One Family, Three Stories
Tammie Works
870-352-8858

Jeffrey

Thinking back on the birth of our oldest son, Jeffrey, who is now 6 years old, the time seems to have flown! Jeffrey was born at 37 weeks. His birth weight was at 8 lbs 10 oz, his lungs were not developed, and he spent about 25 days in the intensive care (NICU). He spent his first week on a ventilator in a medical-induced coma. When the doctors noticed that Jeffrey could not balance his electrolytes, they discharged us and referred us to the nephrology department at Arkansas Children's Hospital (ACH), where we saw Dr. Craig Belsha. A discharge form told us to give him no water; his formula had everything he needed. What a joke!

He was drinking about 4-5 oz every 2-3 hours and not gaining any weight. We made daily trips to ACH for laboratory tests. Those were the hardest days of my life. I will never forget the day that I got the call telling me I had to bring Jeffrey back to ACH for admission. His sodium levels were running in the 170s, which was dangerously high, and they had to figure out why. After only 3 days, they narrowed the problem to diabetes insipidus and gave Jeffrey two doses of the hormone needed to correct the problem. It did not work. To their surprise, and ours, the diagnosis was nephrogenic diabetes insipidus (NDI). Until this time there had been no NDI cases at ACH. For my husband and I it was a true nightmare.

They started Jeffrey on Indomethacin and overnight he changed. I awoke the next morning at 6:30 and looked at my baby. His poor body was so thirsty. The doctors were shocked. They watched him throughout the day, and we went home that afternoon. They followed him closely in clinic with biweekly visits and lab work.

Everything was great for about 18 months. Jeffrey had no vomiting that normally accompanies the disease but he did have terrible side effects from the medication. He started complaining that his stomach hurt, and they referred us to Dr. Casteel, a gasterologlist. After following Jeffrey for only a couple of weeks she started ordering tests. She then found the problem—ulcers, bad ones. They were some of the worst she had ever seen in a child Jeffrey's age. We started the switch that day to the Duril and Amilride, but soon saw that we needed more help to control his urine output, so we added Naprosyn. The switch to the new drugs went smoothly. His sodium levels now run around 134-136, and he is currently on Zantac, Naprosyn, Duril, Amilride and Potassium Chloride.

Justin

Soon, we were expecting another boy. Our hearts sank--not because we weren’t happy for another child, but because we feared that he might have the same experience as Jeffrey. We had genetic testing; the results indicated that I had two genetic mutations that carry NDI. Jeffrey had the same mutations. Now the waiting game began.

Justin was born on January 1, 1996 and weighed in at 8 lbs 3 oz. At birth the nurses drew five tubes of blood from the umbilical cord and sent it with my mother’s blood for testing. Justin was discharged when he was only 2 days old. I breastfed him and offered him water when needed it. Within a week we received the call. Justin also had NDI, and my mother was also a carrier. I was shocked.

Justin’s case was far more intense than Jeffrey’s. Justin had reflux and therefore, little to no weight gain. We were in and out of the hospital twice a month for dehydration. We had baby scales at home and we weighed him twice daily. He would vomit all day, and we would call Dr. Belsha, who would order a "straight admit." We would be at the hospital three to five days, sometimes longer, then go home for a week or so before we would be back again. In May 1996 it was decided that he needed surgery.

Even after the surgery, the poor baby just could not seem to get back on track. We prayed for strength from the Lord and for wisdom and courage. Slowly, Justin began to gain weight and became stronger. Justin is now doing great. He now goes to pre-school twice a week. He is one of the smallest in his class, but he is a happy, active, and for the most part a healthy little boy! His sodium levels run in the 137 range?perfect! He is currently on Diuril, amiloride, Zantac, and Naprosyn.

Jonathan

Our third son, Jonathan, was born June 1, 1998 weighing 7 lbs 14 oz, but only after having a hard go of things. We were hit head-on by a drunk driver when I was 7-months pregnant. I broke my pelvis and was on a walker for 8 weeks and then a cane until Jonathan was born. The doctors were not sure for the first few days if Jonathan would make it, but he did. With the other two boys having NDI the doctors were much more cautious with Jonathan. His breathing was a little off, and he had a larger head than normal (15 inches), so they put him in NICU to watch him more carefully.

Jonathan had hydrocephalus?water on the brain?and needed brain surgery right away. I was numb inside. I thought, isn’t the NDI going to be enough for the little thing to handle? The day before his surgery I woke up and told my husband I wanted Jonathan transferred to ACH. That’s where his kidney specialist was, and they have to be in on the surgery. The doctors at ACH wasted no time in getting treatment started for the DI.

Later that night Dr. Teo, the neurosurgeon, told us that Jonathan did not need surgery at this time. He wanted to wait and watch. We were discharged under strict doctor's care. At this time he was nursing every 2 hours for 20 minutes and getting free water.

It was not long before we noticed that Jonathan had reflux. Not as bad as Justin, but still enough to cause concern. So the medications were started. We weren’t sure if it was the NDI or the hydrocephalus causing the reflux. It was very clear, though, that NDI and hydrocelpalus were not going to work well together. The symptoms of one seemed to mask symptoms of the other. Justin’s sodium levels were running a little higher than normal, but seemed to be staying steady in the 145-147 range. Otherwise he was doing well.

Dr. Teo followed Jonathan for 6 months. On December 15, 1998, we went in for a CT scan. After Dr. Teo measured Justin’s head, told us we had to proceed with surgery. We admitted Jonathan the day before the surgery for IV fluids. We were prepared for the worst. There was a slim chance that Jonathan might not make it through the surgery and even some chance of more brain damage from the surgery itself, but we had total faith in God and the doctor that He had sent our way.

We got to see Jonathan in recovery. He was ready to drink, of course! He did so well that he went straight back to his room. His sodium levels stayed in the normal range, and he went home the next afternoon! There is still a small question of whether the surgery was a complete success, but he suffered no other damage and is progressing slowly but surely. To us it was a total success!

I breastfed Jonathan for 6 1/2 months, which helped. In December we started him on Similac PM 60/40 with Polycose added in. Did he ever start drinking more! He is eating some baby foods now, nothing solid or with any form. He drinks around 64 oz of formula a day and 16 to 20 oz of water and juice. He is now 10 months and weighs 19 lb 6.5 oz with a head circumference of 20.5 inches. We see Dr. Wells every 4 weeks but we don’t have to see Dr. Teo until September. It looks like we are on the downhill side of the mountain now!

The Works Family

Jeffrey, now almost 7, takes five meds twice a day. He weighs 47 lbs and loves the outdoors. You give him a baseball and watch him go! Justin?he is still my little child. He is 3 years old and weighs a heavy 25 lbs. He eats and drinks like a horse, but he tee-tees it all out too! He enjoys life to its fullest and can make a dark room light with his personality. And Jonathan is a blessing. He always has a smile on his face. The people at our church ask us, "Is he always this happy? Doesn't he ever cry?" We just simply smile and say, "Nope! He is just a happy, happy baby!"

To my kids, their life is normal. And we like it that way. We don’t want them to be treated with special care or like they have a handicap. It is only a handicap if you make it one. We have to be watchful but, at the same time, let them live life to the fullest. The Lord has carried us through so much and it is strictly by his hand that we are as strong as we are today.

For those of you who are new to NDI, listen to your child. Look for a doctor that will listen to you and, above all, have faith. Some days are hard, but then one of my boys will come in and hug me and smile at me and tell me how much he loves me. I think "Wow. If he can keep a smile and continue to express love then surely I can too!"