In August 2007, DJ Kuras went to a new pediatrician for his school physical. The doctor noticed that DJ had not grown in two years. He had us call University Pediatric Endocrinology to set up an appointment to check on levels of growth hormones. They could not see him until February 2008.
The pediatrician ordered the needed blood tests, including some extra tests just to be sure of everything. While DJ was on holiday break in December, we took him for the blood work. Two days later we were called by the pediatrician’s office, his blood work showed that his cholesterol was over 900 and his kidneys were failing. They asked us to have more blood drawn to verify results. Two days later we were again called; same results and we needed to see the pediatrician immediately. The pediatrician told us he was leaving the group we were at, but, that a new pediatrician was there and the two of them checked DJ over. They found that his liver and spleen were so enlarged; they were now on the outside of his ribs. His blood pressure was over 200. They sent us to the University Pediatric Nephrology, Gastroenterologist and Genetics Departments associated.
The Nephrology Department, felt it was a form of nephronophthisis, they were not sure what type. They would do blood work regularly to watch levels of kidney function. They wrote scripts for the kidneys and his blood pressure.
The Gastroenterologist Department did not think children could actually have cholesterol that high and the problem was due to kidney function. They would only check his blood work once a year to make sure nothing else went wrong.
The Genetics Department would be sending blood samples to labs around the country to figure out what exact genetic mutation DJ had; giving them the knowledge to know which form or type of nephronophthisis he had.
Endocrinology found DJ had no growth hormone and with the kidney failure, growth hormone injections were started on a daily basis. They also started him on a liquid cholesterol medicine, which tasted horrible and didn’t really help. The doctor arranged a meeting with cholesterol specialists from around the country and they started DJ on a new medicine. It did bring his cholesterol down to the 300’s.
All of the above departments and his pediatrician continued watching his blood work and his blood pressure.
With watching his diet and taking the medicines, his liver and spleen did shrink enough to go back under is rib cage. They are still larger than normal, but, manageable. He is allowed to play sports again, so long as he wears a hard abdominal protector he got for lacrosse.
DJ was practicing lacrosse with his summer team. We had to get him off the field, go home to pack and get to Strong as soon as possible. DJ freaked at first, realizing he had to go for major surgery and when he woke the next morning, he would have someone else’s kidney in him. When he finally realized it would be the best thing for him, we had to get going. He let his friends and family know we were leaving. We left a message for the school as the next day was graduation day for him from 6th grade; he would be going to the Jr/Sr High School the following year. He was also scheduled to get an award from NYS at the graduation.
We arrived at Strong late in the evening and a bed was waiting for DJ. Around 2am, they took him to the surgical unit. We said our goodbyes and DJ said “let’s get this over with” and they took him away. We waited upstairs and a nurse called us several times to say everything was going well. At 5:30am, they called to say the surgery was finished, the kidney was already working and they would let us see him in a couple of hours.
DJ went to the PICU unit to be monitored. He was doing well, except for the fact that he was losing blood all day. They had to keep giving him transfusions. The doctors explained that after major surgeries, there could be leakage and a blood clot. They would go in to repair any leaks and remove the clot.
The morning of 06/24/10, the nurses give DJ his meds and another transfusion. His blood pressure was still up so he was given more blood pressure meds. DJ was taken to the prep area for surgery, his medical history; allergies, meds given, etc. were reviewed by anesthesiologists, the surgeon and the nurses. I did tell everyone that he had gotten the extra dose of blood pressure medicine just prior to coming down for surgery. We were told to go to the Pediatric Waiting Room and someone would let us know when they were done and how things went. We waited and waited and waited. After two hours, I went to the receptionist and asked if she knew anything. She did not. We could not get any phone calls as the waiting room was in the basement and only phones with Verizon service would work. I heard a “Code Blue” page for the surgeon who was removing the clot from DJ, so I went to the receptionist again and asked for update. She thought they must be done and heading right back to the PICU unit. We left the basement and headed to PICU. On the way we met the surgeon and the head of the anesthesiology department. They were looking for us, there was a major problem. They did not hold him in recovery, and were returning DJ to the PICU where he would be kept for several days. As the elevator reached his floor and the doors opened, DJ went into respiratory arrest and it took them 3 minutes and 50 seconds to get DJ back. They used his “A Line” in the main vein in his neck to administer medicine and CPR to bring him back. He was placed on a ventilator and had a breathing tube and penile catheter inserted. They are not sure what damage may have occurred with him gone for that length of time.
As his blood pressure was up and they forgot he had just gotten a second dose prior to surgery and they gave him more blood pressure medicine. They also, gave him more fluids through out his body, which is done after major surgeries, but after seeing pictures of DJ before surgery, they realize he has too much fluid in his body.
After this, changes are made in procedures. All parties involved in prep and surgery, will recheck all medicines given, verify original weight of patient prior to giving them fluids and the patient will remain in recovery even if the are going back to PICU. The phone system has also been upgraded to allow service for all service providers through out the hospital.
For ten days, DJ had the breathing tube and was on the ventilator. Medications are given to keep him “asleep” so he will not fight the breathing tube. He is allergic to the main anti rejection medicine and they have to find a replacement drug. They also start to decrease the fluid in his body. The new medicine is working and he has no reaction to it. After the ten days, they remove the breathing tube and catheter. They also remove him from the ventilator to allow him to wakeup. He is fine; he can breathe easily and on his own. The only damage they can find is through the blood work and with his liver function. Over time with meds and diet they feel it should return to his normal.
The next day he is moved to a regular room. The family is taught how to take blood pressure readings and clean the incisions. I have to contact BC/BS regarding the new anti rejection med as it has to be approved after leaving the hospital. The hospital sends records showing the reaction to the first med and the non reaction to they second. The med is Ok’d and he will continue on it when released.
On the 4th of July, any patient able to be moved is placed in a wheel chair with their IV’s, if needed, and taken to the top level of the parking ramp. DJ and the other patients are able to watch the fireworks. There are smiles everywhere and a happy group goes back to the hospital.
July 6, 2010, DJ is released from the hospital but he has to stay in Rochester for 45 days. He will require blood work and clinic visits twice a week. The doctors want DJ close, in case something shows up in the blood work. Since then, they have changed procedures and if there is nothing wrong with a patient, they are able to go home and return to Rochester on the days of blood work and clinic visits.
In September 2010, DJ’s blood work showed he had Epstein Barres Virus, which if not controlled, can lead to transplant lymphoma. We are told to watch the size of his lymph nodes and continue to watch virus levels in his blood work. In February 2011, we see the Buffalo Nephrology doctor who mentions that DJ’s lymph nodes are larger than normal. I remind him that DJ has the Epstein Barres Virus and ask what we should do. He states to continue watching blood work and come back for next appointment in three months. I am not happy with that answer and contact Rochester Nephrology doctors. They do not know the size of his lymph nodes, but, say if Buffalo doctor says we can wait, that is what they will do. I contact the Transplant doctors and tell them my concern. They tell me to have Buffalo doctor forward size of lymph nodes to them. Within days I receive a call telling us to get to Rochester Strong and the next morning they will do a biopsy on one of DJ’s lymph nodes. We travel through a snow storm with high winds and blowing/drifting snow, but, get DJ to Strong and he is admitted. He is prepped for the surgery, IV placed, meds scheduled and nothing to eat or drink after midnight. When asked, no one knows what time DJ’s surgery is scheduled for. The next morning the doctors from both departments arrive and check DJ over, but, neither knows what time he will be taken for surgery. By lunch time, I call the social worker and ask what is going on. DJ has not had any of his meds nor anything to eat or drink since midnight and no one can see a time for his surgery. She calls both departments and each had thought the other department had scheduled the surgery. It was not going to happen and they were sending us home. She got both to agree that we should not be billed for this stay as we did what they asked. Transplant doctors were now taking charge and would call us with another date.
On March 1, 2011 we go back to Rochester Strong, but, this time they are doing an outpatient surgery of actually removing a lymph node to get a true reading. The surgeon removes a lymph node from DJ’s groin and we go home to wait for results.
When the results are back, the EBV is in his lymph nodes. The Nephrology doctor will now begin to lower DJ’s immune suppressant drugs to allow his body to try to naturally fight the virus and we watch the blood work.
The lower drugs are working and his body is fighting off the virus. To try to totally rid DJ of the virus, they want to lower the drugs more even more. Even though the blood work showed the level of the EBV is below 1000 and considered negative for several weeks. I tell them that my gut is saying it is not the thing to do. They are the doctors and say it will be ok, so we lower the drugs one more time. The blood work starts coming back with diminishing kidney function.
DJ is now scheduled for a kidney biopsy to see if the lower immune suppressant drug level and the body were able to fight off the viruses and if it has realized that the kidney is not his, trying to reject it. The biopsy shows that is exactly what is happening. To fight the rejection, DJ is given high dosages of steroids through his IV for several hours and the immune suppressant drugs are increased. This is making him very nauseous and he is unable to keep anything down.
When blood is taken the next week, it shows that the rejection has not been stopped. As this is the way to stop it, the doctors are confused and start to call around, even to doctors for adult renal patients, for more ideas.
It is decided that they will start DJ on a process called Photopheresis. This is usually only done on adults or teenagers of adult size. As DJ will be the smallest patient for this, the Pheresis Department will do some research.
DJ has a catheter inserted in his upper chest, going into the main artery to his heart, with two tubes hanging outside his chest. The tubes will be used to take his blood out, one third at a time, into the Photopheresis machine to be separated. The machine will then keep the white cells and return all other parts of his blood to his body. The white cells will be treated with a chemical and high dosage of ultra violet rays, to “confuse” the T cells within his white blood cells. Being in a “confused” state, this should stop the T cells from attacking DJ’s transplanted kidney. After treatments, DJ has to wear wrap around sunglasses and sun screen for 24 hours. When home, we have to have all the windows blocked with heavy curtains to keep out all sun light and he can go without the sunglasses.
On the days of treatment, it begins with blood work to verify his blood level and that the fat count in his blood is low. The machine can not distinguish fat cells from white cells and the treatment would not be done, as it could clog the machine. Most days his blood level is too low and he needs a blood transfusion. Once he has blood put into his body, we go to the Pheresis Department within the adult Oncology Department, where all of his blood is now taken out, treated and returned to his body. Since his blood level is so low, they must manually drain the blood remaining in the tubes within the machine and give it back to him. All of this takes between 2 to 3 hours and is very taxing for him and he sleeps a lot.
After two weeks of twice a week treatments, the nurse trying to give DJ a blood transfusion realizes something is wrong with the catheter tubes, as they are leaking. The end of the tube is cracked and when she calls around, finds out there is a batch of defective catheters and DJ has received one. It has to be replaced immediately to keep DJ close to the original schedule of treatments.
Treatments continue through December 2011. The Nephrology doctor wants to stop treatments prior to original schedule and see if rejection is gone. When asked why not complete schedule, they say the blood work looks good and they want to see where things are.
January 2012, another kidney biopsy is scheduled. Results show that the acute rejection was stopped but chronic acute rejection is still ongoing. We need to start treatments all over again. It will be twice a week to begin and reducing to once a month over the next few months until June.
The whole time the Photopheresis treatments are going on, DJ has not been able to play sports, which are everything to him, or even participate in gym in school. The doctors, his school nurse and his teachers are all coming to us worried that he is starting to become depressed. The doctors are afraid this could affect his whole health. I remembered that years earlier we had been given a vest covered with padding and show the renal doctor. We ask the doctor if DJ wears his kidney protector and this vest, could he play sports again. The doctor says yes to travel soccer but not gym class in school. To be able to play a sport at all is a great boost for DJ.
After all treatments and travel soccer is done, DJ has another kidney biopsy done in July and it shows the rejection has been stopped. Unfortunately, the kidney has been damaged enough that DJ is back in Stage 3 kidney failure.
In November, I ask the social worker about needing Medicare Part B & D. An agreement was made that a transplant patient would use their insurance carrier as primary insurer for months 1 through 30 after the transplant. Months 31 through 36 would have Medicare be the primary insurer and the previous insurance, i.e. BC/BS for us, would be secondary. He was approved for Parts A & B right after his transplant, but, as our BC/BS plan covered everything, I was told we would not have take Part B at that time and request it for months 31 through.36. The letter we received from the social worker requested that we be accepted into Medicare Part B & D effective January 23, 2013. When I took the letter and application to Medicare in December 2012, I was informed that Part B can only be applied for January through March of a year and the effective date would be July of that year. I explained that we needed it January through June and he would not be eligible for any Medicare after June 23, 2013. They were sorry, but, rules are rules. Appeals after appeal were denied. On one call, I was told that as my insurance coverage was from my work place and it was in effect since the transplant, I could use their special enrollment form. After the company I work for completed their portion and I completed mine, I turned it in. A week later I received a phone call telling me that the special enrollment form did not apply to kidney transplants and my appeals were done. They told me to appeal to my insurance company.
While talking to BC/BS about situation, they tell me that the actual date for BC/BS to become secondary was December 1, 2012 not January 23, 2013 as stated in the letter I was told to use. They would only be the secondary insurer and no appeals could be made to BC/BS. They told me I would be responsible for the 80% that Medicare would normally cover as primary insurer and the co-pays as written in my BC/BS contract. They would pay the remaining 20% above my co-pays. I asked if that included doctor visits, blood work, testing, x-rays and medicines. A very nice lady, brought in to talk to me, asked if Medicare had told me the rules regarding medicine coverage and I said no. She explained that if your current insurance plan for medicines is same as or better than Medicare Plan D plans, your original insurance plan will continue to cover the medicines.
As of June 1, 2013, DJ is no longer eligible for any part of Medicare until DJ has to start dialysis and/or have another kidney transplant.
For more information on helping DJ and his family find a donor or for additional information on DJ and his disease, please send an email to Cheryle Kuras’s email: [email protected]