Allie's Battle With FSGS
In December 2009, our then 7-year-old daughter, Allie, woke up one morning with very swollen ankles and legs. We took her to the pediatrician as soon as the office opened and the doctor's kind smile changed to a look of concern as soon as I told him about her swollen legs. He immediately took her blood pressure and told us she had a kidney condition and would need to see a nephrologist immediately. Thankfully, her blood pressure was not elevated enough to be admitted to the hospital that morning.
The nephrologist told us that the protein in Allie's urine was high as a result of a condition called Nephrotic Syndrome. She also told us that about 90% of children with this condition respond to steroids within 6 weeks so she guessed Allie would be back to normal by Christmas or New Year's. Allie started prednisone that day to stop the protein leaking. At first she responded to the prednisone and her spilling decreased, but unfortunately it only lasted a couple of days and then it was elevated again. Allie's kidneys were not filtering properly so protein was spilling out and her body was retaining fluids. We called the nephrologist just before Christmas to inform her that the protein levels had increased. The nephrologist said Allie would need to be hospitalized for an infusion to help her decrease the fluid she was retaining.
Our family went to our church's Christmas Eve service and Allie brought a blanket to keep her warm. She slept through most of the service. It was very sad to see her so tired and out of it. At 3AM Christmas morning she began to vomit and within an hour, she began to vomit blood so we had to take her to the emergency room where she was admitted. She stayed in the hospital for 4 days, missing Christmas with her brothers and extended family. That began the course of albumin infusions which would push the extra fluids out of her body. At one point, she had over 15 pounds of water weight on her small body of 40 pounds.
During the month of January, Allie had been in the hospital almost every week day, but came home on weekends only to return the next week. The doctor's continued to increase her predinose dose to try to kick her into remission, but it was not working. She became weaker and weaker each day and eventually could not walk or even hold her head up on her own.
The doctors decided it was time to do a kidney biopsy to try to determine exactly what was causing Allie's kidneys to not filter properly and leak so much protein. However Allie was in such bad shape the biopsy could not be performed until she was "shrunk" down to a reasonable size. So she was admitted again to the hospital for multiple infusions to get her down to a safe size for a biopsy. The biopsy results came back showing she has FSGS, not just Nephrotic Syndrome. Not much is known about FSGS and it is very hard to treat.
Some patients respond immediately to steroids, but then relapse once the steroid dosage is reduced. Others never respond to the steroids, like Allie, and then they move onto much stronger immunosuppresants. Once on these stronger treatments (only a handful are available and none of them are FDA approved for FSGS or Nephrotic Syndrome), some patients respond within weeks, others within months and others none at all. If there is no response, the patient will eventually move into renal failure and need a kidney transplant. One of the toughest part of this disease is that after a kidney transplant, FSGS can return within hours, days or months of receiving the new kidney and it can then destroy the new kidney as well.
With the FSGS diagnosis, Allie's doctors put her on Prograf, a very strong immunosuppresant usually given to transplant patients to prevent rejection of the new organ. It was very difficult to decide to try this medication as one of the many side effects is cancer. But we had no choice because Allie's condition was getting worse and worse. I knew my Allie was in there somewhere, but I could not see her from the outside. Her body looked so different, swollen and weak, and the joy she shared with so many was not visible. It was just heartbreaking.
Once she started began the Prograf and started to be weaned from the steroids, she began to slowly get her strength back, however she was in a wheel chair from February -April. Allie was not able to go to school the second half of her 2nd grade year. She was taught at home by an amazing home-bound teacher. Her body began getting stronger each time the steroid dosage decreased, but her blood work and lab results were not improving. In April she picked up a bug and developed strep in her blood and was hospitalized again for a week.
By the middle of the summer Allie was walking, swimming, running and returning to her old self. She was still swollen, got tired easily and suffered from multiple side effects of the medications and her lab results were still awful. But finally in August (more than 8 months since diagnosis) there was finally some improvement in her lab work. The doctors could actually give a number to how much protein she was spilling. Before then it was so high, the lab could not give it a number.
Thankfully she continued to improve until she reached full remission in October 2010. Throughout her illness she was on up to ten medications at a time. We are so happy and thankful to say that Allie has stayed in remission since October 2010 and is only on one medication right now.
We still need to be very careful to keep Allie from illnesses as her immune system is not as strong as healthy kids her age. And although she is in remission right now, we know that only about 25% of children with FSGS stay in remission. FSGS is the second leading cause of kidney failure in children.
Unfortunately, our family’s story is not unique. NephCure estimates that there are currently 19,306 people living with end-stage-renal-disease (ESRD) due to FSGS. About half of FSGS patients who do not respond to steroids go into ESRD each year, requiring dialysis or transplantation. Approximately 1,000 FSGS patients a year receive kidney transplants however, within hours to weeks after a kidney transplant, FSGS returns in approximately 30-40% of patients. We must find a cure.
Please join our efforts to help find a better treatments and a cure for these two terrible kidney diseases so other kids don't have to suffer like Allie did.
The nephrologist told us that the protein in Allie's urine was high as a result of a condition called Nephrotic Syndrome. She also told us that about 90% of children with this condition respond to steroids within 6 weeks so she guessed Allie would be back to normal by Christmas or New Year's. Allie started prednisone that day to stop the protein leaking. At first she responded to the prednisone and her spilling decreased, but unfortunately it only lasted a couple of days and then it was elevated again. Allie's kidneys were not filtering properly so protein was spilling out and her body was retaining fluids. We called the nephrologist just before Christmas to inform her that the protein levels had increased. The nephrologist said Allie would need to be hospitalized for an infusion to help her decrease the fluid she was retaining.
Our family went to our church's Christmas Eve service and Allie brought a blanket to keep her warm. She slept through most of the service. It was very sad to see her so tired and out of it. At 3AM Christmas morning she began to vomit and within an hour, she began to vomit blood so we had to take her to the emergency room where she was admitted. She stayed in the hospital for 4 days, missing Christmas with her brothers and extended family. That began the course of albumin infusions which would push the extra fluids out of her body. At one point, she had over 15 pounds of water weight on her small body of 40 pounds.
During the month of January, Allie had been in the hospital almost every week day, but came home on weekends only to return the next week. The doctor's continued to increase her predinose dose to try to kick her into remission, but it was not working. She became weaker and weaker each day and eventually could not walk or even hold her head up on her own.
The doctors decided it was time to do a kidney biopsy to try to determine exactly what was causing Allie's kidneys to not filter properly and leak so much protein. However Allie was in such bad shape the biopsy could not be performed until she was "shrunk" down to a reasonable size. So she was admitted again to the hospital for multiple infusions to get her down to a safe size for a biopsy. The biopsy results came back showing she has FSGS, not just Nephrotic Syndrome. Not much is known about FSGS and it is very hard to treat.
Some patients respond immediately to steroids, but then relapse once the steroid dosage is reduced. Others never respond to the steroids, like Allie, and then they move onto much stronger immunosuppresants. Once on these stronger treatments (only a handful are available and none of them are FDA approved for FSGS or Nephrotic Syndrome), some patients respond within weeks, others within months and others none at all. If there is no response, the patient will eventually move into renal failure and need a kidney transplant. One of the toughest part of this disease is that after a kidney transplant, FSGS can return within hours, days or months of receiving the new kidney and it can then destroy the new kidney as well.
With the FSGS diagnosis, Allie's doctors put her on Prograf, a very strong immunosuppresant usually given to transplant patients to prevent rejection of the new organ. It was very difficult to decide to try this medication as one of the many side effects is cancer. But we had no choice because Allie's condition was getting worse and worse. I knew my Allie was in there somewhere, but I could not see her from the outside. Her body looked so different, swollen and weak, and the joy she shared with so many was not visible. It was just heartbreaking.
Once she started began the Prograf and started to be weaned from the steroids, she began to slowly get her strength back, however she was in a wheel chair from February -April. Allie was not able to go to school the second half of her 2nd grade year. She was taught at home by an amazing home-bound teacher. Her body began getting stronger each time the steroid dosage decreased, but her blood work and lab results were not improving. In April she picked up a bug and developed strep in her blood and was hospitalized again for a week.
By the middle of the summer Allie was walking, swimming, running and returning to her old self. She was still swollen, got tired easily and suffered from multiple side effects of the medications and her lab results were still awful. But finally in August (more than 8 months since diagnosis) there was finally some improvement in her lab work. The doctors could actually give a number to how much protein she was spilling. Before then it was so high, the lab could not give it a number.
Thankfully she continued to improve until she reached full remission in October 2010. Throughout her illness she was on up to ten medications at a time. We are so happy and thankful to say that Allie has stayed in remission since October 2010 and is only on one medication right now.
We still need to be very careful to keep Allie from illnesses as her immune system is not as strong as healthy kids her age. And although she is in remission right now, we know that only about 25% of children with FSGS stay in remission. FSGS is the second leading cause of kidney failure in children.
Unfortunately, our family’s story is not unique. NephCure estimates that there are currently 19,306 people living with end-stage-renal-disease (ESRD) due to FSGS. About half of FSGS patients who do not respond to steroids go into ESRD each year, requiring dialysis or transplantation. Approximately 1,000 FSGS patients a year receive kidney transplants however, within hours to weeks after a kidney transplant, FSGS returns in approximately 30-40% of patients. We must find a cure.
Please join our efforts to help find a better treatments and a cure for these two terrible kidney diseases so other kids don't have to suffer like Allie did.
