HLH is an immune disorder than can be classified in two types of forms, the familial form or the reactive also known as acquired form. Familial HLH is also known as FHLH while the other is mostly just known as HLH. Familial HLH is usually the result of a genetic issue while HLH is a reaction that your immune system had to a virus or other type of source such as Juvenile Arthritis. You can learn more about both on
the following website www.histio.org.
My 11 year old son was diagnosed with the reactive form in December of 2009. He is in treatment for it which consists of steroids, immunosuppressive therapy and can involve chemotherapy. The only actual cure is a bone marrow transplant. Julian is in medication induced remission and the hope is that when he is done with his treatment that he will remain in remission.
A very simplistic description of this disorder is that the person gets a virus or an illness and the body develops killer cells to destroy the virus. Once the virus is gone, the body no longer needs these killer cells and gets rid of them. If the body can't get rid of them, they eat away at the body. That is the case with HLH, these killer cells take over and attack organs and bone marrow. Since the bone marrow is attacked, it affects cell production. This is the reason that Julian's platelet count dropped.
When Julian was in a coma, I often thought to myself "I just want to hear him say mom again", "I just want see his smile". So many thoughts like that ran through my head. I can't put into words how awesome it is that Julian is playing basketball and his recent report card is all A's and B's. I will never take for granted hearing him say "mom" again and I find that the sounds of his voice or his kisses on my cheek are like heaven on earth.
Today, Julian hung out with his friends, played video games and is now watching TV while laying on my bed. Life is how it's supposed to be.
Saturday, April 24, 2010
Thursday, April 22, 2010
Introduction
I can only describe this period of my life as a nightmare. There was some stress in my life. I was 34 weeks pregnant and had just been placed on bed rest due to hypertension. I work as an "on call" nurse but have always done full time hours. With my position there is no pay if you do not work. We had a high loan from our 3 IVF attempts and were not expecting for me to not work for a few weeks, so the unexpected time off was a little bit stressful. If only would I have known that it was just the beginning.
My 11 year old son Julian whom is quite active developed fatigue, loss of appetite and a fever. Julian played on three basketball teams and was a quarterback for his football team. This was a straight A over achiever that never stopped. I kept asking him if his throat hurt or if his "stomach" hurt. I was watching and waiting for another "flu" type of symptom to develop but it didnt. After a few days of high fevers he developed jaundice (yellowing of his skin and the white part of his eyes). Now I was really worried!
We took him to our local emergency room and I began to run through my head all of the possible reasons a normal and healthy 11 year old boy would have a problem with his liver. I knew as a nurse that the jaundice meant that there was something going on with his liver. I held back tears and tried to remain calm and reassuring to Julian. They started an IV and drew blood. All of the nurses that he came into contact with (the triage nurse and his assigned nurse) had that look that I got when I knew that it wasn't going to be good for my patient. I felt un-nerved by that look and wondered if I had ever done that to my patients without offering any reassurance. Their silence and lack of reassurance felt as sharp as a knife.
When his labwork came back it confirmed that his liver was failing him. The other thing about his lab results that I found really upsetting was that his platelet count was low. Platelts are the part of your blood that allow clotting and keep you from bleeding. So, immediately the tears flooded me and I couldn't even stop them for Julian's sake. It was well after midnight and he was in and out of sleep. I walked out of the room and went into a bathroom. I didn't want to sob infront of anyone and I really didn't want Julian to hear me.
The emergency room doctor never discussed the lab results with me, she just handed them to me and said that she was going to admit him to the hospital. She vaguely discussed that it could be a virus and that he would need to be watched. She returned shortly after to tell me that my son's pediatrician had decided that he should be transfered to Children's National Medical Center. I felt relief with this and then disapointed at myself for not demanding that from the beginning. We were told that the ambulance to transfer him would arrive by 6am. I layed with him in the stretcher and watched him sleep as we waited. It arrived at 5am.
We were transfered to the emergency room of the new hospital so that they could re-evaluate him and decide where to go from there. We were there until about 2 or 3pm before being admitted into a room. While we were in the emergency room his fever spiked again and he became incontinent of stool. This was really upsetting to him and the crying and anger began for him. I saw the fear in his eyes and I felt so much guilt that I couldn't hide my own at a time when I should be doing nothing but reassuring him. I comforted him as much as I could. Every now and then I would walk into the hall and cry silently.
When we got to his room, I surveyed my surrounding and began to plan out how I would sleep at night. I worried about the scattered contractions that I was having and wondered if my blood pressure was sky high. I began making the phone calls to family, close friends, our pastor. A stream of doctors came through and the consensus seemed to be that he had liver failure related to a virus. They seemed to feel that he would need several weeks to get better but that he would. However, after a few hours his breathing didn't seem quite right to me. I called and asked for the doctors to evaluate my concern. They reassured me that his lungs were clear and he was stable.
Later in the evening I began to move things around in his room so that I could organize our belongings. Suddenly I thought that my water broke. I quietly went into the bathroom fully expecting to confirm that my wather broke but hoping that maybe I urinated on myself. I was shocked to see blood, a lot of blood. I started to sob again. After regaining my composure I walked out of the bathroom and told Julian that I would have to go to another hospital to possibly have the baby.
Julian began to cry and say that he couldn't breathe. I tried to calm him as I felt more blood streaming down my legs. I whispered to my husband that I was bleeding and that I could have a placenta abruption going on. This is when the placenta tears causing significant vaginal bleeding. In most cases the baby only survives if he can be taken our via emergency c-section in less than an hour.
I was relieve that family had arrived to be with Julian and I wouldn't be leaving him alone. I was rushed to the hospital next door. They evaluated me and felt that I my placenta was intact, the bleeding had stopped, so they discharged me. My husband insisted that I go home and actually sleep a few hours (I had been awake for over 24 hours and had no bed rest). I reluctantly went home as he returned to Julian's side. It was around 5 in the morning when he called me and told me to return to the hospital. I sobbed all the way there. I arrived to find out that while I slept, Julian crashed and was placed on life support. He was on medications to sustain his blood pressure and a machine to breathe for him. I began to feel like the life was draining out of me and I was sure that I couldn't handle life without Julian. I sobbed because I was knew that I would have a baby really soon and couldn't handle taking care of a newborn and I didn't want to leave Julian's side.
I held vigil at Julian's side as family and friends waited in the family room. After everyone's insistance, I went to see my OB doctor. He immediately admitted me to deliver the baby. My blood pressure was sky high and he suspected that I was misdiagnosed and did infact have a slow tearing placenta. As he induced me, I couldn't find any happiness inside and felt like it was utterly unfair to bring a baby into the world at this point in time. I wondered if my family was holding Julian's hand the entire time as I would have. Carson was born the evening of December 30th, two days after the nightmare began. I held him for an hour before he developed respiratory distress and was taken to NICU and placed on a maching to help him breathe. He was 5 weeks premature but strong and would be OK after a little bit of time in the NICU. Less than 24 hours after his birth, I left the hospital to be by Julian's side.
Things seemed to be getting worse for Julian. His platelet count dropped to 3,000 (normal is approx 150,00 to 400,000), he had needed multiple blood transfusions and was bleeding in his lungs and into his eyes and had swelling in his brain. His entire body was so swollen and with the bright yellow coloring, my Julian no longer looked like my Julian. I prayed and cried and begged God to save my son. I sat in a chair watching as he deteriorated and the doctors and nurses diligently worked to save his life without ever giving up hope. As an emergency room nurse I had been a part of many codes but I never once put myself in anyone of my patients' families shoes. I wondered if I would ever be the same again.
Then came the unofficial diagnosis: HLH. A rheumatologist brought on only 5 months prior to CNMC (Children's National Medical Center) from Cincinnati's Children's Hospital made the call to treat Julian for HLH even if the tests wouldn't be back for a few weeks to confirm it. Julian began getting better. Today, he is back at school and back to sports with no deficits. He is my miracle. I continue to struggle with the trauma of what happened. I decided to start this blog to put the story of HLH out there and to write about my experiences in the hope that healing can begin and others can be informed about this illness. HLH is short for Hemophagocytic Lymphohistiocytosis. It is an immunilogical disorder that most don't survive.
My 11 year old son Julian whom is quite active developed fatigue, loss of appetite and a fever. Julian played on three basketball teams and was a quarterback for his football team. This was a straight A over achiever that never stopped. I kept asking him if his throat hurt or if his "stomach" hurt. I was watching and waiting for another "flu" type of symptom to develop but it didnt. After a few days of high fevers he developed jaundice (yellowing of his skin and the white part of his eyes). Now I was really worried!
We took him to our local emergency room and I began to run through my head all of the possible reasons a normal and healthy 11 year old boy would have a problem with his liver. I knew as a nurse that the jaundice meant that there was something going on with his liver. I held back tears and tried to remain calm and reassuring to Julian. They started an IV and drew blood. All of the nurses that he came into contact with (the triage nurse and his assigned nurse) had that look that I got when I knew that it wasn't going to be good for my patient. I felt un-nerved by that look and wondered if I had ever done that to my patients without offering any reassurance. Their silence and lack of reassurance felt as sharp as a knife.
When his labwork came back it confirmed that his liver was failing him. The other thing about his lab results that I found really upsetting was that his platelet count was low. Platelts are the part of your blood that allow clotting and keep you from bleeding. So, immediately the tears flooded me and I couldn't even stop them for Julian's sake. It was well after midnight and he was in and out of sleep. I walked out of the room and went into a bathroom. I didn't want to sob infront of anyone and I really didn't want Julian to hear me.
The emergency room doctor never discussed the lab results with me, she just handed them to me and said that she was going to admit him to the hospital. She vaguely discussed that it could be a virus and that he would need to be watched. She returned shortly after to tell me that my son's pediatrician had decided that he should be transfered to Children's National Medical Center. I felt relief with this and then disapointed at myself for not demanding that from the beginning. We were told that the ambulance to transfer him would arrive by 6am. I layed with him in the stretcher and watched him sleep as we waited. It arrived at 5am.
We were transfered to the emergency room of the new hospital so that they could re-evaluate him and decide where to go from there. We were there until about 2 or 3pm before being admitted into a room. While we were in the emergency room his fever spiked again and he became incontinent of stool. This was really upsetting to him and the crying and anger began for him. I saw the fear in his eyes and I felt so much guilt that I couldn't hide my own at a time when I should be doing nothing but reassuring him. I comforted him as much as I could. Every now and then I would walk into the hall and cry silently.
When we got to his room, I surveyed my surrounding and began to plan out how I would sleep at night. I worried about the scattered contractions that I was having and wondered if my blood pressure was sky high. I began making the phone calls to family, close friends, our pastor. A stream of doctors came through and the consensus seemed to be that he had liver failure related to a virus. They seemed to feel that he would need several weeks to get better but that he would. However, after a few hours his breathing didn't seem quite right to me. I called and asked for the doctors to evaluate my concern. They reassured me that his lungs were clear and he was stable.
Later in the evening I began to move things around in his room so that I could organize our belongings. Suddenly I thought that my water broke. I quietly went into the bathroom fully expecting to confirm that my wather broke but hoping that maybe I urinated on myself. I was shocked to see blood, a lot of blood. I started to sob again. After regaining my composure I walked out of the bathroom and told Julian that I would have to go to another hospital to possibly have the baby.
Julian began to cry and say that he couldn't breathe. I tried to calm him as I felt more blood streaming down my legs. I whispered to my husband that I was bleeding and that I could have a placenta abruption going on. This is when the placenta tears causing significant vaginal bleeding. In most cases the baby only survives if he can be taken our via emergency c-section in less than an hour.
I was relieve that family had arrived to be with Julian and I wouldn't be leaving him alone. I was rushed to the hospital next door. They evaluated me and felt that I my placenta was intact, the bleeding had stopped, so they discharged me. My husband insisted that I go home and actually sleep a few hours (I had been awake for over 24 hours and had no bed rest). I reluctantly went home as he returned to Julian's side. It was around 5 in the morning when he called me and told me to return to the hospital. I sobbed all the way there. I arrived to find out that while I slept, Julian crashed and was placed on life support. He was on medications to sustain his blood pressure and a machine to breathe for him. I began to feel like the life was draining out of me and I was sure that I couldn't handle life without Julian. I sobbed because I was knew that I would have a baby really soon and couldn't handle taking care of a newborn and I didn't want to leave Julian's side.
I held vigil at Julian's side as family and friends waited in the family room. After everyone's insistance, I went to see my OB doctor. He immediately admitted me to deliver the baby. My blood pressure was sky high and he suspected that I was misdiagnosed and did infact have a slow tearing placenta. As he induced me, I couldn't find any happiness inside and felt like it was utterly unfair to bring a baby into the world at this point in time. I wondered if my family was holding Julian's hand the entire time as I would have. Carson was born the evening of December 30th, two days after the nightmare began. I held him for an hour before he developed respiratory distress and was taken to NICU and placed on a maching to help him breathe. He was 5 weeks premature but strong and would be OK after a little bit of time in the NICU. Less than 24 hours after his birth, I left the hospital to be by Julian's side.
Things seemed to be getting worse for Julian. His platelet count dropped to 3,000 (normal is approx 150,00 to 400,000), he had needed multiple blood transfusions and was bleeding in his lungs and into his eyes and had swelling in his brain. His entire body was so swollen and with the bright yellow coloring, my Julian no longer looked like my Julian. I prayed and cried and begged God to save my son. I sat in a chair watching as he deteriorated and the doctors and nurses diligently worked to save his life without ever giving up hope. As an emergency room nurse I had been a part of many codes but I never once put myself in anyone of my patients' families shoes. I wondered if I would ever be the same again.
Then came the unofficial diagnosis: HLH. A rheumatologist brought on only 5 months prior to CNMC (Children's National Medical Center) from Cincinnati's Children's Hospital made the call to treat Julian for HLH even if the tests wouldn't be back for a few weeks to confirm it. Julian began getting better. Today, he is back at school and back to sports with no deficits. He is my miracle. I continue to struggle with the trauma of what happened. I decided to start this blog to put the story of HLH out there and to write about my experiences in the hope that healing can begin and others can be informed about this illness. HLH is short for Hemophagocytic Lymphohistiocytosis. It is an immunilogical disorder that most don't survive.
Subscribe to:
Posts (Atom)
