Extreme Home Makeover

Last night I was watching one of my most favorite shows, Extreme Home Makeover. http://abc.go.com/primetime/extrememakeover/

It is one of the only reality shows based on showing the best of people and the best of a community. Recipients of a new house are chosen based on unusual and dire circumstances and I always have that feeling that “there but the grace of God go I”. In addition to some deserving family, there are 100’s of volunteers that participate and the show really does a great job in communicating how the volunteers actually feel good about working so hard for someone else.

The family last night had 4 children with something terribly wrong with them. Their white blood cells attacked any food they ate and caused them to throw up food. The children are fed daily, with feeding tubes directly into their stomachs. In addition, the children had other severe allergies. One of the little girls has had her kidneys shut down repeatedly. Mom and Dad were young, still in their twenties and had been told initially that the disease the children had was NOT hereditary. Dad works full time and is in medical school carrying a full load because he wants to do research to cure his children.

So, in addition to building them a house, the show sent the family to Philadelphia Children’s Hospital where there were doctors who research the condition that the children have. The doctors, after examining the children and spending good time with them, had some new and inventive treatment plans for the family. The big gift was that the children would be able to tolerate some specific foods.

The reason that this struck me so is that we experienced something equally dramatic and life changing this spring when the National Institute for Health in Bethesda, Maryland http://www.nih.gov/ brought Axxx and her parents out for a week of examination and doctors appointments for their Proteus study. . http://www.proteus-syndrome.org/ Axxx’s little brother and I tagged along for the ride.


Axxx in her life has seen 100’s of doctors. Besides the usual specialists that we keep rotating through, hospitals arrange clinics to gather “great minds” together to brain storm on difficult cases. Emanuel Hospital did a clinic for Axxx when she was less than a year old. They presented her case to the 75-80 physicians attending. We brought her and showed her to the doctors, then left for them to talk about her. Once an OHSU department head asked us to come to a clinic and we were eager to do it. It turned out that Axxx was “disease of the week” where we assigned a very small room. Over the course of 2-3 hours more than 100 doctors filtered thru and asked questions. Shriners Hospital invited Axxx to a clinic once or twice along with others. We have no objection to clinics. It is just that we end up talking to arrogant doctors who boldly tell us they are going to do some surgery or another, when they have no idea what the physiology of her tumors is. Or they think she is a lab rat and want to experiment and not see her as a little girl with hopes and dreams and hopefully a future that is rich and wonderful.

So given all the doctors Axxx has seen and how little we have every learned from them we were so eager to speak to doctors who would really know something about her condition, doctors who had experience with other Proteus patients. During the week, Axxx saw every kind of “ologist” that you could think of. This is a government facility and I have to tell you… they knew how to do medicine. Each and every doctor was equally at ease talking to Axxx as they were talking to her parents. Whatever phsyiology they were examining, they knew their stuff. In Portland, when Axxx went to a new doctor, they had to go look in a book. Going to the NIH was such an incredible gift and a relief to talk to someone and have some level of confidence in whatever they were saying.

We learned so much about Axxx’s condition…her prognosis, danger health signals, ways to make her more comfortable now, pain management, practical things like shoe lifts, …. We came home with some reassurance that her condition is not necessarily terminal. Nobody knows what that gift was like unless they have wrestled with the reality of their child not living to adulthood. We came home with the weight of the world lifted. Axxx has many trials ahead to face. We will face them as a family together. And her condition is not Proteus… but it is Proteus-like. All her symptoms are Proteus symptoms. But she is one of 8 who are exactly alike (and Proteus patients named after the Greek god who changed shape at will, are all different). There is no name for her condition. But we have the hope of meeting other people who have exactly what she has.

So on Sunday night when I saw the family on Extreme Home Makeover receive the gift of information and help for their children’s rare medical problem, I know that it was a huge gift.. better than a house, better than a vacation. Because this year we received the same gift… information and help for such a rare medical condition that there are only 8 cases known. We are blessed. DKU