Date Of Birth: December 11th, 2008

Illness: Nephrogenic Diabetes Insipidus

BIOGRAPHY

Reilly was born precisely on his due date weighing in at 7 pounds, 13 ounces and 53 centimetres long, looking just like a little skinned rabbit. However, despite adequate feeds, he failed to grow and thrive.

Our housing was a bit unstable the first 6 month of his life, but at a Community nurse appointment at 7 months he was still only 59 centimetres and 5.2 kilos. The nurse called the local paediatrician as he was so undersized for his weight, who admitted Reilly to the local hospital that day.

After a few hours in surgery with another child he came to examine Reilly. He noted he was quite little, then Reilly proceeded to pee all over him. He ordered blood tests to be taken first thing the next morning. The blood tests and urine tests showed Reilly had dangerously high sodium levels in his blood and almost no sodium in his urine. We were sent that day to Princess Margaret Hospital for Children in Perth to see the Endocrinologist who suspected Reilly may have Central Daibetes Insipidus, or less likely Nephrogenic Diabetes Insipidus, who that night being Friday, admitted him to the hospital on a liquid only diet, being at least 1 third water to bring his sodium levels down and explained not much more could be done as it was Friday night, but ordered tests to be done after the weekend. 

He had several days of a liquid only diet with blood tests every day, and even twice some days to see if his sodium was coming down. On Tuesday he had a cranial ultrasound performed, still possible as his fontanelles had not closed over to ensure there were no tumors putting pressure on his pituitary gland. Thank Goodness this was ruled out! So the following day He had a Water Deprivation Test performed (where they give him a dose of synthetic DDAVP and hold back liquids to see if urine volume goes down and concentration of sodium in it goes up). Unfortunately the test proved that the hormone made him wee more so Central Diabetes Insipidus was ruled out.

This gave us a diagnosis of Nephrogenic Diabetes Insipidus (meaning he produces enough hormone to regulate urine volume and concentration, but his kidneys do not respond to it. Therefore he is virtually unable to draw any water into his blood and any sodium out of his blood). 

We were seen by the renal dietician at the hospital who gave us advice about his diet to keep sodium in his blood to a low and after a renal ultrasound ( which also came back clear) we were discharged with a referral for a cautionary MRI in two weeks time and an appointment with the renal specialist and the neurologist in 4 weeks time. The MRI came back clear, but blood tests ordered for the morning Of Reilly's renal appointment showed his sodium levels were dangerously high again. We were told Reilly would not be going home that day and he was admitted to the hospital again, and placed on a dextrose drip to try and balance his sodium levels carefully (while his levels were high enough to cause brain damage or death, it is also dangerous to bring sodium levels down too quickly. Likewise, a normal saline drip can prove fatal for Reilly).

After the weekend on the drip and having to be recanulated several times, (his veins were tricky due to the hypernaetremic dehydration) his levels were not dropping enough, the specialist decided it was time to try him on a medication Hydrochlorothiazide and he responded to it immediately. So after another 7 days in hospital we were discharged with a presciption for his medicine and more dietary advice, and an invitation to call the renal nurses and renal dietician as needed.

We have had an adventure with Reilly and we cannot take risks. If he gets sick and has vomiting and/or diarrhoea, he needs to be taken immediately to the local hospital for blood tests to monitor his sodium levels. If they are too high he needs to be admitted to hospital on a dextrose drip to keep him hydrated. Also he needs extremely careful monitoring of his diet. He is on a daily allowance of 500 milligrams of sodium. He is on a newborn formula that takes up 40% of this so in terms of food this leaves 300 milligrams of sodium. There are certain foods that are completely off limits - almost ALL takeaways, tuna, baked beans. One vegemite sandwich would account for his TOTAL daily allowance of sodium. However he loves fruit, especially the squishy, juicy varieties. He is starting to grow a little, was 8.27 kilos at his last check up and 69 centimetres, however he is still only the size of a 8 month old and is quite delayed in his development also.

We go every 3 months to see his Renal doctor in Perth for monitoring and Reilly is receiving physiotherapy and occupational therapy to assist with his delayed development. I really don’t want to think about where we would be if our clinic nurse had not picked up just how small Reilly was for his age and am extremely and eternally grateful to EVERYBODY involved in Reilly's care, they have all had an input into the little darling he is today.