We Round the Bend to New Views

When my wife and I went to the Phoenix Perinatal Associates clinic at week 24 we were feeling positive and upbeat, afterall, she had undergone 6 MCA doppler exams and there were no signs that the baby was anemic. Perhaps we were a little naive as to how this all worked and had become complacent in our false sense of security. This visit promised to be far different than the other visits. This visit would cast a dark shadow over our lives that only got darker over the next month. The exam started quite normally and we preceded to joke and tease with our ultrasound technician as she prepared to check the blood flow through our little babies middle cerebral artery. When the ultrasound image came on the screen we caught a sweet profile of our unborn little boy and the technician snapped a picture for our photo album. The technician located the middle cerebral artery and adjusted her machine to focus in and detect the blood flow in cm/sec. Instantly we could see that the peak on the machines graph readout was too high and the mood intensified. This showed us that the babies blood was flowing too fast through the artery and this was the sign we dreaded to see for this meant that the worst was true, the baby was kell positive and rapidly becoming anemic. In conjunction to the MCA doppler the technician also looked for what is called hydrops fetalis. Hydrops fetalis is a severe, life-threatening problem of severe edema (swelling) in the fetus and newborn. Hydrops develops when too much fluid leaves the bloodstream and goes into the tissues. When the mother's antibodies attack the foreign red blood cells, they are broken down and destroyed, resulting in anemia. Hydrops can develop as the baby's organs are unable to compensate for the anemia. The heart begins to fail and large amounts of fluid build up in the baby's tissues and organs. As the technician moved the transducer across the babies chest cavity and abdomen there on the screen we saw what even our untrained eyes could detect. There were large dark areas on the screen, the telltale signs of the hydrops. As with most medical services, the bad news needs to come from the doctor and one was called to come and talk to us about what this exam had revealed. We went to Dr Elliot's office and sat down to have him explain to us where we were headed with the next step in this process and the sobering truth of the complexity of our babies disease hit us full force as we sat there in the doctors office in our new home town of Phoenix.

The First Step

A few troubling weeks had passed since we received the results of my blood test and we now had an appointment with a perinatologist. A perinatologist is an obstetrical subspecialist concerned with the care of the mother and fetus at higher-than-normal risk for complications. The meeting we had was less than informational as the doctor we saw had never dealt with this disease. We had done alot of our own information research on the internet and learned quite alot through medical journals and professional papers and the doctor we saw was able to tell us far less than what we found on our own. This was our first clue that this is a rare medical condition and made us hope that we could find a specialist that had seen and treated this condition. What ever the case, we were determined to not let ourselves needlessly worry and obsess until we knew for sure we needed to do so. Now, what we understand at this point is that the blood from my wife will not cross the placenta and attack our childs blood until week 16 or so. We also knew that to assess the health of the baby no real method existed until the pregnancy was 18 weeks along. The method that would be employeed when we needed was an ultrasound test called an M.C.A Doppler. M.C.A. stands for middle cerebral artery and doppler refers to the reflected sound waves being used to measure blood flow through this artery. If the blood flow through the artery was below a mean velocity then there was a good chance that anemia had not yet begun and we could rest for a week until the next test. We knew that at any given time the MCA Doppler could show that the baby was anemic and this whole ordeal would quickly be elevated to the next level. In the mean time I was promoted at work and given the chance to move to Phoenix, AZ to manage a branch of the company I work for. We decided to take the promotion and we moved to Phoenix where we got connected with the Phoenix Perinatal Associates. We knew that this was a great choice we made when we found out that if we needed we would be under the care of a pioneering doctor in the care of babies with Kell Isoimmunization, Dr William Clewell. And so from week 18 until week 24 we went for the weekly MCA Dopplers and felt like we were almost in the clear because all the exams had been normal. One factor in this whole process was what is called titer levels which refers to the levels of the anti-body in my wife's blood. She unfortunately had titer levels that were higher than anyone had seen. Her anti-kell titer level was 1:1056, the standard titer level that would cause concern and warrant weekly exams is 1:8. Just another bit of bad news in a time in our lives when things were happening that were so insane we didn't know how to deal, again we decided to take it day by day and not get crazy with it. No one wants to think that their unborn child is at risk for anything life threatening and we certainly could not concieve of this fact. Were we dreaming and would we wake up to find that everything was alright?

The Journey Begins

My wife and I were looking forward to our second pregnancy. We had timed this one so that our first child would not quite be 2 yet when his younger sibling was born. It was pretty simple for my wife to get pregnant and we eagerly anticipated the next 40 weeks and the eventual meeting of our next child. In early December we both went to my wife's 8 week exam and were so excited to be this far along in the pregnancy. Time was flying and we were quickly getting to the end of this year and moving towards 2006. The exam went relatively well aside from the doctor instilling the first hint of fear and apprehension in us for something totally unrelated to what we would find out was a major complication... he thought that the uterus was too small when he felt it with his hands. Whatever! During the exam the doctor drew blood to run some standard tests. A couple of days later my wife got a call from the doctor himself(always a sobering call) with the results of her blood test. Bad news. She was told that her blood contained an anti-body called anti-kell. Basically this meant that somehow my wife was exposed to blood at some point that had this kell protein and, her blood, detecting this as a foreign invader(much like a cold virus) created anti-bodies to the kell protein. When this happens there is risk to the developing baby that his blood will contain the kell protein and his blood would be attacked by my wife's blood and destroyed. With the babies red blood cells being destroyed he could become anemic and this would not be good. The big question now was... is this baby going to have a problem or is everything ok. If this baby was kell negative(as my wife is) than we were in the clear, but, if this baby were kell positive than his red blood cells would be destroyed by the anti-bodies. Red blood cells have many different proteins on their surfaces some are more common than others. With the kell protein only about 8% of caucasian males have it and the other 92% do not. The main factor here was my genetic make up and whether or not I had the gene for kell. If I did then was I homozygous(carrying two genes) or heterozygous(carrying one gene). If I carried one gene there would be a 50/50 chance that the baby inherited this gene. If I carried two genes then it would be 100% that the baby inherited the gene. The doctor ordered a blood test to be done on me that would determine if I was kell positive or not. When the results came back our hearts sank, I was kell positive(heterozygous, I carried one gene) and now we knew that this pregnancy would be difficult if the baby was also kell positive. We would not know for a while what the results were so we had some time to ponder this and learn all that we could in order to prepare for the next 7 months.