by caregiver Gail Christensen
My son, Robert, was diagnosed with PPH on May 19, 1997, by accident. On May 16, I took him to the doctor for strep throat and he started taking amoxicillin. When I woke him up for school the next day, he started complaining that his legs were bothering him and he couldn’t walk. I decided to take him to the doctor after I felt his legs and they were ice cold compared to the rest of him. His regular doctor wasn’t available and we saw one of the PA’s there. The PA at the time thought Robert had rheumatic fever and made an appointment to see the cardiologist that Monday, May 19th. The doctor did an EKG and an echo and told us that my 7 and a half year old son had PPH and that the prognosis was about 5 years after diagnosis. I was devastated. My boyfriend asked the doctor about exercise and the doctor looked at us and said that exercise would kill him. He wanted us to take Robert to the Mayo Clinic in Rochester, MN for a confirm ation.
We arrived at the Mayo Clinic on Tuesday, June 3, 1997 and they ran some tests on him the next day. They did the heart catheterization that Thursday and we were told that whatever medications they tried on Robert had no effect whatsoever, however, we found out that he had a hole in his heart. The chief cardiologist explained to us that they did not want to close the hole because it was acting like a “release valve” and equalizing his pressure in his lungs. His pressure at that time was 75, normal pressure at rest should be around 25. They scheduled a lung biopsy for the next day. In recovery after the biopsy, they had to rush him back to surgery because he started losing a lot of blood. After that scare, the doctor explained to us that it is a good thing that he has millions of capillaries in his lungs because most are deformed and/or shut down. Robert started running a fever that Friday night and it finally broke early Sunday morning. While in the hospital, my mo ther who was in NY called to tell me that my dad lost his battle with colon cancer.
We left the Mayo Clinic that week and went back to Rapid City, SD. His cardiologist put him on Dipyridamole and baby aspirin. Over the course of the next few months, his pressures increased slightly and we asked the doctor about moving to a lower altitude. We were told that could possibly prolong his life or maybe not. We decided to move to Tacoma, Washington and his pressure basically stayed at 80. We let him do “kid things” like ride his bike, etc but kept an eye on him to make sure he didn’t get out of breath. Robert lived in Kansas for a year with his dad and then I moved to Salt Lake City, UT in September 2004 and Robert moved back in with me. At this time his pressure was up to 100. I had found out that Robert wasn’t taking his meds all the time while he was living with his dad. His cardiologist at Primary Childrens Hospital in Utah, put Robert on Tracleer in August 2005 and then started him on Revatio in February 2007, at that time his pressure was 115. He was tiring more easily and it was common for Robert to sleep about 12-14 hours sometimes on the weekends.
On June 2, 2007, after sleeping nearly 20 hours, he was still not feeling good and could not even walk 15 feet without resting. I took him to Primary Childrens Hospital and his oxygen sats were 75. They admitted him to the hospital. An echo the next day showed his pressure at 165 and I did not have a good feeling about this at all. Monday night, June 4, his cardiologist told me that Robert had Pulmonary Capillary Hemangiomatosis, (PCH), and he explained that is more severe than the PPH. He also said there was a doctor at LDS Hospital, not too far from Primary Childrens, who knew more about the PCH then he did and Robert was transported there on Wednesday, June 6. He did well being transported and while the doctor was examining him and I was standing outside the room, looking through the glass, I see my son struggling to breath and heard him say he couldn’t breathe. At that time one of the nurses escorted me out of the hallway and about 15 minutes later I was told my so n went into cardiac arrest and they worked on him for almost 45 minutes and could not save him. R.I.P. Robert, I love you to the moon and back…..
We need to find a cure for this deadly disease.