The CGD Café

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For anyone thinking about a BMT for their CGD child.

Let me start at the beginning.  My name is Tom.  In July of 1996, my son, Nick, was born.  It was a great day in my life, even though I was a young father.  From the day we brought Nick home from the hospital, he was running slight fevers.  At about 2 weeks old, we had to take him to the emergency room because his fevers were pretty high.  We were admitted to the hospital that evening with pneumonia.  We were in the hospital for a week, and then were sent home.  We stayed home for a couple of days, then had to go back to the emergency room for the same problem.  This time we stayed for 2 weeks.  IV antibiotics, breathing treatments, the whole nine yards.  After our discharge, the doctors knew something was not right with Nick.  It took a few months of different tests to finally figure out that Nick, who was 4 months old at this time, had Chronic Granulomatous Disease.  He was started on Actimmune injections 3 times a week, Bactrim, and Sporanox. 

Months went by without any additional problems.  Nick was rather healthy since, at that time, we were told only about 2% of his cells worked correctly.  We were young parents and tried to make sure Nick stayed healthy by keeping him away from a lot of the things that can make you sick.  After a few years, we sent Nick to a daycare, pre-school, and then to public school in our small town.  Nick would have a few infections here and there, but normally only a few days in the hospital, or oral antibiotics would take care of it.

When Nick was about 3 years old, we had a pool in the yard.  We were very careful to make sure the chlorine level was always good, and made sure the pool stayed clean.  At that time, Nick would run around with no shoes or socks.  He loved to be in his bare feet all the time.  Nick got a small cut between his toes that we did not find at first, until it was infected.  We don’t know if he cut his foot in the pool, or in the yard, or in the house.  Nick started running fevers and to the hospital we went.  Nick was on IV antibiotics in the hospital for a week, and then he got his first PICC line and we were sent home.  Who would have thought it would take three months of at home IV therapy to get rid of the infection from such a small cut.

When Nick was about six years old, we went to Disney World in Florida.  He got to see the Ocean for the first time, and just had a blast.  At this time, Nick was sick from something, but did not know what at the time.  When we returned home, we went to his Immunologist because he was running over 105 fevers.  We were admitted to the hospital for fever of unknown origin.  This is not new for a lot of CGD kids, as I am sure you are aware.  After approximately 17 days in the hospital, Nick finally had a BM full of blood.  Come to find out, Nick had Colitis.  It was pretty bad too.  Nick was put on high dose Steroids and after a day, we were sent home.  The fevers were gone, and Nick was eating, which we were not used to.  Every time the doctors would lower the steroids, he would start to run slight fevers again.  Nick was on high dose Steroids for approximately one year.  I will tell you, we thought someone ate our son.

Over the next few years, Nick had a few infections and problems, a few short hospital stays, but nothing very serious.  He had perianal abscesses, sinus infections (lots of sinus problems), a few short stays for fever of unknown origins, some skin problems due to photosensitivity caused by Voriconazole which included pre-cancerous cells on his face and some other minor things.  We always considered ourselves lucky with Nick because we always tried to give him a normal life, let him do things with his friends, and just be a kid.  He was great at this because he knew exactly what to stay away from, and what he could and could not do.

In November of 2003, we had our daughter, Olivia.  We were so scared of having another child, but were excited she was a girl.  We saved her cord blood just in case she was a match for Nick.  After we saved the umbilical cord, we found out Nick and Olivia were a perfect match.  We also found out Olivia was a carrier of CGD.  We thought it was great news they were a match, but decided we would just store the cord blood until later on, just in case we needed it.  Like I said, Nick really did live a normal life, and was not very sick very often.

In August of 2008, we went to see Nicks Immunologist.  Once again he suggested Nick have a BMT.  This time, there was more sincerity in his voice.  Almost like he had lost another patient.  We agreed to talk to the Hematology/Oncology doctors and get more information.  We met with the transplant coordinator, and then the Hematologist.  We included Nick in all the meetings, let him ask questions and told him of possible complications.  After a few weeks of talking it over, we all decided, Nick included (he was twelve at the time) that this was probably the best thing to do so Nick would not have to take his medicine anymore, and could finally get to do all the things his friends could.  We decided to wait till summer, and then Nick would only miss his 8^th grade year in school, then be able to return for his freshman year in high school.

A month before Nick was supposed to have his transplant, Nick was taken off his Actimmune injections.  He was so happy since he was scared to death of needles.  We scheduled the transplant process to begin on June 15, first day of Chemo.  On June 13, Nick woke up with a fever of 102.6.  We called to Hematologist and we were admitted to the hospital for tests.  At this point, the transplant was postponed.  On June 19, we decided to have a meeting with the hematologist and Immunologist to find out if we should just wait a while.  We all decided that Nick was doing better, and would go on with the transplant.  On June 23, Nick started his first round of chemo.  Nick started Dylantin on June 22, then Busulfen on June 23.  On June 27, he started Cytoxen and on June 29, he started AtGam.  Day 0 ended up being July 2. 

Nick was very sick with the chemo, and developed very bad mouth sores.  The pain management of morphine, oxycodone and a few others were doing a pretty good job though.  Nick started running fevers over 105 on July 10.  On July 12, Nick got a bad rash, was having joint pain, his hands and feet hurt and was still running fevers.  These were signs of engraftment.  Nick was started on high dose steroids and within 24 hours, the fevers were gone.  On July 29, day +27, Nick was discharged and sent home.  This was fantastic.  We had to go to clinic 3 times a week after that, but we were all home again.  Nick had not seen Olivia in a month, and was so happy to be able to hug her, and talk with her like he used to before all this crazy stuff.

After a few more weeks, Nick’s platelet count would not go up.  For some reason, his platelets were not coming back very fast.  After a few more tests, we found out Nick had the BK virus and CMV.  Lots of people have these viruses, which is a form of the Herpes virus that causes chicken pox and fever blisters.  They started him on more IV medications, and upped his IV fluids. 

The end of September Nick was having more problems with these viruses.  The docs just could not get them under control.  Nick started peeing blood, and was clotting rather badly.  It got to the point that is completely obstructed his urethra and he could not pee.  We rushed into clinic and he was catheterized.  They flushed his bladder and we talked them into removing the catheter.  That evening, Nick started having more problems.  Come to find out, the entire lining of his bladder had “sloughed” off and had formed a large clot.  We were admitted to the hospital and Nick had a Suprapubic Catheter surgically placed in his bladder.  The doctors were able to put GMCSF and the antivirals directly into his bladder to get rid of these darn viruses.  After 17 days in the hospital, they were able to remove the suprapubic and send up home. 

Nicks urine was still bloody after we went home, but was not having any clotting.  We were home for a whole week, then he started clotting again.  I rushed him to the ER 45 minutes away and they were able to break up the clot and place a regular catheter.  This was around 11:30 on a Thursday night, so we were able to talk to urology first thing Friday.  They decided to wait a few days before placing another suprapubic because the surgeons were all out of town on a conference. During these few days, they were constantly changing the catheter and flushing his bladder manually, which was very painful. Finally on the following Tuesday, Nick received another suprapubic.  When he came out of surgery, he had the regular catheter and the suprapubic.  We thought this would only be for a day or two, but the suprapubic would not drain correctly.  After about 5 trips to the O.R., they were finally able to get the suprapubic to drain properly and were finally able to remove the catheter.  During this time, with all the manual irrigations, something must not have been sterile because Nick ended up with a fungal infection in his bladder.  This was approximately two and a half weeks with this catheter.  During this time, Nick would not move, it hurt too much with the catheter moving in his penis.  Nick ended up with fluid around his lungs and was having problems with his O2 stats.

We ended up in the PICU for a week.  During this week, we watched Nick get a little better every day.  He did not need as much oxygen, and seemed to be getting in better spirits.  After a week in the PICU, we were sent back to the regular floor.  He seemed to be doing so much better, the docs determined this must be just inflammation in his lungs from not moving around very much and we determined, since he did not have the regular catheter anymore, he would have physical therapy to help him move around.  We were on the floor for one week, he had been taken off all his antibiotics and they left just the antifungals going.  We had about 2 really good days where we got to see the real Nick again.  The funny Nick we had not seen in a few weeks. 

Late evening on November 12, Nick started having a lot of problems breathing again.  The PICU doctors came up and had an X-ray done.  Nicks lungs did not look good at all.  We went back to the PICU early in the morning of November 13.  After about an hour of trying to stabilize his breathing, the doctors determined the best bet would be to put him on a ventilator.  Nick did not know what was going on, he had not slept at all, was hallucinating, and was using all his energy just to breathe.  I told Nick they were going to sedate him, and when he woke up, he would be able to breathe easier again. 

Early morning on November 14, Nicks blood pressure and oxygen saturation levels plummeted.  We were told that Nick was going to die.  We decided to have the family come up to see Nick one last time and say their goodbyes.  After the family said goodbye, my wife and I sat with Nick as the doctors stopped all the blood pressure meds that they said was keeping him alive.  After sitting with Nick, lying with him in his bed, crying, and saying goodbye, I looked at the time.  It had been about 4 hours since they stopped the meds.  I asked the nurse if this was expected, and she said no and had the doctor come in and talk to us.  Nick was septic, his lungs were shot, there was no reason for him to still be there.  We took it as a miracle.  Nick was not ready to go.  He was always a fighter.  Sunday morning, the doctors told us that Nick had humbled them.  They took him off all his anti-rejection meds to give him a fighting chance at whatever the infection was, started a lot of antibiotics and antifungals, and antivirals and told us he still had a very slight chance of recovery.  We had already decided that Nick had been on this earth for 13 years and we knew what he would want.  We went into this transplant for a better life.  At this time, Nick was going into liver and kidney failure, was very swollen from water retention since his kidneys were not working correctly, and had an infection that they could not culture or find.  We still thought he had a fighting chance.  At this time, Nick was on approximately 40 medications, down from before.

 The next few days were very hard.  It seemed Nick was in there fighting, and we thought he was getting better.  The doctors all still said it was just a matter of time.  We talked with the doctors and they stated he would give us a sign if he could not do it anymore, and that sign would tell us to stop the vent.  On November 18^th, we received our sign.  Nicks O2 saturation went down again, and the ventilator was not able to help anymore.  We had to make the hardest decision in our lives, and let our 13 year old son die.  We had the nursed remove all of his IVs and once that was done, they removed the vent.  Having to sit there and watch your child take his last breath is the last thing a parent should ever do.  I know I will never get that out of my mind, and I will see that for the rest of my life.  Nick passed away at 9:20 pm on November 18.

The reason for writing this is not to discourage anyone who is contemplating a BMT, but to let you know that there could be consequences.  We always thought Nick was invincible, but now we know he was not.  Please make sure you know what you are getting into, and know the complications, heartache and other problems you may go through before you make this decision.  This decision will stay with you for the rest of your life.  Like I had said before, Nick led a pretty normal life, minimal complication from CGD, and was the happiest, strongest, and smartest child I have ever met.  If we would not have done the BMT, who knows if it would have still ended on November 18^th at 9:20 pm, or if he would have lived a long, normal life.

This is just a small portion of what Nick went through during his BMT.  If you would like to read more, please visit our Caringbridge site at http://www.caringbridge.org/visit/nickb.  You can also email me if you have any questions at tbeauman@msn.com.