Then There Were Two

I thought I would write a post about the medical options for treatment for Anna's condition. The main reason I thought to write this post was based upon people's common reactions. When they see or look at Anna and ask about the treatment for her condition, I think they shudder inside when we tell them that typically amputation is the best treatment option. They are probably thinking, "Amputation is a treatment option?" Well, actually it is a treatment option. Common responses are "Well surely they can do something these days, with all the medical advances out there" or "Surely they can do something so you don't have to amputate her leg", or "I'm sure they will be able to fix everything with all the medical technology out there"... you get the idea.

These comments can become tiresome. The reality is that we know the options. It's our daughter. What parent would take the decision to amputate lightly? So comments like "surely they can do something" are frustrating. I think perhaps people respond this way to comfort themselves rather than the person addressed. They are taken aback and want to say something reassuring. I can understand this and so I don't blame them. Since their comments aren't exactly reassuring to us, I hope they are at least reassuring to them! All that said, I'm sure I would have reacted the same way four months ago.

I would like to provide a quick run-down on treatment to hopefully dispell any myths. I should start by saying that this is worded through our opinions, research, and discussions with others. I think our research has been thorough and unbiased but some may disagree, and that's ok. Neither treatment option is ideal; we will explain where we are in the decision-making process at the end.


Updated 9/15: A letter from a reader with FH about her parents' decision and how it has affected her.  



Choice #1- Amputation of the affected leg.

Child has the surgery done when they are 12 months old, before they are walking and before they will have a memory of the surgery. They have a prosthesis fitted two months after they are healed. Most children start walking immediately.

Pros:

• Complete mobility and function of the leg comparable to that of a normal leg.
• Ability to walk, run, swim, skate, etc.
• Only one surgery and the child can go about his or her normal life (a minority of children with complicated cases may need more surgery down the road).
• Minimal complications and risks.
• Child looks 'normal' when wearing pants and you often can't even tell he has a prosthesis.
• Children are resilient and can adjust easily with early intervention. The child grows up with a prosthesis and knows nothing else. He does not feel sorry for himself.

Cons:

• Child does not have two real legs and may feel self-conscious.
• Child has to switch legs for swimming, heavy running, etc.
• Having to deal with stares, questions, etc. that accompany being different.
• Having to adjust to a new prosthesis each year.
• The fittings and costs associated with prostheses.

Checkout this video of a boy with F.H. getting his first leg and walking for the first time. (I couldn't embed this video.)

And this second video below is of a little boy on his special "blades" that he has for around the house when he's tired of his walking legs.

Choice #2- Limb Lengthening and foot reconstruction surgeries.

Child is put through a series of intense surgeries over the course of ~15 years to lengthen the leg bone and muscle and reconstruct the ankle and foot. This type of treatment for FH cases like Anna's (mild to severe) is somewhat controversial in the medical field; some doctors will perform it and others will not recommend it.

Pros:

• Leg may be salvaged. 
• Child could end up with normal function of the leg once they are an adult.
• Parents don't have to make the difficult decision for their own child to amputate before the child is old enough to participate in the decision-making process.

Cons:

• A child like Anna is put through a minimum of 3-4 lengthening surgical procedures. Most children need more surgeries to address later complications (8 surgeries is the average according to the current medical research).
• Each surgery requires living with a device called an external fixator (though there have been some new developments regarding the fixator being internal vs. external) that is attached to the bone in the leg for approximately 8 months. Screws are turned that stretch the bone and muscle every night to lengthen the leg (painful). Child is x-rayed bi-weekly (travelling to West Palm Beach or Baltimore if you want to go to one of the few doctors in the country with significant, skilled experience) to see how the bone is growing. Child undergoes up to 2 hours a day of intense physical therapy during this long rehabilitation process. This happens multiple times throughout the child's life. Mobility is impaired through these periods due to the surgeries, fixators, and rehabilitation. Many children are confined to a wheelchair and/or use a walker during these periods.

• The child can have her first surgery and external fixation process as early as 18 months - a very young age to go through such stress and pain over the course of months.

High rate of complications. Infections, fractures, nerve damange, and other unexpected complications. Sometimes lengthenings have to be stopped to avoid permanent damage. Long term side effects are concerning to us and are not reversible.

Results are not guaranteed since there isn't a way to predict how the bone will respond to the lengthening. The shape of the resulting leg does not match the other leg (because of the stretching) and ends up having what some would call 'disfiguring' scars.

• Because results are not guaranteed and most patients given the latest procedures have not been followed for more than 25 years (since it's a newer treatment), chronic pain down the road is a big question mark. This chronic neuropathic pain is not alleviated well by medication. There have been cases of people reporting so much pain in their 30s that they ended up amputating their leg anyway. There have been teens who have decided to amputate instead of continue with treatment. I know of several cases personally. Adjustment to amputation as a teen or adult is more difficult than as a child.

• The life of the family in many ways centers on the child and their treatment. Other siblings may not receive as much attention from mom, who is assisting her affected child throughout the rehabilitation process. Parents may be split up for long periods of time during surgeries and rehabilitation. Some people choose to relocate their families closer to the hospital/clinic when undergoing treatment

• It is not uncommon for children to be taken out of school for months due to the reality of their care.

• The child has to wear special braces or shoes with lifts in between surgeries as they grow to make up for the increasing leg difference. This makes it obvious to others that the child has a medical condition.

• Financial cost is significant to say the least. If you can't afford to have the surgeries done by the few skilled, EXPERIENCED surgeons out there, your child is more likely to experience the above negatives.

• This recent study shows just a few of the after effects following successful lengthening- including a remaining leg length disrepancy, a majority of patients with limps, valgus ankle deformity, calph atrophy, and residual bowing of the tibia.

Overall, the majority of the medical field recommend amputation as the best treatment option for cases like Anna with a 27% leg length discrepancy that would require aggressive lengthening (3-4 lengthenings). Many people still choose to pursue the journey of limb lengthening in order to try and save their child's leg. Every family is different. After much thought and prayer, we feel the best medical option for Anna would be a prosthesis. We wouldn't feel comfortable putting her through 15 years of surgeries and limiting her mobility through much of her childhood for a less than optimal result- one that could end up in significant pain or dysfunction down the road. 

We think a prosthesis would give Anna the best chance of having a normal childhood and maximum mobility without endless hours of physical therapy, out of state medical trips, and separating her from her friends, family, and school. To a smaller degree of importance, we also don't feel that choosing to center our family's life around Anna's medical condition when there is another great option out there, would be the healthiest choice for her or the family. The option that results in a stable, healthy home environment for Anna will likely benefit her more than trying to save her leg at all costs. Children with a prosthesis are just as "normal" as other children. Anna does not need a lengthened leg to be whole or beautiful. Since children with a prosthesis have close to the same mobility as those without one, we feel that for us, the aesthetic benefits of having two real legs/fitting in with how one is "supposed" to look, and any emotional attachment we might have to to her foot is not worth the pain and limitations Anna would experience throughout her life. Yes, we would love if she didn't have to go through this in the first place, but we also recognize that looking like everyone else is not what is most important, or eternal.

Does that mean it's easy? No. Who wants to make the decision to amputate their child's leg? All sorts of questions run through my mind. Having a girl makes it even more complicated. Society is less accepting of women who are physically different, because women are supposed to be the picture of beauty and femininity. How will she wear shoes? What will she do at the beach? For the prom? How will her self image be? How will people treat her, view her, etc.? Some of these may sound like small concerns, but the day-to-day challenges are what I think about. Thankfully, we have talked with lots of other parents who chose amputation and none of them have ever regretted their decision, not even for a minute. Their children are well-adjusted, active, and HAPPY. They live full lives and face few limitations. They do all the things other kids can do. Over and over I am told it is the parent who has the hardest time with their child being "different" and wanting their child to be "normal." Talking to real-life families has been perhaps the most confirming and encouraging on this journey of decision-making.

It is not an easy decision either way, and I feel for parents in similar situations. Both options need endless amounts of love, grace.... and constant running to our Savior, leaving everything at the cross for Him to make whole.

To those of you who are reading this, and who believe what Ben and I believe about Jesus and take him at his Word, don't worry... we are not holding fast to the medical field. Man's abilities are limited. God is limitless. He is the Great Physician. And we are actively believing him to intervene on our behalf. We also feel that we need to pursue the best available treatment for Anna. This is a difficult line to walk... balancing faith with reason. It is so easy to fall to one side or the other, when we still have so much to learn and when we know that our child's quality of life is hanging in the balance. It's one thing to make a life-changing decision for yourself. It is another to make that decision for someone else.