The months leading up to the scheduled hospitalisation for Stephen's bone marrow transplant were happy times for Stephen and the family. We welcomed a new addition to our family, a baby girl, and Stephen soon forgotten about his lengthy stay in the hospital in the company of his two loving sisters. There was hope initially that Stephen's little sister would be able to donate her cord blood to Stephen, but it was not meant to be. A matching cord blood from a sibling donor would have significantly increased his chances for a successful and stable engraftment.
Meanwhile, the doctors also made another attempt to look for a compatible live donor of bone marrow cells for Stephen's transplant. Although we have already found a matching cord blood unit from an unrelated donor for Stephen's transplant, engraftments in marrow transplants usually occurs faster than engraftments in cord blood transplants. Hence the marrow cells from a matching live donor could potentially shorten the waiting period between the actual transplantation of the donor cells and engraftment. This waiting period is a crucial time when the body has zero immune cells to combat any infection, so a shorter waiting period would minimise the risk of infection.
With new donors being registered everyday (worldwide), there was also a chance that the search may find an exceptionally compatible HLA-type match for Stephen. All these measures was done in an effort to give Stephen the best possible chance for his transplant. Although Stephen was out of danger (ie. infection-free) and happily playing at home, our minds were constantly worrying about the risk of Stephen picking up yet another infection while waiting for his transplant to begin. He simply cannot afford another infection as it may put his transplant in jeopardy again.
A few days after the open lung biopsy, we were informed by the doctors that they have a positive diagnosis from the tissue samples that they had extracted from Stephen's lung and it was granuloma. Granuloma can be described as a ball-like formations or a collection of immune cells that are a result of the immune system's attempt to encase and isolate foreign substances that it cannot eliminate in the body. This news was a huge relief to us because they didn't find any active fungal infection in Stephen's lungs. Normally, the doctors would treat granuloma by prescribing immune suppressant drugs, but in Stephen's case, it was difficult to treat it because it was the result of his immune deficiency. Only a bone marrow transplant would permanently resolve his granuloma problem.
By end September 2012, we sat down for another meeting with the doctors and we were briefed that the risk involved in giving Stephen a bone marrow transplant will be higher due to the presence of granuloma in his lungs. The granuloma could potentially complicate the transplant procedure because nobody knows what would happen to it when Stephen's body is stripped of its immunity. Furthermore, there weren't that many medical records or journals of bone marrow transplants given to patients with granuloma in the lungs, hence there will be some uncertainty going into the procedure. However, we knew that the higher risk was still far better than not having a transplant at all. We were just happy that there was still hope for a cure, so we gave our full support for the transplant to proceed.
Stephen spent another couple of weeks in the hospital to recuperate from his biopsy procedure before being sent home to rest, gather strength and spend some quality time with his family before he begins his transplant which was scheduled to start in early January 2013. By the time we brought him home in mid October 2012, he had already spent over 70 days in the hospital.
The open lung biopsy procedure was a nail-biting 6-hour long wait. After obtaining the tissue samples that they needed, the doctors also went on to implant a long term catheter called a Hickman line into Stephen's chest to facilitate the introduction of fluids, blood and drugs intravenously into him without the need for needles. This catheter was also necessary for his much anticipated bone marrow transplant.
When we finally got to see Stephen in the intensive care unit, we were shocked to see so many tubes going into his body. In total, we counted 9 tubes going into Stephen. There were tubes in his nose, mouth, neck, hands, chest, back torso and urinary tract. The sight of Stephen lying there still unconscious from the anaesthesia, and the sounds coming from the ventilator and monitors were quite surreal for us. Words alone cannot express how we felt then, so here is a picture of Stephen taken right after his procedure:
There is not a single instance that we didn't wish for a chance to take our son's place in all that he has had to go through, and this was another one of those times. Though it pains us to see him in this state, we were just glad that he has pulled through the procedure. We were also hoping that the doctors will be able to find out what's infecting his lungs this time.
Despite making a difficult decision to proceed with the open lung biopsy, we still had to deal with the possibility that it may not yield any results for a diagnosis. However, the doctors also sat us down to explain that if they can confirm that it is indeed a fungal infection in Stephen's lungs, there will not be many choices of drugs that they have left to treat Stephen with as he is already on two of the strongest antifungal drugs available at their disposal.
The doctors then went on to explain that there is a possibility that they may not be able to resolve the fungal infection, let alone give Stephen a life-saving transplant. Fungus cells to an immune-deficient patient's body are not unlike cancer cells in a healthy person's body. They are difficult to eliminate and they can spread to any part of the body. Once a fungus infects the body of the host, it can easily spread to the vital organs like the heart or brain if it is not treated quickly with the right drugs. If this was the case with Stephen, it may be too late to stop the fungus in him and we may have to consider palliative care for him.
This sobering news was without a doubt the lowest point that we have experienced since we discovered Stephen's condition over 2 years ago. There was no time to lose, Stephen was wheeled into the operating theatre not long after that.
The change in intravenous drug did not manage to reverse the allergy that Stephen had developed and he had to endure a few more 'accidents' before we were able to conclusively say that he was allergic to milk. There were times when he would vomit midway through his stoma bag change, and this presented a very terrible situation for us because Stephen would be lying in a pool of his own barf and we had to prevent any contact between Stephen and his own stool at the same time, plus we had to take care not to soil all the tubes that connected him to his IV pumps. The allergy had also greatly limited Stephen's options of food prepared by the hospital, hence he quickly got tired of the food and started losing weight at an increasing rate.
Aside from his allergy problems, Stephen also started showing signs of breathing problems. His breathing rate was getting faster and the intervals between each breath was shorter. He was breathing at a rate of about 60 breaths per minute, and this was almost twice the average breathing rate for a normal child.
It soon became clear to us that Stephen's lungs were slowly losing their efficiency due to the mass that was growing in them. The cause of the mass in his lungs was still unknown and therefore it became quite urgent for us to find out what it was. We quickly decided that the risk of an open lung biopsy was much lower than the risk that the rapid breathing had presented, so we gave our full support to the doctors for an open lung biopsy.
After spending weeks staying in the hospital and receiving medical treatment that did not lead to any diagnosis or improvement of Stephen's condition, we were at a loss of what to do. But just as we thought that things couldn't get any worse, Stephen developed an allergy to milk during his current stay in the hospital. The allergy caused Stephen to regurgitate everything that he consumed (emptying out his gut in the process) and the sudden loss of fluids in his body would induce a fever in him shortly after. The problem with the fever was that the doctors were unable to tell whether the fever was due to the body's reaction to milk or a response to a potential infection.
After a few rounds of consuming food containing milk, followed closely by vomiting and fever, we became very discerning of what we gave Stephen to eat and drink. Even trace amounts of milk can lead to a big purge followed by hours of suffering for Stephen. It was also normal procedure for the doctors to call for a series of blood tests after each round of purging and fever to look for infection markers in his body. This resulted in multiple needle puncture wounds all around his arms and feet. I cannot begin to describe the discomfort and pain Stephen felt after each episode of consuming milk or milk products. We could not even give him a drink of water after each purge because it will all come back out again. We will never know the exact cause of this new allergy although there is a theory that one of his drugs had caused him to develop this allergy. Nonetheless, this hit Stephen really hard because milk was his favourite beverage.
Fortunately for us, we had an all-star team of doctors looking after Stephen and they knew exactly what to do. They changed his intravenous antifungal drug and began making plans for an open lung biopsy.
After a quick discussion with the doctors, we decided to go with the less risky method of extracting tissue samples from Stephen's lung, the needle biopsy. Fortunately, there were no complications throughout the procedure and Stephen had a rather short and uneventful recovery. In fact, he recovered so quickly that he was allowed to go home the very next day, less than 24 hours after his procedure.
About a week later however, we were given the bad news that the needle biopsy didn't yield any conclusive results. Now even though we did not have a clear diagnosis, there was still a high chance that the patches were the result of a fungal infection, so plans were put in place to cover Stephen with stronger antifungal drugs. By the end July 2012, Stephen was admitted into the hospital once again to start his treatment on a new intravenous antifungal drug. If all goes according to plan, the infection in Stephen's lungs would be cleared within six weeks and he can begin his bone marrow transplant straight after that.
Fast forward six weeks of continuous treatment in the hospital, Stephen was wheeled into the CT Suite for another scan to check the progress of his lungs. Unfortunately, the result of the scan wasn't even close to what we had expected. The scan showed that the patch had not only grown in size, but it had spread into both lungs. At that point, it would not be an understatement to say that we were totally at a loss of what to do.
The procedure to take tissue samples from Stephen's lungs was called a biopsy. It involves puncturing the lung with a needle or a scalpel to take tissue samples. This was a very tough decision for us to make because we were worried about the long term damage to Stephen's lungs.
Many parents would cringe at the thought of letting doctors operate on your child, but we had to make a quick decision as the biopsy was necessary to find out what was causing Stephen's lungs to be patchy. If it was an active infection in the lung, the type of infection had to be diagnosed quickly in order to prescribe the right drugs to treat it. The longer we waited the worst the infection could become and the doctors might not be able to treat the infection if it gets out of control.
As if the decision wasn't hard enough, there were two methods of biopsies to choose from, a needle or an open biopsy. A needle biopsy, as the name suggests, involves using a needle to core out tissue from the lung. This method was less risky and it will only create a small hole in Stephen's lung. An open lung biopsy would create an opening big enough for a surgical grabber to go in and grab out tissue samples from the lung. This method was more risky as it creates a bigger hole, but it also allows the doctors to see and pin-point the infection more accurately and take out sufficient tissue samples. This in turn would lead to a higher chance of a correct diagnosis.
By May 2012, we were mentally preparing ourselves for a long haul stay at the hospital with Stephen. We have kept our spirits high, persisted with his trice weekly injections at home, religiously followed his oral medication schedule and maintained his stoma as well as we could.
The doctor's visit was rather routine, we took another round of blood from Stephen (for testing) and did a chest X-ray prior to seeing the good doctor, who subsequently cleared Stephen's physical checks and blood tests. However, upon reviewing the X-ray, he mentioned that the X-ray was different this time. They knew that Stephen's lungs bore scars from his first lung infection in 2011, but his lungs looked a little more patchy when compared to his previous few chest scans. When this news hit us, our hearts just sank into our stomachs.
Fast forward about a month later and Stephen was admitted into the hospital again. This time, it was not for a bone marrow transplant (as we had hoped), but for a lung biopsy. Stephen's doctors needed to find out what was the cause of the patches in his lungs, and the best way to do so was to take some tissue samples from him.
My sincere apologies to all my blog readers for not posting in the last 18 odd months, things got a bit rough at home and hence time was rather scarce for me and my family. I shall continue my story from where I left off.
After Stephen's colostomy in March 2012, it took another couple of months before his doctors began discussing the timeline for his bone marrow transplant. This was done to give Stephen's perianal wounds and stoma (from the colostomy) sufficient time to heal properly so that they will not pose any risks to the transplant procedure. If all goes well, we should be able to start the transplant by around June 2012.
With lots of care and support, we managed to pull Stephen out of his depressive state and it wasn't long before he regained his appetite and normal physical activity at home. The downside of this was that the more food Stephen ate, the more motion he passed, and this required more frequent bag drains. The stoma bags that we were placing on Stephen also had shorter lifespans too because the adhesives were wearing out faster due of his increased activity (leading to more frequent bag changes). Add this to our daily list of chores and medicine feeds and it was a full day's work everyday for the both of us parents. May 2012 could not have arrived any slower for us, but the thought of giving Stephen his transplant soon kept us going.
The time finally came for the doctors to review Stephen once again whether he was well enough for a bone marrow transplant. Hence all our hopes for a speedy transplant were pinned on just a few routine blood tests and an X-ray.