Sue and Lou, both medical professionals, took exception to the way I portrayed doctors on my posts about Doah, especially the one about his recent emergency room visit, which made me momentarily revisit the question of whether or not we should conserve him (i.e. legally become his guardians since he is now an adult). I supposed my characterizations were a bit one-sided since I was dealing with some of the negative (from a parent's point of view) aspects of the emergency medical situation. Obviously, my children would not be alive today if there had not been some very special medical professionals in their lives. I promised Sue and Lou on that earlier post to show the other side of the picture. So, here is the first of my posts for them.
Noelle is 33 years old, twelve years more than we were told she might live after she survived her first surgery, principally because crede, pushing on the abdomen and squeezing the bladder, killed off the bladder in about 20 years. When Noelle was three, intermittent catheterization was found not to introduce a sufficient number of bacteria into the urinary tract to cause infection and therefore could replace crede for keeping spina bifida children dry. She is now heading toward a long, full life. When she works, she does copyediting and has been a great proofreader of several of my books. She completed two years of college as an English major; Shakespeare has been her love since she was in grammar school. She lives independently in an apartment in Salts, 20-30 minutes from where Donnie and I live. She is mostly occupied these days with her significant other who fell into a diabetic coma, likely brought on by renal failure, in April 2006. From then until December 2008, he was in and out of a coma and hospitalized 5 hours away, which made it very difficult for her to see him because she cannot ride for five hours; she has to self-catheterize every four hours, and that complicates trips of any type. When he was awake and aware, he needed a breathing machine and made a request for extraordinary care; he did not want to have the plug pulled. Last Christmas (2008), he was finally stable enough to be taken off the respirator and transferred to a nursing home in Malek City, near Noelle. He still has no functioning kidneys and is not a candidate for transplant, so he needs dialysis three days a week. Given his medical needs, right now there is no hope of his ever coming home, but then Noelle had only a 50% chance to survive her initial surgery and her life expectancy was only 21 years. So, why should Noelle not have hope? At least, it is easier for her to maintain daily contact and provide emotional support when he is so much closer and not on a respirator. One measures good things by different instrumentations when one lives at the very edge of life.
Noelle was born with myelomeningeocele, the most severe form of spina bifida, and, as is sometimes present, she did have Arnold Chiari malformation. Soon after birth, she developed hydrocephalus (water-on-the-brain), for which a ventriculo-peritoneal shunt was placed. (Actually, her first shunt was ventriculo-atrial, ending in the heart instead of the abdomen, but somewhere along the line between shunt #1 and shunt #12, it became a VP type.) Around age 4, she had a grand mal seizure and was determined to have epilepsy although that is under control except for an occasional absence seizure, the greatest significance of which is that the state of California will not allow her to drive (and should not do so; she understands that). From birth, she has had a neurogenic bladder, the reason for the need to self-catheterize every four hours; otherwise, she would be wet all the time. She has also had a neurogenic bowel; we have tried a variety of bowel-management programs but the amount of enervation is too great for any of them to work, so she wears adult diapers. She is also paraplegic, having limited sensation between chest and hip and none at all below the hip, so until she went to college, she wore long-leg braces from chest down. Now, it has become easier for her to manage being in a wheel chair. We are delighted that she was able to stay in braces more than 20 years (doctors had suggested a wheelchair from age 5) because it gave her such a feeling of independence: wading in the ocean, swinging on swings, sliding down slides, and even roller-skating with her kindergarten peers. As a teenager, she was not the top-dog on the totem pole, ended up out of school for medical reasons for almost an entire year, and during that time began slowing pulling out her hair, a psychological disorder called trichotillomania. She still struggles with this and generally covers up the damage with a hat.
Noelle's special medical providers:
MAJ (USAF) Wayne Paullus was Noelle's first neurosurgeon. I was an Army officer stationed at Goodfellow Air Force Base when Noelle was born. The tiny town of San Angelo, Texas had limited facilities, and so Noelle was air-lifted 2.5 hours away to San Antonio's Wilford Hall Medical Center. This was in the early years of shunts and saving spina bifida children and well before they could be promised a full life. An Air Force hospital, in particular, was not highly experienced in such defects. Perhaps Noelle should have been sent, instead, to a children's hospital, in which case the surgery would have been a quick 45-minute sew-up-the-open-sac exercise. Instead, Dr. Paullus spent more than four hours in surgery. Since he had never seen a spina bifida case, he treated her as if she had been hit with a grenade or mortar. He spent hours isolating every single working nerve and made sure that it had a clean pathway and would not be damaged by his repair job. No pediatric neurosurgeon that I know has ever done that. As a result of Dr. Paullus's "ignorance," Noelle ended up as "the most mobile child in all of California with her level of lesion" (according to a neurosurgeon at Stanford University Hospital 14 years later). God bless you, Dr. Paullus! Where are you, Dr. Paullus? We have lost track of Dr. Paullus over the years. I had a US Air Force General, a former student of mine, promise to track him down. Now, I need to track her down so that she can track him down!
Dr. Scott Moffett was the senior pediatrician at Fort Devens, Massachusetts, a post to which I was assigned after short stints in Alabama and Arizona. Noelle was Dr. Moffett's special patient, and when Children's Hospital wanted to put her on prophylactic medication to prevent urine infections (typical for children with neurogenic bladders), he was concerned about her developing resistance to antibiotics from overuse and offered to check her urine daily if Children's Hospital agreed. The agreement was made -- and 20 years later, Noelle is in very good shape compared to nearly all of her peers who took the medication: many are now dying from simple diseases because they are antibiotic-resistant. While we were at Ft. Devens, Noelle developed whooping cough, a rather routine illness back then (now there is vaccination) except for children with Chairi malformation who often ended up with tracheotomies and/or brain damage; some even died. Dr. Moffett admitted Noelle into the tiny post hospital and had a second bed put in her room. It was his. Noelle had a rough time with the whooping cough, but Dr. Moffett was never far from her room during the day, and nights he slept on the second bed. Two weeks later, Noelle was home and well! (A few years later, the nursing faculty at Renboro Hospital, which specialized in spina bifida care among other defects, told me that they had had no Arnold Chiari children survive whooping cough totally unaffected the way Noelle had.) God bless you, Dr. Moffett, wherever you are.
Andi Kush was a special caregiver we met in Lower Burrell, Pennsylvania when we lived nearby and our children were attending Lower Burrell Christian Academy. (Yeah, I know -- odd that an atheist at the time would send her children to a Christian school. That decision was a no-brainer: the academics were the best around; for the same reason, I had sent our older daughter, Lizzie, to a Catholic school when we lived in Massachusetts. I counteracted the religious teachings at home, a not-so-great decision, the long-term effects of which I am still dealing with.) Andi was an extraordinary physical therapist. Noelle needed surgery before she could get braces and waited eagerly for them. In the interim, Andi made sure that her muscles were in great condition so that she would not have to build them up after surgery. The first day that Noelle got her braces, she was walking, thanks to Andi. (Later, Andi helped Doah learn to walk and pushed me into making contact with the doctor in Cininnati who ultimately saved him.) Andi was more than a physical therapist; she was my therapist -- always willing to talk about anything related to raising my special children, never judging or criticizing, always open to the impossible (like figuring out how to help Noelle learn to roller skate safely). Andi remained in touch for years, and I would sometimes call her for a sanity check, especially when someone was telling me that I should not expect Noelle or Doah to do something that I knew that they could with a little inspiration and a lot of perspiration. I lost contact with Andi when we went to Jordan, and that was a very sad loss. Her contact information no longer works. (Sniffle, sniffle!)
Our next stop after Renboro was Washington, DC, where I had been offered a position with the State Department, and Dr. Ronald Uscinski . Little did I know the night that Ron did shunt repair #9 on Noelle how deeply he would affect our lives. Noelle had ended up rather unexpectedly at Georgetown University Hospital, and Ron had been pulled out of bed at 2 a.m. for surgery with no access to any records, all of which were at Noelle's previous hospital. "OK, Mom," he said, "You're on. Spill out the contents of your memory." And that is how it was with Ron. He trusted that parents knew their children perhaps even better than the doctors who never lived with them. When I needed someone to support me and prepare all the international paperwork to bring Shura, a spina bifida child artist from Siberia, where he was hospitalized and dying, to the USA, Ron was the person I turned to, and he did not let me down. When I needed someone to support me in standing up to the neurosurgeon at the hospital in the USA where Shura ended up and not allowing repair of Shura's lipocele for fear that the surgery would make him worse off, Ron was the person I turned to, and he did not let me down. After all that, I could never forget Ron. Fortunately, I don't need to. I am on his e-mail distribution list for all his latest crazy ideas and maverick agendae!
Dr. Nina Scribanu, the only emigre Eastern European (Romanian, to be precise) doctor I know who was able to be licensed in the USA without going back to school, was another Georgetown doctor. The director of the Genetics Clinic, she had much interaction with the spina bifida clinic patients. The capacity of Nina to be compassionate was unbounded, and a good example of that is the evening that Noelle was admitted (I don't remember for what), and there was no bed available. The nursing staff put a blanket on the floor until a bed could be found, and Nina sat on that blanket with her for nearly three hours (of course, she would periodically nudge the staff to keep searching for a bed, which, of course, they were doing with great energy, considering that a doctor sitting on the floor after hours was constantly in their sight -- and not just any doctor, but the clinic director). Nina spoke excellent English and even better Russian (my Romanian is limited to reading very general kinds of things), and during the time we lived in Washington, she and I would go to Russian movies and then back to her house for tea, where her husband also spoke Russian. She kept in touch for a number of years, and when I learned that a lab in England had found a connection between Noelle's and Doah's defects, a weak X chromosome (we had been assured over and over for ten years that there was no connection, but I refused to believe that), Nina flew to England to find out more! A few years later when we were living in California, I was back at Georgetown University to lecture and for the sake of old times dropped by the clinic. The coordinator told me that Noelle's records were still active. "She is so special to us that we just are not yet become ready to archive them."
So, Sue and Lou, I hope that provides another, very different picture of what we have experienced from medical professionals in our lives. Yes, we have had some raw deals, but we have had some people who can only be described as God's angels.
Coming tomorrow: the angels that God sent to Doah!