Jordan & Jenna

MMA Mut 0

We would like to introduce everyone to 2 of the strongest, most loving children we have ever known. It has been a trying 9 years, but out of that time a very close family has been formed. Our first daughter, Jordan, was born June 11, 1996. I carried her full term at age 36, and we were blessed with a perfect, beautiful little girl. I had a mostly normal pregnancy, except for gestational diabetes, and a few panic attacks. Jordan liked to sit very high, and I suffered from Sinusitis constantly, not a good combination. She was delivered by cesarean section, due to lack of dilation which caused her heart rate to drop. Prior to any difficulty, the decision was made to go ahead with the Csection. Jordan breastfed without difficulty slept and interacted normally until day 3. That morning she started to breathe a bit heavier, and started to sleep more. When the nurses took her down for her discharge physical, I voiced my concerns. We got ready to leave, and a couple of hours later, an alarmed doctor came to the room to speak with us. They had attempted to move her into the NICU, but they had no beds available, and were planning on moving her to another hospital. By the time we arrived in the NICU, she was in an isolette, ready for transport. Our miracles started there. They wanted to move her to John Hopkins Bayview, a satellite of the main hospital. We had not wanted to let her go there because of previous experiences, and only gave in when the Dr at Bayview asked to talk to us on the phone. He said, “Let me take care of your daughter.” The attending physician happened to be a metabolic specialist named Dr Alpan. He met us at the door, and told us he thought our little one had a metabolic disorder, and if she could hold her own for the next couple of hours, he would do everything he could to pull her through. Jordan’s labs came back with ammonia of 1050, and off we went to John Hopkins main hospital, where a team met us again. Jordan had a line placed, and she held her own through the hemodialysis. She stayed in the NICU for the next 3 weeks, and day by day blossomed back into the little girl we had let go for a discharge physical at 3 days old. Jordan’s first 3 years were, looking back now, fairly uneventful after a rough start. She had a few admissions a year, for dehydration/gastroenteritis, but otherwise had no developmental delays. She walked and talked on time, and was normal other than regulating the protein intake. She drank her formula from a sippy cup and attended regular preschools. Since then at about age 5, she was diagnosed with kidney problems. She would be admitted for dehydration, although nothing in the daily routine had changed. In many talks with her Drs, we figured out that she was not concentrating her urine. No one ever asked the right questions. You go to the ER, they asked if she had been peeing, but we never thought about the color. No matter how hot it was, it never got any darker. Since then, she had been categorized as having kidney failure (ESRD), although currently her creatinine level has hit a plateau at 2.1. Jordan has a history of chronic pancreatitis, one admission lasting 45 days, after a shortened Make a Wish trip to Disney. After doing an ERCP, they found a rare defect called an annular pancreas. They did a rework of her intestines around that defect and since we haven’t had one episode. Jordan caught the flu in January of this year, and suffered a stroke. She initially couldn’t walk or talk, but has been very determined as usual, and is now talking very well, and although quite dystonic in her lower extremities, has been walking pretty well. She has balance problems along with the dystonia. Her Dr’s are recommending botox injections to help with that. All in all, Jordan is so strong. She never complains, except for when she gets cramps in her feet at night (we’re told it’s a kidney failure symptom), and has worked very hard to keep up her schooling. She missed a half of the 2nd and 3rd grade, is in extended school year for the summer, and will attend the 4th grade this year.

Jenna, our second daughter, was diagnosed prenatally. We hadn’t planned on a sister for Jordan, but Jordan was wishing really hard for one. She got her wish, on 5 June 2001. Jenna was born at Hopkins, after a normal pregnancy. I was 41, but didn’t have any gestational diabetes or panic attacks with her. She was immediately placed in the NICU, was by all tests a perfect little girl. They put her on the required meds, and restricted diet and we took her home within 1-1/2 weeks. Jenna’s first year was an easy one. The usual admissions for vomiting and dehydration. but by 2 was already diagnosed a few times with pancreatitis. She has been through every test and scope there is, and she doesn’t have any defects in her digestive system or pancreas. Jenna is now 4, and has spent many days as an inpatient. She is significantly delayed; we think she is going through 2, 3, and 4 now. We have her placed in a restrictive school now, so she can learn some social interaction skills, but can’t keep her well enough to keep her there. She was in a different school last year, less restrictive, and she wasn’t very cooperative. She missed 2/3’s of the year. Jenna has had a stretch of admissions for the last 2 years that have added up to over 40 some. One or two weeks in, 2-3 days at home, back in. Recently, we had almost 5 wks home (one overnight admission to hydrate her), but ended with another almost 3 wk admission. She came home 2 days ago, after spending a week of that in the PICU. She has a pattern of stop eating, stop pooping, and start vomiting. We have tried lactinex for belly bugs, augmentin, erythromycin (back on that one again), and baby food. Jenna always has multiple loose stools, but after trying to add fiber/pectin with baby food once a day, she became impacted. That got her a ticket to the PICU. She was hypertensive, her glucose increased and required an insulin drip, and her lipase/amylase levels went high enough to again diagnose her with pancreatitis. Jenna is also very anemic. Her hematocrit/hemoglobin levels drop to nothing when she gets sick. She has had 7 transfusions already this year. Her last 2 admissions her liver enzymes have sky rocketed, but scans of her kidneys and liver show nothing to be concerned about. Any suggestions/referrals/information on any of these mysteries is welcome. Her Dr is the most wonderful woman, and is one of the smartest people I’ve ever met. She tells us everything, and admits to being puzzled. We have consulted with every other service in the hospital and still have many questions. Jenna has had a rough life. She speaks very clearly most of the time, but only says things when she wants to. She walks like a toddler, wobbly a lot, and we won’t let her on steps without assistance. She demonstrates echolalia and developmental delay. She is lovable, and determined. She gets into everything, but definitely has a world of her own. We can’t blame her for shutting people out; she’s been through a lot. Both girls have portacath, they are both g tube fed 24 hr/day using the Enterite pump, and allowed 4gms of protein per day. Their formula is Pediatric Vivonex, H2O. Polycose is added for one, not the other, and their meds. Jordan gets over a gallon/24hrs, Jenna 1/2g/24hrs. Meds include, Bicitra, Carnitine for both – Polycitra K, Claritin, E-mycin, and miralax for Jen. Epogen, Atenolol, extra salt, clonzapam, and Artane for Jordan. Both girls have a low heat tolerance and a natural aversion to foods that contain protein. Jordan complains about the smell of cooking meat, so we grill outside often. They both seem to prefer certain food textures, and like salty/sour taste rather than sweet. We also have to watch fat content due to their history of Pancreatitis.

We refer to our girls as “high maintenance”; we are always exhausted, but wouldn’t trade them for anything. We are proud of them both, for all of their strength. When we wonder how we can keep going, we look at the strength they have and we know we can do it. They are wonderful. At the end of each day, when we put them in their beds and they say “I love you”, it’s all worth it

Joey and Kathy
Baltimore MD

From the Summer 2005 OAA Newsletter