Wilms’ tumor: How much do you know about it?

Wilms’ tumourmost often affectschildren ages threeto four years old.

The outlook for most children with Wilms’ tumor is good. (online)

“There are incalculable resources in the human spirit, once it has been set free.”— Hubert Humphrey

It is an unimaginable anguish to have your child diagnosed with a life-ending illness. Yet, for many families, this news is part of their living. Undoubtedly, the health-care struggles have been magnified, especially during the novel coronavirus pandemic.

People oftentimes move in and out of our lives for a reason as life is a fraught with many obstacles; some of which leave us gutted. However, there is always hope, and we should never surrender to the pain and suffering which exist.

Earlier this year a parent shared with me that her five-year-old daughter was diagnosed with Wilms’ tumour. At first I was clueless about this type of tumour, so I decided to do some research and turned to two trusted online sources.

The Mayo Clinic defines Wilms’ tumour as a rare kidney cancer that primarily affects children. Also known as nephroblastoma, it is the most common cancer of the kidneys in children. Wilms’ tumour most often affects children ages three to four years and becomes much less common after age five. Interestingly, girls are more likely to be diagnosed with Wilms’ tumour than boys. Wilms’ tumor most often occurs in just one kidney, though it can sometimes be found in both kidneys at the same time.

The mother of the affected five-year-old recounts the family’s journey from diagnosis to treatment: “As a homeschool mother of six young children, the diagnosis created a tremendous wave of confusion in our family. Fortunately for my daughter I was able to camp out with her in the hospital at the University Hospital of the West Indies. Unfortunately, my daughter and I spent almost two months in the hospital away from home, my husband, and the other children.

“The big C is not a diagnosis anyone wants to hear for themselves, worst of all for their infant child. There was nothing in her medical history that predisposed her to such a disease and the only noticeable sign was a lump on her left side and rapid weight loss. It was somewhat comforting, however, to learn that the prognosis was overwhelmingly positive as the success rate of the medical treatment and complete recovery rate for this particular cancer was significantly high.

“The treatment included a combination of approaches, namely chemotherapy and/or radiotherapy and surgery. Another comforting bit of information for us was that the tumor had not spread to any other organs and so the cancer was at an early to moderate stage of development, which meant that the treatment would be less aggressive.

“Despite that, we dreaded the often-spoken-about chemotherapy. While it was painful to see my little baby endure this, once we understood more about the treatment we were better able to manage the side effects and some of the hospital staff were very helpful, encouraging, and supportive. This made the journey less excruciating for my daughter and for the rest of the family.

“There were some obviously difficult moments, however. The COVID restrictions prevented anyone other than the parents from visiting the patient, so my daughter’s siblings were only able to see her occasionally at the security post. This was the first time the family had been separated for an extended period and that was painful to endure.

“The side effects of the chemotherapy caused intense suffering to my little five-year-old. Some of the adverse effects include severe jaw pain, dehydration, faecal infection, diarrhoea, unbearable stomach pain, and alopecia. Having completed the first stage of her treatment, she is looking toward surgery now and that comes with its own challenges.

“Our faith in the Almighty, the loving support from family and friends are the things that keep us stable and give us hope. Encouragement and hope thrives also because of the many other children we met in hospital with similar and even worse conditions and the resilience and courage they and their parents and guardians show. There is strength within the human mind that can inadvertently take over when faced with tremendous hardship and pain. An unwavering belief in the superior being and the power of the community helps us realise and make use of this inner strength.”

TYPES OF WILMS’ TUMORS

There are two kinds of Wilms’ tumors, grouped by how the cells look under a microscope. One is favourable histology, more than nine out of 10 Wilms’ tumors fall into this group. It means there isn’t a lot of difference among the cancer cells, and children with this type have a good chance of being cured. The other is unfavourable or anaplastic histology. This type has a variety of deformed cancer cells and can be much harder to cure.

Symptoms

Signs and symptoms of Wilms’ tumor vary widely, and some children do not show any obvious signs. However, most children with Wilms’ tumor experience one or more of these signs and symptoms: an abdominal mass you can feel, abdominal swelling, blood in urine, and pain. The Mayo Clinic adds that other signs include fever, nausea or vomiting or both, constipation, loss of appetite, high blood pressure, and shortness of breath.

Causes

It is not clear what causes Wilms’ tumor, but in rare cases heredity may play a role.

Cancer begins when cells develop errors in their DNA. The errors allow the cells to grow and divide uncontrollably and to go on living when other cells die. The accumulating cells then form a tumor.

In Wilms’ tumor, this process occurs in the kidney cells, and in rare cases, the errors in DNA that lead to Wilms’ tumor are passed from a parent to the child. In most cases, however, there is no identified connection between parents and children that may lead to cancer.

One of the factors that may increase the risk of Wilms’ tumor include being of African descent. In the United States, African-American children have a slightly higher risk of developing Wilms’ tumor than children of other races, while Asian-American children appear to have a lower risk than children of other races.

Another factor is family history. If someone in your child’s family has had Wilms’ tumor, then your child has an increased risk of developing the disease.

This type of tumor may also occurs more frequently in children with certain abnormalities or syndromes present at birth, including aniridia. This is a condition in which the iris, the coloured portion of the eye, forms only partially or not at all.

Hemihypertrophy, charcterised by one side of the body or a part of the body being noticeably larger than the other side, is another cause of Wilms’ tumor.

Wilms’ tumor can also occur as part of rare syndromes, including WAGR syndrome, which includes Wilms’ tumor, aniridia, genital and urinary system abnormalities, and intellectual disabilities; Denys-Drash syndrome, which includes Wilms’ tumor, kidney disease, and male pseudohermaphroditism, a condition in which the individual has a single chromosomal and gonadal sex but combines features of both sexes in the external genitalia, causing doubt as to the true sex; Beckwith-Wiedemann syndrome, an overgrowth syndrome, which means that affected infants are larger than normal, and some may be taller than their peers during childhood. Other signs may include abdominal organs that jut out into the base of the umbilical cord, a large tongue, enlarged internal organs, and ear abnormalities. There is also an increased risk of tumors, including a type of liver cancer called hepatoblastoma.

WILMS’ TUMOR TREATMENT

The online source WebMD states that how a Wilms’ tumor is treated depends on how much the cancer has spread, and treatment can include surgery, chemotherapy, and radiation therapy.

If surgery is the preferred course then your doctor may do one of these procedures: partial nephrectomy, which is the removal of the tumor and some healthy tissue around it; radical nephrectomy, which is the removal of the affected kidney, the ureter, the adrenal gland on top of the kidney, and nearby tissue; and removal of both kidneys, after which your child would then need to undergo regular dialysis treatment to filter waste out of their blood. Once they are healthy enough, they may become candidates for a kidney transplant.

WILMS CANCER FOUNDATION

The Wilms Cancer Foundation (WCF) is a US-based charitable organisation that supports and represents the needs of children, families, and health-care organisations affected by this type of cancer.

Through its programmes, the foundation aims to provide every child, family, and health-care organisation with the support they need to tackle Wilms and save children’s lives.

WILMS’ TUMOR OUTLOOK

The outlook for most children with Wilms’ tumor is good. Up to 90 per cent of children who have a tumor with favourable histology can be cured. If the tumor has unfavorable histology, the cure rate is lower.

Once cancer treatment ends, your child will have regular doctors’ visits to make sure he or she stays healthy. If there’s something you do not understand, make sure to ask your paediatrician. This will help you to understand how best to support your child during this challenging time.

In the midst of disease and sickness, which are constant in the world, there is an urgent need to raise awareness of Wilms’ tumour, especially in Jamaica.

Even with the deficiencies in our health-care system, we pause to pay tribute to our hard-working health-care professionals who turn up and give of their best.

We empathise with those parents who are journeying with their children who have been diagnosed with Wilms’ tumour and any other type of cancer or disease. To the children who are suffering from this rare type of cancer we pray for your full and speedy recovery.

Jesus said, “Come to me, all you who are weary and burdened, and I will give you rest. Take my yoke upon you and learn from me, for I am gentle and humble in heart, and you will find rest for your souls. For my yoke is easy and my burden is light.” (Matthew 11:28-30)

Wayne Campbell is an educator and social commentator with an interest in development policies as they affect culture and or gender issues. Send comments to the Jamaica Observer or [email protected]

Wayne Campbell (online)


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