Month 1 -3
Our son Nathan was a healthy boy up to 11 months old. He developed what appeared to be the flu, but continued to grow worse. Initially, we took Nathan to his pediatrician's office. The pediatrician then sent us to a lab for blood work. We were waiting to hear from the pediatrician what the lab report revealed, but the following day we received news that the blood had clotted and blood would have to be redrawn. The pediatrician anxiously urged us to take Nathan to Cardinal Glennon Children's Hospital, "Where they know what they are doing!". We took him in with his brother who had an early morning appointment with Glennon's orthopedic department. The nurse in orthopedics took one look at Nathan and asked what was wrong with him...I told her that after Joey's appointment, we were to take Nathan to the Lab for blood work. She then informed us that Nathan needed to see an emergency room doctor ASAP. She then made arrangements for Nathan to be seen immediately...long hours later after desperate attempts to draw blood and put in a Central Line, which they finally succeeded to do in PICU Surgery, a diagnosis was made very quickly. We were introduced to Dr. Craig Belsha, who gave us as much information as he possibly could, explaining over and over again what was happening and how we were going to attempt to arrest the progress of the disease. Nathan's kidney function had fallen to approximately 10% and he was seriously dehydrated. He was admitted for six weeks and was ready to leave when he caught Roto Virus; this set him back another six weeks. Plasma and transfusions were the primary treatments at this time. Also during this time, I noticed his right leg turning purple and reported it the the resident, after an ultrasound, we discovered he had developed a blood clot in his leg. This was treated with Coudamin for several months, which interfered with his ability to get well also. After several months, he was taken off Coudamin as the clot had shrunk significantly. When Nathan was finally discharged after approximately 12 weeks, the plan was to go home, leave the Central line in his neck, and come back in three days for labs...
Within a week, Nathan was back in the hospital. This
time, he developed kidney problems, along with high blood pressure. His blood
pressure wasn't responding to the oral medications, so they had to be given
through his line. Dialysis was needed so the surgeons inserted a peritnatal
dialysis tube in his tummy, along with 4 different high blood pressure medicines.
Plasma infusions continued each day twice a day, some days he also received
packed red blood cells. This continued for another 6 weeks. Nathan was then
taken out of the hospital and plasma was continued as an outpatient every
other day.
Month
6
Within 3 weeks, Nathan was taken back to the emergency
room... He had developed an infection from his central line, and a high fever
developed quickly. Within minutes, his fever was up to 105. Seizures started
to follow, and it took two hours to stop the seizures. No kidney problems
occurred, but the high blood pressure continued for some time, and a slight
paralysis developed, which may or may not be temporary. Plasma is being continued,
and it seems to keep the HUS at bay.
Month 7
Back in the hospital, but using plasma to head off what appeared to be the start of a problem...Successful. No full blown HUS episode. Paralysis has subsided, anti-epilepsy drugs are now being used, along with 4 high blood pressure medicines Summary of First Year 182 Days in the Hospital 70 to 80 of those days were in ICU...very frightening at times
Summary of First Year
182 Days in the Hospital 70 to 80 of those days were
in ICU...very frightening at times
Year 2
We are now very proactive, and run his labs up to three times a week, twice a week when he seems to rebound. We have been able to prevent life-threatening Atypical HUS attacks...while we have been in the hospital a lot, the extreme severity has been avoided...the good news is that we have saved the kidneys...the bad news is that in saving the kidneys, the HUS has a place to clot, and it results in Recurring HUS attacks.
Year 2 Summary
A much better year then year one....only 94 days
in the hospital (only?), only 9 days in ICU unit. Average week consists of
3 plasma transfusions a week...when hospitalized, 14 plasma transfusions a
week...Still on high doses of high blood pressure meds....about 23 doses of
various medicines administered daily. It had been decided to remove the double-lumen
broviach, (his Central Line), and replace it with a port that fits just under
the skin and is used to administer his treatments, drugs, what ever he needs.
It is supposed to be safer that a line that hangs outside of the body. We
can attest to that as one time in the PICU the nurse was attempting to insert
TPA in one of the lumens, (this is used to clear blood clots from the lines),
and the line sprung a leak! Surgeons were quickly summoned to repair the damage
as this would mean Nathan couldn't receive his blood pressure medicines for
four hours...if his blood pressure continued to climb, they would have to
perform emergency surgery. The angels were with us that day and all was well...but
for a while, this time at home, crawling around on the carpet he had sweat
so much the tape securing his broviach had come loose and he kneeled on the
l ine and it burst, spurting blood everywhere! The emergency room nurses must
have had a good laugh out of my solution...I tied it in knots and put a clothes
pin on each end to secure it to stop the bleeding! In this second year he
also discontinued dialysis but the tube was left in "just in case", after
six months, one day he uncapped the tube and as luck would have it, came down
with peritinitis...we removed the tube at this time.
Year 3 Summary
Each year brings Nathan more strength. This year he had completely fallen off the growth chart for three year old boys and the only thing we could get in him was half and half, yes the stuff you put in your coffee...lots of calories but a kidney that kept freaking out over little things like potassium, calcium and oh yes, protein. We had to treat the half and half to keep Nathan healthy and finally even this wasn't enough. We had a feeding tube put in his stomach, after about six weeks the tube was removed and another "permanent" device called a Mickey Button replaced it. We now feed him four times a day through this "button" and a nine hour feeding at night, a special formula made by Nestle called Renal-Cal.. By the way Nestle is a great compassionate company; it seems the insurance companies don't pay for formulas to keep the kidneys healthy so Nestle very generously supplies Nathan with four cases of formula a month. I've no idea how many hospitalizations we had this year, much less than last as the doctors became convinced Nathaniel's frequent, severe line infections were imbedded in the port itself and couldn't be exterminated. Therefore, the port was removed and a new one replaced the old on the other side of his chest. This and the consistently steady diet that is optimal for kidney patients have made an incredible difference in Nathan's health. P.S. he's back on the growth chart! Oh! We also received permission to go to Florida for a one week vacation...Nathan wasn't allowed to drive from Missouri, he had to fly...and we were loaded down with information on the nearest children's hospital and the local nephrologists, but we went without incident, a good time was had by all.
Year 4 Summary
Last year we attempted to go 10 days between plasma infusions and relapsed into an episode in November, the day after Thanksgiving. We finally got back to every other day on treatments and came home just before Christmas! We had one bout of diarrhea that resulted in a short five-day stay, but it has been clear sailing ever since. You wouldn't believe his labs now...we have been off of Epogyn, (a hormone to help produce red blood cells), now for several months and most of his blood work looks great, for an ATYPICAL HUS patient We were able to travel again this summer and just came back from a car trip to the Rockies in Colorado . Nathan now weighs 18.3 kilos and is looking like the chubby, toddler, picture of health. We are going to occupational therapy as Nathan's skills are quite behind, he is making progress, but the therapist thinks there might be neurological reasons for his difficulties with fine motor skills. We are now again attempting the ten day spread between transfusions---this is our first week, we look for success because his numbers are so much better than last year when we attempted the change. It's nearing the end of July, and Nathan is enrolled in preschool for this upcoming year...we weren't able to meet the doctors' criteria of no more than seven kids in the class, but as he is doing so much better, we are praying for the best.