In May of 1998, Kieran began life as a happy, healthy, 10 pound 7 ounce baby boy. He stayed large for the first 4-5 months of life then gradually moved to an average size where he has remained.
Vanessa went back to work in late July. A few weeks after that, Kieran began to eat a combination of formula and breast milk. (Until that time, he had been breast fed only.) By late September or early October, Kieran's skin had developed a bright red rash, known as eczema.
We took Kieran to the doctor in October. She gave us what were to be the first of many lotions and ointments and told us to give them a try. When his skin did not respond to them, she gave us a few more and referred him to a specialist. By this time we had found out that many babies suffer from eczema and that the vast majority grow out of it after a few years. We had also removed all milk products from his diet, which had helped his skin, but not cleared it up.
To make a long story short, we were very careful to be sure that Kieran ate no milk, eggs, or wheat. With the help of a dietician, we learned that there were a lot of things that Kieran could eat and which he enjoyed. At that point, around 9-10 months old, Rice Crispies became one of Kieran's favourite foods. This strict diet, in combination with new dry wrap skin treatments that we'd learned from dermatology nurses, helped his skin a bit, but never cleared it up.
A few weeks after Kieran's first birthday, Frank took Kieran to the allergy clinic for his regular eczema check-up. The dermatologist who looked at Kieran noticed that in addition to eczema, Kieran had small purplish/red spots on his skin. (To us bad skin was bad skin.) He ordered a blood test to be taken.
The results came back quickly. Kieran's platelet count was 18,000 (18) instead of the usual 250,000 (250)... very low. In addition, the platelets themselves were small in size. More blood tests followed as the doctors tried to pin down the cause of this abnormality. Meanwhile, Kieran spent a week in one hospital and 10 days in another as they tried to get (and keep) his eczema under control. His skin condition only seemed to improve when he was on antibiotics, and would rapidly deteriorate whenever a course was completed. After a few weeks, we finally got a firm diagnosis. Kieran has Wiskott-Aldrich Syndrome (WAS).
With this new understanding of the cause of Kieran's eczema, his treatment changed a bit. In addition to the daily bathing, medicating, and bandaging of his skin, Kieran was given a repeat prescription of antibiotics to take every day. He also continued to take an oral antihistamine to help combat the itching.
At this point, a hematologist at the Sick Children's hospital here in Edinburgh became Kieran's coordinating doctor. Knowing that boys with WAS typically have trouble with their immune systems, she had Kieran's blood tested to find out how well he had reacted to the vaccines he'd had so far. Unfortunately, his body had produced almost no antibodies as a result of his vaccines. Consequently, she made arrangements for Kieran to be given regular infusions of immunoglobulin, or antibodies, gathered from the blood of blood donors. She hoped that by receiving other people's immunities, Kieran would be better able to fight off infections.
Kieran received his first infusion in mid July, 1999. The results were remarkable. Firstly, his color, which had always been too pale, became a bit pinker and healthy looking. Additionally, we were able to take him off of the daily oral antibiotic and antihistamine. (He still got an antibiotic two days a week and an antihistimine occasionally, as needed.)
We were able to reduce the strength of Kieran's steriod ointments. Also, since his skin was no longer infected, we were able to switch bandaging methods at that time. We began treating him with wet wraps which made his skin soft and smooth. Other than wearing wraps every day, Kieran looked like any other normal little boy.
In January, 2000, Kieran's skin began to flare up again. It didn't seem to matter how strong a steriod ointment we used, his skin continued to worsen. Finally, we decided to use the dry bandage technique we'd been taught the year before. Overnight, Kieran's skin improved. Literally overnight. We were shocked at the improvement we saw the next day.
After a bit of experimentation, we found that the best treatment for Kieran's skin was for him to be treated with dry wraps most of the time and wet wraps occasionally. Once again we were able to use a minimal amount of steroid ointment and his skin was usually very good.
Also in January, 2000, we met with specialist in childhood immunology. He questioned us in detail about Kieran's medical history then presented us with information he had on WAS. He also stated that Kieran seemed to have a fairly severe case of WAS and recommended that if we wished Kieran to have a bone marrow transplant, the sooner we had it done the better it would be for Kieran. We assured him that should a suitable bone marrow donor be found, we would like Kieran to have a transplant.
Subsequent to that meeting, we stayed in contact with the specialist and his team in Newcastle upon Tyne as they made preparations for a bone marrow transplant. We were able to go down to see the hospital in February (where, naturally, Kieran was required to give lots of blood for tests and have a chest x-ray taken.) In March, at the request of the Newcastle doctors, Kieran gave more blood and a urine sample at the Sick Children's hospital here in Edinburgh. As one result of the tests, the doctors have confirmed that Kieran seems to have gotten a 50/50 mixing of his tissue types from both Frank and Vanessa, verifying our suspicions that neither of us would be a suitable donor for Kieran.
In early July we were notified that Newcastle had located a suitable donor. Kieran was admitted to the hospital in Newcastle in late August for placement of a central line into his chest. Shortly thereafter his chemotherapy began. The purpose of the chemotherapy was to kill off as much of Kieran's own bone marrow and immune system as possible. On September 12, Kieran received the donor's bone marrow and the slow healing process began. Kieran progressed steadily and was released into a "half way" house in Newcastle in late October. Three weeks later, he was declared well enough to return home. (See transplant diary for details.)
As we moved into the half way house, Kieran's eczema flared up again (it had subsided during the time that Kieran had no immune system.) However, we were pleased to discover that it could be controlled with much weaker hydrocortisone ointments/creams than we'd been using pre-transplant (only 0.5% or 1%). We continued treating his skin as necessary for the next few months. By spring, the eczema was gone and it hasn't returned. In October, we also began the process of slowly introducing Kieran to the foods that he'd been sensitive to pre-transplant. We were pleased to discover that they didn't seem to cause him trouble anymore.
Medical treatment after transplant included immunoglobulin infusions every three weeks as well as a few preventative oral medications. In addition, since Kieran still had his central line in place, a nurse came by twice a week to flush his lines. The line was removed in January when it developed a leak.
As the new year progressed we watched Kieran's platelet count slowly fall. In March his count finally fell below ten. At that point, Kieran's doctors implemented a new treatment. Kieran was given a course of steroids and he began getting larger immunoglobulin infusions more frequently. Kieran's platelet counts improved, but then started to fall again once he had completed the steroids.
In April, Kieran developed a urinary tract infection and his neutrophil count plunged. The hospital in Edinburgh started Kieran on some antibiotics, resumed the steroids, then transferred him to the hospital in Newcastle. Not knowing which problem developed first, the doctors decided to get a bone marrow sample to find out how Kieran's bone marrow was faring. The sampling showed that there were no problems: both Kieran's own marrow and the donor's marrow peacefully co-existed within his body. As a confirmation of good health, once the urinary tract infection cleared up, Kieran's neutrophil count returned to normal and he returned home.
Unfortunately, once the steroids stopped, Kieran's platelet count started falling again. In May, Kieran started having persistant diarrhea. Late in the month, Kieran also began to be lethargic, unable to keep up his active routine. Blood tests and stool samples showed nothing out of the ordinary, however. Finally, in early June, Kieran developed a fever and was once again admitted to hospital in Newcastle.
We spent a long three weeks in the hospital that month. The doctors tried several different antibiotics before finding some that seemed to help. Meanwhile, Kieran's temperature chart looked like a roller coaster: low while the pain killer was working, high as it wore off. He was given several platelet and whole blood infusions to keep his body working properly. During this time, Kieran ate little and was thoroughly miserable. Finally, Kieran had some biopsies taken so that the doctors could find out what was wrong. At the same time, surgeons put another central line into Kieran's chest to aid in future treatment.
The doctors weren't able to pin down exactly what it was that had caused all this trouble. However, they did determine a few things that weren't causing the problem and from that they were able to prescribe a treatment. Soon Kieran was discharged, still on antibiotics and once again on steriods for his platelets. Ten days after we returned home, he seemed to be back to full strength.
In the past year, Kieran has done exceedingly well. His skin is perfect. He eats a normal diet. He has only had one minor fever and he's full of energy. He is still on a number of medications, but he has had no further problems. His central line was replaced with a porta-cath this month when the former developed a leak. The porta-cath is completely under Kieran's skin so he should have less chance of infection and the chance to go swimming.
Kieran's doctors are pleased with Kieran's health with the sole exception of his platelet count which is still low. He is still receiving treatment for that issue via continued steroids and frequent high-dose immunoglobulin infusions. However, we have no complaints. From what the doctors have told us, it should be simply a matter of time before even that factor is under control.
