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Emily, age 2

• 1998 Jul 31: Emily, 26 months old, begins losing her balance when walking. She is otherwise fine and in a good mood. Her appetite is good, no fever, no vomiting, no lethargy, no runny nose or coughing. As she is quite verbal, she would have told us if she had had a headache or other pains. This is the Friday before a long weekend. Because she seems otherwise well, we decide to just wait a few days and hope it resolves itself.

• 1998 Aug 4: By Tuesday, her gait has not improved. We call our family doctor’s office. The nurse thinks that she may have an ear infection and that we should come in to see the doctor. The family doctor, perplexed by the absence of other symptoms (such as those suggesting an ear infection), refers us to a pediatrician the very next day.

[In retrospect, we were and still are extremely grateful that the family doctor advanced our case to a specialist so quickly. In the following months we would read and hear of so many cases where brain tumours were misdiagnosed as ear infections, flu, and even behaviour problems!]

• 1998 Aug 5: The pediatrician notices a slight eye tremor (nystagmus), indicative of a cerebellum problem, and immediately refers us to Toronto’s Hospital for Sick Children (HSC) Emergency, where a CT scan reveals an abnormal chicken-egg-sized mass in Emily’s posterior fossa. We are admitted in anticipation of surgery. Our world has suddenly collapsed around us. Yet Emily has been amazing, cooperating for two sets of CT’s without any need for sedation; she is only 26 months old!

[That night was the worst night of our entire lives. Neither of us slept a wink; we just wanted to turn back the clock, wake up from this horrible nightmare, and go back to our peaceful lives of just a few days ago. Just a short while ago everything had seemed so perfect – we were about ten days away from the expected birth of our second child – and now suddenly everything had fallen apart.]

• 1998 Aug 6: MRI confirms CT results. One of the neurosurgeons tells us that because Emily's condition is not as urgent as it might be (no hydrocephalus, no lethargy, etc.), "we have the luxury of time". Surgery won't need to be tomorrow (Friday) but can wait until Monday morning. We have stumbled into a frightening world where "luxury" means brain surgery in four days... The neurosurgeon explains that in many cases, by the time kids are diagnosed with brain tumours they are often suffering from high intra-cranial pressure and need intervention immediately to prevent coma.

• 1998 Aug 7: Our staff neurosurgeon meets with us to discuss the scan results and the treatment plan. The first course of action will be to do brain surgery to try and remove as much of the tumour as possible. The tumour is quite deep and possibly partly grown into the brain stem, which is inoperable. In addition to the usual risks of any surgery (infection, bleeding), there are risks of operating in that area of the brain. We are to prepare ourselves for the possibility of some post-operative "neural deficits", which might include partial facial paralysis and inability to swallow. The latter would mean that Emily would require a feeding tube. We are also told that in the post-operative period there is a common (about 30%) complication of cerebro-spinal fluid (CSF) build-up in the skull which causes pressure on the brain. This pressure is relieved by draining the excess fluid with a tube called a "shunt". Insertion of the shunt would require additional surgery. Our neurosurgeon will also put Emily on Decadron (dexamethasone) which is a steroid which shrinks brain tissue and thus helps relieve intracranial pressure.

• 1998 Aug 9: Day before surgery. By this time (barely over a week since the initial symptoms) Emily can no longer walk, and has developed a tremor in her hands ("intention tremor") so severe she is unable to feed herself. One of the neurosurgeons meets with us to discuss tomorrow's surgery, answer our questions and obtain our consent. He is wonderful and extremely patient at explaining the procedure and answering our questions. While we are still anxious on the whole idea of "brain surgery", this pre-operative discussion goes a long way in removing some of our fears and educating us about what will be involved in Emily's surgery (our previous knowledge about neurosurgery was basically nil). So that we are not alarmed, we are also warned what Emily might look like coming out of surgery. She may have her head shaved, be wearing a turban-sized bandage, and have lots of tubes and wires coming from her. Also, her symptoms may become worse initially before they start to get better.

• 1998 Aug 10: Neurosurgeons perform a craniotomy, with subtotal resection of the mass (a pea-sized portion is situated in the brain stem and could not be removed). We are told the mass appears malignant, but we will have to wait until official results from pathology which might take a week or so. The operation takes about five hours, during which we wait anxiously in the surgical waiting room and try to eat a little (we have zero appetite). Our first opportunity to see Emily after the surgery is in the post-anesthetic care unit (recovery room) later that afternoon. She is mostly unconscious but looks very good considering everything we had been warned about. Only a 1cm strip of hair was shaved along the incision line which is at the back of her head. The incision is closed with dissolving stitches and steri-strips. She has no big head bandage. In addition to Decadron, she has also been given ancef, an antibiotic. After an hour or so, Emily is transferred to the critical care unit (CCU) where she is expected to spend the next 24 hours or so. When we see her in CCU, she is somewhat conscious but very drowsy. Owing to a slight fever, she has an ice pack on the top of her head. As for monitoring and intubation, her heart (ECG) and respiration are monitored, blood pressure measured through an arterial line, Foley catheter to drain urine from the bladder. She of course has an IV for fluids and medication. She is receiving morphine for pain as well as the Decadron. By around 19:00 that evening, she says her first words after surgery (she comments on the red light on her toe, where the oxygen-saturation monitor has been attached). As for the need for a shunt, all we can do is wait and observe her over the next few days and weeks.

• 1998 Aug 11: This morning Emily is transferred from CCU to her room in the neurosurgery ward. She continues to receive morphine for pain management. She is now more alert, but any movement of her head or neck causes her significant discomfort. A physiotherapist drops by to check Emily's muscle tone and reflexes. Reflexes are a bit low, but they are symmetric which is a good sign that the surgery hasn't caused damage. An occupational therapist comes by to introduce herself. In the next few days she will be assessing Emily's arm movement and play skills. The neurosurgeons say that Emily is looking great. She is coming along well. Today she has a post-op CT scan.

• 1998 Aug 12: Emily is off the morphine and is receiving codeine and acetaminophen (Tylenol) for pain management. Today she takes something by mouth for the first time after surgery. She is able to drink a little water and eats a cracker. We are extremely relieved that Emily's swallowing reflex appears unaffected by the surgery. Emily also feels more like chatting today. She talks quite a bit and very coherently. Again we are very happy that her speech and memory appear unaffected also. At today's session with the physiotherapist, Emily is able to hold her head up unassisted, turn it side-to-side (with crying) and take a few steps of assisted walking but is quite unstable.

• 1998 Aug 13: At rounds this morning, neurosurgery staff come to see Emily and report on yesterday's CT scan. It is appears there is some evidence of fluid build-up. If it becomes progressively worse, Emily might require a shunt. For now we can just wait and see. One of our neurosurgeons recommends reducing the I.V. hydration rate to see if it will encourage Emily to drink more.

• 1998 Aug 14: During the night of the 13th to the 14th, Emily's heart monitor alarm starts to go off every few minutes signalling bradycardia (low heart rate). Normally, Emily's resting heart rate is around 100 to 120 beats per minute. This night it keeps dipping into the 60s. We are again worried that Emily's condition is taking a turn for the worse. Our nurse attends and after manually checking Emily's pulse and blood pressure assures us that Emily is okay. While low heart rate can be a possible sign of intra-cranial pressure, the situation is not serious at this point. She encourages us to try and get some sleep. It is now almost 3:30 (am). This afternoon, Emily has another CT scan. This scan is an extended scan which includes not only her head but also her chest and abdomen. The purpose is to check for spread or other tumours elsewhere in her body. She needs to take oral contrast and requires sedation (Nembutal) for the procedure. While her ventricles are slightly enlarged which is an indication of hydrocephalus, it is not serious enough to warrant a shunt just yet. The pressure might become worse or it may resolve by itself. Emily also has x-rays of specific joints: left wrist, knees and ankles.

• 1998 Aug 15: Emily's recovery continues. She still needs codeine in addition to the acetaminophen. In the evening she volunteers to us that her "hand is not strong anymore" (her own words) as she tries to make a fist with her left hand. Again, this is something to observe. In other news, our second child is due any time now. Around 22:30 Sylvie's Braxton-Hicks contractions, which have been increasing in frequency and intensity over the past week, become more pronounced. We mentally try to prepare ourselves for the logistics of the impending birth.

• 1998 Aug 16: Sylvie's contractions continue into the night. By 2:00 a.m. it appears labour has begun. Because we'd been told that the time between onset of labour and delivery for a non-first-time baby is on average about 10 hours, we debate whether Sylvie should stay and labour with Lester as long as possible here in Emily's hospital room, or go to her scheduled hospital to labour alone. We know that the hospital's policy is not to admit a labouring mother until she is well dilated and almost ready to enter active labour. If labour has only just begun, Sylvie could be turned away to face several hours of labour by herself wandering the darkened halls or sitting in the closed cafeteria. Since Emily is asleep and the contractions are quite strong (though irregular), we decide to go to Sylvie's hospital anyway for assessment. At 2:30 a.m. Lester drives Sylvie to Women's College Hospital (WCH), fortunately only a few blocks away. Lester returns immediately to Emily's bedside. The whole hospital experience has made Emily very frightened and "clingy". Only Dad or Mom can console her when she cries, and we don't want her to wake up and find us both gone. Our second child, David, is born at 3:05 a.m., only 15 minutes after Lester left Sylvie at WCH. Good thing we didn't wait!

• 1998 Aug 17: Emily has a bone scan in the Nuclear Medicine department. She is injected with a radioactive isotope a few hours before the actual imaging process. The isotope is taken up in areas of fast growth - growth plates in her bones and cancer cells. We return a few hours later for the actual scan. While Emily cried through the entire imaging process, she did not resist being held still by the nurse for the image capture. No sedation was needed. Our neurosurgeon comes by to assess Emily and inspect the bulge in the back of her head. He confirms that it is CSF build-up. He asks if Emily has been vomiting (she hasn't), and decides to wait a little more. No shunt yet.

[Today Sylvie was discharged from Women's College Hospital and Emily met her brother David for the first time. While we had talked with Emily at great length about the new baby to prepare her for his arrival, we never imagined that David’s birth would coincide with her hospitalization and surgery. In the days after she was diagnosed we were very concerned that she might make a false connection between these two events and resent her new sibling. We were extremely relieved that Emily took a great liking to and interest in her baby brother. While in the hospital, interaction with David provided Emily with a wonderful diversion from the hospital procedures. Many times over the next few weeks Emily would ask to "see David" and we would bring his recliner onto her hospital bed for her to play with him. David is a relatively laid-back little guy in spite of the circumstances. He spends the first two weeks of his life with us in Emily’s hospital room. The HSC staff is wonderfully accommodating.]

One week after her surgery, Emily meets one-day-old David.

That evening, our oncologist meets with us to deliver preliminary pathology results: the pathologist has been debating between a diagnosis of "medulloblastoma" or of "rhabdoid", and will most likely be signing a diagnosis of "medulloblastoma with rhabdoid features". Current accepted chemotherapy for medulloblastoma is the Baby POG protocol, which, in conjunction with radiation, has demonstrated a favorable cure rate (over 50%). We ask our oncologist what would happen if there were a recurrence of the tumour after chemo and radiation. We are told that patients are "not salvageable" at that point [we would later develop a great respect for our oncologist, but at that moment her use of the term "not salvageable" (a standard oncology term) in reference to a child seemed terribly cold and detached]. We are devastated at the reality that there is an almost 50% likelihood that Emily will die from this cancer, though apparently it could be worse. Medulloblastoma is the most common pediatric brain tumour, and has considerably better prognosis than rhabdoid, which has a cure rate of less than 20%.

Our oncologist volunteers that we may also try non-conventional treatments (shark cartilage, essiac, etc) if we wish so long as they do not conflict with concurrent treatment. Before trying something, we should check with her first to ensure there is no conflict. Our oncologist also volunteers that she does not believe the often-heard suspicion that pharmaceutical companies are witholding a cure for cancer for fear of losing the huge industry associated with cancer therapy. We feel it is a positive sign that she has chosen to volunteer her point of view on these topics up front and is willing to discuss them with us. (We later learn that she has a sensitive understanding of a family's psychological need to try alternative therapies (especially in cases where conventional treatment has failed), even if she herself doesn't believe in the effectiveness of many of these treatments and is infuriated by some of the unscrupulous people who take advantage of these vulnerable families.)

Strangely enough, not a single staff member has yet mentioned the "C" word when speaking of Emily's condition. The discussion is one of malignancy, oncology, chemotherapy and radiation. The truth is, Emily has cancer. We wonder to ourselves if the avoidance of this doom-filled term is intentional, or if it is simply that "cancer" is too general a term in this field of specialization.

• 1998 Aug 19: Emily has another CT scan today to check the size of her ventricles. She is able to do it without sedation. Her ventricles are not further enlarged. Our neurosurgeon tells us that Emily will probably get by without needing a shunt. Hooray! Emily is able to walk with assistance (hand holding).

• 1998 Aug 20: Our oncologist comes by with the solemn news that "there's been a bit of a glitch". Revised pathology and final diagnosis: intra-cranial rhabdoid. DNA testing shows monosomy 22 in tumour. Definitely rhabdoid, not medulloblastoma (we later learn many rhabdoid tumours were probably misdiagnosed as medulloblastoma in the past). Chemo protocol will be changed from Baby POG to ICE (ifosfamide, carboplatin, etoposide), to be administered IV over three days on an in-patient basis, repeating the cycle every three weeks (or as soon as blood counts recover). We are told the cure rate for rhabdoid is extremely low, and virtually non-existent without cranio-spinal radiation. As devastating as the medulloblastoma diagnosis was, this revised diagnosis seems an order of magnitude worse. As Emily is too young for radiation, the plan at this point: ten to twelve cycles of ICE to attempt to control the tumour until Emily reaches age three, then proceed with radiation.
  
  Why has ICE been chosen as the treatment? At this time, there is very little treatment information available on rhabdoid.  [Michael had been diagnosed only one month before, and we didn't know about his webpage, which at that time would not have had much information on survivors anyhow].  The few rhabdoid journal articles available show that rhabdoid brain tumour patients have usually been treated with the Baby POG protocol (used for medulloblastoma) and few have survived more than a few months post-surgery.  Hospital for Sick Children has treated six rhabdoid cases before.  Only one has had long-term survival, and this was after using ICE as well as full craniospinal radiation.  Given how few long-term rhabdoid survivors have been documented, our oncologist naturally has chosen ICE for Emily as well.
  
  [We also discussed giving localized radiation to the posterior fossa, but it was not presented as a favourable option.  The hospital was reluctant because they felt that doing full craniospinal radiation later (which they basically considered essential for cure) would cause too much damage to the previously-irradiated brain tissues.]

• 1998 Aug 21: Emily's recovery from the craniotomy is progressing well. The staff even say that we may be able to get a weekend pass. We think that it would probably be more stressful for Emily to go home only to have to return to the hospital a day or so later. We therefore elect not to pursue the weekend pass.

• 1998 Aug 24: Today is the surgery for the port-a-cath (central venous line) insertion, through which Emily will receive chemo (and blood-products, if necessary). When her line is not accessed everything will be hidden under her skin, so she can bathe or swim without any problems. While Emily is still in the operating room a bone marrow aspiration and bone biopsy is also done (from her hip bone) to rule out spread of cancer. Emily receives morphine for pain management.

• 1998 Aug 25: Begin chemo: cycle 1 of ICE. Emily shows no adverse side effects. Emily is now receiving codeine for pain management.

Emily continues to ask to "see David".

• 1998 Aug 27: Because the drug carboplatin can cause hearing loss, Emily has an audiogram (hearing test) today to measure her baseline hearing sensitivity. Emily is still very scared of new hospital procedures. She is uncooperative and refuses to participate. Emily is supposed to look when she hears a sound, but she just clutches Dad's shoulder and buries her head. The audiologist is very patient and understanding. Eventually she uses a different machine which can measure cochlear function without the need for patient cooperation. A tube which looks like the tip of a hearing aid is inserted into each of Emily's ears one at a time. The machine then generates a series of tones and measures the ear's response (I don't know how this works). All Emily needs to do now is to be quiet while the machine makes its measurements. At first Emily screams, but she eventually tires and the audiologist does her work. Emily has "normal cochlear function".

• 1998 Aug 28: We return home after living at the hospital for nearly a month. We are grateful that Emily has escaped the need for a shunt. In the following week she is weaned off dexamethasone (a corticosteroid which reduces swelling in the brain, but has the unfortunate side effect, in Emily, of radical mood swings).

[Emily would go from a contented mood to being upset in literally five seconds. This would happen several times a day. She would be upset for 45 minutes at a time during which she would cry and scream; she was inconsolable and could not be reasoned with. She screamed so much during this post-dexamethasone period that she screamed herself hoarse. Her voice remained hoarse for a few months.]

• 1998 Sep 3: Emily has a fever; we must return to hospital. She is admitted for IV antibiotics (Tobramycin and Piperacillin).

• 1998 Sep 5: While still in hospital, Emily's port begins malfunctioning (first no blood return, later it doesn't flush). Over the next few days Emily has a chest X-ray and a lineogram. A lineogram is like a "video" X-ray enabling the radiologist to see motion rather than just a still image. The lineogram reveals two separate problems: a clot in port reservoir, and a sleeve clot on the catheter. Urokinase injection is given in the port to try to clear the clot, without success.

• 1998 Sep 6: Urokinase is tried a second time in the port, again without success. Then heparin infusion is tried (in a peripheral IV, since the line is no longer working at all now). No results, and unfortunately with this level of heparin Emily's bloodwork must be monitored every 6 hours (from a vein in her hand, arm, foot, anyplace they can find that draws blood).

• 1998 Sep 7: The heparin is not yet at a therapeutic level and needs to be increased.

• 1998 Sep 8: A venogram (similar to a lineogram) is scheduled, and they must start a second peripheral IV for this procedure. Emily now has IV's in both hands. Venogram reveals no additional clots in chest. There is talk of surgery to remove the port altogether and insert a new one. We are very discouraged, as it's been barely two weeks since she had surgery for the first port and all we got out of it was one chemo cycle. A clot specialist comes by and recommends systemic urokinase infusion (rather than urokinase only in the port reservoir).

• 1998 Sep10: Second lineogram reveals that both clots have been cleared! Port is tried, works perfectly. Meanwhile, however, Emily's craniotomy wound has become infected. Start IV antibiotic (cloxacillin) plus topical cream (Fucidin).

• 1998 Sep 12: Port stops working again (no blood return). Start again with urokinase, peripheral IV's, bloodwork from veins in hands and feet. We are so discouraged. Talk of removing port begins again.

• 1998 Sep 14: Third lineogram show no clots. Line is tried, and it works!

• 1998 Sep 15: Emily has a cough and nasal congestion. N-P (naso-pharyngeal) swab tests positive for para-flu (croup) (as if she didn't have enough to deal with…)

• 1998 Sep 18: Begin chemo: cycle 2 of ICE.

Because of her croup, Emily is isolated in her hospital room, but she is allowed to bring a toy from the playroom. She has great fun riding a Barney car, despite her IV pole trailing behind her.

• 1998 Sep 20: Emily accidentally kneels on the IV tubing and pulls out her Huber needle. As the port wasn't heparin locked, a new Huber must be inserted right away to prevent clotting, without the luxury of an Emla patch (local anaesthetic). Ouch.

• 1998 Sep 21: Finally discharged home, after nearly three weeks of problems. Now that she has been off dexamethasone for a while and has recovered further from the surgery, Emily's disposition improves noticeably. Her ataxia (loss of balance) subsides. She begins to lose her hair, but luckily at her age she really doesn’t care, and besides, new little brother David is also without hair. We begin administering G-CSF at home to help speed recovery of her white cells. This is to be given by daily subcutaneous injection through an insuflon (a small catheter in her arm) for 7 to 10 days following every chemo cycle.

Home at last!

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