Monday 7 February 2005. Xavier at last returned to the St Luc University Clinics for the transplant, which would be carried out on the 10th day after the chemotherapy that would destroy all his bone marrow cells so that the donor’s cells can replace them. We were expected at the day hospital at 9h30 where we were to meet the team who would be looking after Xavier.   Our departure for the hospital was like setting out on an expedition; as well as his pyjamas and wash bag we took with us his high chair, his little table and two small chairs, his toy box and his favourite toys. We had prepared everything the day before according to the hospital’s instructions.   Despite all our preparations, we arrived almost an hour late. We were ready at 8h30 and his godmother arrived to look after Alexander whilst we were away, but loading everything into the car took much longer than we anticipated because we had to use a certain amount of ingenuity to fit everything into the car. During the journey, his mum phoned to apologise for being late.   This rather chaotic morning didn’t seem to bother Xavier at all, despite the fact that he hadn’t eaten anything because of the extra tests (thorax X-Ray, blood test) and the anaesthetic that he had to have before the tube was fitted in his central tract. As soon as we got to his room in paediatric haematology, he delighted in touching everything while his grandma undressed him so that he wouldn’t sense his parents’ worry.   His explorations were short-lived and given how late we were we didn’t meet anyone and were soon taken to the operating theatre corridor were we stayed for nearly an hour waiting for Xavier’s turn to come.

	During this time, Dr Largent came to examine Xavier and to meet his parents.   At about midday, Xavier was at last taken for his anaesthetic, accompanied by his mum. The anaesthetic was administered through a drip to avoid using the mask that had frightened him last time he had an anaesthetic at St Luc’s. After spending a bit of time in the recovery room, he was transferred straight to the sterile unit (unit 56).   An hour later, his grandma returned to help his mum rearrange his room, which wasn’t really laid out for a baby. It’s a standard 20m² room with a sealed off area behind which there is a small washroom with a standard washbasin with a mirror and shelves as well as an individual toilet separated by a door. A baby cot had been put in the bedroom, which contained a bed, a table, a chair, a reclining chair, a huge set of scales, a medical trolley and a trolley for a drip. On shelves above the bed there is a television, a video and a CD/cassette/radio player. With the addition of the high chair and Xavier’s table and chairs, the area felt a bit cramped. In the sealed off area, which has two doors – one leading to the bedroom, the other to the corridor – hang the green overalls that the doctors and nurses will put on before entering the bedroom. Adjacent to the bedroom is a glass-screened visiting room enabling visits to talk to the occupants using an interphone. Access to this area is from a corridor behind the sterile unit.   We wanted to make Xavier’s play area bigger so we moved things around, leaving the centre of the room clear. We also asked for the medical trolley and the trolley for the drip to be put in the sealed off area.  As there is no nappy-changing area, Xavier will have to be changed on the bed, so his mum asked for the stereo to be removed in order to put nappies and changing things in its place. The telephone, blood pressure apparatus, stethoscope and anything else lying around was put out of Xavier’s reach.   Meanwhile, Professor Vermylen and his team came to see Xavier, who will be examined by Dr Carlier before the glucose drip is fitted. Professor Vermylen took the time to explain the programme Xavier would follow to his mum, after which she asked the team to leave us to settle in calmly.   His dad returned at the end of the afternoon to spend a few hours with Xavier, whilst his grandma took his mum to get something to eat because she’d be in there until the end of the morning the following day. Grandma wouldn’t be able to come early because she’d have to organise Alexander’s care with the nursery nurse who will look after him when his godmother and grandma are not able to.

	Xavier will be monitored by Professor Ch Vermylen and her team, which comprises three supervisors and an assistant:   • Dr V. Largent   • Dr B. Brichard   • Dr Ch Chandrain   • Dr Carlier   For the six sterile rooms, A-M Servaty’s nursing staff is as follows:   During the week :   • in the morning: two nurses,   one auxiliary nurse,   one “ALE” auxiliary nurse (with no patient-contact),   • in the afternoon: two nurses,   or one nurse and one auxiliary nurse,   • at night: one nurse.   At the weekend:   • during the day: one nurse,   one auxiliary nurse,   • at night: one nurse.

	Basic tests during his stay:   Taking the blood pressure and pulse: several times a day, Taking the temperature and weight: several times a day, Blood test: three times a week, Thorax X-Ray: once a week, Bacteriological samples: once a week, Urines and faeces samples: once a week.       Chemotherapy from the 8th to the 15th of February:   44 mg of Busulfan each day orally: the first 4 days, 440 mg of Cyclosporine (= Endoxan) each day by IV (1h): the next 4 days.   This treatment will destroy Xavier’s   bone marrow cells.     The day before the transplant, 16 February:   Administering of Cyclosporine (anti-rejection): 22 mg per day intravenously   This treatment diminishes the immune system in order to avoid rejection.     TRANSPLANT : THURSDAY 17 FEBRUARY 2005

	All of the medication that Xavier was taking is discontinued except:   Xantac morning and evening Motilium before each meal.       During the first phase of chemotherapy (Busulfan).   For digestive decontamination, the gilded staphilococcus and strétoscopes: - Bacitracin, - Polymiscine, - Sporanox.   To prevent convulsions: - Rivotril   As antiviral preventive: - Zovirax   For the stage with the fall of the white globules (infections): - Lendoglobine (plasma):  twice per week,   For stage with the fall of the immune system (antibody): - Cendoglobine (antibody):  once per week.     During the second chemotherapy (Endoxan)   It will be added by intravenous:   for vesical protection (the night before 2nd chimio): - Hyperhydratation - Mesna   to prevent vomiting (powerful anti-vomitories): - Novoban + Litican if necessary:  three times per day       The day before the transplantation, he will also take:   As an anti-rejection, each day by IV: - Cyclosporine,   To prevent mouth ulcers, fungal infections, etc.: - Corsodyl (mouth wash)

	Throughout the therapy, he will be receive fluids intravenously by:   Glucion (glucose/hydration).       From the day after the transplant onwards, he will have additional nutrition by IV:   Total Parental Nutrition   (Vitamins, trace elements, glucose and proteins)

	Xavier reacted very well to this first phase of chemotherapy although he had much less of an appetite, but on the other hand he drank much more than before. At the end of these four first days, he was in good shape overall and his weight was 11kg 550 grams, he didn’t have any nausea and didn’t vomit, his blood pressure remained between 7.5 and 8.5 and his temperature varied between 36,7° and 37,7°. He only had a runny nose (Neobacitracine).   Xavier remains very active, happy and smiling, for the moment our special outfits and even our masks aren’t a problem for him but he is starting to find that time is dragging by since he’s cooped up in his room. Sometimes he takes his mom by the hand and insistently points to the door to make her understand that he wants to go out, or when his grandmother comes back to see him in the afternoon, he holds out her cane and heads towards the door, not understanding her refusal because in the past when his grandmother arrived it always meant walks through the hallways of St. Luc. Each time one of us leaves his room, he cries his eyes out; his dad is the only one who can leave without causing this drama. Even if we know that we have no other choice, it breaks our heart each time it happens.   On the second day his occasional problems of balance and trembling (linked to his disease) reappeared and he doesn’t get on very well with his physical therapist, since contrary to what was planned, an intern physical therapist is taking care of Xavier.   On the morning of the third day, Xavier had to return to the operating room to replace his center lane due to an incident that occurred while he was taking his morning bath. After the operation he entered his room in tears, because his mom went with him to the operating room but had to be disinfected before going into the unit, whereas he was brought to his room by a door with direct access.   Our first contact with the staff was not very smooth because they had different opinions about our presence in Xavier’s room 24 hours a day, but now we are more tolerated. We obey the rules and don’t make too much fuss, even if they are restrictive. Nonetheless, several things are inconsistent.   On the first day, Xavier’s mom established some rules of conduct in order to preserve Xavier’s daily structure and to keep a certain harmony in his schedule:   -         Around 8 a.m., his grandmother comes to give him a bath and get him dressed, while the nurse and/or the nurse’s aid change the beds, takes care of his medication, and checks his vital signs and his scheduled tests.   -         Around 9:30 a.m., he has his breakfast of two sandwiches, jam, and a glass of mandarin juice, then plays with his grandmother until his mom comes back from getting something to eat and freshening up.    -         Around noon his mom gives him his lunch and he eats it half-heartedly, even though the dietician calls his room every day to make up the menu based upon his very restrictive diet and his tastes, but he doesn’t like the texture.   -         In the afternoon, he takes a nap with his mom who asked that during this time any treatment and visits by staff be avoided so that he won’t be disturbed. Professor Vermijlen and her team share this point of view and try to respect this as much as possible and come to examine Xavier before or after his nap.   -         The rest of the day, apart from his treatment, is filled with games and giggles: Xavier surprises all of us with his love of life and his courage.   -         Around 6:30 p.m. his dad takes over, Xavier plays for about another hour or two before dinner and then his dad puts him to bed before his mom comes back around 10:30 p.m.   -         In the evening, while Xavier is sleeping, his mom uses the time to disinfect his toys, his high chair, his table, etc.   As for Alexander, his mom’s absence doesn’t seem to affect him too much because she takes care of him every evening. When she arrives he is high-spirited, his mom cuddles him and then gives him his last bottle before putting him to bed. Tuesday he met Nathalie, the child minder from the Dragonflies service and everything went well but he still hasn’t gotten better even though he’s been taking antibiotics. Friday he had a fever, and his godmother took him to St. Michel at the end of the afternoon. Dr. Soumoy is on holiday so the paediatrician on duty examined him and said that he should have a new x-ray and blood test, which indicate that he has rhino bronchitis and a seromucous ear infection. He has to stop taking antibiotics and is prescribed respiratory physical therapy. Although he is sick, Alexander continues to gain weight and develop; he is now able to turn over on his stomach, he babbles constantly and remains very cheerful.   Here are some pictures of Xavier in his room “Click on the picture to enlarge.”

	Xavier also did well in this phase but his poor balance increased and he sometimes had bouts of trembling, especially in his hands, which are the inherent problems associated with Krabbe’s disease and which appeared on the first day of hospitalization. He surprised all of us by remaining fidgety and in a good mood throughout this period; he continued to have his breakfast, no longer ate lunch and picked at his dinner because he still didn’t like the texture. His weight, blood pressure and temperature remained stable.   When his little legs can no longer carry him, Xavier holds out his arms to be picked up so that he can continue whatever he’s doing. His day always starts with a bath that is a special moment because he always loves playing in the water. In general, Xavier is very active in the morning until noon – 1:00 p.m. and in the evening from 5:00-10:00 p.m. between these two periods he takes a nap except for very rare disturbances like the first day when he had his respiratory physical therapy because he was a little congested.   This first week at St. Luc was rather chaotic for us; it’s coming to an end and there is still nothing arranged for his mobility physical therapy even though it’s essential in this disease. We respect our schedule as best as we can in order to keep the structure that we have set up to preserve Xavier, Saturday afternoon his dad comes to take over for his mom but once again the transition isn’t made without tears. It pains her to leave him and she knows that his crying is short-lived because Xavier is with his dad but she stays behind the door and can’t bring herself to leave until he’s calmed down.   Before going home his mom and grandmother buy several things for the hospital, especially pyjamas for Xavier because it’s very difficult to find ones with both tops and bottoms that button up the front and are in his size. Alexander, who spent the afternoon with his grandmother, is doing much better and receives a lot of cuddles from his mom who smothers him with kisses. Although she knows that she’s doing the right thing, she can’t help but feel very guilty that she spends so little time with him and that her thoughts are always with Xavier. These 24 hours go by at full speed; there are so many things to attend to before going back to St. Luc that his mom doesn’t even have the time to relax.   A new week begins, it’s already the third day of chemotherapy and Xavier increasingly has bouts of tiredness but these alternate with periods of intense activity and even if he wants to be carried around, he continues to laugh and play. For the first time he is nauseous and vomits. Xavier is still congested and has to continue the respiratory physical therapy.   The fourth and last day goes well; he is no longer nauseous but is very tired. At the end of the morning, his mom meets Gaby, the team psychologist who is very charming and makes a very good impression, even on Xavier. She is sorry for the lack of organization during the first week due to the holiday week (Carnival).   When he wakes up around 4:30 p.m. he needs an hour to feel fine, but the intern comes for his respiratory and mobility physical therapy although it was agreed that she could come in the morning so that the session would be productive. Xavier is not at all receptive, he can’t stand well on his legs and the session doesn’t last very long.   It’s his godmother and godfather’s turn to come and visit him and Xavier thinks it's very funny to speak to them via the intercom that reminds him of a telephone, so he absolutely has to grab it…   The day before the transplant, there is no chemotherapy, and Xavier is introduced to Muriel, the teacher at the Escale School of St. Luc, who will come 30 minutes to one hour per day to take care of early-learning games. Around 11:00 a.m. L… comes for his physical therapy but unfortunately the session doesn’t go well because Xavier is not at all receptive. His mom takes thus decides to make a schedule so that his early-learning games and his physical therapy can take place without encroaching on each other.   Here are some pictures of Xavier surrounded by his parents and of his grandmother giving him a bath: “Click on the picture to enlarge.”     2005 07.feb 09.feb 11.feb 14.feb 16.feb HG * 11,1 10,7 10,6 10,06 9,1 WC * 12.970 8.610 8.720 3.500 1.800 PL * 261.000 263.000 247.000 251.000 205.000   Legend* HG  Hemoglobin The patient is considered anaemic if it is below 12.   WC White Cells Normal value is between 4.000 and 10.000   PL Platelets Normal value is between 150.000 and 400.000

	For this capital day, nothing is changed in Xavier’s habit, he is in a very good although quite weak. He continues to laugh and play even if its small legs do not carry him any more.  At the end of the morning, his grandmother leaves the room before the transplant, during which Xavier will remain calmly in the arms of his mom…     THURSDAY 17 FEBRUARY FROM 12:00 – 12:30 P.M. UMBILICAL CORD BLOOD TRANSPLANTATION