When Kate was 18 months old she suffered a serious episode of Cyclical Vomiting Plus Syndrome. We did not know what it was called at the time, we called these episodes of frequent acute illness her ‘episodes’. We had been seeking help for Kate for months. We were looking for guidance and leadership for her diagnostically and with care for her multiple medical conditions. We had excellent specialists involved, but without a unifying diagnosis, no one seemed prepared to take the lead on Kate’s case.
April 23rd, 2009
Kate and I arrived to the emergency department at 5:30 p.m. It was early evening, and there was an line up literally out the doors of the emergency department. Kids with their parents looking feverish, snotty, coughing, crying and generally unhappy. As I stood in line with Kate, she had become very quiet. She dozed against my shoulder and didn’t seem to have the energy to lift her head. The fight had gone out of her. For a moment I thought to myself, “maybe I’m wrong about this – maybe she’s fine and really not that bad – this is a long line up, perhaps we should just go home”. As we arrived at the triage desk, a young mom rushed by yelling for a nurse. Her daughter had cut her finger on a sharp can. There was a bloody bandage on the finger and a flap of skin that looked pretty gross. But the bleeding had stopped and the nurse calmly guided the mother to a seat and told her the finger looked ok and that she would need to wait her turn for triage. Kate and I approached the desk, handed over our health card, and in my fatigued state I tried to briefly relay what had been going on for the past several days/weeks. It was/is almost impossible to relate what is ‘wrong’ with Kate in a short conversation. I should have asked, “Do you have 20 minutes”?
When I mentioned that Kate was being followed by metabolics at CHEO that seemed to get things moving a little more quickly. Kate was taken back for a triage assessment at which point everything moved at an accelerated pace. Kate had high blood pressure,her oxygen saturation levels were in the mid 70s, and she was tachycardic (very high heart rate). She was marked as ’emergent’ on her chart and Kate was taken to a ‘resus room’ which is an area of the emergency department where children are taken for immediate treatment. We were soon surrounded by doctors and nurses who were assessing and treating Kate. Bloodwork was immediately taken and the team worked to get an IV into Kate so that they could provide IV bolus, which is a large volume of fluid given over a short period of time. Kate was acidotic – indicative of severe dehydration.
The metabolics team was called, and the resident ordered a full metabolic work up on kate. The ICU (Intensive Care Unit) was also called for a consult and ordered a portable chest x-ray for Kate as they were considering pneumonia and started immediately treating her with an antibiotic. Zofran was also ordered to combat the nausea and dry heaving. The emergency team also considered administering chloral hydrate – the sedative that had been prescribed for Kate which we had not yet used. The idea was breaking the ‘cycle’ of pain and irritability she was in. Neurology was consulted, and felt this was ok, but as her parents we objected and expressed our concern that with low O2 saturation and the pH levels she had, we were not comfortable sedating her.
By 11:30 p.m. that evening, Kate had received 5 IV bolus of fluid. She was still in emergency resus and was being transferred to the PICU (Pediatric Intensive Care Unit). Dr.C had been paged and was coming to see Kate in the morning.
At 2 a.m. Kate and I arrived at the PICU. Kate was now on oxygen to help facilitate her O2 saturation. She was acutely acidodic with a very high anion discrepancy. She received further IV bolus and now had potassium added to the fluid in order to balance her electrolytes.
At 3 a.m. the team required further bloodwork to assess Kate’s condition. Kate’s body was in a such a state of dehydration and shock that the nurses were unable to locate a vein to get blood. Kate was poked in the arms, wrists, hands, feet, and head multiple times. Despite her severe state, Kate cried – producing no tears. Then she slowly began to give up and not fight any longer. I held her each time she was poked and tried to reassure her, knowing that this was all so awful, but necessary. The anesthesiology resident was called to try and access a vein – with no success – and at this point I had asked to excuse myself to a chair. I couldn’t restrain Kate any longer. The toll on me emotionally was too much. The VAT (veinous access team) were called – with no success. Finally, after about 2 hours of attempts to draw blood the team decided to try an arterial blood draw which was performed by the anesthesiology resident. Blood was finally taken.
Over the course of that night, I lay in bed with Kate stroking her head. She was sleepy, but fitful and clearly exhausted. It seemed I could not comfort her, and I felt at a loss of what to do.
The next morning was a flurry of activity with consultations with Dr.C (metabolics); Dr.D (neurology); Dr.G (nephrology); and, Dr.S (endocrinology) – all specialists that continue to follow Kate to this day as part of the medical team at CHEO. More blood was taken and urine samples were analyzed. Kate was scheduled for a CT and MRI Spectroscopy. (MRI Spectroscopy measures biochemical changes in the brain. While MRI identifies anatomical abnormalities, MR spectroscopy compares the chemical composition of normal brain tissue with abnormal tumor tissue). I called Dr.S and he came to see Kate that afternoon to discuss the situation – but mostly to support me, for which I am ever grateful. The focus for that day was stabilizing the severe acidosis due to dehydration. My focus was on trying to make Kate more comfortable and to have the team help her with her pain.
Kate had a difficult night of restless ‘sleeping’. She moaned during the night as was clearly uncomfortable. She seemed less responsive than the previous day. She put up no fight with the nurses or bloodwork. What was most unsettling to me is that she would look at me, but not seem to really see me. Her oxygen saturation levels were still very low and the team was conferring on what to do next. It was decided that they would do another chest xray, and that a lumbar puncture might be appropriate to check for meningitis. Nephrology were considering many renal failure scenarios based on Kate’s urinalysis and metabolic concerns. Kate’s labs were indicative of a condition called renal tubular acidosis, which is not a diagnosis but a symptom of a further condition. In Kate’s case it was finally determined to be transient (occuring in isolation and not chronic). Kate’s unrine had protein in it and hypercalciurea (a significant loss of calcium), a condition she continues to be treated for to this day. The team felt this might help explain the acidosis, but it was not telling the full story, so an immediate ultrasound of Kate’s kidneys was ordered.
That evening, Kate’s CT results came back and appeared normal. A LP (lumbar puncture) was ordered for that evening – those results were also normal. Kate was starting to be a bit more awake. She was opening her eyes and watching me. She was begining to sip from a bottle. As long as she was given medication for discomfort, she seemed to settle and be able to sleep. I made a list of what was being considered:
– elevated aldosterone levels
– Sistenosis (lab samples sent for testing)
– Pearson Syndrome (now ruled to be ‘not likely’)
– Thiamine Resistant Megaloblastic Anemia (awaiting test results)
– Mitochondrial DNA deletion sequencing (now ruled ‘not likely’)
– lactate levels
– echocardiogram to examine enlarged heart that was identified via chest xray in PICU
– sideroblastic anemia
– developmental delay
– delayed myelnation on MRI
– lethargy and irritability (ongoing)
I also added to my list that our biggest question and issue for discussion was how to manage further episodes – a plan, and to have a letter for emergency to give to triage.
Kate had stabilized enough to move her from PICU to the floor on 4 East. I was nervous about the move. You get into a sort of state with the constant care and fishbowl existance of PICU that you become a bit dependant on the level of care. Kate was still not well, but she was stable and she became a little more of our responsibility to care for.
She remained on oxygen and IV fluids. I was beyond exhausted, but unwilling to leave Kate. Brian and I finally decided on a ‘day’ shift/ ‘night’ shift role that we continue to this day. I would do the day when there was the most action with specialists, and Brian would stay the night with Kate. We entered a routine of coaxing fluids and food into her, weighing diapers, administering medication, facilitating exams by nurses and the medical team. Kate was slowly recovering and there was nothing to be done but to support her as she slowly came out of her episode. Exams and tests were ordered and my mom arrived to lend support as well. An MRA was added to the list of tests as was testing for biotinase deficiency and a skin biopsy. (MRA, magnetic resonance angiography is used to generate images of the arteries in order to evaluate them abnormal narrowing, occlusion or narrowing and risk for rupture.)
The team had been conferring about Kate’s case during this admission and were clearly in agreement with us that something needed to be done to better facilitate the coordination of her care. A social worker from neurology was assigned to Kate’s case, and Kate was referred to the palliative care program.
A note about pediatric palliative care:
When people hear that Kate is a palliative care patient, they often looked shocked and concerned. It’s important to know that pediatric palliative care is different from that of the adult population. Palliative care in the peds population does involve ‘end of life’ care, but it is much more thant that. Palliative care patients in the pediatric population are children who have life limiting disease, and who require pain management and respite support. Kate qualified for both of these.
Kate’s medical team also organized the much awaited and requested medical meeting to discuss her case. As her parents, we asked for 3 specific items to be discussed:
1) That we be referred for a second opinion concerning Kate’s case, and that the following be discussed:
– where would we go for this second opinion – when will this happen – what specialties will we meet with
2) That we wanted a crisis letter for medical triage at CHEO emergency department which would include: an intervention/treatment guide for Kate’s episodes.
3) We asked for a discussion about better managing Kate’s discomfort/irritability/pain during her episodes.
Kate continues to have discomfort and difficulty sleeping. Her IV is moved from her foot to her hand as the 1st site has become swollen and red. Kate successfully manages a general anesthetic administered so that the team can image for a MRI, MRS and MRA.
The team conference is held and we are invited to come in at the end of the meeting to hear what the team has decided for Kate.
Kate will be referred to Sick Kids in Toronto to examine bone marrow failure and her bloodwork will be examined by a lab in Boston specifically for sideroblastic anemia.
Kate will also be seen at Sick Kids by neurology and metabolics by a colleague of Dr.C.
Dr.B, Kate’s gastroenterologist has outlined further tests for Kate to explore the GI issues she is having, i.e. cyclical vomiting.
The referral letters will take 1-3 months and we will be supported by CHEO social work in making any arrangements.
Dr.Sp is now Kate’s palliative care doctor. He gives us a tour of Rogers House and discusses his role in pain management and palliative care support for Kate.
CHEO has also committed that Kate will have better coordination within hospital upon discharge, and that her discharge summary from this hospitalization will included a triage letter for the emergency department.
On May 4th, Kate is discharged from CHEO. She has not returned to her ‘baseline’ and is still unwell, but she is stable and has been continuing to improve. We discuss sleeping issues, discomfort, and fluid intake with the discharge planning nurse and clarify the procedures for the next episode Kate might (will) have.
We took Kate home after 2 weeks in hospital, but it was not to last long.