This is Sarah. She is a strong, strong girl. At the age of 3 (pictured), she was diagnosed with Ulcerative Colitis, an inflammatory bowel disease in which the body’s own immune system attacks the soft lining of the large intestine, causing inflammation and bleeding, after I (her dad) found blood in one of her diapers. Later, blood tests and biopsies would give indications that it was in fact Crohn’s Disease. This proved, to me at least, that when my doctors had told me that my own Crohn’s Disease could not be genetically inherited, they were wrong. As it would later come to light, there is an approximately 9% chance. Lucky girl.
Sarah was prescribed Pentasa, and that’s how we found out that she was allergic to Pentasa. Her symptoms increased tenfold. She was bleeding so badly that she became anemic in a day or two on the new medication. She was admitted to the nearby children’s hospital, where I moved in with her for almost two weeks. She was fed intravenously and pumped full of IV steroids. We watched SO MUCH Veggie Tales. I can still recite the script to Madame Blueberry and sing all of the songs ten years later. After over a week of eating nothing but what was dripped into her arm and lots of steroids, Sarah felt much better and was discharged from the hospital.
So, my daughter was allergic to Pentasa, which means she was allergic to 5-ASA. This eliminated something like 45% of all available drugs for Colitis/ Crohn’s Disease right out of the gate (we’ve since tried 5-ASA class drugs again just to be certain. Sure enough she was hospitalized again, requiring a blood transfusion that time.) She was started on 6-mercaptopurine, an immuno-suppressant drug designed to prevent the body from tearing apart its own guts. For as long as she’s been on 6MP, she’s gotten at least 4 colds per year. We have to be careful about vaccinating her. If there’s a cold at school, she will bring it home. Otherwise, though, the drug worked great for her. It didn’t exacerbate her symptoms the way that 5-ASA did. She was able to eat, play, and be a kid again.
I met my current wife. We started dating. She and my daughter became huge friends right away. ”Hi I’m Sarah Angelica. I’m three and a half. Do you want to play a game?” She’s “mom” now. Bio-mom has been out of the picture for awhile.
While Sarah was on 6MP, she developed idiopathic thrombocytopenia, or ITP. This is a disorder in which the spleen, which cleans and filters the blood, destroys healthy platelets and reduces the body’s overall platelet count way below a normal level. This prevents blood from clotting properly or at all. You might imagine that the combination of Crohn’s, which causes internal intestinal bleeding, with ITP, which prevents blood from clotting, isn’t a good one. Lucky for my daughter, she’s got the right blood type to get WinRHo, and I’ve got very good insurance. WinRHo is an infusion drug that falsely flags red blood cells with a protein that makes them “look like” platelets to the spleen, so red blood cells are destroyed instead of platelets. The tradeoff is a risk of anemia and the side effects of the infusion itself. She had no side effects to the first 5 or 6 infusions but at her most recent one she started shivering in the 83 degree doctor’s office and had a terrible migraine that lasted into the next day. She was grabbing her head and crying. But, it keeps her from bleeding to death and until her reactions become dangerous, she’ll keep getting it, about twice a year.
Sarah stayed healthy on the 6MP for a number of years, maintaining a reasonable remission with a flare now and again that we controlled with Prednisone. My wife (then fiance) and I bought my childhood home (my parents had been forced to sell when they divorced). Sarah moved into my childhood bedroom. We were very optimistic. Sarah was excited to start attending her new school and even had some of my old teachers. My wife and I were married in the kitchen in front of a small group of family and friends. We took Sarah with us on our honeymoon to Disney World. The stress melted off of Sarah and she was as happy, healthy, and strong as I’ve ever seen her before or since.
It didn’t last.
Eventually the 6MP started to metabolise differently as Sarah got older, and at around age 10 one of her weekly blood tests showed that the drug was producing too much of a metabolite that was toxic to her liver. The specialist we were seeing at the time recommended Remicade, an infusion drug with a side effects and warning list a mile long. We were terrified to try it, and we didn’t. Our daughter was in remission at the time. We decided not to risk it. Even though the meds were becoming dangerous in her system, she was still healthy in terms of her Crohn’s Disease. We decided to try going off of meds entirely and moderating her Crohn’s with diet, using steroids when necessary. For awhile–almost a year–this worked. Then it didn’t anymore.
My daughter, now age 11, was admitted to the nearby children’s hospital and she was bleeding BAD. She was pale as a sheet, covered in bruises that stood out against her anemic skin like spots on a dalmatian because her blood clotting disorder makes her bruise easily. She was exhausted from blood loss and pain. Sarah had gone from remission to deathly ill in about a week, maybe two. She didn’t tell us right away when her symptoms came back. Sarah’s got a huge tolerance for pain, of necessity, and doesn’t like to complain. My now pregnant wife and I took turns staying in her room despite my objections that she shouldn’t be staying in the hospital but at home resting. Truth be told, I was running out of PTO at work and it was necessary.
She got a blood transfusion. The head specialist at the hospital pushed the Remicade again. Not to derail the story but I have a history with this man. He treated me when I was little. My parents once questioned him about a treatment decision–wanting to know the pros and cons–and he told them, over my hospital bed in 1984, that if they wanted to question him he would stop treating me and they could find another doctor. Now, you might imagine that my parents were a pain in the ass to him and had provoked such a stark and strange reaction. They were not and had not. He simply is a man who wants to dictate how things will be. I told him that I preferred IV steroids and bowel rest and that my daughter’s treating specialist agreed with me. Later that day, while I was home taking a shower and packing some of my daughter’s books to bring back to her hospital room, he went into the room with my wife and daughter and threatened them with a PICC line, listing all of the risks and embellishing each one, if we wouldn’t agree to Remicade.
We did get the PICC line so that my daughter could be fed intravenously during her bowel rest so she could keep her strength up. We also spoke to the patient advocate and removed the head specialist from her case. We have never spoken to him again.
Sarah did get better on the IV steroids and bowel rest, but not better enough. Her specialist told us that it was probably going to come down to either Remicade or total colectomy. After some tears, we opted for the Remicade infusion. (At this point, my daughter had 6 IV lines going into her at various locations, including the central line. On more than one occasion I had had to correct one nurse or another on the IV pump settings to prevent an overdose of one med or another, because of the complicated net of tubes and 4 separate pumps that operated them at the time.) The day arrived and with a LOT of trepidation and anticipatory worry, the Remicade was infused. We ground our teeth for the entire hour it took to complete the dose. My daughter got well again within hours. It was a miraculous effect. Some color even returned to her cheeks. My wife, now 6 months pregnant, went home to sleep properly for probably the first time in over a week.
My daughter got 2 more Remicade infusions over the next few months. Her color came back and she was able to eat and to sleep without waking up to use the bathroom (really, to go bleed into the toilet at 10PM, 12AM, 1AM, 2AM, 3AM…) She stopped missing school. She participated in the Lego League robotics competition and another after school science/engineering program. She marched with the band. She sang in the chorus.
After the third infusion, Sarah started to complain that her shoulders were hurting her. At first, I thought she was trying to avoid chores, but she insisted. We gave her Tylenol and saw her pediatrician, who thought they might be growing pains or Crohn’s related joint problems.
One night, in the middle of the night, my wife went into labor. She thought they were false contractions because they just didn’t hurt much, she said. They were over an hour apart. She told me to go back to sleep. In the morning, they were still an hour apart. I went to work. She called me at noon. I raced home and the baby was almost born in the car on the way to the hospital. My new youngest daughter, Elizabeth, was absolutely beautiful. Sarah came and visited her in the hospital and instantly fell in love with her, too. She’s like a little mother to her to this day. The baby had severe jaundice and had to stay in the hospital, which was considered minor as jaundice is easily treated. Unfortunately, the hospital was a little too cavalier about it in the end. My wife boarded in the room with her and watched while Elizabeth lay in an incubator under bright blue lights to reduce her bilirubin count, with a blindfold to protect her eyes.
My oldest daughter’s shoulders kept hurting. Then, her ankles hurt. Then her knees. Her hips. Two itchy “bug bites” she had on her wrist suddenly spread out to the size of quarters and she developed 12 more on her abdomen and chest. After a week and a few visits to her baffled pediatrician, she was unable to walk and had to be carried everywhere. She was admitted to the children’s hospital again, under care of the Infectious Disease department. I insisted it was the Remicade. The doctors told me that her symptoms weren’t consistent with a drug reaction.
My oldest daughter developed psoriasis-like sores, big, red, itchy, peeling patches all over her arms, legs, between her fingers and toes, on her scalp, belly, chest, back… everywhere. She developed blisters on the bottoms of her feet and ulcers in her mouth. Where her scalp was inflamed, hair fell out (thank goodness never very much of it.) Biopsies were taken and cultures were grown and parasites were tested for. She was quarantined to her room. She hid under her blankets when dozens of medical students would parade into her room and want to look at her arms, her legs, her rear, and take pictures. Students were in her room literally every hour including all night long. After 3 days of this I told the charge nurse that enough was enough and I didn’t want her to be a teaching case anymore, but that request was soundly ignored. I stayed in Sarah’s room every day, leaving only for a few hours at a time to visit my wife and newborn infant in another hospital, 50 miles away.
Elizabeth (we call her “Izzy”) was sent home with my wife from the hospital. I left my oldest’s hospital room for half a day to go wait for the discharge paperwork and drive them home. The baby seemed more tired than she should be and slept literally all the time. Her skin was a very dark brown but we wrote it off as a newborn thing since nobody at the hospital seemed concerned. She slept through her first night home, which is never supposed to happen. The hospital doctor assured us she was just recovering from the jaundice and would perk up in a day or two.
At the children’s hospital, eventually a pharmacy student found a published journal article that described Sarah’s strange condition symptom-for-symptom. It was a rare Remicade reaction experienced by around 0.3% of recipients. A serum reaction, plus. No more Remicade for her as the next dose might kill her. Sarah still has daily pain throughout her body to this day, as well as scars from the lesions on her skin. Rarely, she’ll wake up immobilized by pain and once again need to be carried in order to get around. There has been no diagnosis, but neuropathic therapy and orthotics have been prescribed.
The baby kept sleeping all the time. It was difficult to wake her up to eat. We saw a pediatrician (we’ve since fired her), and she ordered a blood test which showed bilirubin levels just below the danger point for brain damage. She said to wait until the next morning and see how the baby was doing. If we had listened to her, I have no doubt in my mind that Izzy would have suffered kernicterus which is a horribly debilitating and very preventable condition in which the brain is terribly damaged by bilirubin diffusing into the spinal fluid from the blood due to very high concentrations, and then precipitating out of the baby’s spinal fluid onto the brain’s surface. The resulting lesions can cause mental retardation, palsy, partial paralysis, and death. We would find out later that Izzy’s bilirubin levels were so high that it had begun to diffuse into her spinal fluid across the blood-brain barrier.
Luckily we didn’t listen and we brought the baby to the emergency room of her birth hospital, reasoning that her records were there and that the doctors there already knew about her treatment course. We wanted her back under those Bili lights ASAP and no backtalk. We walked in with a comatose 5 day old infant. The charge nurse and the triage nurse stared at us like a live rattlesnake. They were afraid to touch her. They had apparently no experience between them with sick neonates and no OB residents were in attendance, just a pediatric physician’s assistant. Eventually they put us in a trauma room way in the back of the ER and handed us an adult O2 mask, then told us to “blow it on her face for awhile, see if she wakes up), then left us in there alone for an hour and a half. The PA finally had time for us and came down to do a blood culture to rule out meningitis. She reassured us and told us not to worry, because jaundice is no big deal. Somewhat later, I think it may have been 3 or 4 hours, they finally put her back under the Bili lights, probably to shut us up. The hospital pediatrician later confirmed that her levels could have risen to a very dangerous point if she hadn’t been given the lights that night. We don’t know what the count was at its peak but we know it was quite high at the last draw before treatment.
While she was prepping the site for the blood draw, the physician’s assistant contaminated it with an ungloved finger. My wife is a nurse and I’m in nursing school. We know she screwed it up. At the time, we were too tired and shell shocked to make a fuss. The test came back positive for Strep A. I insisted on a second culture, which they drew, but they started IV antibiotics anyway, with all their attendant side effects, just in case (the second culture came back negative but only after 5 days of IV antibiotics for no reason on a sick neonate). During this time she also had a lumbar puncture (spinal tap) in order to test for any infection of the spinal fluid, and that’s how we found out, incidentally, that bilirubin had infiltrated there. We insisted the baby be transferred to the regional leading NICU. She spent a week there, which was blessedly little time, thank God, compared to some of the other poor babies in there. She’s fine now, except that at age 2 she’s still not talking yet. She’s been evaluated by early intervention workers who say she’s within normal development ranges, but we will always wonder if she was injured by the hospital’s laid back attitude toward her jaundice.
So, colectomy for the oldest then, since Remicade was out. Except that we weren’t going to do that. I was too afraid that cutting out the diseased colon would just cause the disease to migrate to the small bowel. This happens for many patients and my daughter was only 11, too early in my opinion to be cutting her guts out and curtailing her future treatment options. I called my old childhood specialist (not the jerk who had treated me in hospital once and who had threatened my wife and daughter with a PICC Line, the one who had saved my life by suggesting dietary changes when no other doctors were even considering diet as a part of treatment) in Boston. He suggested we combine 6MP with Allopurinol, a gout medication. The allopurinol competes for some of the same digestive enzymes that 6MP is metabolized by, and thereby prevents much of the toxic metabolites of 6MP from being formed. In other words, it keeps 6MP from poisoning her liver. After she was released from the hospital with topical medications for her rash and Tramadol for her total body skeletal pain, we put her on the 6MP/Allopurinol combo. It worked great.
For awhile.
Sarah got sick again after about a year of the 6MP/Allopurinol cocktail working great. We put her on high dose steroids, hoping it was just a cyclical flare we could fend off and then resume her previous therapy. She got better. Her joints stopped hurting. She felt like a million bucks. Loading somebody up with corticosteroids will do that, as long as they haven’t been on them too long.
Eventually, slowly because Sarah has always been very sensitive to Prednisone withdrawal, we were able to wean her back off. Near the end of July 2011, she took her last dose. She was still on the 6MP/Allopurinol cocktail. Once again, Sarah felt fine.
For three weeks.
As of this writing, it’s been three days since she got sick again. Sarah was in terrible abdominal pain. She started bleeding. She has bruises all over her body and she has petechia (like little red freckles) all over her neck and chest from burst capillaries. She turned 13 on Wednesday (today 8/10/2011). She’s back on steroids again, but it’s been too little time since the last course. Side effects are already showing up. She’s got the moon face, the acne. No doubt her bone density has dropped, but we haven’t had a scan yet. We’re terrified to try Humira. Clinical trials are too far away. Her specialist in Boston, my childhood specialist who saved my life, has just gone on indefinite sabbatical.
On the recommendation of another Crohn’s sufferer on reddit, I purchased a book called Breaking the Vicious Cycle, which advocates a sort of modified keto diet. I’ve only just started reading, but we’re optimistic since I’ve had great success in controlling my own condition with diet in the past, though this diet is radically different from what I’m used to.