Our Story
RYAN's STORY
Ryan's first episode of Hemolytic Uremic Syndrome (HUS) happened when he was 10 months old. He was diagnosed with the Typical form of HUS caused by E.coli. Ryan was on a strict fluid restriction, which only allowed him to drink what he had put out in urine. He did not need any other treatments at that time. That hospital stay was a mere 7 days. The nephrologists closely monitored him. At first he was going every couple months, eventually we were only going twice a year. The typical form of HUS doesn't recur so we were only monitoring him to be sure that there was no permanent damage to his kidneys. The only sign of any problems was that he had protein in his urine, but the doctors said that was normal for a child with his medical history.
On July 5th, 2002, about 2 years after his first episode, our lives changed forever. For the previous week, Ryan had been sick on and off. He had vomited a couple of times, but always acted fine afterward. It had been an extremely hot week, so I figured he was just playing too hard for the weather. On July 5th, Ryan woke up later than normal around 10 am. When I changed his diaper I noticed his urine looked a little dark so I called his doctor to have him checked out. We went to the doctor that morning. The doctor had said that everything looked fine; he probably had a touch of the stomach bug, possibly a urinary tract infection. She sent us home with instructions to have a urinalysis and blood test done the following day. When we got home from the doctor's Ryan wanted to take a nap. He had only been up for a couple of hours and was tired again. I decided that I wasn't going to wait for the following day for lab tests. I had seen him act the same way when he was 10 months old and I was not dismissing his illness for something less. We went to the emergency room. I refused an IV, which the doctor wanted immediately thinking he was dehydrated. I had a gut feeling it was his kidneys again and knew that last thing he needed was fluid. I wanted lab work done before any IV was placed. We waited about 30 minutes for the lab results. Just from looking at the doctor's face I knew my gut feeling was right on target. Ryan's BUN was 125, creatinine was 1.3, his platelet count was 50,000, LD was over 4,000. Ryan had HUS again, but we couldn't understand how because typical HUS doesn't recur.
That's when we were told that Ryan has Atypical HUS. That day Ryan had a catheter placed for dialysis and plasmapheresis. The protocol for a child was to do dialysis as needed and pheresis every day for a week. That's exactly what was done. He had 5 days of dialysis and 7 days of pheresis. He was in the ICU the entire time. After the first couple of days my husband and I took turns staying over so one of us could be home for our younger daughter. Things started to improve with each day of treatment. We remained in the ICU, even after his numbers began to improve because of his blood pressure. There were times his blood pressure was as high as 180/120. They had to put a nasal gastric tube in for nutrition and because he refused any meds by mouth. By July 19th we were moved onto the floor, out of the ICU. His blood pressure was still high, but he was stable. We were discharged on July 26th. Ryan was on about 5 different medications for his blood pressure and vomiting. We were to follow up with the nephrologists twice a week.
Everything was going well. His numbers remained within their limits, blood pressure was stable. We went to two follow up visits with his doctors. His lab work on his visit August 1st looked good. A mere 2 days later it all started again. He began vomiting and acting lethargic again. We went to the emergency room on August 3rd. This time they placed a permanent catheter for pheresis and dialysis. He only needed dialysis for the first few days. He had pheresis everyday over the next several weeks. His blood pressure was very hard to keep stable. He was on so many meds it was hard to keep track of them all. We skipped one day of pheresis after about 3 weeks, so he was now on a 6 times per week pheresis schedule. Again my husband and I took turns staying overnight with him so we had very little time together. We spent 5 weeks in the hospital this time. The first 3 weeks were in the ICU and the following 2 weeks were on the regular floor. I followed his numbers very closely. It seemed like they were stuck and not getting better for the longest time. His blood pressure had been stabilized so we finally convinced the doctor's to discharge Ryan. We went home on September 8th.
We were on a strict outpatient schedule with pheresis 6 times a week. We tried to decrease frequency of treatments a couple of times and his numbers got worse. We tried plasma infusions and his body didn't tolerate that, his blood pressure would go extremely high with that extra fluid. We did have a couple of hospitalizations, just a couple days here or there for increased blood pressure or to try a new medication. Ryan also had a G-tube put in on October 23rd. It wasn't until November that we tried skipping treatments again.
It is now January 2003, six months since the start of all this. We're down to pheresis 4 days a week and hoping to try 3 days a week pretty soon. At one point Ryan was on 14 medications, he is now on 5. Ryan has a record in the pheresis unit at the hospital we go to. To present date he has had 127 pheresis treatments. There were times when I thought that things were never going to get better, but I was wrong. It is the hardest thing a family could ever deal with. the doctor's don't have all the answers to your questions you drive yourself crazy with what ifs and whys. When I am at the end of my rope, ready to throw in the towel; I take a look at Ryan. He is the one that is actually going through this illness, being hooked up for phereisis for 2 ½ hours 4 days a week. When I need a pick up I just look at his adorable little face and see that he still has a smile. He's laughing, he's playing, and he is showing the world that he is going to beat this thing. It may take his whole lifetime before there's any better treatment or even a cure for this illness, but Ryan is not letting it beat him. He wakes up each day and he may only be 3 years old, but he knows he's sick, he knows he is different from other children. It doesn't stop him. It doesn't slow him down. If this illness hasn't stopped him, I will not let it stop me.
We will beat this.
Nathan's Story
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. We took him in after he was growing more and more lethargic.
The HUS diagnosis was made very quickly. He was admitted for 6 weeks. Plasma and transfusions were the primary treatments at this time.
Month 4
Within a week, Nathan was back in the hospital. This time, he developed kidney problems, along with high blood pressure. Dialyis was needed, along with 4 different high blood pressure medicines. Plasma infusions continued each day.
THis continued for another 6 weeks. Nathan was then taken out of the hospital, and plasma was continued as on outpatient.
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 appearred 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 1st 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 Blood numbers twice a week..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 that year one....Only 94 days in the hospital (only?) And 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
Year 3-6
Very much like year 2.
Year 7:
While Nathan's overall health has improved, he finally lost the battle to the kidneys, and we are now on Peritoneal dialysis for him.
If you have a story you would like to share, E-Mail it to SaturnRacer1@cs.com
AMANDA's STORY
Our youngest daughter of three Amanda was diagnosed with Atypical HUS at the age of 5 weeks old. We first noticed her appetite decreasing, some vomiting and diarrhea. We took her to her pediatrician where she got some lab work, and was sent home. Her appetite had decreased to nothing and she was not drinking at all, so we called her pediatrician at home who instructed us to give her some pedialyte, and call if vomiting persists. She refused to drink anything and became lethargic, so we took her to the emergency room, and they admitted her for anemia, jaundice, and extreme dehydration. After two days in our local hospital she was diagnosed with HUS, and airlifted to the University Of Chicago hospital where she stayed for approx. 3 weeks. She was given several transfusions of Packed RBC, Fresh Frozen Plasma, and was started on the medicine Lasix. She suffered very little kidney damage this time and recovered completely. It wasn't until she was 5 months old that she had her first recurring episode. After being admitted to U of C again, she was given more RBC, Platelets, FFP transfusions, and an increase in BP meds, iron, and vitamins through an NG tube. After 2 1/2 weeks she was released, and her kidneys had suffered more damage than the first episode. The very next day after she was released we were back in the emergency room again, as her episode had not totally subsided. She needed to be admitted again, but this time we decided to take her to Children's Memorial in Chicago, as the nephrologists at U of C were out of ideas, and answers. For the next 8 months, life as we knew it was a living nightmare. Amanda spent 3 out of 4 weeks in the hospital, and had uncontrollable high blood pressure between every episode. At this point she has had a total of 8 active episodes of atypical HUS. The worst one came when she was 9 months old. This episode shut down the kidneys completely again which left her with lots of excess fluid in her system. It quickly turned into pulmonary edema which caused her great efforts just to be able to breathe. At the worst point, her oxygen level dropped in to the 40’s when she lost consciousness and turned blue in color. They intubated her on the spot, and rushed her to the PICU where they immediately started emergency dialysis, and placed her on a ventilator. During dialysis, her heart quit beating as she went into congested heart failure. After resuscitating her, they placed her in a chemically induced coma for the next 10 days to allow her body to try and heal. Being the fighter that she is, she managed to pull through, and was released again after another 5-week stay. She was home for almost 2 months, when another episode struck. At this point her kidneys were in end stage renal failure, and completely shut down for what turned out to be for good. She started full time Pd dialysis at 13 months of age. She is still NG fed the majority of her nutrition due to her lack of appetite, and is on too many meds to list now. Because of the lack of kidney function, she hasn't had an active episode for over a year now. Amanda needs a kidney transplant, but the doctors seem confident that the HUS will definitely come back again, so we are waiting to allow her body to heal completely, and for more answers from the medical community. Amanda is 2 1/2 years old now, and is a very smart and happy little miracle baby. Since she has been home, she has only had a few minor hospital stays due to imbalances in her electrolytes, which caused her to have seizures. We thank God for her everyday and we feel very blessed that we still have her. Thank you for reading our story, please feel free to contact us with yours or any information you can share about research, updates etc. etc. You can reach us at thomas.vicari@verizon.com
Tennesha's Story
Our daughter Tannesha is now 12 years old. By the grace of God she is doing
well on dialysis at this time. My husband and I adopted Tannesha at age 5. She
was on peritoneal dialysis at the time. We were told her diagnosis was
membranoproliferative glomerulonephritis. Her kidneys began to fail at 5 months
of age. She began dialysis at age 3. She had peritonitis two times in two years,
and was placed on Hemo-dialysis. We listed her for a transplant, and I was
tested to see if I could donate. I was thrilled to be a match. On January 9th
1996 I donated a kidney to Tannesha with great hopes of a life without being
hooked up to a machine. The fifth day post- transplant Tannesha presented with
flu like symptoms. Then everything feel apart very quickly. Her platelets
crashed, blood pressure elevated to dangerous levels, red blood count quickly
dropped. I had know idea what was going on. Finally after several test a
diagnosis. HUS, what is that? Tannesha began to lose the kidney I gave
her in five days. This had nothing to do with rejection. Plasma Pheresis,
what's that? What planned to be a five day stay in the hospital, with plenty of
rest for me, turned into 2 months of confusion, fear, acceptance, and
determination.By 1998 Tannesha was in complete renal failure. We had done eight
months of plasma pherisis after her transplant with no success. Ohio would not
list her again, but the University of Minnesota did. We were told it would be a
long wait. Six months later we were called for a kidney , a perfect match.
After a 12 hour surgery ,Tannesha had a new kidney. Would the disease come
back? Yes it did , two months later. She was so sick .Plasma pheresis
again, hooked up to a machine everyday.By July of 2000 Tannesha was back
on dialysis. Her prognosis is poor. She is not transplantable because of her
atypical Hus returning again. I did not want to here that, nor accept it.
Tannesha is 4ft. tall and weighs 50 pounds. She has a leg graft for Hemo
dialysis, and a g-tube for nutrition. Her legs hurt most of the time from renal
rickets. But guess what!!! God has given her the ability to enjoy life, despite
three times a week on a machine. She is taking Horse back riding lessons,
ballet, and loves to swim. We choose to home school . That works best for us. I
met Tannesha while working in the hospital. I knew she was a gift
form God , and that John and I would adopt her. I try to enjoy her everyday,
for I do not know what tomorrow will bring. She has a strong faith
and is not afraid to die.I pray for research of this disease. It may not affect
many children,but to me one is enough.Here are some contacts you might want
to look into for information on Atypical HUS. Dr Remuzzi - GREMUZZI@marionergi.it he is a doctor
in Italy. Dr Mauer - mauer002@maroon.tc.umn . He has a
passion for research and is located at the University of Minnesota. I was told
that we spend more on lipstick in this country than on researching Atypical HUS.
The difficulty is that there are no two cases alike. If we pull our resources
together, who knows what will happen. An FYI Tannesha takes a supplement
called Juice Plus. It is food in a capsule, high in antioxidants and
enzymes. It has helped her to stay free from common childhood illnesses. If you
want any info let me know. Also if anyone has severe GI problems (abnormal
colonoscopy) similar to chrons disease, but related to HUS I would be interested
in any info.
jjtv@earthlink.net
