appealed to the Mr. Richard Steinke and his secretary, Sarah Tate, (Maryland State Board of Education) for some help.  A few days later, Dr. Robin Church, director of the Kennedy Krieger School (part of the Kennedy Krieger Institute which is part of Johns Hopkins Hospital System) was visiting with Mr. Steinke. She took over dealing with us, set up testing with Dr. Martha Denkla, an expert on learning disabilities and ADHD, and had Joseph tested. We took Joseph in off of the DDAVP (he was to have been tested on it and then again off if but the school district, at the last minute, decided against it). He was drinking and peeing every 20 minutes around the clock for 4 days, including during the testing. Dyslexia (which runs in the family), ADHD and memory difficulties were diagnosed. Joseph began Kennedy Krieger several months later.

Kennedy Krieger wrote an IEP (Individualized Education Plan) for Joseph's learning problems, and also including a plan to deal with his DI.  He is allowed to go to the bathroom and drink water as needed. He is the last pick up in the morning for the bus and last drop off in the afternoon and he is allowed to get off of the bus in the morning to go to the bathroom as well as drink water on the bus. He does his morning needle in school and they are aware of his DI problems and help in making sure that the DI is managed during school hours.

Joseph also has constant pupil dilation, wears glasses, has a constant hand tremor, gets overheated easily, when he exerts himself he gets red in the face and you can feel the heat rising off of him, and has a low frustration level when it is "needle time." He is also overweight and seems to be hungry much of the time.

In July of 1996, we made the decision to try him on the tablets. He started out on the .1 pills. Within a week, he was complaining that he couldn't see. I took him to the eye doctors and his eyesight had changed considerably, to the point where they were just amazed and he needed a new prescription for glasses. He had to go back every 2-3 months to be checked. Finally, in April of 1997, after an increase in memory problems (including in school), behavior difficulties, changes with his eyes, and a significant increase in urination and thirst, we opted to have him go back to his needles. He was actually asking to go back as he said that he felt better. He was up to 15 - .2 DDAVP tablets a day. He was put into Johns Hopkins and put back on his needles. What blessed relief for all of us!!

His teachers noticed a change immediately. He was not as "sluggish," he was more alert, had stopped forgetting what he was doing in the middle of an assignment, less drinking and peeing and felt so much better. In fact, his eyeglass prescription was changed again, for the better!  He still goes for MRI's, is seen by Dr. Claude Migeon, head of pediatric endocrinology at Johns Hopkins, and is GROWING!!. He takes L-arginine on and off along with his DDAVP. It appears to decrease his thirst and urine output as well as his pupil dilation and hand tremor (all of which become better on the DDAVP and much better along with the L-arginine). Dr. Brenner monitors that aspect of Joseph's DI.

He still attends Kennedy Krieger and is learning by leaps and bounds.  He is in art therapy to help him cope with the chronic illness. You can tell when it is needle time or if he is late on a dose as his frustration level decreases markedly and he can't seem to remember things as well. He still draws and injects himself which gives him the freedom to spend nights away from home or go to camp or various programs. We keep medication at places that he regularly visits: grandma and granddad's, church, school, etc.... We make sure that wherever we go we carry 2 bottles of meds, extra needles and alcohol wipes in case a bottle gets broken or the box is empty.

He wears a medic alert and we always explain why he needs a bathroom. We carry a case of water in the car so that there is water all of the time in the summer. In the winter, we just carry one bottle. And, we explain to every doctor who has seen him outside of his DI, that he has this and that it has NOTHING to do with insulin.
His DDAVP runs about $1200 per month without insurance (we have medical insurance through my husband's job) and we are NOT on an HMO.  He has never dehydrated severely except under hospital testing conditions. We have been very blessed to have wonderful doctors who suspected the DI immediately and are knowledgeable and understanding about DI. Doctors who have been willing to help us in our quest to make Joseph's life better. And, family who understands and helps (especially Grandma and Granddad who babysit the younger children when Joseph has to go to the doctor).

Joseph still attends Kennedy Krieger, who have been absolutely wonderful, and is doing very well there. We still deal with the "dumb, stupid needles" when he gets angry with having to do a needle. It is not easy at times raising a DI child. So many things must be balanced, even on good days.

Since Joseph's birth, we have gone on to have 2 more children. When the fourth came, we had not gone through genetic testing yet. Anytime it appeared that she was nursing "too much" we kind of went into a mild panic: "Oh no! Another DI!" That, thank God, is not the case. It is tough enough at times with only one DI child.

We don't know what the future will bring in terms of his ability to provide medical care, insurance or learning. Out biggest fear is that the DDAVP will stop being as efficient as it is as he grows (he is already on a high dose), and what kind of job opportunities he will have that will understand this disease and accommodate him. We just thank God, that he has caring doctors, teachers and a loving family who do understand right now.