“Everything changed when I was 8 and went through a living nightmare”
Rita Agarwal, MD, FAAP, FASA
We go to work every day trying to provide best possible care to our patients, we consider the type of anesthetic, the best technique, the ideal way to provider perioperative analgesia and how to minimize postoperative complication. Few of us think about the long-term consequences of the surgery that our patient may be undergoing. If we like and trust our surgeons we also tend to trust their judgement, even if the operation appears to be new, radical, or innovative. Pediatrics regularly presents Family Partnership Essays that includes physicians, patients and or family members and others in who may have been involved in the care of the child. This particular article is very thought provoking and I encourage everyone to try and read it. It is short and powerful and describes a parent and her now adult child’s experience with limb lengthening surgery. The article is coauthored by Lindsay Ellingsworth current Orthopedic surgeon and a medical ethicist.
Original article
David Hootnick, Lindsay Ellingsworth, Rene Mauchin, Amy Caruso Brown. "It Occupied Her Entire Childhood": Looking Back on Limb-Lengthening. Pediatrics. 2021 Feb;147(2):e20201055. doi: 10.1542/peds.2020-1055. Epub 2021 Jan 5. PMID: 33402527
Lindsay was born in the late 80s and was diagnosed with an abnormal femur, which is less common and more difficult to treat than the discrepancy in the length of the tibia or fibula. Her left leg was 8-10 cm shorter than her right, however her foot and lower part of her leg looked perfect. Rene, Lindsay’s mother, describes her horror at initially being told that they should amputate and use a prosthesis. They sought several opinions that last 2 of which recommended limb lengthening surgery.
Lindsay Ellingsworth writes “Despite this, I was raised like any other child and never felt that I was different or was treated differently. This is how I was born; I did not know anything else… When my parents bought me a bike for my fifth birthday, I hopped right on it and rode it with one leg without ever being taught how. It was natural adaptation…”. “Everything changed when I turned 8 and went through a living nightmare: my first limb lengthening surgery.” She describes waking up screaming in pain night after night, wondering what was wrong with her that she had to experience so much pain, medications that did not work and multiple complications that lead to more surgery. Pain and her external fixator dominated her childhood, children teasing, adults staring, regularly adjusting the screws, dressing changes and more surgery. She ultimately underwent 13 operations including back and neck surgery as a young adult. While Lindsay gained significant length in her affected limb, she developed scoliosis, knee instability cervical and lumbar imbalances and hip abnormalities. She met her current orthopedic surgeon while studying to be a nurse, a career she later had to give up due to ongoing pain and disability. She is now on Social Security Disability and continues to suffer from chronic pain.
Rene Mauchin describes the efforts she and her husband made to try and make the right decisions in a distressing and bewildering circumstances. They were encouraged by their new, experienced, and well published surgeon to try this procedure that would save their beloved little girl’s leg. It is clear reading through this account that despite presumably good intentions on the part of everyone involved in the Lindsay’s care, there were major missed opportunities. “With each new complication, our doctors always focused on how to fix it and how to get to the goal, even when the goal seemed to be moving further from our reach. We did not know that stopping was possible….” As physicians we are “fixers” by nature, training, and education. It is so much harder to step back from the immediate crisis and look at the big picture.
David Hootnick, Lindsay’s current surgeon briefly reviews the current literature on limb lengthening surgery, including literature that proves many patients with certain types of limb lengthening discrepancy (fibular hemimelia) have a better quality of life, undergo less surgery, are more active, and have less pain if they undergo an amputation versus limb lengthening surgery.1 He admits that many surgeons bring their own preferences and may weigh the new and innovative more heavily that the evidence would indicate. In addition, making these decision for their young children may be swayed by depictions in popular media. Who can forget the classic scene in Forrest Gump when a young Forrest in leg braces (for unclear medical indications) is being chased by bullies. “Run Forrest run”, yells Jenny.
There are other studies that show equal or better outcomes between the 2 techniques and Ann Caruso Brown the medical ethicist on the article discusses the importance of shared decision making when there are 2 different approaches to a medical problem and no obvious correct choice.2,3 She implores doctors to inform families of all the risks and benefits including possible complications and their consequences particularly for such life altering decisions. “We have to ask parents, and children when they are old enough to participate, to envision the worst-case scenario as well, asking themselves, “If neither path produces the desire outcome, which decision can we best live with? Which set of complications is more acceptable to us? Will it be harder knowing that we tried something innovative, and it failed, or that we took a more conservative approach?”
References
1. McCarthy JJ, Glancy GL, Chnag FM, Eilert RE: Fibular hemimelia: comparison of outcome measurments after amputation and lengthening. J Bone Joint Surg Am 2000; 82: 1732-5
2. Birch JG, Paley D, Herzenberg JE, Morton A, Ward S, Riddle R, Specht S, Cummings D, Tulchin-Francis K: Amputation Versus Staged Reconstruction for Severe Fibular Hemimelia: Assessment of Psychosocial and Quality-of-Life Status and Physical Functioning in Childhood. JB JS Open Access 2019; 4: e0053
3. Walker JL, Knapp D, Minter C, Boakes JL, Salazar JC, Sanders JO, Lubicky JP, Drvaric DM, Davids JR: Adult outcomes following amputation or lengthening for fibular deficiency. J Bone Joint Surg Am 2009; 91: 797-804