Sarah Hyland, who plays the eldest daughter Haley Dunphy on the award-winning comedy series “Modern Family,” just revealed in an article in Self that she underwent a second kidney transplant last year. The 28-year-old Hyland was born with congenital kidney dysplasia that was severe enough to require a kidney transplant in 2012. The donor was her dad.
Unfortunately, in October 2016, Hyland’s body began to reject the transplanted kidney. She experienced severe fatigue, frequent fevers and infections and her serum creatinine climbed higher than it was before the transplant. Despite multiple hospital admissions and treatments, they were unable to rescue the kidney, and in February 2017 she started dialysis. Because Hyland was tethered to a dialysis machine for four hours per session, three days a week, she picked a dialysis center close to the “Modern Family” set, so she could arrange her shooting schedule around her treatments.
Her body underwent a dramatic transformation. She lost a lot of weight, but her face was swollen due to the immunosuppressant medications. In May 2017, she became the victim of bodyshamers who accused her of promoting anorexia. But Sarah fought back, tweeting:
“I have been told that I can’t work out. Which, for me, is very upsetting. I love to be STRONG (I’ll be using that word a lot). Strength is everything. Being strong has gotten me where I am. Both mentally and physically. I am not a fan of ‘being skinny’… I don’t mind when you say that I look pregnant. Or fat. Because I know that my face is swollen from my medication that is saving my life. For those on Prednisone I know what you’re going through, and I commend you sticking it out as I have.”
Later that month, her transplanted kidney was removed, and a second transplant was enabled by her brother Ian, 23, as the donor. Nevertheless, Sarah became depressed: “When a family member gives you a second chance at life, and it fails, it almost feels like it’s your fault. It’s not. But it does. For a long time, I was contemplating suicide, because I didn’t want to fail my little brother like I failed my dad.”
Talking about her thoughts helped her and she encourages others to do likewise. “It’s not shameful,” she says. “For anybody that wants to reach out to somebody but doesn’t really know how because they’re too proud or they think that they’ll be looked upon as weak, it’s not a shameful thing to say. It’s not a shameful thing to share.”
She underwent her second kidney transplant in September 2017. She’s doing well with her immunosuppressive drugs regimen. Hyland also suffers from endometriosis and has undergone six surgeries in the past 16 months to treat it.
In summary, she tells Self: “My name is Sarah, I have two of the most amazing dogs in the entire world. I have the best boyfriend ever… I have the greatest family one could ask for. I’m on a show that is absolutely unbelievable and surreal.”
“Oh, and also, I was born wrong. Physically. In a way that’s not good for your body to keep going. Oh, and also, I have endometriosis. Oh, and also, I have kidney failure. Oh, and also, I’m a two-time kidney recipient. Oh, and also, I had a frickin’ hernia for almost a year that went unnoticed.”
“That list doesn’t stop… But that list doesn’t hold me back from anything. I won’t let it.”
What is Kidney Dysplasia?
According to the U.S. National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK) congenital anomalies of the kidney and urinary tract (CAKUT) account for approximately 20-30% of all anomalies found in the prenatal period. Defects can be bilateral or unilateral, and additional renal defects can coexist in an individual child. CAKUT plays a causative role in 30 -50% of cases of end-stage renal disease (ESRD) in children.
Although kidney dysplasia can be associated with a number of genetic disorders, more commonly it occurs as a sporadic malformation. Cystic renal dysplasia is a renal parenchymal malformation in which the fetal kidney contains primitive ducts and cysts. Non-renal tissue, such as fat, cartilage, and hematopoietic tissue may also be present.
Kidney dysplasia is a relatively common condition. Researchers estimate that kidney dysplasia affects about one in 4,000 babies. This estimate may be low because some people with kidney dysplasia are never diagnosed with the condition. About half of the babies diagnosed with this condition have other urinary tract defects.
Multicystic dysplastic kidney (MCDK) is the most severe form of cystic renal dysplasia. Macroscopically, there are multiple noncommunicating cysts separated by dysplastic tissue. Some functional renal tissue may be present, but the amount is typically minimal. The ureter may be absent or atretic. The disorder can be unilateral or bilateral. If the contralateral kidney is normal, a compensatory hyperplasia of that kidney is often present, which begins in utero and continues for several years. The other kidney can have other genitourinary defects including hypoplasia, vesicoureteral reflux (VUR), ureterocele, ureteropelvic junction (UPJ) obstruction, and so on. VUR is the most common and occurs in about 20% of affected individuals. Extrarenal malformations may also be present, including heart defects, myelomeningocele, and intestinal or esophageal atresia.
What Causes Renal Dysplasia?
The cause of MCDK is unclear at the present time. Studies by Hwang et al. and Caruana et al. suggest an underlying genetic predisposition in some patients. Hwang found that MCKD was associated with mutations in CHD1L, ROBO2, HNF1B, and SALL1 genes. Caruana found copy-number variation (CNV) in 10% of their patients with CAKUT.
Environmental factors, such as vitamin deficiency and teratogens, may also be involved. Prenatal exposure to ACE inhibitors and angiotensin II receptor blockers has been associated with fetal renal abnormalities. Animal models have demonstrated that vitamin A deficiency is associated with urogenital malformations and renal hypoplasia.
What are the signs of kidney dysplasia?
Many babies with unilateral kidney dysplasia have no signs of the condition. In some cases, the affected kidney is enlarged at birth and palpable as a flank mass. Occasionally, it can cause pain.
What are the complications of kidney dysplasia?
In most cases of MCDK, the natural course is involution of the dysplastic kidney. However, the complications of kidney dysplasia can include:
- Hydronephrosis of the functioning kidney, often due to abnormalities mentioned above
- Chronic kidney disease (CKD) and kidney failure
- Urinary tract infection. Recurring UTIs can also lead to kidney damage
- High blood pressure
- A slightly increased chance of developing kidney cancer, primarily Wilms’ tumor
How is kidney dysplasia treated?
If the condition is limited to one kidney and there are no other signs of kidney dysplasia, no treatment may be necessary. However, the infant should have regular checkups that include:
- checking blood pressure
- testing blood to measure kidney function
- testing urine for albumin
- performing periodic ultrasounds to monitor the damaged kidney and to make sure the functioning kidney continues to grow and remains healthy
What is the long-term outlook for a child with kidney dysplasia in only one kidney?
The long-term outlook for a child with kidney dysplasia affecting only one kidney is generally good. A person with one working kidney, a condition called solitary kidney, can grow normally and may have few, if any, health problems.
The affected kidney may shrink as the child grows. By age 10, the affected kidney may no longer be visible on x-ray or ultrasound. Children and adults with only one working kidney should have regular checkups to test for high blood pressure and kidney damage. A child with urinary tract problems that lead to failure of the working kidney may eventually need dialysis or a kidney transplant.
What is the long-term outlook for a child with kidney dysplasia in both kidneys?
A child with kidney dysplasia in both kidneys
- is more likely to develop CKD
- needs close follow-up with a pediatric nephrologist
- may eventually need dialysis or a kidney transplant
Some clinical trials for patients with MCKD are available and can be found at ClinicalTrials.gov.
Michele R. Berman, MD, and Mark S. Boguski, MD, PhD, are a wife and husband team of physicians who have trained and taught at some of the top medical schools in the country including Harvard, Johns Hopkins, and Washington University in St. Louis. Their mission is both a journalistic and educational one: to report on common diseases affecting uncommon people and summarize the evidence-based medicine behind the headlines.
Source: MedicalNewsToday.com
