This is likely to be a long post so I'm sorry to write a novel but I need to get this down. My 18 month old daughter went through something very frightening this week. For the two weeks before this happened I was noticing small signs but I never put two and two together. Little things had changed like her portwine stain was lighter. I thought that perhaps this was a delayed response to the laser treatments she'd received. Her lips turned blue one morning because it was cold but they were not purple, they were blue. Once she warmed up she looked more normal but her lips still looked rather pale and I figured the warmer it got, the brighter they would get. Last Saturday I had her in the tub with my three and six year old. That is when I realized something was really wrong. Her siblings were pink and had very red lips. Her skin was pale, void of any color and her lips were as pale as ever. On Sunday I voiced this concern to a friend of mine who agreed that she looked pale. She and I reviewed some pictures I had taken of the baby in February and her lips were rose red, her stork bite in the middle of her forehead was visible and she had a vibrant color to her skin. It was a stark contrast and I began to worry.
On Monday morning (April 4th) I called my regular ped first thing. My office is large so you have to leave a voicemail for the triage nurse and wait for her to call back. She returned my call around 10:00AM that day and after hearing my report scheduled an appointment for Wednesday (April 6th) at 10:15AM. I asked her if there were any over the counter fingerstick hemoglobin tests on the market because I needed an answer and she said no. My older daughter has dance at 10:30AM on Mondays so I took both girls and sat down to watch in the studio. Normally, my 18 month old would running all around and I would have to take her out of the studio to run up and down the sidewalk. This day she sat in my lap the entire time. Again I felt a sense of forboding because this wasn't like her and she looked like a full blown vampire. I got home around 11:30AM and decided that Wednesday was too far away and I wanted her to be seen that day. I did not wish to go through the rigamarole of calling my ped's office and leaving a message and waiting for the nurse to call me back because it was nearly lunchtime and I knew it wouldn't be until after 1:30PM that she would even get back to me. Instead I called a small pediatric office near my home that I had never been to. They gave me an appointment at 2:30PM that day.
I took both girls and saw the doctor who was very thorough and wonderful. She explained the she would order a CBC, metabolic panel and an iron study that I could take to Children's hospital at my leisure in the next couple of days. She agreed that Bridget looked pale and examined her. Her liver and spleen were not enlarged and no masses were felt in her abdomen. She was about to send me on my way when I asked her to please do a finger stick and she thought that information wouldn't tell us anything and that the veinopuncture hemoglobin she would receive at the hospital would be more accurate. I agreed but I thought just knowing she was anemic would give me some peace of mind. I figured she is pale because she's anemic and I'll start her on some Floradix and she'll be fine.
That was not to be. Five minutes later the same pediatrician entered the exam room collected but I could tell she was very worried. She told me that I had very good mama instincts and that I needed to go home and pack a bag for us and go straight to the ER because her hemoglobin was only 5.6. She told me they would admit me and likely transfuse her. She did not wish to speculate what might be causing this anemia but she looked afraid for us.
I walked out with the two girls fearing the absolute worst and crying in the parking lot. I was so afraid she had leukemia. I called my husband and told him the situation and then packed a bag after calling my father to stay with our other children and hubby, Bridget and I were off to Children's Hospital ER. We were in the ER for 6 hours before we got any answers. They did a lot of blood tests and then did not want to speak to us about the results until they consulted with hematology. That made me think it was bad news. Our ER nurse came in and said they were still waiting for the consult and I just begged to tell me something and she rubbed my arm and said, "it isn't leukemia but we don't know what it is". Well, that it wasn't leukemia made me feel better and about 20 minutes later the pediatric ER fellow came in and said her iron and folate were normal and that all her other cell counts were normal but that her hemoglobin was 5.1 and that they would be admitting her into the hospital. She also explained that they would probably transfuse her. This was scary because we do not vaccinate and she is not protected against Hep B. When I made the choice to forego that particular immunization I knew there was always a small chance that my baby would require a blood transfusion but it never occurred to me it would be under circumstances like this.
By the time we got into our room in the Peckham Center for Childhood Cancer and Blood Disorders (that was a scary sign to read!), it was 11:00PM. We were all exhausted. The baby had fallen asleep in my arms and an orderly pushed my in a wheelchair holding her to our room. Once there, the nurse explained all the rules and what they would be doing and then a hematology resident and intern came in to talk to us. They asked lots of questions and seemed particularly giddy and interested in our daughter's case. I liked that. My husband went and got something for us to eat and then left to go home. Only one parent is allowed to stay in the room. Once he left the resident came back to tell me what he thought she had. He wrote it on the white board. He explained that he couldn't say this was her diagnosis but he thought that this is what she had and he turned out to be correct. The next morning we waited for the hematology fellow and attending to turn the resident's best guess into a diagnosis.
They got to our room at 11:00AM and explained that they believed she had Transient Erythroblastopenia of Childhood (TEC). It is a condition in which the bone marrow temporarily shuts down the production of red blood cells. It is extremely rare for a child to present with hemoglobin this low and not have it be an iron deficiency. The hematologist told me that a child has about a 5 in one million chance of developing TEC that causes such a slip in hemoglobin. They believe that it may happen more often but that the marrow kicks back into gear more rapidly preventing the child from becoming anemic so know one ever knows it happened. They also explained they are not entirely sure why this happens. There are some ties to viruses, mainly parvovirus (Fifth's disease), EBV, CMV and hep A. They tested her for parvo virus and we are still waiting on the results. I don't think it will be positive because I have seen Fifth's disease and she didn't have the rash.
They also explained that her latest CBC revealed a further slip of her hemoglobin. It was down to 4.3 from 5.1 just 10 hours earlier so they felt a transfusion was imperative. They said we could wait another 12 hours to see where her hemoglobin was then but that her reticulocytes were almost non-existent and that because they tell the marrow to make red blood cells, it wasn't likely that her number would be up later in the day. They explained that the lower her hemoglobin got the greater the stress on her heart. We chose to transfuse her ASAP. It was hard and she cried the first time. They have to take vitals a lot during a blood trasfusion and she hated being hooked up and having a blood pressure cuff on her arm or leg. She made it through the first one without any reaction so they gave her another transfusion in the middle of the night from the same unit of blood. That one was much easier because she slept through the whole thing. The next morning her hemoglobin was up to 8.2! They said we could go home with that number but that there was still 150 ml of the unit of blood she'd already received and that it would be better to tank her up in the hopes of preventing her needing another blood transfusion from a different donor before her marrow recovered. Once she received the third transfusion, we had to wait an hour to observe her and we were discharged on Wednesday night at around 5:45PM!
On Friday morning I returned for a CBC and retic count and her hemoglobin was 12.4 and her retic had rebounded slightly from 0.3 to 0.4. I go back with her next Friday for the same tests.
So, that is our tale. It was rough but I'm so thankful. We won the blood disorder lottery. There were many families in that ward that had not been as fortunate. There were many families that had been there for months. I felt guilty when we left only two days. I have NEVER received such superior medical care (other than my midwife!). All of the nurses were sympathetic and amazing with the baby. The entire hematology team was beyond my wildest dreams. Her doctor was so respectful and loved that we asked so many questions. He stayed in the room and talked with us for over an hour the second day we were there and for 30 minutes the day before. I left feeling very happy with the care my daughter received. I would love to hear from anyone whose child has had TEC. Is there anyone else out there?