ARPKD/CHF, A Transplant Story

June 28, 2013 / admin / 5 Comments / Living with ARPKD/CHF

My name is Katie; I am a single mother to a four-year-old boy named Joseph who was diagnosed with ARPKD/CHF at sixteen months of age. He received a kidney/liver transplant when he was three years old.

This is our story.

I knew from the day that I had Joseph that something was wrong. Call it maternal instinct, call it a paranoid first time mother, call it whatever you want. I would tell my family and other mothers my feelings and everyone told me I was “crazy.” Joseph was always sick as a baby; he caught every cold and flu bug. I had brought this to our pediatrician’s attention at one of his well-child checkups, but she told me it was normal.

At sixteen months of age Joseph came down with another flu and a slight fever so I put him down for a nap. An hour later he awoke covered in petechiae. I knew this looked serious so I rushed him to the ER. They drew blood and said Joseph’s immune system was bottomed out, his hemoglobin was low and his platelets were very low. Within an hour Joseph and I were headed to the Children’s Hospital by ambulance.

They put us in isolation and ran every test you could think of, spinal tap, bone marrow biopsy, bone marrow aspiration, you name it and he had it done! I was a total mess and in so much shock that I honestly don’t remember two whole days of that six-day stay. Finally they decided to do an abdominal ultrasound and discovered Joseph’s kidneys were covered in cysts, there was one cyst on his enlarged liver, and he had an enlarged spleen. The doctors still didn’t know the cause of all of his symptoms.

Following the ultrasound and after I got Joseph calmed down, I left him with my parents and went to the hospital cafeteria. I sat down, flipped open my phone went to Google and typed “kidney and liver cysts,” the first thing that popped up was Polycystic Kidney Disease. I read and read, I knew in my heart that I was right; something was wrong with my baby and this was it! I picked at my salad and went back up to Joseph’s room to wait for the doctors. An hour later our doctor approached me with a printout and before I looked I said ”its PKD.” She looked at me puzzled then told me that Joseph had ARPKD, but his kidneys and liver had 100% function and there was no way to tell when a transplant would be needed. So a day later we were discharged.

I went home and found the ARPKD Facebook group. We continued to go to checkups with renal and GI at Children’s Hospital. At one point GI thought Joseph had Hepatitis A, they said that’s the only thing that could explain the liver and spleen issues. I went home and posted on the group about Joseph having ARPKD and Hepatitis A and an amazing woman (Colleen Zak) responded telling me about CHF and the ARPKD/CHF Alliance. The information on CHF from their site is actually what finally got the GI doctor to see Joseph’s issues as ONE DISEASE!! ARPKD/CHF is a liver AND kidney disease!

Fast-forward one and a half years later and Joseph only had 20% kidney function and horrible portal hypertension, causing Joseph’s abdomen to fill with ascites. We are transferred to UCSF for a transplant evaluation, and a few months later at 13% kidney function, the renal team decided it was time to come up with a dialysis plan. Then Joseph’s bilirubin started to climb, and his PT and PTT were way off. Our liver teams words were, “his liver has declared itself, he needs a transplant.”

Joseph was listed for a combined liver and kidney transplant. We were told that Joseph’s blood type is AB Negative (the most rare), and the team had no guess as to when my precious baby boy would get new organs. After much debating the renal team decided try to kill two birds with one stone and use peritoneal dialysis to remove both toxins from Joseph’s blood and the ascites in his abdomen, so Joseph was admitted for a peritoneal catheter to be placed.

Usually when a PD catheter is placed it’s not used for at least two weeks so it can heal, we thought we had a lot more time than that, but the surgical anesthesia wiped out the rest of Joseph’s kidney function and two days after surgery we started dialysis. I needed peritoneal dialysis machine training, which is normally done as outpatient, so we had to stay in the hospital until I was fully trained. I dove head first into learning as much as I could, as fast as I could, so that I could bring my baby home!! The great news was that the dialysis was working, it removed both the toxins and a liter or more of ascites from his abdomen a day!

Two weeks later, the night before we were to be released to go home, our night nurse walked in and introduced herself; she said it would be a calm night because we were going home tomorrow. She opened the computer to look at her instructions, I was looking over her shoulder and we both noticed a TON of lab work ordered! Right then an X-Ray Technician came to the door and said that she needed a chest x-ray for Joseph’s liver transplant. At this point I had had enough! I told her Joseph already had a chest x-ray and that’s how he got listed. They weren’t bothering my kid anymore, we were going home in the morning (or so I thought.) I noticed something click when the x-ray tech turned to me and said “I’ll come back if they really need it.” Then it clicked for me, in total disbelief I turned to look at our nurse who was standing wide eyed and asked “do they have organs for my son!?” She was just as shocked as I was and left to go ask what the heck was going on!

At this point it was 10 pm. I picked up the phone and called my parents and asked them to come to the hospital ASAP. About that time, one of the Surgical Fellows walked in and said that our surgeon had accepted the organs for Joseph! I broke down and this might sound strange, but I was filled with fear and not one ounce of anything else. I was hysterical! I thought we would have more time! I hadn’t done any research on transplants because most kids are on a transplant list for six plus months! The fellow patted me on the back as I sobbed and asked “how long have you been waiting?” When I responded “two effin weeks!!!!” she looked at me like I was crazy!

After she left I picked up the phone and called the only person I knew at the time whose child had a combination transplant. I had met her in the ARPKD/CHF Alliance Facebook support group, her name was Denise and she was amazing! Luckily she was from my area and her son had a transplant at the same hospital! The phone rang and rang and finally she answered; “Denise, they have organs for Joseph!” I said inaudibly. At that moment Joseph’s IV machine started beeping, so I pressed the nurses call button and returned to the phone conversation. I had to gather myself and say it again. “Denise, they have organs for Joseph!” She screamed from excitement! I wasn’t excited; honestly I thought Joseph was going to die. She explained that the surgeons had to look at the organs to make sure that they were healthy. I held on to that hope that they weren’t, so Joseph wouldn’t have surgery right then!

At that moment our charge nurse Julie, who Joseph and I had grown very close with, came flying into the room. She had heard me freaking out after I had pushed the nurse call button “What is going on?” Julie asked. “They have organs for Joseph.” I sobbed. Julie began jumping up and down screaming from joy and excitement, “That’s great news!” she said. “No Julie, he’s going to die,” I sobbed again. Julie stopped, walked over to me, put one hand on each shoulder and shook me, hard. She looked me in the eyes and said “IS THAT WHAT YOU THINK!?” I nodded my head. Again she shook me and looked me in the eyes, this time we were both crying. “Listen to me” she said, “if they don’t do this surgery he will die.” That’s all it took, I took a deep breath and decided that being a pile of tears wasn’t helping anyone; I needed to be strong for Joseph. I had gotten him through all the other hardships and I WOULD get him through this.

When Julie left and I walked over to my precious baby, my world, my everything, and just watched him sleep for what seemed like forever. Then I grabbed his hand, fell to the floor and prayed. My parents arrived and tried to comfort me but at the time it didn’t help much. The team of surgeons came in and immediately I asked, “so you still have to go look at the organs right? This isn’t a for sure thing yet?” The head surgeon responded “The donor is an 8 year old boy from Sacramento who died from a head trauma, there is no reason that these organs wouldn’t be in great shape and they’re a perfect match. The surgery is scheduled for 9 a.m.”

I don’t remember much of that night, but watching Joseph sleep and wanting to wake him up so badly so that we could spend what I thought could be our last few hours together. I fought that urge all night knowing if he was going to make it through the nine-hour surgery, he needed to rest. Nine a.m. came way too fast; I wasn’t ready, although I don’t think anyone really ever is. They let me lay on the hospital bed with Joseph on top of me as they took us down to the pre-anesthesia room. As we rolled out the door of our room, every doctor, nurse, staff member that had had anything to do with Joseph lined the hallway and hugged us and cheered us on; I swear to you it was just like a movie.

They got us downstairs and gave Joseph some versed, then the anesthesiologist picked Joseph up in his arms with his favorite blanket and a smile on his face (from the versed of course) and walked my beautiful baby down the hall and into the O.R. That eight and a half hours was the longest, most gut wrenching experience of my life and is still the longest time I have ever been away from my son.

The amount of emotions you go through during a time like that are unreal! Fear, excitement, numbness, more fear and a few more emotions that have no name! The surgeons and nurse in the O.R. called about every two hours to let me know things were going well and that Joseph was perfectly stable. Eight and a half hours later Joseph was wheeled into the PICU and I was allowed to go see him. I walked in and although he was intubated and heavily sedated, he looked AMAZING!!!

My parents came in for about an hour then went home to get some rest. Joseph couldn’t talk with the tube in his mouth, but was responding by shaking his head. When the nurse asked if she could do anything to make him feel better he frowned and pointed to the intubation tube, so they decided to remove it. He did fine and in two days was out of ICU and back on the floor. At four days post-transplant he was standing up, walking around and playing. Eleven days post-transplant we were headed home!! We started out on eleven meds twice a day and now, nine months post-transplant we are down to five.

We have been very lucky, there have been a few bumps in the road but overall he is doing AMAZING! Our doctors call him the combo transplant poster child! The only thing that you see that is different than an average four year old is his nasal gastric tube to help with his fluid intake, which keeps his new kidney happy.

Do you want to know what I see? I see a happy, healthy, playful, brilliant child that was given a chance, because someone who lost their baby decided to give the most amazing gifts ever, by donating their boy’s organs. Not only did that little boy save my son, but another child received the donor’s pancreas, and another child received the donor’s other kidney. I am forever grateful to the donor’s family and ask only one thing of whoever is reading this. If you aren’t an organ donor, register today, and encourage your family and friends to do the same! You can’t take them with you, but you can be someone’s hero!

Thank you all for taking the time to read this!